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Developmental eld reassignment in unilateral cleft lip:
Reconstruction of the premaxilla
Michael H. Carstens

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Division of Plastic Surgery, Saint Louis University, USA

Address for correspondence: Michael H Carstens, Division of Plastic Surgery, Saint Louis University, USA. E-mail: [email protected]

Th

n the 21st century the greatest stimulus for progress

in cleft surgery will come from more a more accurate
model of facial development and how clefts originate.
Victor Veau[1] accurately predicted this: All cleft surgery
is merely applied embryology. The revolution in
developmental biology has not yet been incorporated into
surgical practice. The drawings and terminology used in
textbooks today are based on the work of Wilhelm His in
the 1870s.[2] Cleft repairs are therefore designed based on
the anatomy as it appears in the newborn. Measurements
are taken and the tissues are geometrically manipulated.
But the anatomy of a cleft as seen at the time of birth is far
different from its original configuration in the embryonic
face. From its onset at gastrulation, the clefting event
unleashes pathologic processes that predictably alter the
original embryonic anatomy over time to produce what
we recognize at term as a cleft lip. Left uncorrected, these
processes will remain operative throughout facial growth.
This explains why geometric cleft repairs relapse over
time, requiring revision.

Developmental field repair (DFR) is based upon the

neuromeric model of craniofacial embryology.[3-4] The
goals of DFR surgery are: (1) resolution of all pathologic
processes of clefting (deficiency, displacement, division
and distortion); (2) dissection along embryonic separation
planes (subperiosteal release); (3) preservation of blood
supply to the alveolar mucoperiosteum; (4) primary
unification of the dental arch; and (5) reassignment of all
developmental fields to their correct relationships. Before
describing the surgical technique, certain basic concepts
of field theory should be understood.
Craniofacial anatomy results from the assembly of specific,
75

identifiable developmental fields. Fields are discrete units

composed of ectoderm, mesoderm, endoderm and neural
crest. At each level of the embryo all germ layers specific to
that level can be referenced back to developmental zones
of the neuraxis known as a neuromeres. These are named
using the nomenclature of neuroembryology. The master
plan of the entire embryo is determined by 6 prosomeres
(prosencephalon-forebrain), 2 mesomeres (mesencephalon
midbrain), 12 rhombomeres (rhomboencephalon-hindbrain
and 31 myelomeres (spinal cord).[5-7]

RUBENSTEIN

Applications of neuromeric anatomy provide a potential

embryonic map of all craniofacial structures with
important implications for diagnosis and surgery. Exclusive
of the cranial base (basisphenoid and posterior) and
parietal bone, the craniofacial skeleton is made exclusively
from neural crest. Thus the cell populations producing the
ethmoid, presphenoid, premaxilla and vomer all originate
in antero-posterior order from the neural folds in genetic
register with the 1st rhombomere (abbreviated r1). The
inferior turbinate, palatine bone, maxilla, alisphenoid
(greater wing) and zygoma arise from the neural crest
of the 2nd rhombomere (r2). The squamous temporal,
mandible, malleus and incus are r3 neural crest bones.

Non-neural crest craniofacial bones come from paraxial

mesoderm (PAM) lying immediately adjacent to the neural
tube. PAM is divided into individual units shaped like
popcorn balls called somitomeres, each one in register with
its corresponding neuromere. The first seven somitomeres
(Sm) contain the myoblasts for all muscles of the orbit
and the first three pharyngeal arches. For example, the
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Carstens

mandible comes from r3 neural crest and all muscles

originating from it arise from Sm3. Beginning with Sm8 all
somitomeres undergo a further transformation into somites,
each having a dermatome, myotome and sclerotome. The
first four somites (derived from Sm8-Sm11) produce the
cranial base posterior to the sphenoid, the muscles of the
tongue and part of the sternocleidomastoid and trapezius.
These are called occipital somites.

The pathologic anatomy of cleft formation

The pathologic anatomy of unilateral and bilateral
labiomaxillary clefts stems from a tissue deficiency state
localized to the lower lateral piriform fossa. The tissue at
fault is the mesenchyme of the ipsilateral premaxilla. Neural
crest stem cells responsible for synthesis of the presphenoid
and ethmoid arise from the mesencephalic neural folds and
are genetically identified with the 1st rhombomere (r1). Note
that the basisphenoid is not neural crest in origin; it comes
from paraxial mesoderm from somitomere 1 (Sm1). Sm1 lies
just outside the neural tube at level r1 and is in register with
it. Immediately caudal to this population are neural crest
cells immediately above the rostral rhomboencephalon in
register with the 2nd rhombomere (r2). The most rostral
zone of r2 (herein referred to as r2) is the likely source
material for the vomer and PMx. The more caudal zone of
r2 produces inferior turbinate (IT), palatine bone (P), maxilla
(Mx), alisphenoid (AS) and zygoma (Z).

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Disturbances at a particular neuromeric level can affect

individual or multiple fields to be deficient or absent.
Thus, isolated cleft palate (unassociated with cleft lip)
represents a deficiency state of the vomer. This occurs as
a spectrum. As the vomer is progressively smaller, it lifts
away from the plane of the palatal shelves and the cleft
extends forward toward the incisive foramen. In mild
cases of cleft palate associated with Pierre Robin sequence,
a reduction in the horizontal plate of the r2 palatine bone
is seen. Soft palate muscles are consequently normal
but divided. As the pathology worsens, reduction in the
horizontal plate of the r2 maxilla creates the well-known
horse-shoe palate cleft. Submucous cleft palate, on the
other hand, involves pathology in the 3rd pharyngeal arch.
Somitomeres 6 and 7 contain the myoblasts of levator,
uvulus, palatopharyngeus and superior constrictor. These
can be globally affected. Frequently, persistent VPI follows
a seemingly simple palatoplasty, requiring further surgery.
Failure to stratify cleft palate by embryologic mechanism
explains much of the confusion currently extent in the speech
and surgical literature.

presents the reconstructive application of these principles

to the surgery of labiomaxillary clefts.

Th

Finally. Treacher-Collins syndrome provides an example

in which all r2 developmental fields of the midface are
affected: the maxillary, palatine and zygomatic bones
are all small. The septum, vomer and premaxilla (being
r1 structures) are unaffected. For this reason, the central
midface projects normally while the dimensions of the
palate, maxilla and zygoma are constricted.

Developmental fields form in a specific spatio-temporal

sequence. Each one builds upon its predecessors.
Making a face is much akin to assembling a house with
magical pieces of Lego, each one of which will grow
over time. Imagine a Lego house made from 20 pieces (4
on the floor and 5 stories high). All pieces are growing
independently. If a cornerstone piece is removed, the 19
remaining pieces undergo a deformation and the house
tilts into the deficiency site. The missing Lego piece in cleft
lip is the premaxilla. The physiologic basis of DFR is the
reconstruction of the premaxillary field.[8-10] This chapter
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

These cell populations migrate forward into the

developing face in a strict temporo-spatial order. The
sphenoid is laid down first, followed by the ethmoid. In
like manner, formation of PMx is the prerequisite for the
appearance of V. Formation of the PMx and V requires the
pre-existence of the perpendicular plate of the ethmoid
(PPE). The function of the PPE is to provide a cellular
scaffold by which r2 neural crest cells can reach the
midline.[10] [Figures 1-2] In holoprosencephaly (HPE) the
PPE can be absent. PMx and V cannot develop correctly;
a wide bilateral cleft results.[11-13]

The piriform fossa of humans and some high primates

is assembled as the fusion of the frontal process of the
premaxilla (PMxF) and the frontal process of the maxilla
(MxF). In all other vertebrate skulls PMxF and MxF are
readily visible as two distinct entities. [12] Evolution
foreshortened the human snout. The two fields became
superimposed, PMxF becoming telescoped internal to MxF.
This lamination is responsible for the strength of the
piriform rim. Plating of the piriform buttress in fracture
repairs takes advantage of this bicortical anatomy.

PIRIFORM
The developmental field in which the PMx resides consists
of an epithelium and a mesenchyme. Formation of the
PMx results from interactions between these tissues. The
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Developmental eld reassignment in unilateral cleft lip

Th

2007 Carstens
2007 Walters

2007 Carstens
2007 Walters

Figure 1: Developmental eld map of the face. All neuromeres are color coded. Neural crest cells forming ethmoid, presphenoid, vomer
and premaxilla share common embryologic origin from 1st rhombomere (violet). Maxilla, zygoma, alisphenoid come from 2nd rhombomere
(blue). Nostril sill is union of r1 and r2. All craniofacial sutures contain neural crest cells. Craniofacial elds are neuroembryologic and follow
suture boundaries

premaxilla has several anatomic subcomponents, these are

assembled in a strict sequence.[14-15] In dental terminology
the central incisor is called A and the lateral incisor is
called B. A erupts before B. Therefore the central incisor
field (PMxA) is biologically older than the lateral incisor
field (PMxB). Neural crest mesenchyme flows forward
along the previously-established PPE. It first encounters the
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epithelium corresponding to PmxA and then spills over into

zone PMxB. The time sequence of dental eruption (central
incisor A > lateral incisor B) is a manifestation of the relative
biologic maturity of the mesenchymal field within which
each tooth develops. The frontal process field (PMxF) is a
vertical offshoot of PMxB; this subfield is the biologically
newest tissue.
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Carstens

mesoderm from the 2nd somitomere forms the anterior

half of the squamous temporal bone and the cranial half
of the parotid gland. All derivatives from level r2 can be
mapped out within the sensory distribution of V2, the
nucleus of which resides within r2.[19-24]
The developmental anatomy of the nose, prolabium
and premaxilla has been previously described in terms
of neuromeric theory by this author.[3-4,8,9] In contrast to
the rest of the body, all dermis and submucosa of the head
originates from neural crest cells, not from a dermatome
associated with a somite.[25] Pre-dermal neural crest arises
from three distinct zones of the embryo. The dermis
of the forehead, nose and vestibular lining come from
the caudal prosencephalon (above prosomeres p4-p1).
This prosencephalic neural crest (PNC) migrates forward
like a gigantic glacier to occupy the neural folds above
prosomeres p6 and p5. The alar half of p6 and p5 creates
the telencephalon (cerebrum). The basal halves of p6 and
p5 plus all remaining prosomeres (p4-p1) synthesize the
diencephalon (epithalamus, thalamus and hypothalamus).
The neural folds above p6 and p5 are sterile. They
contain the pituitary, olfactory and optic placodes, but
no neural crest. When PNC flows forward into this zone
the placodes are activated and dermis is formed. Nasal
vestibular lining from the cribriform plate forward to the
internal nasal valve comes from p6 PNC. All remaining
frontonasal dermis from the internal nasal valve forward
to the hairline comes from p5 PNC.

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Pathology affecting the PMx occurs as a spectrum based

on this original developmental pattern. A deficiency
state of the PMx will first occur in the most distal aspect
of the frontal process (ie. at its most cranial extent). As
the mesenchymal deficit worsens, frontal process will
be reduced in a cranial-caudal gradient. Scooping out
of the piriform rim results; the nasal lining is pulled
down as well. This causes depression of the alar base
and a downward-lateral displacement of the lateral crus.
Biologic signals from PMxF regulate epithelial stability
and therefore affect lip formation (vide infra). When the
signal strength is minimally disturbed the lip is normal
despite the piriform distortion. Therefore the forme fruste
manifestation of premaxillary deficiency is a cleft lip nose
with a perfectly normal lip.[16]
Once the frontal process is eliminated, the deficiency
state shows up in the lateral incisor field. Progressive
degrees of PMx deficiency in the lateral incisor field
cause incremental loss of alveolar bone. Normal alveolar
development follows a gradient. It begins at the incisive
foramen and progresses forward. Mild deficiency causes
notching on the labial surface. As the deficiency worsens
the notch deepens backward toward the incisive foramen.
A critical lack of alveolar bone mass results in outright
failure of lateral incisor development.

Th

BMP-4 signals from this field are directly implicated in the

mechanism of fusion between the lateral lip element and
the prolabium.[17-18] BMP-4 emanating from PmxL forms
a cranio-caudal chemical gradient. The strength of this
gradient depends upon the total amount of available BMP4; this in turn is proportional to the overall mesenchymal
mass of PMxB. Reduction in mass of the lateral incisor
field results in a diminution of the total BMP-4 signal. Lip
fusion follows this same gradient. Mild weakness of the
BMP-4 gradient will result in notching of the vermilion. As
the situation worsens the extent of the lip cleft worsens
in a cranial direction. The clinical spectrum of the socalled minimal cleft lip deformity faithfully reproduces
this biologic sequence.[16]

In summation, variations in clefts involving the primary

palate and the lip can be understood as interactions
between deep plane fields of the premaxilla, the maxilla
and superficial plane field of the lateral lip element. The
mesenchyme of the lip has a different embryologic origin.
It is genetically identified with the 2nd rhombomere.
Neural crest cells from r2 provide the dermis and the
subcutaneous tissue of the alar base, while paraxial
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

PNC skin shares sensory innervation with the dura of the

underlying frontal lobe. V1, the sensory nerve for this
common zone has its nucleus with the 1st rhombomere
(r1). The neuroanatomy is analogous for the rest of the
face. Rhombomeres r2 and r3 contain neurons supplying
all skin and dura innervated by V2 and V3. This is the
embryologic basis for the treatment of migraine headaches
with Botox injected into peripheral trigger points.

