GIGANTISM
DEFINITION
Gigantism, excessive growth in stature, well beyond the average for
the individual’s heredity and environmental conditions. Tall stature may
result from hereditary, dietary, or other factors. Gigantism is caused
by disease or disorder in those parts of the endocrine system that regulate
growth and development. Androgen deficiency, for example, delays the
closure of end plates, or epiphyses, of the long bones, which usually takes
place when full growth is achieved. If the pituitary gland functions normally,
producing appropriate amounts of growth hormone, while epiphyseal
closure is delayed, the growth period of the bones will be prolonged.
Gigantism associated with androgen deficiency is more frequent in men
than in women and may be genetic.
Another type of gigantism associated with endocrine disorder
is pituitary gigantism, caused by hypersecretion of growth hormone
(somatotropin), during childhood or adolescence, prior to epiphyseal
closure. Pituitary gigantism is usually associated with a tumor of the
pituitary gland.
In pituitary gigantism, growth is gradual but continuous and
consistent; the affected person, with bones in normal proportion, may attain
a height of eight feet. Muscles may be well developed but later undergo
some atrophy or weakening. The life span of pituitary giants is shorter than
normal because of their greater susceptibility to infection and metabolic
disorders. Treatment by surgery or irradiation of the pituitary gland curtails
further growth, but stature cannot be reduced once gigantism has occurred.
Gigantism is a rare condition that causes abnormal growth in
children. This change is most notable in terms of height, but girth is
affected as well. It occurs when the child’s pituitary gland makes too much
growth hormone, which is also known as somatotropin.
CAUSES
From the time someone is born, the way their body grows is
controlled by hormones produced by the pituitary gland in the brain. The
most important hormone for growth is called growth hormone, also known
as human growth hormone, HGH or GH.
Most children with gigantism have too much growth hormone, which
makes them grow too much, too fast. Gigantism is almost always caused
by a benign tumor, also known as an adenoma, growing in the pituitary
gland. Usually, there is no obvious reason for this, although it may be due
to rare genetic conditions.
There are also rare genetic conditions that can cause gigantism
without the child having an adenoma. Examples include Sotos syndrome,
Beckwith-Wiedemann syndrome, and Weaver syndrome.
Gigantism is caused by an overproduction of growth hormone (GH) that
occurs in childhood before the bone growth plates have closed. Diseases,
disorders and conditions that can cause GH overproduction leading to
gigantism include:
Benign tumors of the pituitary gland, which press on the pituitary
gland and cause excessive GH production. This is the most
common cause of gigantism.
Carney complex (rare gene mutation that causes a high risk of
certain tumors including pituitary adenoma)
McCune-Albright syndrome (syndrome caused by a gene
mutation)
Multiple endocrine neoplasia type 1 (inherited disorder that
causes tumors to form in endocrine glands and makes them
hyperactive)
Neurofibromatosis (genetic disorder causing tumor growth)
SIGNS AND SYMPTONS
The main symptom associated with gigantism is large body stature
with increased height compared to peers. Muscles and organs may be
enlarged as well. Other symptoms include:
Delay in puberty
Physical changes similar to patients with acromegaly, including:
o Abnormal enlargement of the hands and feet
o Widening of distal fingers and toes, called "paddle" toes
o Hands feel like soft bread dough
Changes in facial features, which can be quite prominent:
o Enlarged forehead and jaw
o Pronounced under bite
o Spreading teeth
o Enlarged tongue, nose and lips
Deepening of the voice in males
Skin changes:
o Increased number of skin tags
o Oily skin and excessive sweating
Increased tissue in the wrists can lead to nerve compression, which
may cause carpal tunnel syndrome
Children with gigantism may also have flat noses and large heads,
lips, or tongues. The symptoms may depend on the size of the pituitary
gland tumor. As the tumor grows, it may press on nerves in the brain. Many
people experience headaches, vision problems, or nausea from tumors in
this area. Other symptoms of gigantism may include:
severe or recurrent headaches
weakness
insomnia and other sleep disorders
irregular menstrual periods in girls
deafness
TREATMENT
Treatments for gigantism aim to stop or slow your child’s production
of growth hormones.
Surgery
Surgery is the best form of treatment and cures 80% of the
cases of gigantism. The surgeon will gain access the pituitary gland
using the transsphenoidal approach—so named because the route
crosses, or transects, the sphenoid bone. This bone is located behind
the nose, mostly within the skull.
