SYNDROMIC VIGNETTES IN ANAESTHESIA
Anaesthesia for prune belly syndrome
Adrian Bösenberg, MBChB, DA(SA) FFA (SA)
Department of Anaesthesia, University of Cape Town, South Africa
Synopsis of patient: An 18month old, 12.8kg male presented Figure 1a and 1b: Typical wrinkled skin of the infant’s abdomen at rest
for laparotomy. He is a known ‘prune belly’ syndrome and had under anaesthesia.
undescended testes. The purpose of the laparotomy was to lo-
cate his testes and perform a bilateral orchidopexy if possible.
His mother gave a history that he suffered from recurrent chest
infections but his chest was clear on the day of surgery. He had
no cardiac abnormalities. His abdominal musculature was de-
ficient and had no tone. It seemed to consist of folds of loose
redundant skin (Fig1a and b). On straining or coughing the ab-
dominal wall was very protuberant. (Fig 2). He was known to
have bilateral hydronephrosis with compromised renal func-
tion. His electrolytes were within normal limits and the urea
slightly elevated. Haemoglobin was 9.8gm. (Haematocrit 28).
His clotting profile was normal. No premedication was given.
He received halothane anaesthesia supplemented with an epi- 1A
dural at T11-12. Relaxants were not considered necessary. An
epidural infusion of 0.2% bupivacaine at 1.5ml/hour was used
for 48 hours and the postoperative course was uneventful.
PRUNE BELLY SYNDROME
Prune belly syndrome is rare with an estimated occurrence of
1:40000 births. The name is derived from the characteristic
wrinkled appearance of the abdomen that resembles a prune.
The syndrome usually consists of a triad of congenital anoma-
lies - deficiency of the abdominal wall musculature, cryp-
torchidism and urinary tract anomalies.1-3 The syndrome is not
confined to this triad and may also involve the respiratory,
cardiovascular, skeletal and gastrointestinal systems in 75% 1B
of affected individuals.1-3
More than 95% are boys and there is no obvious racial pre-
dilection. Females usually only have abdominal wall involve-
Figure 2: Abdominal appearance of the same infant while coughing prior to
ment. The prognosis may vary from death in utero or a nor- extubation.
mal life expectancy.2 Survival depends on the severity of the
renal and pulmonary impairment with 20% dying within the
first month of life and 50% within 2 years. The syndrome may
also be referred to as Eagle-Barrett syndrome, Triad syndrome
or abdominal musculature syndrome.1,2
Although prune belly syndrome is considered a distinct
entity by most investigators, there is no consensus as to its
pathogenesis despite extensive study of clinical cases and
pathological material.2 The embryological theory suggests that
Correspondence:
Professor A Bosenberg
email: bosie@[Link]
Southern African Journal of Anaesthesia & Analgesia - May 2004 10
SYNDROMIC VIGNETTES IN ANAESTHESIA
there is an aberration of mesenchymal development at around evaluation of renal function is therefore important. Bilateral
six weeks gestation whilst others consider the distal urinary undescended testes are characteristic. The urinary tract anoma-
tract obstruction as the primary underlying mechanism. A vas- lies, if present, are not as severe in affected females.
cular aetiology is a third proposal, which attempts to explain Gastro-intestinal manifestations: Chronic constipation
the lower limb abnormalities, but these are probably second- may be a presenting feature. Malrotation and imperforate anus
ary to iliac vessel compression by the dilated urinary tract are also common presenting problems. Reflux may present an
rather than a primary vascular insufficiency. additional anaesthetic risk.
Cardio-respiratory manifestations: Pulmonary hypopla- Anaesthetic considerations: In view of the abnormal ab-
sia may occur as a consequence of oligohydramnios caused dominal musculature, ventilatory support should be manda-
by the distal urinary tract obstruction in utero. If severe, the tory as spontaneous ventilation may be significantly impaired
pulmonary hypoplasia may lead to stillbirth or demise in the by general anaesthesia. Muscle relaxants are probably not nec-
early neonatal period. essary in view of the lax abdominal wall. The response to
In those presenting for surgery respiratory complications muscle relaxants is said to be normal but compromised renal
are the leading cause of postoperative morbidity.3 These chil- function may affect elimination. All drugs dependant on the
dren have an ineffective cough because of the deficient ab- kidneys for excretion should be used with caution.
dominal musculature and the relative flat diaphragm. Pectus Some authors advise the use of neuromuscular blockers
excavatum may compound the problem.4 The risk of respira- when ventilating these patients to reduce the risk of pneu-
tory infection is increased as a result. Opiate analgesics should mothorax or pneumomediastinum particularly in those indi-
therefore be used with caution, and regional anaesthesia is con- vidual with pulmonary hypoplasia.3 All respiratory depressants
sidered the best option.4 should be used with caution.3,8
Laryngeal atresia5 and difficult intubation6 have been de-
scribed in association with this syndrome. The airway should Conclusion
be secured rapidly wherever possible, to reduce the risk of Although this syndrome is rare, a knowledge of the patho-
regurgitation. physiology involved is essential to plan an appropriate anaes-
Cardiac defects may be seen in approximately 10% of af- thetic.8 Perioperative management can go a long way to pre-
fected individuals. The most commonly encountered defects venting serious morbidity or even death.3
are similar to those seen in children with cardiac defects in
the general population i.e. patent ductus arteriosus, atrial sep- References
tal defects, ventricular septal defects and Tetralogy of Fallot. 1. Jennings RW. Prune belly syndrome. Semin Paediatr Surg.2000;
Prophylactic antibiotic cover is advised. 9:115-20.
Orthopaedic manifestations: The prevalence of muscu- 2. Wheatley JM, Stephens FD, Hutson JM. Prune belly syndrome:
loskeletal problems requiring orthopaedic intervention is in ongoing controversies regarding pathogenesis and management.
the order of 45%.7 The involvement may be congenital or de- Semin Paediatr Surg 1996; 5: 95-106.
velopmental. Congenital anomalies affect the lower limbs in 3. Henderson AM, Vallis CJ, Sumner E. Anaesthesia and prune belly
particular and include club feet, limb deficiencies, hip dys- syndrome. Anaesthesia 1987; 42: 54-60.
plasias and vertebral malformations. The suggested mecha- 4. Heisler DB, Lebowitz P, Barst SM. Pectus excavatum repair in a
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structed urinary tract. The developmental defects may be sec- 69.
ondary to renal disease (renal osteodystrophy) or include 5. Lyon AJ. Congenital atresia of the larynx in association with prune
scoliosis and pectus carinatum or pectus excavatum.4 These belly syndrome. J Army Med Corps 1983;120:118
defects support a mesenchymal defect. 6. Baris S, Karakaya D, Ustun E, Tur A, Rizalar R. Complicated air-
Genito-urinary manifestations: Distal urinary tract ob- way management of prune belly syndrome. Paediatr Anaes 2001;
struction is usually not detectable clinically, suggesting a func- 11:501-4.
tional rather than a physical cause for the obstruction. This 7. Loder RT, Guiboux JP, Bloom DA, Hensinger RN. Musculo-skel-
may lead to severe dilatation of the bladder and ureters caus- etal aspects of prune belly syndrome. Am J Dis Child 1992; 146:
ing bilateral hydronephrosis. Kidneys are often small and dys- 1224-9
plastic. Affected individuals are prone to urinary tract infec- 8. Holder JP. Pathophysiologic and anaesthetic correlations of the
tions, increasing the likelihood of renal failure. Preoperative prune belly syndrome. AANA J 1989;57:137-41.
Southern African Journal of Anaesthesia & Analgesia - May 2004 11