Design of surgical incisions for cleft repair follows this

embryology. The boundary between these two fields
is sharply demarcated within the naris. The skin of the
anterior columella and philtrum is thus a p5 derivative.
This skin is supplied by terminal branches of the
internal carotid artery, the anterior ethmoid arteries and
innervated by V1.[3] The skin making up the floor of the
nose has a different origin. It extends from the base of
the columella laterally and makes contact with the alar
base. The medial (terminal) branch of the sphenopalatine
artery innervates this skin. The innervation is from V2 and
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Developmental eld reassignment in unilateral cleft lip

is shared with the ipsilateral incisors. Continuity between

the p5 skin of the lateral columella and the r2 skin of the
nasal floor makes possible the elevation of a skin-cartilage
flap containing the medial crura with long skin flaps. Many
years ago Vissarianov described this flap as a means of
secondary cleft reconstruction.[26-27]

The premaxillary developmental field consists of lining

(present in its totality) and mesenchyme (missing).
Lining can be recreated by: (1) subperiosteal dissection
of the primary palate (r2 premaxilla and r2 maxilla); (2)
subperiochondrial dissection of the p6 septum (sufficient
to reduce the septum into the midline; (3) subperiosteal
elevation of r2 vomer to close the nasal floor (sufficient to
reduce the septum into the midline); and (4) subperiosteal
rescue of the r2 nasal floor skin from the lateral prolabium.
When these flaps are elevated and sutured, the primary
palate becomes a box lined with neural crest stem cells,
all of which carry membrane-bound BMP receptors.[29-31]

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Based on signals from the underlying premaxilla, the r2

alar base produces a tongue of tissue that makes contact
with the r1 lateral prolabium. This skin bridge sets up the
floor of the nose. It also provides a mechanical platform by
which mesenchyme from the lateral lip element can make
contact with the p5 mesenchyme of the prolabium. Lip
closure thus occurs. This process involves mesenchymal
flow from the lateral lip element toward the prolabium.
For this to occur, the epithelium covering the lateral lip
element, the r2-r1 skin bridge and prolabium must undergo
a genetically-induced breakdown. BMP-4 produced by the
premaxillary field causes de-repression of Sonic hedgehog
(SHH), a gene localized to these overlying epithelia. The
protein product of SHH causes epithelial breakdown. Thus,
absence or deficiency of an appropriate BMP-4 signal will
lead to restricted expression of SHH, abnormal persistence
of epithelium and failure of mesenchymal fusion.[28-29]

a set of Lego pieces, all growing over time. If one piece of

the set is missing, with subsequent growth the remaining
pieces will collapse into the deficiency site. Only when
all the Lego pieces are in place can harmonious facial
development occur.

Th

The volume of the PMx determines whether or not lip

closure can occur. First, a small premaxillar field manufactures
small amounts of BMP-4. The amount of BMP-4 produced is
critical to produce the epithelial breakdown necessary to
permit mesenchymal merger. Second, when the premaxilla
is too small, the physical distance between it and maxilla
will exceed a critical dimension. Epithelial bridge formation
between the alar base and the prolabium cannot occur. If this
critical distance exists at the level of the incisive foramen,
a cleft of the secondary palate will form. This is because
the horizontal repositioning of the palatal shelf from the
maxilla must make contact with the vomer just posterior
to the incisive foramen. The process is just like a zipper. If
initial contact is not made, fusion of the palatal shelf to the
vomer cannot take place. Even if initial contact is made, a
secondary palatal cleft can still result due to displacement
of the vomer away from the midline. The vomer can become
warped by the inequality of growth forces on either side of
the cleft. Thus, the zipper may get started anteriorly but, as
the process proceeds posteriorly, when it encounters the
deviated vomer, a palatal cleft will open up.[3]
Reconstruction of the premaxilla
Cleft surgery that does not reconstruct the missing PMx
does not solve the biologic problem. The pediatric face is
79

Mesenchymal replacement can be undertaken using

two basic mechanisms. Autogenous bone graft from
rib or hip provides mesenchymal cells originating from
paraxial mesoderm. Incorporation of the graft into the
primary palate occurs by osteoconduction. Implantation
of recombinant human bone morphogenetic protein-2
(rhBMP-2) in combination with an activated collagen
sponge (ACS) results in morphogen-based recruitment of
stem cells from the environment into the sponge. Stem cell
concentration and differentiation into osteoblasts results
in the formation of bone native to the site. This process
is known as osteoinduction.[32] Extensive pre-clinical work
by Boyne demonstrated the ability of rhBMP-2 to form
membranous bone, including reconstitution of surgicallycreated cleft palate defects in primates.[33-34] Studies
demonstrating efficacy in maxillary lift combined with
absence of donor site morbidity resulted in FDA approval
for this indication.[35]

The technique of facial bone reconstruction using

rhBMP-2/ACS implantation is known as in situ osteogenesis
(ISO) and has been previously reported by this author
and co-workers.[36] Resynthesis of a 12 cm mandibular
defect in a 9 year old demonstrated the ability of ISO to
function effectively outside the range of blood supply
associated with a critical-size defect.[37] BMP-2 mediated
osteoinduction is accompanied by extensive recruitment
of blood vessels from local environment.[38] For this
reason selected bone defects can be resynthesized using
ISO alone, without recourse to microsurgical tissue
transplantation.
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Carstens

2007 Carstens
2007 Walters

Figure 2: Developmental anatomy of premaxilla. Spatio-temporal distribution of

neural crest (arrow) demonstrates frontal process as most recent derivative.
Clefting affects the premaxillary elds in reverse order; piriform fossa deciency
is therefore the forme fruste manifestation of a cleft

Th

Distraction techniques have been successfully applied

to ISO-regenerated bone. In a number #7 lateral cleft
with a foreshortened mandibular body and absent
ramus, distraction of the recipient periosteal chamber
in a posterior and superior direction permitted
synthesis of 3.5 cm of mandibular ramus with eventual
articulation with the skull base via a pseudoarthrosis.[39]
Distraction-assisted in situ osteogenesis (DISO) will be an
alternative treatment to rib grafts in the reconstruction
of the Pruzansky III mandibular defects in craniofacial
microsomia. The bone produced will be membranous.
The dissection is less problematic. There is avoidance of
unpredictable growth of the rib graft outside the natural
periosteal environment. Finally, the chest wall donor site
is obviated. Histology of ISO-produced membranous
bone is indistinguishable from that of the recipient site
in both mandible and maxilla.
Alveolar clefts are lined with mucoperiosteum
containing neural crest stem cells. Blood supply is
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

Figure 3: Medial and lateral sections of the nasal cavity demonstrate the
neuromeric origins of the bone elds. The biosynthetic pathway of the r2 MNC
lays down the premaxilla and vomer along the axis of the nasopalatine nerve
and the medial branch of the sphenopalatine artery (the absolute terminus of
the external carotid). Fields affects by the Tessier clefts are indicated by yellow
stars, eg. the #3 cleft is a selective knock-out of the inferior turbinate. Because
the p5 lacrimal bone is built upon IT the #3 cleft destroys the lacrimal system.
The cleft premaxilla is zone #2

excellent and the dimensions modest. For this reason,

this author and co-workers reported 50 alveolar clefts
successfully treated with rhBMP-2.[40] Precise surgical
technique of implant placement and of soft tissue
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Developmental eld reassignment in unilateral cleft lip

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Figure 4: Cleft site showing retraction of p5 lateral crus vestibular pulled

downward into the premaxillary deciency site (just in front of the inferior
turbinate). LLC is programmed by p5 nasal skin; ULC in programmed by p6
vestibular epithelium. If the LLC is not released and additional tissue provided,
the nasal vestibule will be reduced in surface area 30-40% compared to the
non-cleft side. Standard cleft repairs lead to respiratory dysfunction

Th

Figure 5a: Surgical marking and incisions for DFR. Point #3 is 6-10 mm from
the normal philtral column. As medial incision for NPP-LCC ap reaches the
columellar base, it is interrupted by medially-based rectangular Reinisch ap.
Lateral nasal wall incision separates p5 vestibular mucosa (sky-blue) from r2
nasal skin (yellow)

closure was emphasized in the current series of 200

cleft sites.[41] Long-term outcome of 43 cleft sites was
assessed at one year post grafting with 3-D CT. The
series was comprised of 23 unilateral clefts (6 primary
and 17 secondary) and 12 bilateral clefts (3 primary
and 9 secondary). Complete transverse fill (unification
of the dental arch) was achieved in all cases. Vertical
fill was improved to 75-100% when an inert bulking
agent (tricalcium phosphate) was included. For these
reasons, primary cleft repair using developmental field
reassignment technique can ideally be combined with
ISO to achieve primary unification of the dental arch
without donor site morbidity.
81

Figure 5b-d: DFR surgical design. Operative sequence demonstrates

elevation and transposition of NPP-LCC (non-philtral philtrum/lateral columella
chondrocutaneous) ap into deciency site created by release and cephalic
rotation of p5 lateral crus. This reconstructs the soft tissue corresponding to
mssing lateral premaxillary elds

Philosophy of Developmental Field Reassignment

(DFR): the 4 Ds
Faulty embryogenesis of the premaxillary field affects the
position and shape of surrounding fields in four distinct
ways: Division of force vectors causes unequal growth,
Deficiency-related collapse of partner fields into the void,
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Carstens

dental arch into a normal relationship. Fifth, reassign all

developmental fields into correct relationships with each
other. When properly executed, these principles result
in the restoration of the functional matrix. When all the
bone-forming soft tissue fields are spatially correct, all
force vectors exerted upon bone will be correctly aligned.
Subsequent growth of the face is directed back toward
the normal.[44-46]

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A new algorithm: Is lateral nasal wall deciency

relative or absolute?
In keeping with Victor Veau, cleft repair is a constant
exploration of natures experiments. Developmental field
reassignment surgery is deliberately designed to address
a tissue deficit of the lateral nasal wall and alveolus, the
product of a congenitally small premaxillary field. That the
lateral crus be entrapped cannot be in doubt. Its release
into a normal position occasions a triangular tissue gap
that must be filled. Proper airway reconstruction is the
name of the game. At the same time, the alveolar cleft (a
six-sided box) must be reconstructed. This requires flap
coverage for its nasal surface (the top of the box). Can
these two goals be accomplished with the same tissue?
A skin graft alone (particularly anterior auricular skin
and cartilage) will effectively support lateral alar crus
advancement. It cannot provide vascular coverage for the
alveolar cleft roof. At first blush, the LLC-NPP flap would
seem big enough to accomplish both goals. After 7 years
of work with this technique this author has concluded that
paring from the prolabium is not sufficient. Premaxillary
field soft tissues are not only relatively deficient from the
lateral nasal wall: an absolute tissue deficit exists. Use
of the premaxillary tissue mismatched to the prolabium
is not always sufficient to solve the problem. The new
algorithm of DFR surgery now calls for optional composite
grafting of the lateral wall, followed by elevation and
transposition of the LCC-NPP flap across the nasal surface
of the alveolar cleft. The decision to graft is based upon
the relative size of the defect versus that of the flap.

Figure 6: Orbicularis oris is structurally and funcionally bilaminar. Anatomic

dissection by Park demonstrates supercial orbicularis oris (SOO) to attach to the
philtral column as a dilator. Deep orbicularis oris (DOO is separated from SOO by
a plane of fat and axial vasculature. DOO is a sphincter. It runs in the plane deep
to the p5 mesenchyme of the philtrum and is thus continuous with itself across
the midline. All pharyngeal arch muscles follow the same pattern: deep plane
muscles appear earlier than supercial, caudal before cranial and lateral before
medial. The very last facial muscle to appear is the oblique head of SOO

Th

Displacement of partner fields away from the midline.