Using precise surgical instruments, the surgeon will make an
incision through your child’s nasal cavity to create an opening in the
sphenoid bone. Once the surgeon gains access to sphenoid sinus
(the air-filled area behind the sphenoid bone), further incisions will be
made until a hole is created in the sella turcica—the bone that cradles
and protects the pituitary gland.
After the pituitary gland is in the operative field, removal of the
tumor can proceed. The surgeon will use high magnification to readily
distinguish normal pituitary tissue from the tumor.
Microsurgery uses a powerful operating microscope to help
distinguish between tiny structures in and around the pituitary
gland.
Endoscopic surgery uses small tubes and a tiny camera to help
remove the tumor in small pieces.
Most patients are able to return home the day after their surgery
for removal of a pituitary adenoma, and nasal packing is seldom
required.
Treatment with a prescription medication is possible if surgery
does not cure the child’s gigantism or if surgery is not recommended.
Medicines are also sometimes prescribed before surgery to improve the
likelihood of a good outcome.
Octreotide or lanreotide are synthetic forms of the hormone
somatostatin and stop the release of growth hormone. They are
often effective for the long-term control of gigantism, but they can
only be administered by injection every two to four weeks. If the
tumor is particularly large, these drugs may be administered
before surgery. Due to the side effects of these drugs and their
expense, surgery to achieve a long-term cure is preferable.
Bromocriptine and cabergoline are from a class of drugs called
dopamine agonists. They can lower IGF-1 and growth hormone
levels in about half of the people treated with them (although the
symptoms could improve even if your IGF-1 and growth hormone
levels do not decrease). Although not as effective as the synthetic
hormones octreotide and lanreotide, they are less expensive and
more convenient to administer, because no injection is required.
They can be combined with octreotide in children with no adverse
effects on long-term health.
Pegvisomant is a recently developed drug that blocks the action of
growth hormone in your body, thereby lowering IGF-1 levels. It
must be administered by subcutaneous (beneath the skin)
injection daily. It is another option if the child does not respond to
surgery or other medications, or if the child cannot tolerate these
treatments for other reasons.
Gamma Knife
Gamma Knife radiosurgery is a highly advanced form of
radiotherapy that is used to achieve similar results to the traditional
surgical techniques. However, with Gamma Knife it can take several
years for growth hormone and levels to return to normal, rather than
days or weeks as with traditional surgery. It is typically a treatment of
last resort in patients with gigantism.
The ‘knife’ in this surgery is actually made up of many small
beams of radiation focused on a single point. Each individual beam
too weak enough to damage healthy tissue, but at the point where the
beams converge they deliver a dose of radiation that is lethal to the
tumor.
Gamma Knife is an outpatient procedure, does not involve any
incisions, and requires only brief sedation under general anesthetic.
However, there are additional considerations for pediatric Gamma
Knife surgery.
NURSING INTERVENTION
Nursing priorities are to help the client cope with physical changes,
pace activities to prevent fatigue, relieve discomfort from headaches,
abdominal distention r/t organ enlargement, and skeletal pain.
Prepare for radiation therapy of pituitary gland, if prescribed.
Postoperatively: Monitor VS, LOC, perform frequent neuro
assessments, monitor for signs of increased ICP, and meningitis.
Elevate HOB
Administer corticosteriods as prescribed, on time
Monitor for adrenal insufficiency
Monitor nasal drainage for cerebrospinal fluid (CSF).
Monitor for F & E values
Monitor for diabetes insipidus due to ADH disturbances
–Avoid water intoxification
Remind to avoid drinking from straws, sneezing, coughing, and
bending over to prevent graft dislodgement.
Provide understanding and compassionate support to families and
clients.
Encourage parents to express their feelings.
Encourage clients to share a sense of helplessness, shame, fear
associated with disease manifestations.
Acting as an advocate and liaison clients and families, with other
health care team members.
Encourage clients to socialize with their surroundings.
Encourage client involvement in recreational and diversionary
activities are age-appropriate.
Encourage self-care and activities when strength permits.
References:
https://www.healthline.com/health/gigantism#causes
https://www.healthdirect.gov.au/gigantism
http://pituitary.ucla.edu/resources
https://www.barrowneuro.org/specialty/gigantism/#:~:text=Pharmacological%20Treatment
%20and%20Hormone%20Therapy&text=Octreotide%20or%20lanreotide%20are
%20synthetic,every%20two%20to%20four%20weeks
https://www.britannica.com/science/gigantism
https://nanda-nursing-care-plan.blogspot.com/2014/07/gigantism-nursing-diagnosis-altered.html