Distortion over time of structures such as the septum.
DFR repair addresses these 4 Ds in reverse order. All
distortions should be corrected. The displaced soft tissues
if the face should be centralized into the midline. The
deficiency site should be restored, using neighboring
soft tissues and bone graft or ISO; and division should
be closed via unification of soft tissues.[42]

SURGICAL TECHNIQUE OF DFR

The execution of DFR surgery is based upon the concepts
of Sotereanos.[43] Its guiding principles are five.[6-7] (1)
Correct all pathologic states in the first operation (as
above). (2) Respect embryonic developmental planes
during dissection, ie. subperiosteal release of the soft
tissue envelope for a tension-free closure. (3) Conserve
blood supply to the periosteum, safeguarding it for future
membranous bone synthesis. Fourth, align and unite the
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

This operation consists of markings, a five-step dissection

sequence and a five-step closure.
Preparation and marking
The DFR operation is comprehensive, providing
simultaneous correction of the lip, nose and primary
palate. A more extensive dissection is required. The
82

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Developmental eld reassignment in unilateral cleft lip

Figure 7: Four-step nasal incision sequence achieves advancement of both medial and lateral crura. I have recently modied the infracartilagenous incision, making
it discontinuous along the lateral crus to mimimize contracture. Nasal tip is reconstructed by simultaneous elevation of both the medial and lateral crura. The Delaire
sutures are shown. #1 is placed in the levator insertion into the SOO one nger-breadth lateral to the alar base. This centralizes the lip-cheek ap. The non-cleft side
is done rst to the anterior nasal spine (a drill hole is helpful). #2 is placed in the nasalis and is optional (decision made at the end of the case). It adjusts alar base
position and nostril sill curvature. #3 suspends the oblique head of SOO from the columellar base. It establishes the aesthetic drape of the lip

Th

operation takes longer to perform (about 3-4 hours for a

unilateral cleft). For this reason, patients come to surgery
at 4-6 months of age. Prior to operation the dental arch
is prepared with splinting (a form of infant presurgical
orthopedics); this is begun as early as two weeks after
birth. The emphasis of presurgical splinting is: (1)
promoting anterior growth of the retro-displaced cleft
maxilla; and (2) maintenance of the space in the alveolar
cleft. If satisfactory maxillary shelf repositioning does not
result by age 3 months, a lip adhesion procedure is carried
out. When satisfactory dental arch correction is achieved,
DFR repair is performed. This usually occurs 3-4 months
after the lip adhesion.
The patient receives antibiotics and corticosteroids
for swelling. A central V2 block is performed at the
83

pterygopalatine fossa using 0.25% Marcaine (bupivicaine

hydrochloride). Approximately 3-5 cc per side is sufficient
(maximum dose for 0.25% bupivicaine being 1 cc/kg). At
the end of the case, the central block is reinforced by
blocking the infraorbital nerves with bupivicaine, 1-2 cc
per side. The child returns to recovery pain-free. The initial
block ensures that substance P (a critical mediator in the
pain cascade released in response to surgical trauma) will
not be produced. In the absence of substance P the entire
postop pain response is altered.
The surgical fields of the unilateral cleft are defined as
follows. FIGURE (In neuromeric terms: A =p6 (red) +
p5 (turquoise), B = r2 (pale gold), C is r2 (yellow) and D
is r2 + r4 (yellow + orange). The prolabium is divided
into two zones. Zone A is the true philtrum; it measures
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Carstens

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

UCL Double Layer Periosteoplasty: Step 1

Figure 8: NPP-LCC ap has dual blood supply. Behind the surgical tape are:
(1) lateral branches from the ipsilateral anterior ethmoid artery of the columella;
and (2) vessels running along the dorsal surface of the lower lateral cartilage
that anastomose the nasopalatine artery with the lateral nasal artery. In front
of the tape, the nasopalatine (medial sphenopalatine) artery emerges from the
septopremaxillary junction. This tissue, otherwise called the B ap, reconstructs
the nasal oor (roof of the alveolar cleft) and the lateral nasal wall. This
corresponds to the missing premaxillary eld

2007 Carstens
2007 Walters

Figure 10a: Anterior closure of unilateral alveolar cleft using sliding sulcus aps.
On the cleft side two incisions are required. (1) A medial-to-lateral L-shaped
releasing incision (red line) is placed in the gingival sulcus. It runs from the cleft
lateral to the buttress. There, it ascends vertically to the zygomatico-maxillary
junction. The subperiosteal ap is elevated widely up to the infraorbital foramen.
Because the periosteum is an intact sheet, the ap is stiff, being tethered to the
orbital rim. (2) A counter-incision (green line) is begun at the level of the nasal
oor and brought transversely from the piriform to the buttress. This releases the
lower (alveolar) mucoperiosteal ap. It freely translates two tooth units medially.
This closes the anterior wall of the alveolar cleft

UCL Double Layer Periosteoplasty: Step 3

2007 Carstens
2007 Walters

Th

Figure 9: Dissection of alveolar cleft. Elevation and division of mucoperiosteal

aps provides closure of nasal oor and oral aspect of primary palate. R2
premaxillary aps (violet) are sutured to r2 maxillary aps (yellow), providing
4 walls of the box. The cleft-side sliding sulcus ap closes off the front of the
box. Backwall closure occurs when vomerine mucoperiosteum is elevated and
sutured to the palatal shelf MxP. Septum is reduced and secured to midline. Cleft
site box lled with osteoinductive cytokine rhBMP-2 or autogenous bone graft.
This reconstructs the missing premaxillary Lego piece

the width of the columella. It contains paired anterior

ethmoid arteries (the terminal branches of the internal
carotid system) and paired terminal branches of V1.
Thus zone A contains two neurovascular developmental
fields. Zone B of the prolabium is all tissue lateral to the
philtrum: the non-philtral probium (NPP). The nasopalatine
(medial sphenopalatine) artery, the terminal branch from
the internal maxillary system, supplies the NPP. Bilateral
clefts have two NPP zones. The cleft-side alar base is zone
C. The lateral lip element below the alar groove is zone
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

2007 Carstens
2007 Walters

Figure 10b: Bilateral sliding sulcus aps are illustrated. Although I started out
with this technique, I no longer do a gingival release on the non-cleft side.
A one-sided release is sufcient. On the non-cleft side, wide subperiosteal
mobilization is combined with a full-thickness vertical releasing incision up
the buttress. This creates a huge, mobile bipedicle ap. The entire soft tissue
complex becomes centralized without tension. Nota bene: Surgeons working
under extreme condition (very wide clefts in remote areas with no access to
orthodontic management) bilateral sliding sulcus aps are very effective. They
will close any size defect

D. It contains a sphincter layer, the deep orbicularis oris

(DOO) and the dilator layer, the superficial orbicularis oris
(SOO) muscles. DOO develops in conjunction with the oral
mucosa while SOO develops with the skin. A layer of fat
conveniently separates these two layers.[47-48]
The true destination of NPP soft tissue is in the nasal
84

Developmental eld reassignment in unilateral cleft lip

Th

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Figure 11 (Case1): Bilateral cleft lip with right unilateral cleft of primary palate and bilateral clefts of secondary palate. Soft tissue relationships one year after DFR
demonstrating stability of nasal tip projection. Comparison of CT scans at 3 months and 9 months show bony union of primary palate. Periapical view of alveolar cleft
site shows reconstitution of the periodontal ligament, a neural crest structure. This is not seen with conventional iliac crest graft. Serial 3-D skulls taken 9 months
apart support the Delaire dissection concept: wide subperiosteal undermining repositions the osteogenic soft tissue elds into a correct, centralized relationship.
Subsequent osteosynthesis of membranous bone now occurs in a correct centric distribution. Developmental eld reassignment promotes normal membranous bone
formation after the surgical procedure

floor and the lateral nasal wall. NPP represents the soft
tissue envelope corresponding to the lateral zone of
the premaxilla, PMxB + PMxF. Nasal floor soft tissue
corresponds to the lateral incisor field, PMxB. Soft tissue
of the lateral nasal wall between the inferior turbinate and
85

the lateral crus corresponds to the frontal process field,

PMxF. Lateral wall soft tissue corresponds to the frontal
process field, PMxF. Nasal floor soft tissue corresponds
to the lateral incisor field, PMxB. The rationale of DFR
surgery is to reassign the misplaced fields of the premaxilla
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Carstens

Figure 12 (Case 2): Post op CT scans of piriform fossa in this challenging cleft demonstrate leveling out of the nasal oor to support the alar base. Consolidation of
the alveolar cleft during primary DFR surgery maintains dimensions of the dental arch without collapse even after palatoplasty. Note symmetry of columellar-alara
relaltionships seen in lateral views

Th

into correct position. When the NPP flap is combined

with the reflected mucoperiosteum from the margins of
the bony cleft a surgical pocket is created. This site is
subsequently filled with bone graft or a bone-producing
cytokine (rhBMP-2) such that missing premaxillary fields
are re-synthesized.

The NPP flap functions just like a boxtop to cover the

alveolar cleft. It is almost always adequate. I am convinced
that the secret identity of NPP is the original PMxB.
Because of the cleft this tissue is shipwrecked on the
premaxilla. Unfortunately, NPP is frequently insufficient
to replace PMxF. Every time the lateral crus is released
and advanced a soft tissue defect appears. The defect
represents missing PMxF. In my clinical experience,
this is almost always the size of the auricular cymba. A
composite graft of anterior auricular skin and cartilage
from the cymba is the most reliable replacement for this
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

tissue. The cymba graft, in combination with the NPP

flap, provides more than enough tissue to reconstruct
the missing premaxilla.
Markings are carried out using a modification of the
Millard system. (Paired anterior ethmoid neurovascular
bundles define the true philtrum; this equals the width of
the columella.). First to be marked is point 2, the junction
of the normal philtral column with the white roll. The
width of the columella at its base is then measured. This
distance (usually 6-10 mm) is subsequently marked along
the white roll medial to the philtral column as point 3.
Distance 2-3 is the width of the philtrum; this contains
the two anterior ethmoid arteries (approximately 4-6 mm
apart). The midpoint of the philtrum (point 1 in the Millard
system or nadir of cupids bow) is irrelevant. Point 13,
defined by the bulge of the footplate of the medial crus on
the non-cleft side, marks the shoulder of the columella.
86

Th

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Developmental eld reassignment in unilateral cleft lip

Figure 13 (Case 3): MD Secondary deformities resulting from displacement of tissue from the midline corrected with subperiosteal centralization. Note changes in
the non-cleft alar base after DFR. Residual sag in left alar contour will need cartilage batten graft in the future. The straight-line scar in DFR repair simulates philtral
column.

The corresponding point 12 on the cleft side will be found

displaced caudally and internally with respect to point 13.
Total philtral height 2-13 should equal 3-12. Points 4 and
10 mark the midpoint of the alar bases on non-cleft and
cleft sides, respectively.
On the lateral side, points 9 and 11 mark the terminus of
skin within the nasal cavity. This is located just anterior to
87

the inferior turbinate. Point 9 is the tip of flap D while point

11 is the tip of the future nostril sill C, so-called because it
is in continuity with the alar base C. The dimensions of C
can be roughly mapped out by measuring the nostril sill on
the non-cleft side. This is the distance from the midpoint of
the non-cleft alar base (point 4) to the ipsilateral footplate
point 13. Point 8 marks the natural transition of the white
roll. Distance 8-9 should match 3-12.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

Carstens

layer of fat. The caudal margin of the two muscles defines

the white roll and contains the labial artery.[47]

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Step 2. Medial dissection: the NPP-lateral columellar

advancement flap
The medial margin of the cleft (zone B in our diagram)
consists of prolabial tissue lateral to the true philtrum.
This is in continuity with lateral columellar skin and the
medial crus of the alar cartilage. Conveniently, the blood
supply of these two units is a watershed permitting
disection of a very long flap. The B flap has two parts: (1) a
skin/mucosa flap of the non-philtral philtrum (NPP) and (2)
a chondrocutaneous flap containing the lateral columellar
skin and medial crus (LCC). B = NPP + LCC

Figure 13a: Vissiarnarov ap harvested from scar in case 3 simulates non-philtral

philtrum and lls lateral piriform defect created by advancement of the lateral crus.
Note large pedicle from the external carotid-based nasopalatine artery. These
aps can be harvested in virtually all secondary cleft reconstructions

Dissection sequence
Step 1. Lateral dissection: rescuing the nostril sill
The lateral lip is tensed with a single hook and the skinmucosa margin is incised proceeding upward from 8 to 9,
located just below and anterior to the inferior turbinate.
From here the incision swings around laterally to 10,
the midpoint of the alar base, (but not beyond it at this
point). In this manner, the lateral lip flap D is separated
from the alar base C. This step separates orbicularis
from the nasalis. From point 9, a second, more internal,
incision defines the triangular flap C. The base width of
C can be deduced by measuring the dimensions on the
non-cleft side.

Th

From the lip a lateral vermilion flap (L) is pared off and
dissected down to the alveolar cleft margin. Proper paring
of L includes a small strip of lip skin 2-3 mm wide because
it is rolled-in, with an abnormal relationship to the
underlying muscle. If the surgeon does not do this, the
skin will pucker inward at the final closure. The L flap is
the most optional tissue of the entire DFR operation. It is
best brought medially and interposed between the nasal
vestibular lining and the B flap. It can also be used as a
free graft to the lateral nasal wall. No tissue is sacrificed
in DFR.
With its vermilion stripped off, the lateral lip margin is
now entered and the orbicularis is split into its deep
(constrictor) and superficial (dilator) components. The
deep orbicularis oris (DOO) is shaped like the letter J and
separated from the superficial orbicularis oris (SOO) by a
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

B has a rich blood supply. The NPP component is

irrigated by the nasopalatine artery. The LCC component
gets supply from 2-3 lateral branches of the ipsilateral
anterior ethmoid. In addition, vessels in continuity with
the lateral nasal system from the facial artery run along
the surface of the alar cartilage itself. Once elevated, B
is surprisingly long, reaching all the way across the cleft
to the lateral nasal rim. [Figure B] is inset into the gap
created by advancement of the p5 lateral vestibular lining
(and the lateral crus). This achieves two important surgical
goals: (1) cephalic advancement of the medial crus with
increased tip projection; and (2) soft tissue augmentation
of the lateral nasal wall. Inset of B permits release of
the tethered lateral vestibular lining. Repositioning the
lateral crus now occurs in what would otherwise be a
Y-V pattern without the Y-V closure. Natural alar cartilage
anatomy is accomplished without pinching together an
already-deficient lining.
The future cleft-sided philtral column is determined by
understanding the embryology of the prolabium. The
true philtrum lies between points 2 and 3 and consists
of two fields (each supplied by a separate branch of the
anterior ethmoid artery). The philtral dermis comes from
p5 prosencephalic neural crest and is innervated by V1.
The width of the philtrum is roughly equal to that of the
columella, generally measuring 6-10 mm. Because the
philtrum develops on top of the r2 premaxilla, it receives
additional blood supply from the terminal branches of
the sphenopalatine artery (SPA) emerging at the level of
the septopremaxillary junction. Thus, the philtrum has a
dual blood supply. All remaining prolabial skin and mucosa
(the NPP) is an r2 derivative, supplied by the nasopalatine
artery and innervated by V2. The prolabium of a bilateral
cleft thus consists of four developmental units based on
88

Th

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Developmental eld reassignment in unilateral cleft lip

Figure 14 (Case 4): Developmental eld reassignment of soft tissue elds in bilateral lip/palate cleft repair demonstrates normalization of Cupids bow. Contrary to
current dogma the philtrum and columella are not short in the cleft condition; they are mismatched. By recognizing and reconstructing the missing premaxillary eld,
DFR corrects deciency-induced eld mismatch

embryology, blood supply and innervation: two central

philtral A fields and two lateral B fields.
The embryology of the columella is as follows. The anterior
centrally-located skin comes from p5 and contains the paired
anterior ethmoid arteries. On either side of that central
swatch, the columellar skin extends backwards toward the
89

septum. Just beneath the lateral columellar pillars lie the

medial crura of the lower lateral cartilages (LLC). Because
these pillars serve as the biologic template for the cartilage
to form, dissection of skin away from the crura is extremely
difficult. The LLCs are thus p5 neural crest derivatives. The
upper lateral cartilages lie above the p6 vestibular lining and
are therefore of p6 neural crest origin.
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Carstens

The NPP-LCC flap now resembles a long boot, shaped like

Italy. The toe of the boot extends along the prolabial
margin. Beneath the heel of the boot lies the footplate
of the alar cartilage. This landmark corresponds to point
12. Grasping the heel of B provides instant access to the
medial aspect of the medial crus. This is a safe plane
permitting dissection of the alar cartilage right into the
nasal tip, with the following caveat. The B flap gets blood
supply from the nasopalatine artery via 2-3 branches
emerging at the junction of the premaxilla and vomer.
Gentle spreading along the medial border of the cartilage
will reveal these branches and preserve them. Additional
blood supply descends along the skin. Formerly I would
elevate these NPP-LCC flaps completely, never having an
issue with ischemia. Now however, I think it prudent to
preserve the nasopalatine branches when possible. This
type of blunt dissection is more than sufficient to advance
the nasal tip.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Correction of the cleft lip nose requires releasing the alar

cartilage from two points of entrapment.[49] The deficiency
state of the premaxilla causes a mechanical deformation
of perfectly normal p5 skin on both sides of the nasal
introitus. In the lateral nasal wall, the r2 skin overlying the
premaxillary frontal process (PMxF) is reduced or absent.
Consequently, p5 skin containing the lateral crus of the
alar cartilage is dragged down into this sinkhole located
just in front of the inferior turbinate. The lateral crus is
therefore flattened. On the medial side, the deficiency
of the r2 premaxillary lateral incisor field (PmxB) creates
a sinkhole into which the p5 lateral columellar skin
is displaced. This displaces the medial crus of the alar
cartilage downward and inward compared with the noncleft side.
The LCC-NPP flap is elevated as follows. Under tension, the
NPP skin is elevated in continuity with the lateral columellar
wall. The lateral (internal) incision is made first, separating
the skin from the mucosa all the way to the junction
between the premaxilla and vomer. Although this incision
will eventually be carried up in front of the septum, it is
advisable to stop here. Tension can thus be maintained on
the NPP while it is separated from the philtrum proper.

Th

Next, a second incision, parallel to the first, ascends from

point 3 to the base of the columella. It then extends
into the nose along the lateral sidewall of the columella.
Previously I would continue this incision along the
side of the columella (just anterior to the edge of the
medial crus) and thence bring it directly into the nose
as an infracartilagenous incision. This design made
me concerned about possible scar contracture. At the
suggestion of Dr. John Reinisch, I began to break up the
incision by elevating a medially-based rectangular skin flap
occupying the caudal half of the columella. Just beneath
the Reinisch flap lies the footplate of the lateral nasal
cartilage. At its cephalic margin, the incision is resumed
along lateral columella. At the level of the intermediate
crus the incision transitions beneath the soft triangle
into a standard infracartilagenous incision and is then
continued all the way to the piriform margin.

The anterior incision of the NPP-LCC flap is not sufficient to

advance it. A second parallel incision is required. FIGURE It
starts in the membranous septum about halfway up. It is
then carried downward to the vomer and then re-directed
forward to meet the lateral margin of the B flap. Note
that the membranous septal incision provides immediate
exposure of the cartilaginous septum.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

The septum is now dissected out and freed from the

maxillary crest until it sits passively in the midline. As
growth proceeds the centralized septum will no longer
constitute an abnormal force vector tethering the nasal
tip.
Step 3. Nasal dissection: open-closed rhinoplasty
At this juncture, a standard infracartilagenous incision is
made. This incision is brought all the way to the piriform
rim, following the natural fold between the nasal skin
and the vestibular skin. The caudal extent of this incision
terminates at the internal border of the triangular nostril
sill flap B. The exposure gained via the lateral columellainfracartilaginous incision allows a complete dissection of
the dorsal nasal skin envelope as described by McComb.[50]
The success of the McComb dissection is really an
embryonic field separation. The deep layer comprises
the p6 vestibular epithelium and p6 neural crest upper
lateral cartilages, the blood supply to which comes from
below via the ICA. The superficial layer is p5 nasal skin
and the lower lateral nasal cartilages, the blood supply to
which is also of ICA derivation. Interposed between these
two layers, like a sandwich, is the SMAS layer of facial
muscles derived from the 2nd pharyngeal arch (r4-r5). The
myoblasts come from somitomere 4. The nasal muscles are
compartmentalized by r4 neural crest fascia (the SMAS).
The blood supply to this intermediate 2nd arch layer is
from the facial artery (ECA). This mesenchyme provides
an additional source of blood supply to the alar cartilages.
Careful dissection of the lateral columellar walls from
the columella discloses vessels running along the medial
90

Developmental eld reassignment in unilateral cleft lip

surface of the medial crura. These anastomose the lateral

nasal vessels with the nasopalatine vessels.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Along the lateral nasal rim the dissection is carried

right down to the piriform margin. The proper plane
for separation is achieved by hugging the surface of the
cartilage. The overlying r4 SMAS layer and the p5 skin
layer are left in anatomic continuity. Very little bleeding is
occasioned by this approach. As one reaches the piriform
rim the periosteum is incised and stripped vertically.
Subperiosteal elevation of the soft tissues lateral to the
piriform rim preserves the facial artery arcade. The nasal
skin envelope is then liberated cephalically all the way to
the nasal bones.

these solutions made use of an anteriorly-based inferior

turbinate flap as described by Noordhoff.[53-54] Although this
tissue worked adequately, I had several reservations about
it: (1) the dissection is subtle and difficult to teach; (2)
the tissue type is distinct and not native to the nasal rim;
and (3) healing of the donor site can be accompanied by
crusting and bleeding; and (4) it did not make embryologic
sense. Proper dissection and inset of flap NPP combined
with use of a composite for FTSG made the turbinate flap
unnecessary. The key to getting the most out of NPP is to
include the prolabial vermilion with the skin.

Despite these maneuvers, the vestibular lining is still

tight! The media crus has been completely released and
advanced into the nasal tip but the lateral crus being
remains splayed out and tethered. Releasing the lateral
crus from the vestibular lining has been advocated in the
past. This is technically difficult because it violates the
embryology (recall that the alar cartilage arises as a neural
crest response to a pattern embedded in the vestibular
epithilium). The size and shape of the cleft-side alar
cartilage has been demonstrated to be normal compared
with the non-cleft side.[51] Consequently, the p5 vestibular
lining program must be normal as well. Studies at UNC
demonstrate that the overall surface area of the repaired
cleft nostril is reduced by 30%.[52] The site and dimensions
of the deficit correspond to the soft tissues of PMxF.

Th

The soft tissues of the alveolar cleft PMxB can be found

on the prolabium. The LCC-NPP flap effectively provides
a vascularized roof for the bony cleft. Lateral nasal
wall release uncovers a defect that frequently exceed
the reach of LCC-NPP. The release begins at the inferior
turbinate and continues along the junction of vestibular
and nasal skin. At the apex of the lateral crus it becomes
V-shaped. Due to the prior McComb dissection the lateral
crus is advanced cephalically into symmetry with the
normal side. The internal nasal valves will appear equal.
Left behind is a raw spot roughly the size of the ear
cymba. A composite graft using anterior skin and cartilage
from the cymba provides a sturdy reconstruction for the
airway. The LCC-NPP flap, inset without tension across the
alveolus, comes to rest at the foot of the cymba graft.
Exit the turbinate ap
As the DFR evolved, I employed various solutions to
patch the defect created by the vestibular release. One of
91

Step 4. Intraoral dissection: sliding sulcus S flap

The rationale and design of the sliding sulcus mucoperiosteal
flap stem directly from pioneering work at the University
of Pittsburgh by Sotereanos.[43] This technique involves
a gingival release on the cleft side carried out from
the alveolar cleft to the buttress.[42] This permits wide
subperiosteal dissection over the entire face of the maxilla
below the infraorbital foramen as described by Delaire.[55
56]
A 45 degree backcut up the buttress is performed. The
attached gingival is released. The flap is covered on its
undersurface by a sheet of periosteum, rendering it rather
stiff. The Sotereanos maneuver is a mobilization of the S
flap using a counter-incision in the periosteal sheet itself
parallel to the gum line. The counter-incision, located halfway from the gingival margin to the infraorbital foramen,
is made by just scoring through periosteum. It extends
from the piriform margin straight lateral to the buttress.
At this point it joins up with the previous backcut. These
two incisions make a right angle: the periosteal counterincision is transverse across the maxilla and the buttress
incision is vertical. The combination of these two incisions
releases the lower mucoperiosteal flap S from the upper
mucoperiosteum attached to the orbit.
When S is released, it advances mesially about two tooth
units. The margin of S that was freed from the lateral
border of the cleft now extends across the alveolar cleft
without tension and is sutured to the mucoperiosteum of
the premaxilla. In this way, like a sliding door, the S flap
seals up the anterior aspect of the alveolar cleft.
On the noncleft side, a similar subperiosteal dissection
is done without recourse to a gingival release. A large
bipedicle flap is created, permitting centralization of the
previously lateralized soft tissue envelope. Note that when
cleft lip occurs without an alveolar cleft, bilateral S flaps,
elevated without gingival release, permit proper tensionfree centralization of midface soft tissues.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

Carstens

mucoperiosteal flap elevated directly off the lateral incisor.

The space will fill in nicely. If one desires, the flap donor
site can be grafted with Alloderm.
Limited resources and difficult logistics in economically
developing countries force surgeons treating cleft patients
to adopt different and creative strategies, particularly
when the alveolar cleft is wide. These children often have
no access to pre-surgical orthopedics. They may never
receive orthodontics. They do possess a ready source of
bone graft from the rib. In such cases, achievement of a
consolidated arch and elimination of oronasal fistula are
reasonable goals for primary cleft surgery. The two key
factors for success in these patients are: (1) ability to make
a simple acrylic splint and secure it--2 mm screws work
perfectly well; and (2) meticulous dissection of the alveolar
cleft with attention to its vascular anatomy.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

The S flap then comes in two varieties, each of which has a

specific benefit for the patient. The simple S flap is elevated
in the subperiosteal plane across the entire face of the
maxilla between two points of vertical release: the alveolar
cleft and lateral wall of the buttress. It remains attached
to the teeth. A gingival releasing incision is not required.
By virtue of its ability to centralize the soft tissues of the
entire midface, the simple S flap fulfills two important
functions: (1) the creation of an esthetic soft tissue drape;
and (2) the biologically active osteogeneic potential of the
mucoperiosteal stem cells is transferred forward, thus
enabling bone deposition to take place in centrically for
better facial skeletal projection. The drawback of the simple
S flap is that it cannot produce a mucoperiosteal flap to
cover over the anterior aspect of the alveolar cleft. In such
cases, a rectangular mucoperiosteal flap can be harvested
immediately lateral to the alveolar cleft and brought over
for closure. This design works for narrow clefts.

Th

The compound S flap (as described by Sotereanos)

implies a full gingival releasing incision all the way from
the alveolar cleft back to the tuberosity. This involves
additional operating time. Gingival release from an
alveolus containing erupted primary or secondary
dentition is technically simple. The bone is solid and
the surgical plane easy to follow. Infants are a different
matter. Prior to the 4th month of life maxillary and alveolar
bone is too soft to work with. DFR should be performed
between ages 4-6 months. Dissection is facilitated using
loupe magnification, a #15C or Beaver blade, an amalgam
packer and Molt (#9) or Louisville periosteal elevators.
A compound S flap can be mobilized two full tooth
widths. The main advantage of the compound S flap is
the dramatic increase in mucoperiosteal flap length it
provides. This is generally the width of two dental units.
The anterior aspect of the alveolar cleft is thus covered
with stem cell-containing mucoperiosteum. Disadvantages
of S flap relate to the temporary anatomic disruption of
periocoronal attachments.
The S flap has distinct indications for use in primary and
secondary cleft repair. These are of vital importance,
particularly when one must operate under conditions that
are less than ideal. In a primary cleft, whenever possible,
pre-surgical orthodontic control of the arch should be
accomplished. This may also involve a lip adhesion.
The entire effort is directed toward normalizing arch
dimensions. In these cases, compound S flaps are not
required. Closing the anterior wall of the alveolar cleft
can be accomplished with a rectangular superiorly-based
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

If a dental splint can be fabricated, lip adhesion can narrow

the cleft down to dimensions permitting simultaneous
DFR repair and alveolar grafting with a simple S flap.
When this is not possible, a compound S flap will close
the gap. In some very wide primary unilateral clefts I have
used bilateral compound S flaps to successfully close gaps
of 14-16 mm. Of course, this maneuver disconnects the
frenulum from the midline. Postoperative remodeling of
the alveolus reestablishes symmetry. In such cases, arch
stability justifies the extensive intraoral dissection.
Secondary cleft reconstruction follows the same rules
but with the proviso that gingival release incisions are
fast and easy to accomplish. The floor of the alveolar
reconstruction has already been provided by a pre-existent
palatal repair. When orthodontic capability exists, such
clefts should first be expanded to the original width prior
to grafting. But when the patient has no access to ortho,
S flap alveolar reconstruction can be combined with
DFR to produce a permanent, aesthetically pleasing and
physiologic result in one stage.
Step 5. Dissection of the primary palate
Beginning with the lateral nasal wall, the mucoperiosteum
is elevated off the maxillary alveolar bone. The palatal
margin is undermined as well. This results in a large
mucoperiosteal envelope. This must be separated into
nasal and oral components. This is done at the level of the
shoulder of the alveolus. The incision can be extended
backward a few mm along the edge of the palatal shelf.
On the medial side, the septal mucoperichondrium and
the vomerine mucoperiosteum are elevated in continuity.
92

Developmental eld reassignment in unilateral cleft lip

The surgeon then proceeds forward to elevate the

mucoperiosteum off the premaxilla. Separation of the
envelope occurs at the shoulder of the premaxilla. A
vomer mucoperiosteal flap is elevated and sutured to the
nasal mucosa of the cleft maxilla.

This principle is exactly the same as in prolabial death

reported by Millard, a combination of bilateral rotation
backcuts (destroying the anterior ethmoid supply to
the philtrum) and elevation away from the underlying
premaxilla.[55] In bilateral cleft lip and palate, the anatomy
of both prolabium and premaxilla consists of paired
angiosomes. Disruption can occur in one or the other
with impunity, but not both.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

A six-sided box is created. The medial and lateral walls

are raw bone. The floor is oral mucoperiosteum. The
anterior wall is the sliding sulcus mucoperiosteum. The
roof is the nasal mucoperiosteum + the NPP flap. The
posterior wall is the vomer flap. These flaps are loaded
with undifferentiated mesenchymal stem cells (MSC). The
cambium layer of the periosteum is especially rich in stem
cells. All neural crest MSCs contain membrane-bound
receptors for BMP. This pocket is now ready to be filled
with rib graft (iliac crest graft in older children) or with
an rhBMP-2/ACS implant.

to the GPA. This permits downward dissection of the

mucoperiosteum from the shoulder of the premaxilla or
upward dissection from the oral margin. The premaxilla
remains alive, based on its other sources. In bilateral
palate clefts, no such palatal anastomosis exists. The
mucoperiosteum can only be reflected upward. If this is
done on both sides and if the prolabium is simultaneously
elevated, premaxillary necrosis can ensue.

Th

Alveolar reconstruction, both primary and secondary,

demands precise knowledge regarding the vascular
anatomy of mucoperiosteum bordering the alveolar
cleft. This is a neglected area of cleft surgery. The blood
supply to the premaxilla is the most complex (and
potentially treacherous). It is derived from three sources.
(1) Premaxilla is an r2 derivative. Like all mesenchymal
structures caudal to r1, premaxilla is supplied from the
external carotid. The vascular axis of the vomer and
premaxilla is the medial sphenopalatine (nasopalatine)
artery. PMx mucoperiosteum supplied by the SPA envelops
the bone from above-downward, like a cloak thrown over a
chair. (2) From below, PMx mucoperiosteum is continuous
with that of the r2 maxillary palatal shelf (MxP), the
arterial axis of which is the external carotid-based greater
palatine artery. (3) a second anastomosis exists within the
mucoperiosteum covering the anterior superior aspect of
the premaxilla. This zone is in contact with the soft tissues
of the prolabium, a p5 derivative supplied by the internal
carotid-based anterior ethmoid arteries.
Premaxillary survival depends upon maintenance of
sufficient contact between the bone and its enveloping
mucoperiosteum. Alveolar cleft reconstruction cannot be
accomplished without stripping away the vascular coverage
of PMx, to a greater or lesser degree. Thus, premaxillary
vascular anatomy dictates the direction in which the
mucoperiosteum must be elevated. Surgical strategy for
this maneuver is a direct consequence of the cleft type.
In unilateral palate clefts, the medial alveolar cleft margin
is a continuous zone of external carotid from the SPA
93

Fortunately, the robust blood supply of the maxilla comes

to our rescue. Injection studies demonstrate the maxilla
to be supplied by the pharyngeal branch of the facial
artery and facial branch of the ascending pharyngeal
artery.[56] It is independent of its mucoperiosteal cover.
Meticulous dissection of the lateral alveolar cleft creates
a continuous mucoperiosteal sheet uniting the lateral
nasal wall with that of the hard palate. This contains a
mirror-image external carotid anastomosis, this time
between the medial sphenopalatine and greater palatine
arteries. This can be stripped downward with impunity. It
is a long flap, extending from just in front of the inferior
turbinate all the way to the hard palate. The lateral alveolar
mucoperiosteal flap is of sufficient size to close the entire
floor of the alveolar cleft.
What then should be done for children BCL(P)? Must
one sacrifice arch reconstruction to facial aesthetics or
vice versa? The answer (no!) lies in a staged approach
with careful dissection. Primary lip reconstruction must
not violate the tissue plane separating the prolabium
and mucoperiosteum. Even in a bilateral dissection,
50% of the premaxilla will remain perfused by both SPA
and AEA vessels. Lateral nasal wall reconstruction with
the cymba composite graft is appropriate, Liberation
of the medial crura must be done without entering the
territory of the medial sphenopalatine artery. If this
appears difficult, nasal elevation and lip revision may
have to be staged later. Alveolar cleft closure should
be done by elevating the premaxillary mucoperiosteal
flap upward and the maxillary mucoperiosteal flap
downward.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

Carstens

The tip is positioned anatomically using the Cronin nasal

retractor (Padgett Instruments). The medial crura are
battened together with 5-0 PDS. Suture suspension and
modification of the alar cartilage can be readily executed
by means of the open-closed approach as per the surgeons
preference. The authors approach is predicated on
the establishment of normal field relationships. Wide
dissection of the nasal soft tissues combined with release
from their piriform prison allows all fields to be passively
held in position by a nasal stent inserted at the conclusion
of the procedure. The author finds the Koken-type silicon
stents (Porex Corp, Newman, Georgia) easy to use. The
stent is placed at the end of the surgery. Closure of the
nostril incision starts at the intermediate crus working
medially down to the Reinisch flap. One then proceeds
laterally to the margin of the lateral nasal wall. This
involves placing 2-3 sutures over 5 mm.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Wide bilateral clefts require alternative strategies. Two

principles to avoid trouble in these patients are: (1)
meticulous primary closure of the nasal floor; and (2)
delayed grafting of the alveolar cleft. A wide alveolar
roof defect requires big nasal floor flaps. These are best
harvested at the primary surgery, before other tissues get
in the way. The key point here is to not cut the lateral
mucoperiosteal flap until the medial flap is harvested
from the premaxilla. Once the dimensions of medial flap
are known, a superiorly-based lateral flap of sufficient
size is elevated to complete the anterior nasal closure.
At this point we are still left with a wide alveolar floor
defect and not enough tissue to close it. What does one
do? One waits it out, using a palatal acrylic splint (secured
with pins or 2 mm screws) to maintain the transverse arch
dimensions and prevent collapse.
6 months later, at palatoplasty, the long mucoperiosteal
flaps elevated from the hard palate provided ideal coverage
for the alveolar cleft floor. Bilateral compound S flaps are
raised. These will adequately seal off the anterior face of
the alveolar cleft. The graft (using rhBMP-2 or rib) is placed.
Once again, care must be taken to prevent transverse
collapse until the bone graft has hardened. Intraoral
splinting is thus continued for another 2-3 months.

Th

Secondary grafting in bilateral cases follows the same

principles. The vascular anatomy may have been altered
(primary surgery may have elevated the prolabium
away from the premaxilla). In such cases some degree
of revascularization to the premaxilla occurs over
time. Nonetheless, dissection of the premaxillary
mucoperiosteum must be sparing. All that is required
is elevation of sufficient tissue to close the soft tissue
defect and place the graft. Nasal tip elevation can be
safely accomplished using DFR technique and cymba
grafts. The key point is to preserve soft tissue continuity
between the vomer, the septal mucoperichondrium and
the premaxilla: this is the site of entry of blood supply
into the premaxilla.

Closure sequence
Step 1. Elevation of the nasal tip
The lateral crus is stabilized into symmetry with the
normal side using a transcutaneous 4-0 chromic mattress
stitch. The V-shaped limbs of the releasing incision are
closed. The donor site is reconstructed using a fullthickness retroauricular graft or a composite graft of
anterior ear skin and cartilage. By trial and error I prefer
the cymba as the donor site.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

The remainder of the lateral nasal incision will be filled by

inset of the B flap. Reconstruction of the frontal process
of the premaxilla adds the missing tissue to the lateral
nasal wall (see below). This frequently involves placement
of a composite for full thickness graft into the defect. The
medial crural complex is then elevated with respect to the
septum with 4-0 vicryl. This also closes the membranous
septum counter-incision.
Step 2. Soft tissue reconstruction of the premaxilla
The roof of the missing premaxillary field is reconstructed
based on meticulous closure of the nasal floor. This is
carried out using a mouth gag, starting anteriorly at
the incisive foramen. Because the space is tight using
5-0 Vicryl on a small P-2 needle is helpful. The medial
vomerine and lateral nasal mucoperiosteal flaps are closed
all the way posterior to the end of the vomer flap. This
provides correct orientation for inset of the B flap. The
posterior margin of B is sutured from medial to lateral along
the newly-united nostril floor. The tip of B is eventually
inset into the donor site of the lateral crural advancement
flap. Next, the alar nasal skin flap C is sutured to the
anterior margin of B. The surface area of the lateral nasal
wall is now restored.
The floor of the missing premaxillary field is reconstructed
when the medial and lateral mucoperiosteal flaps
harvested from the walls of the alveolar cleft are turned
down into the mouth using 4-0 vicryl. The cleft side
sliding sulcus flap S is advanced and secured using 4-0
Vicryl PS-2 around the dental units. Three or four such
sutures will suffice. Optional suspension of the sulcus to
94

Developmental eld reassignment in unilateral cleft lip

maintain height can be done by passing a 3-0 vicryl up

to the nasal floor and then back down into the sulcus as
a mattress suture.

Orbicularis closure: Three or 4 sutures of 4-0 PDS are

required for the DOO layer. The SOO layer is closed with
5-0 PDS making sure the loop is at the level of the dermalepidermal junction.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

Step 3. Osseous reconstruction of the premaxilla: In situ

osteogenesis (ISO)
The primary palate is reconstructed with Infuse bone
graft (Memphis, Tennessee Sofamor-Danek). The implant is
prepared by soaking a Helistat activated collagen sponge
(ACS) (Plainsfield, N.J. Integra Life Siences) of preselected
size with reconstituted rhBMP-2 applied uniformly over the
ACS. The minimum time required for binding is 15 minutes;
however, I usually wait 30 minutes. The Infuse comes in
three kit sizes 4.2 cc, 5.6 cc and 8.4 cc. These are used as
follows: primary unilateral (small), primary bilateral (medium),
secondary unilateral (medium) and secondary bilateral (large).
The volume of the implant should match that of the defect.
Mastergraft tricalcium phosphate is useful for bulking up
the implantation site. It acts passively, a space-occupying
agent. One could use freeze-dried bone as well (the ideal
bulking agent does not yet exist.) The Mastergraft is wrapped
with the ACS collagen sponge like a fajita or sushi roll and
placed into the defect. I like to place a vertical component
into the alveolar walls and a horizontal component below
the nasal floor. The pocket created by DFR dissection can be
filled with iliac crest graft as well.

Delaire 2: The purpose of this suture is to control the

height and curvature of the cleft side nostril. This is
accomplished by connection of the nasalis to the anterior
nasal spine However this step is optional at this point. If
performed, care should be taken to not tighten this and
inadvertently narrow the nostril. When the remaining
sutures are placed a, decision can be made if a Delaire 2
is warranted. I usually decide upon a Delaire 2 at the end
of the case to accentuate curvature

Th

Step 4. Closure of the lip: Delaire concepts modified

Many years ago pioneering work by Delaire demonstrated
that wide subperiosteal dissection yield significant aesthetic
benefits of tissue draping and, at the same time, did not
impair maxillary growth.[57-58] Indeed, when compared with
supraperiosteal release, this approach is developmentally
correct because it separates the osteogenic functional
matrix (the periosteum and overlying soft tissues) from the
product (the premaxilla and maxilla).
The suture sequence is as follows:
Delaire 1: Centralization of the bilaterally displaced soft
tissues is accomplished with 4-0 nylon sutures placed from
below the flap to the paranasalis (levator) insertion into the
superficial orbicularis oris (SOO). This insertion does not
involve the alar base. It is generally located one fingerbreadth
lateral to the alar base. The non-cleft side is sutured first
to below the anterior nasal spine. This suture serves as a
reference point for a similar suture from the cleft side.
Suspension of the DOO: This suture sets the depth of the
sulcus on the cleft side. At the most cephalic margin the
DOO is suspended from the septum with a 4-0 PDS.
95

Delaire 3: The oblique head of SOO sets the aesthetic drape

of the lip. The 5-0 PDS is obliquely passed upward from
the cephalic edge through the base of the columella and
then back down to the SOO as a mattress suture.
Step 5 Final adjustments: finessing the nostril floor
After closure of the lip, the alar base C is, at time,
compressed medially by the movement of the neighboring
D flap (lateral lip element). If so, the alar base must
be translocated laterally. This can be accomplished by
excision of a crescent of skin from the lateral lip element.
C is then elevated and secured to the lateral via a buried
5-0 PDS suture. At times it is necessary to elevate the
ala completely in order obtain sufficiently lateralization.
p The tip of the nostril sill flap C is now sutured just
posterior to the columellar shoulder. Continuity between
the columellar shoulder and the nostril sill is now
reestablished. Perialar suturing with inverted 5-0 PDS
sutures restores the alar crease very nicely.
A final caveat concerns the lining of the lateral nasal defect.
Experience with this technique shows that prevention of
post operative contraction is paramount. This requires
placement of a Porex silicon conformer sized to the
patient. The stent should be sutured in place with 4-0
nylon for 8 weeks. It is important that the B flap not be
sutured to the sidewall of C with any tension. If tension
exists I have found it prudent to use a small pinch graft
of retroauricular skin.

DISCUSSION
Whenever a surgical procedure for a congenital condition
in a growing child leads to a predictable pattern of relapse
over time two, inescapable conclusions must be drawn: (1)
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

Carstens

The membranous bones of the maxilla and premaxilla

are just a product of the soft tissue functional matrix
that surrounds them. As the soft tissue grows, new bone
is deposited and old bone resorbed according to the
mechanical forces placed on the bone. The purpose of
subperiosteal centralization in DFR is to change the biologic
relationship of the bone product to the functional matrix.
Over time, the former will adapt to the latter in its new,
centralized position. Appreciation of the subperiosteal
plane as the correct approach to surgically separate the
functional matrix from its product (bone) ensures tensionfree release and accuracy of muscle repair (individual
muscle units can be identified by tugging on them from
below). Thirty years of work by Delaire and others confirm
the clinical accuracy and safety of this approach. Readers
are also advised to study the pioneering work on primary
bone grafting done by Rosenstein.[59-60] This 30 year followup of dental development in patients treated with careful
presurgical orthodontics and rib grafting demonstrates
clearly the safety and advantages of gaining control over
the alveolar cleft at primary surgery.

is
a PD
si F
te is
ho a
st va
(w ed ilab
w by le
w. M f
m e or
ed d fr
kn kno ee
ow w do
.c Pu wn
om b lo
). lica ad
tio fr
ns om

the biologic rationale of the procedure is incorrect; and

(2) the anatomic pattern of relapse points an accusatory
finger at the pathology. DFR surgery represents a new
form of thinking about clefts by identification and
rearrangement of specific developmental fields. The
primary pathology of cleft lip is hypoplasia or absence of
the distal premaxilla. DFR is designed to reconstruct the
missing premaxilla using osteoinductive technologies for
stem cell concentration and differentiation such as rhBMP
2 or by conventional osteoconductive technique (bone
grafting). DFR incisions are made on the basis of vascular
supply and embryology, not geometric manipulation.
For this reason the design of DFR presents a series of
specific solutions that speak to problems in cleft surgery
hitherto inadequately addressed: distortion of the nasal
envelope and septum, displacement of soft tissues away
from the midline, the entrapped position of the medial
crus and the hidden nostril sill. DFR treats mechanisms,
not appearance.

The extraoral design of DFR is invariable; it is the same for

both unilateral and bilateral clefts. Developmental fields
are recognized, separated and rearranged into correct
anatomic relationships. The intraoral design varies with
cleft type. If no alveolar cleft is present, subperiosteal
release and zygomaticomaxillary buttress backcut are
sufficient to achieve centralization of the soft tissue
envelope. Gingival release is not necessary. If a primary
palate cleft exists, a complete sliding sulcus dissection
with gingival release will allow for mesial translocation
and coverage of the alveolar cleft.

Th

DFR includes a straight-line repair, resulting in an

anatomically correct philtral column. The distorted and
seemingly foreshortened philtrum in clefts is familiar
to all plastic surgeons. For many years, the rotationadvancement technique has been used to reorient the
philtrum. So why is philtral rotation not required in DFR?
The appearance of the philtrum in clefts stems from the
overall malposition of the soft tissue in a falsely lateralized
state. Thus, the philtrum and columella appear to be
short, but are actually displaced into the nasal tip. DFR
achieves de-rotation of the philtrum and columella by
the following: (1) field separation; and (2) subperiosteal
mobilization. The entire soft tissue complex in a unilateral
cleft is laterally displaced around the corner of the
premaxilla. When mobilized correctly off the bone it is
brought forward around the curve of the bone and drops
right into place.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

The concepts and techniques of DFR are applicable to all

forms of clefts. Because it separates out osseous pathology
from soft tissue pathology, DFR is a true cut as you go
technique. In secondary cleft surgery, DFR is capable of
rescuing previously violated fields and reuniting them
into their correct functional relationships. Sliding sulcus
flaps function as elastic flaps as defined by Goldstein[61-62]
and can readily be brought into the midline. Abbe flaps
are virtually unnecessary, even in salvage cases involving
total loss of the philtrum.
DFR is a practical application of developmental anatomy. It
provides a means to analyze membranous bone formation
and periosteal physiology. It embodies concepts of facial
growth central to orthodontics. It makes use of neuromeric
theory to map out and manipulate developmental fields.
Proper implementation and study of cleft repair using
DFR will provide a forum for dialog among craniofacial
surgeons orthodontists and developmental biologists.

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with recombinant human bone morphogenetic protein. J Craniofac
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Chin M, Ng T, Tom WK, Carstens MH. 5th International Conference
on Distraction Osteogenesis in Craniofacial Surgery. Paris,
France; June, 2006.
Carstens MH. The sliding sulcus procedure: Simultaneous repair
of unilateral clefts of the lip and primary palate-a new technique.
J Craniofac Surg 1999;10:415-29.
Demas PN, Sotereanos GC. Closure of alveolar clefts with
Corticocancellous block grafts and marrow: A retrospective study.
J Oral Maxillofac Surg 1988;46:682-7.
Delaire J. The potential role of facial muscles in monitoring
maxillary growth and morphogenesis. In: Carlson DS, McNamara
JA Jr, editors. Muscle Adaptation and Craniofacial Growth.
(Center for Human Growth and Development, Craniofacial Growth
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p. 157-80.

Th

is
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4. Carstens MH. Neural tube programming and craniofacial cleft

formation. I. The neuromeric organization of the head and neck.
Eur J Paediatr Neurol 2004;8:181-210.
5. Puelles L, Rubenstein JL. Development of the central nervous
system. In: Rossant J, Tam PP. Mouse development: Patterning,
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6. Puelles L, Rubenstein JL. Forebrain gene expression domains and
the evolving prosomeric model. Trends Neurosci 2003;26:46976.
7. Rubenstein JL, Puelles L. Development of the nervous system.
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errors of metabolism: The molecular basis of clinical disorders of
morphogenesis. Oxford University Press: Oxford, England; 2004.
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8. Carstens MG. Functional matrix cleft repair: A common strategy for
unilateral and bilateral clefts. J Craniofac Surg 2000;11:437-69.
9. Carstens MH. Function matrix cleft repair: Principles and
techniques. Clin Plast Surg 2004;31:159-89.
10. Lemire RJ, Cohen MM Jr, Beckwith JB, Kokich VG, Siebert JR.
The facial features of holoprosencephaly in anencephalic human
specimens. I. Historical review and associated malformations.
Teratology 1981;23:297-303.
11. Siebert JR, Kokich VG, Beckwith JB, Cohen MM Jr, Lemire RJ.
The facial features of holoprosencephaly in anencephalic human
specimens. II. Craniofacial anatomy. Teratology 1981;23:305-15.
12. Liem KF Jr, Tremml G, Roelink H, Jessell TM. Dorsal differentiation
of neural plate cells induced by BMP-mediated signals from
epidermal ectoderm. Cell 1995;82:969-79.
13. Liem, KF, Bemis WE, Walker WF, Grande L. Functional anatomy
of the vertebrates: An evolutionary perspective, 4rd ed. Thompson:
Belmont, California; 2006.
14. Barteczko K, Jacob M. A re-evaluation of the premaxillary bone
in humans. Anat Embryol (Berlin) 2004;207:417-37.
15. Mooney MP, Siegel IP, Kimes KR, Todhunter J. Premaxillary
development in normal and cleft lip and palate human fetuses
using three-dimensional computer reconstruction. Cleft Lip Palate
J 1991;28:49-54.
16. Carstens MH. The spectrum of minimal clefting: Process-oriented
cleft management in the presence of an intact alveolus. J
Craniofac Surg 2000;11:270-94.
17. Gong SG, Guo C. BMP4 gene is expressed at the putative site
of fusion in the midfacial region. Differentiation 2003;71:228-36.
18. Ashique AM, Fu K, Richman JM. Endogenous bone morphogenetic
proteins regulate outgrowth and epithelial survival during avian lip
fusion. Development 2002;129:4647-60.
19. Lumsden A, Krumauf R. Patterning the vertebrate neuraxis.
Science 1996;274:1109-15.
20. Noden DM. Patterning of avian craniofacial muscles. Dev Biol
1986;116:347-56.
21. Noden DM. Interactions and fates of avian craniofacial
mesenchyme. Development 1988;103:121-40.
22. Noden DM. Origins and patterns of craniofacial mesenchymal
tissues. J Craniofac Genet Dev Biol 1991;11:192-213.
23. Noden DM, Trainor PA. Relations and interactions between cranial
mesoderm and neural crest populations. J Anat 2005;207:575601.
24. Helms JA, Cordero D, Tapadia MD. New insights into craniofacial
morphogenesis. Development 2005;132:851-61.
25. Carlson BR. Human embryology and developmental biology, 3rd
ed. Mosby: St. Louis; 2004.
26. Vissarionov VA. Analysis of the results of reconstructive plastic
surgery of unilateral clefts of the upper lip based on data of the
surgical section 1970-1977. In: Pathogenesis, treatment and
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40.

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45.

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reference as to the anatomy of the premaxilla. Previous papers
describing the non-existence of the human premaxilla debunked.
Butler, AB, Hodos W. Comparative Vertebrate Neuroanatomy. New
York: Wiley-Liss, 1997. Fundamental conservation of neuroembryologic
developmental units is described. Functional model relating motor and
sensory neural structures serving between axial and pharyngeal arch
musculature in alternating pairs of somitomeres.
Carlson BR. Human Embryology and Developmental Biology 3rd ed.
Philadelphia: Mosby, 2004. Very clear account of how an organism
forms with a good dose of modern biology. Exceptional illustrations.
Francis-West PH, Robson I, Evans DJ. Craniofacial development:
the tissue and molecular interactions that control development of the

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46. Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and
palate. I. Normal development and cleft palate. Br J Oral Maxillofac
Surg 1992;30:287-95.
47. Moss ML, Vilmann H, Das Gupta G, Skalak R. Craniofacial
growth in space-time. In: Carlson DS, McNamara Jr JA, editors.
Craniofacial biology. Center for Human Growth and Development,
University of Michigan: Ann Arbor, MI; 1981.
48. Park GC. The importance of accurate orbicularis repair of the
orbicularis oris muscle in unilateral cleft lip. Plast Reconstr Surg
1995;96:780-8.
49. Carstens MH. Correction of the bilateral cleft using the sliding
sulcus technique. J Craniofac Surg 2000;11:137-67.
50. Carstens MH. Correction of the unilateral cleft lip nasal deformity
using the sliding sulcus procedure. J Craniofac Surg 1999;10:34664.
51. McComb H. Treatment of the unilateral cleft lip nose. Plast
Reconstr Surg 1975;55:596-601.

52. Stenstrom SJ. The alar cartilage and the nasal deformity in
unilateral cleft lip. Plast Reconstr Surg 1966;38:223-31.
53. Drake AF, Davis JU, Warren DW. Nasal airway size in cleft and
noncleft children. Laryngoscope 1993;103:915-7.

54. Noordhoff MS, Chen YR, Chen KT. The surgical technique for the
complete unilateral cleft lip-nasal deformity. Op Tech Plast Surg
1995;2:164-74.
55. Noordhoff MS. The surgical technique for the unilateral cleft

lip-nasal deformity. Noordhoff Craniofacial Foundation: Taipei,

Taiwan; 1997.
56. Millard DR. Cleft Craft II. Bilateral and rare deformities. Little,
Brown: Boston; 1977. p. 259-65.
57. Siebert JW, Angrigiani C, McCarthy JG, Longaker MT. Blood

supply of the Lefort I maxillary segment: an anatomic study. Plast

Reconstr Surg 1997;100:843-51.
58. Delaire J, Precious D, Gordeef A. The advantage of wide
subperiosteal exposure in primary surgical correction of labial
maxillary clefts. Scand J Plast Reconstr Surg Hand Surg

1989;22:147-51.
59. Precious DA, Delaire J. Surgical considerations in patients with cleft
deformities. In: Bell WH, editor. Modern practice in orthognathic
and reconstructive surgery. WB Saunders: Philadelphia; 1992. p.

390-425.
60. Rosenstein SW, Grassechi M, Dado DV. A long-term retrospective
assessment of facial growth, secondary surgery need and
maxillary lateral incisor status in a surgical-orthodontic protocol
for complete clefts. Plast Reconstr Surg 2003;111:1-13.

61. Rosenstein SW. Early bone grafting of alveolar cleft deformities.

J Oral Maxillofac Surg 2003;61:1078-81.
62. Goldstein MH. A tissue expanding vermilion myocutaneous ap
for lip repair. Plast Reconstr Surg 1984;73:768-70.
63. Goldstein MH. The elastic ap for lip repair. Plast Reconstr Surg
1990;85:446-52.

head. Adv Anat Embryol Cell Biol 2003; 169:1-138. New concepts of
developmental biology applied to the head are succinctly reviewed.

Futayama D. Evoution. Sunderland, Massachusetts: Sinauer, 2005. Up-

to-date discussion of the mechanisms underlying evolution, including

Hox genes and the dawn of modern evolutionary developmental

biology.

Gilbert S. Developmental Biology, 8th ed. Sunderland, Massachusetts,

2006. Written for advanced undergrads, this outstanding text is

sumptuously-illustrated; the accompanying DVD and website, www.

devbio.com, have a wealth of detail including real-time movies of key
developmental processes. This is the medical professionals go-to
source to understand molecular embryology.

Hall BK. The Neural Crest in Development and Evolution. New York:

Springer, 1997. Very readable discussion of the 4th germ layer and the
source of nearly all craniofacial bones.

Hanken J, Hall BK. The Skull. Chicago: University of Chicago Press,

1993. This series includes 3 volumes (Development, Patterns of

Structural and Systematic Diversity, Functional and Evolutionary

Mechanisms) summarizing the entire literature.

Huang R, Zhi Q, Patel K, Wilting J, Christ b. Contribution of single
somites to the skeleton and muscles of the occipital and cervical regions
in avian embryos. Anat Embryol 2000; 202:375-383. Mapping study of
neural crest cells from a single somite leading was later expanded.

Hu D, Marcucio RS, Helms JA. A zone of frontonasal ectoderm

regulated patterning and growth in the face. Development 2003;

130:1749-1758. Neural crest derivatives such as bone and cartilage

involve exchange of information between epithelia and mesenchyme.
The model reported here may explain frontal bone development as
programming by p5 skin/dermis and underlying p5 brain covered by
r1 dura. The presence of two distinct templates may map the frontal

ANNOTATED BIBLIOGRAPHY

bone into two distinct laminae, the interface between is a potential

space, the frontal sinus.

Basic science/embryology
These references provide a basis for understanding the
neuromeric system and developmental fields.

Jacobson AG. Somitomeres: mesodermal segments in the head and

trunk. In: Hanken J, Hall, BK (eds). The Vertebrate Skull. Vol I. Pp. 42-76.
Chicago: Univ Chicago Press, 1993. Clear discussion of somitomeres.
Both Carlson and Gilbert make use of this work in discussing how

Barteczko K, Jacob M. A re-evaluation of the premaxillary bone in

embryonic tissues are segmentally organized. Fate maps of craniofacial

humans. Anat Embryol (Berlin) 2004; 207:417-437. The denitive

mesenchymal derivatives are based on this concept.

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Developmental eld reassignment in unilateral cleft lip

Puelles L, Rubenstein JLR. Forebrain gene expression domains and

Normal and Pathologic Development. Copenhagen, Denmark:

the evolving prosomeric model. Trends Neurosci 2003;26:469-476.

Munksgaard, 1999. Slim volume packed with important ideas. Careful

Revised version of prosomeric theory.

analysis of craniofacial bone ossication is the conceptual basis for the

Rubenstein, JLR, Regionalization of the prosencephalic neural plate.

Lego model of sequential developmental eld assembly. Not afraid

Annu Rev Neurosci 1998; 21:445-477. Gene product mapping used

to speculateahead of its time.

to dene prosomeres.

LeDouarin NM, Kalcheim C. The Neural Crest, 2nd ed. Cambridge, UK:

Tapadia MD, Cordero DR, Helms JA. Its all in your head: new insights

Cambridge University Press, 1999. Gerard Couly and Nicole LeDourain

into craniofacial development and deformation. J Anat 2005;207:461

revolutionized neural crest embryology with the quail-chick chimera

477. Excellent synthesis of new ideas, including the premandibular

model. This laboratory has mapped out the origins and fate of neural

arch. Illustrations are rst rate.

crest cells throughout the embryo.

Cleft Lip and Palate Surgery

Liem, KF, Bemis WE, Walker WF, Grande L. Functional anatomy of the

These references cover the bases of developmental eld theory

Vertebrates: An Evolutionary Perspective, 3rd ed. Belmont, California:

Carstens MH. The sliding sulcus procedure: simultaneous repair

Thompson, 2001. No theory of human craniofacial development can

of unilateral clefts of the lip and primary palate-a new technique. J

exist without reference to other vertebrates. The 4th edition includes

Craniofac Surg 1999; 10:415-434. Principles of periosteal anatomy are

contributions of Hox genes and neuromeres.

applied to surgical correction of soft tissues on both sides of the cleft.

Lumsden A, Krumauf R. Patterning the vertebrate neuraxis. Science

Bardachs contributions are discussed in a new context. The resulting

1996; 274:1109-15. Key reference correlating neural content of

centralization of the facial functional matrix leads to more anatomic

rhombomeres.

deposition of membranous bone over time. Process theory (a model

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Kjaer I, Keeling JW, Fischer-Hansen B. The Prenatal Human Cranium

Morriss-Kay GM, Wilkie AOM. Growth and the normal skull vault

of cleft formation as a series of pathologic mechanisms/processes

and its alteraion in craniosynostosis: insights from Human genetics

acting over time) was presented. Surgical correction would therefore

and experimental studies. J Anat 2005; 207:637-653. Morriss-Kay

become a logical correction of each one of these processes such that

demonstrated in previous papers the origin of the parietal bone from

the patient would grow into the repair.

PAM in mammals versus neural crest.

Carstens MH. Correction of the unilateral cleft lip nasal deformity

Muller F, ORahilly. The timing and sequence of appearance of

using the sliding sulcus procedure. J Craniofac Surg 1999; 10:346

neuromeres and their derivatives in staged human embryos. Acta

364. Contribution of the decient piriform platform to alar cartilage

Anat 1997;158:83-99. The master plan of cranio-caudal development

malposition is combined with concepts of mechanical displacement of

the premaxilla and maxilla. Soft tissue deciency of the lateral nasal

ORahilly R, Muller F. Developmental Stages in Human Embryos.

wall not yet linked to the premaxillary eld.

Washington, D.C.: Carnegie Institution Publication 637, 1987. The

Carstens MH. Correction of the bilateral cleft using the sliding sulcus

Carnegie staging system for human embryos is described here. This

technique. J Craniofac Surg 2000; 11:136-167. Commonality of

reference is used by embryologists world-wide.

pathology between unilateral and bilateral clefts is emphasized

ORahilly R, Muller F. The Embryonic Human Brain: An Atlas

but incision design reflects lack of understanding of soft tissue

of Developmental Stages, 2 nd ed. New York: Wiley-Liss, 1999.

developmental eld anatomy. Embryonic origin of facial musculature

Visualization of neuroaantomy in motion visually comprehensible for

and the anatomy of the philtrum are discussed.

any surgeon.

Carstens MH. The spectrum of minimal clefting: process-oriented cleft

Th

is described. This is the basis of homeotic-gene specic levels.

Osumi-Yamashita N, Ninomiya Y, Doi H, Eto K. The contribution of

management in the presence of an intact alveolus. J Craniofac Surg

both forebrain and midbrain crest cells to the mesenchyme in the

2000;11:270-94. For the rst time I was able to grasp the sequential

frontonasal mass of mouse embryos. Dev Biol 1994;164:409-419.

assembly of soft tissues around the cleft. The genetic signalling

Timing of when PNC, MNC and RNC populations complete migration

relationships between the underlying premaxillary field and the

comes from this lab.

overlying soft tissues was as yet unknown. By this time I was aware

Osumi-Yamashita N, Ninomiya Y, Eto K. Mammalian craniofacial

of neuromeric theory but had not yet envisioned the layering off facial

embryology in vitro. In J Dev Biol 1997;41:187-194.

soft tissue elds over bone elds.

Puelles L. Brain segmentation and forebrain development in amniotes.

Carstens MH. Sequential cleft management with the sliding sulcus

Brain Res Bull 2001; 55:695-710. Luis Puelles and John Rubenstein

technique and alveolar extension palatoplasty. J Craniofac Surg 2000;

discovered and mapped out the segmental organization of the forebrain

11:437-469. As described by Delaire, cleft palate shares with cleft lip

(prosencephalon). Neural crest in association with a given prosomere

common pathophysiologic mechanisms that, over time, produce the

creates external mesenchymal structures (forehead, nose, philtrum, eye),

nal anatomic state. Both buccal and lingual aspects of the alveolus

the development of which coincides with the corresponding zones in the

have a similar pattern of blood supply. Preservation of the entire

brain. Anomalies are often shared. The brain predicts the face.

alveolar mucoperiosteum as a osteosynthetic developmental eld

99

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Carstens
with references.

AEP procedure prevents residual anterior stula. The importance of

Schendel SA, Tessier P, Tulasne J-F. Facial clefting disorders and

Schweckendiecks work is discussed.

craniofacial dysostoses: skeletal considerations. In: Turvey TA, Vig

Carstens MH. Functional matrix cleft repair. J Craniofac Surg

KWL, Fonseca RJ. Facial Clefts and Craniosynostosis: Principles and

Carstens MH. Function Matrix Cleft Repair: Principles and Techniques.

Management. 1996. Philadelphia: WB Saunders, pp. 95-128. Good

Clin Plast Surg 2004; 31:159-189. Detailed discussion of how to do

skull photos of the synostoses.

it. As my understanding of developmental elds improved the FMR

Tessier P. Fentes orbito-faciales verticales et obliques (colobomas)

became the DFR (Developmental Field Repair). All the principles of

completes et frustes. Ann Chir Plast 1969;19:301-311. Original report

FMR are preserved in the current technique. In DFR (as discussed

based on his empiric observations helped to organize facial clefts into

here), dissection of the philtrum and the lateral nasal wall are

recognizeable patterns.

combined with the correct placement of the A1 ap and the deliberate

Tessier P. Anatomical classications of facial, cranio-facial and laterofacial

reconstruction of the premaxilla with rhBMP-2.

clefts. J Maxillofac Surg 1976;4:69-92. Seminal contribution in English

Carstens MH. Development of the facial midline. J Craniofac Surg

establishing a geographic schematization of facial clefts. Developmental

2002;13:129-87. This paper discusses two topics, the neuroembryology

eld model categorizes clefts into a neuromeric distribution explains

of the facial placodes and the neuromeric mapping of neural crest

Tessiers observations on a neuroembryologic basis.

derivatives in the face. A three-dimensional model of facial development

Vissiarionov VA. Analysis of the results of reconstructive plastic surgery

based on Carnegie stages is presented.

of unilateral clefts of the upper lip based on data of the surgical section

Carstens MH. Neural tube programming and craniofacial cleft

1970-1977. In: Pathogenesis, treatment and prophylaxis of cosmetic

formation. I. The neuromeric organization of the head and neck. Eur

deformities and deciencies. Moscow: Institute of Cosmetology; 1982,

J Paed Neurol 2004; 8:181-210. Save for some confusion about the

pp. 118-21

number and relative contributions of occipital somites this paper

Recombinant Human Bone Morphogenetic Protein-2

constitutes my rst-pass introduction into the neuromeric system.

Relevant preclinical studies and clinical applications in the maxilla

Delaire J. The potential role of facial muscles in monitoring maxillary

and mandible.

growth and morphogenesis. In Carlson DS, McNamara Jr JA (eds).

Boyne PJ. Animal studies of application of rhBMP-2 in maxillofacial

Muscle Adaptation and Craniofacial Growth. Ann Arbor, MI: University

reconstruction. Bone 1996; 19(suppl):83S-92S. Early work proving

of Michigan, 1978, pp.157-180 (Center for Human Growth and

BMP resynthesizes membranous bone.

Development, Craniofacial Growth Monograph No. 8).

Boyne PJ. A feasibility study evaluating rhBMP-2/absorbable collagen

is
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m e or
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kn kno ee
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). lica ad
tio fr
ns om

is a key point of the surgery. The additional length gained by the

Delaire J, Precious D, Gordeef a. The advantage of wide subperiosteal

sponge for maxillary sinus oor augmentation. Int J Periodontic

exposure in primary surgical correction of labial maxillary clefts.

Restorative Dent 1997;17:11-25. Fiirst clinical application in craniofacial

Scand J Plast Recons Surg 1989; 22:147-151. Pioneering work

skeleton was to support maxillary implants.

on subperiosteal dissection directly following basic science of

Boyne PJ. Application of bone morphogenetic proteins in the treatment

membranous bone formation represented a functional approach to

of clinical oral and maxillofacial osseous defects. J bone Joint Surg

2001; 83A(suppl)S146-S150. Demonstrates potential breadth of
applications for Infuse.

door for me to visualize cleft formation as a 4-D process acting over

Boyne PJ, Nath R, Nakamura A. Human recombinant BMP-2 in

time. DFR surgery is a direct descendent of this work.

osseous reconstruction of simulated cleft palate defects. Br J Oral

Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and palate

Maxillofac Surg 1998; 36:84-90. Immediate grafting with BMP

I. Normal development and cleft palate. Br J Oral Surg 1992;30:287

maintains the dental arch in primates. This was a key idea for eventual

95. Force vector concept and its effects on bone structure presented

incorporation into DFR.

and illustrated with clarity.

Boyne PJ, Nakamura A, Shabahang S. Evaluation of long-term effect

Moss ML, Vilmann H, Das Gupta G, Skalak R. Craniofacial growth

of function on rhBMP-2 regenerated hemimandibulectomy defects. Br

in space-time. In Carlson DS, McNamara Jr JA (eds). Craniofacial

J Oral Maxillofac Surg 1999; 37:344-352.

biology. Ann Arbor, MI: Center for Human Growth and Development,

Carstens MH, Chin M, Li J. In situ osteogenesis (ISO): Regeneration

University of Michigan, 1981. Balanced facial growth requireds that all

of 10-cm defect in porcine model using recombinant human bone

soft tissue developmental elds become physiologically centralized to

morphogenetic protein-2 (rhBMP-2) and Helistat absorbable collagen

synthesize facial bone in the correct position over time.

sponge J Craniofac Surg 2005; 16: 1033-42.

Precious DA, Delaire J. Surgical considerations in patients with

Carstens M, Chin M, Ng T, Tom WK. Reconstruction of #7 facial cleft with

cleft deformities. In: Bell WH (ed.). Modern practice in orthognathic

distraction assisted in situ osteogenesis (DISO): Role of recombinant

and reconstructive surgery. Philadelphia, WB Saunders; 1992; p.

human bone morphogenetic protein-2 with Helistat activated collagen

390-425. Good discussion of Delaires technique is presented along

sponge. J Craniofac Surg 2005 16(6): 1023-1032.

Th

cleft repair. Delaire was the rst to emphasize physiological principles

of soft tissue-bone interaction over time. Reading Delaire opened the

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Developmental eld reassignment in unilateral cleft lip

Chao M, Donovan T, Sotelo C, Carstens MH. In situ osteogenesis

Ruberte J, Carretero A, Navarro M, Marcucio RS, Noden DM.

of hemimandible in a 9-year-old boy: osteoinduction via stem cell

Morphogenesis of blood vessels in the head muscles of avian

concentration. J Craniofac Surg 2006; 17:405-412. 12 cm segmental

embryos: spatial, temporal and VEGF expression analysis. Dev

mandibular defect reconstructed with BMP-directed ISO. Free bula

Dynam 2003;227:470-483. Important concept suggesting anatomic

transfer was avoided. Response of the regenerate to surrounding soft

basis of peripheral arterial system via Schwann cell induction from

tissue forces was to remodel into a replica of the normal side.

peripheral nerves. BMP-directed neural crest release of VEGF to

Chin M, Carstens MH. Distraction osteogenesis with bone

promote rapid vascularization of surgical site was a concept derived

morphogenetic protein enhancement: facial cleft repair in humans. Proc

from this work.

4 International Congress on Cranial and Facial Distraction Processes.

Seeherman H, Wozney J, Li R. Bone morphogenetic protein delivery

Paris, France, 197-200, 2003. DISO was invented with the original #2,

systems. Spine 2002; 27(165):516-523. Carriers required to retain

th

BMP-2 in the target environment are reviewed.

Chin M, Ng T, Tom WK, Carstens MH. Repair of alveolar clefts with

Urist, MR, Strates BS. Bone morphogenetic protein. J Dent Res 1971;

recombinant human bone morphogenetic protein. J Craniofac Surg

50:1392-1406. The original report. PJ Boyne studied under Urist.

2005; 16(5):778-789. Original study done using DFR concepts in

Valentin-Opran, Wozney J, Csiima C, Lilly L, Reidel GE. Clinical

is
a PD
si F
te is
ho a
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(w ed ilab
w by le
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m e or
ed d fr
kn kno ee
ow w do
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om b lo
). lica ad
tio fr
ns om

#7 cleft repair which we published 2 years later.

evaluation of recombinant human bone morphogenetic protein-2.

Ebara S, Nakayama K. Mechanisms for the action of bone

Clin Orthop Related Res 2002;395:110-120. Relevant clinical data

morphogenetic proteins and regulation of their activity. Spine 2002

succinctly summarized. Very useful reference.

27(16S):S10-S15. Induction of osteoclasts from monocytes sets up

Wozney J. Overview of bone morphogenetic proteins. Spine

negative feedback loop.

2002;27(165):52-58. Physical characteristics of BMP discussed.

Th

secondary cleft cases.

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EPILOGUE FOR COLLEAGUES IN INDIA AND

SOUTHEAST ASIA

is
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ns om

I should like to depart at this point from traditional

academic writing to address the reader in a very personal
way. Many of you must treat children and adults with clefts
under the most demanding of circumstances. Poverty,
poor communication, distance from medical centers
and difficulties in transportation negatively affect every
aspect of your treatment plan. The same is true for your
patients, no matter how motivated they might be. Time
taken to bring a child in for treatment from a remote
location may present an intolerable stress on families
living in these circumstances. For many, if not most, of the
worlds children with clefts, these factors make careful,
well-regulated multidisciplinary care a distant mirage so
easy to talk about, so difficult to achieve.

DFR was born in post-war Nicaragua, under the harshest

of conditions. I was assigned there to work for the
Pan American Health Organization (WHO) as a surgical
consultant in 1990-1992. At that time, the backlog of
plastic surgery cases was estimated at 10,000 patients.
Problems were not only logistical but also the creation
of manpower. Working with Professor Arturo Gomez at
the Universidad Nacional de Nicaragua in Leon and with
a small group of lay people, the first medical foundation
in the history of Nicaragua was formed. Noel Icaza, an
angricultural engineer, gave it the name Nicaplast. We
traveled around the country, operating and working within
various cities to create support for this apparently crazy
idea. Nicaragua had descended into chaos. The gross
national product was just above that of Haiti, making
this formerly active exporting nation the second-poorest
of all the Americas. Given this situation, the creation of
resources to resuscitate reconstructive surgery seemed
an impossible task.

For these patients the surgical team must set priorities

to accomplish the maximum physiologic correction with
the minimum of surgeries. The goals at the first stage
are: (1) a functional nasal airway; (2) a united dental arch
without collapse; (3) subperiosteal dissection designed
to promote centric facial growth; and (5) soft tissue
correction using developmental field concepts to place
scars into embryonic separation planes. The goals of the
follow-up stage are: (1) meticulous three-layer closure
of the soft palate; and (2) use of AEP (alveolar extension
palatoplasty) flaps to prevent anterior fistula. Should the
patient be unable to return for further work, this should
provide at the very least an intact oral cavity in some
degree of alignment, a working airway, reasonable speech
and an appearance that is socially acceptable.

Th

Developmental Field Reassignment cleft surgery was

designed with these circumstances in mind. It is a high
intellect/low technology method. The only structures
missing are a lateral nasal wall soft tissue defect (equal
to the cymba) and an alveolar bone defect (equal to
the housing of the lateral incisor). Reconstruction of
these vital elements is within the hands of all cleft
surgeons. There are only two requirements for DFR to be
properly performed: (1) in-depth anatomic knowledge of
embryologic field anatomy; and (2) the technical ability
to make and secure an acrylic palatal splint. How one
achieves alveolar reconstruction is irrelevant. One may use
cancellous graft (rib in young patients) + platelet enriched
plasma. One may use rhBMP-2. What really matters is to
follow biologic principles.
Indian J Plast Surg January-June 2007 Vol 40 Issue 1

At this point, Carlos and Vivian Pellas stepped in. These

remarkable people, from a highly educated and wealthy
family, survived a commercial airline crash in 1989. 170
perished at the scene. The Pellas were evacuated to
Miami where they underwent numerous surgeries for
burns and fractures including free tissue transfer. Upon
recuperating they returned to Nicaragua determined
to help children with burns and deformities. Their
support for Nicaplast resulted in the establishment
of new residency training at Unan-Leon, improved
infrastucture and a complete modernization of the
pediatric burn unit in the capital city, Managua. Partner
relationships were constructed with Interplast, the
University of Wisconsin, Physicians for Peace and the
AO Foundation in Basel, Switzerland. Each one of these
entities contributed toward expanded reconstructive
surgical care and advanced education. In time, a new
foundation, under the leadership of Vivian Pellas, was
established. The Asociacion Pro Ninos Quemados de
Nicaragua (APROQUEN) now has direct responsibility
for burn care in that country.
The residency was particularly innovative. The longest in
Latin America, it demands full general surgery + 3 years of
plastic surgery, preparing one graduate every three years.
Participation by the resident in all activities with visiting
teams created a broad exposure. Mini-fellowships were
created. Each graduate undergoes final oral examination in
English with the participation of national and international
102

Developmental eld reassignment in unilateral cleft lip

faculty. All three graduates remain in the public sector.

Dr. Gustavo Herdocia work at UNAN-Leon while Dr. Mario
Perez and Dr. Leonel Briceno staff the APROQUEN burn
unit. Over 4000 surgeries and 100,000 outpatients have
benefited from these efforts without a single dollar from
abroad.

I write this to you, the reader, to propose that improvements

in cleft care can always be made, even under difficult
circumstances. Our patients speak for themselves. The
primary task is to make it possible for the national medical
teams to achieve the logistical support required to provide
the care. And, in this regard, innovative efforts in the
private sector, backed by lay people with the desire to
help, to bypass roadblocks and provide better service.

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But human resources are not enough. While Nicaplast

continues its valuable work in Leon, APROQUEN has
surged forward as a modern-day model of a medical
foundation with a bold strategy: the creation of a financial
engine to finance the clinical work and cost of running
a 1st-class burn center in an extremely poor country. In
1972 a terrible earthquake destroyed much of the capital
city, including the main hospital for the country. Extensive
outside aid was lost to corruption; the hospital was never
rebuilt. 4 temporary structures built 35 years ago continue
to provide care to the population. A high quality center
of excellence was nonexistent.

12 years ago I proposed this plan to Carlos and Vivian

Pellas over rum on a muggy Managua night over several
rounds of Flor de Cana Rum. In 2005 the Hospital
Metropolitano Vivian Pellas opened its doors. HMVP exists
because people cared deeply, refused to give up hope and
worked unceasingly to make it happen. And the stunning
fact of the entire Nicaragua experience is that it happened
from within. Information regarding these foundations is
available to all surgeons on the Internet.

Th

Burns are a socioeconomic disease. Nicaragua has

1000 new burn cases per month, 10% of which require
admission. Given the many needs in the health sector,
public resources are grossly inadequate for these patients.
Knowing this, APROQUEN partnered with other investors
to build the most modern (albeit small) medical facility in
the country, with state-of-the-art=radiology, surgery and
intensive care. The facility would serve the international
community and private patients. It would be the very best
in Central America. Trips to Miami would be avoided. But
the key concept is that that APROQUEN would maintain
51% ownershipand a percentage of profits would
thus directed to the burn service and reinvested for the
foundation.

As I look backward and contemplate the evolution of

developmental field theory and the DFR repair, I realize
that it followed a number of steps, some forward and some
backward. The driving force behind innovation comes
from restless curiosity and the capacity to demand that
anatomy must make sense. Nature is trying to tell us her
secrets if we can only learn to listen. As we understand
how the face is constructed, we will learn the sequence
that leads to cleft formation. This inevitably will direct us
toward better techniques and better results. As a cleftaffected person I know the reality behind the surgeries
we perform for our patients. We must push forward to do
better. Hopefully, DFR surgery will lead you to question old
assumptions, strive for perfection and (most importantly)
find your own innovations.

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Indian J Plast Surg January-June 2007 Vol 40 Issue 1