Indian J Surg DOI 10.1007/s12262-013-0805-8
IMAGES IN SURGERY
Omphalocele Major with Absent Lower Limb Vijay Kumar Kundal & Mufique Gajdhar & Raksha Kundal & Reyaz Ahmed & Leela Dhar Agrawal
Received: 10 October 2012 / Accepted: 2 January 2013 # Association of Surgeons of India 2013
Abstract A newborn delivered by Caesarian section presented with an absent anterior abdominal wall and visible bowel loops and liver. The defect was covered by a thin membrane. The patient had associated absent left lower limb and right foot fusion defect. The patient was haemodynamically stable; general condition was average. No genito-urinary abnormality was detected. The anal opening was present normally, and the patient passed meconium immediately after birth. A diagnosis of omphalocele major with amelia was made. The patient was initially managed by topical application of povidone–iodine for eschar formation and epithelisation of the sac. The patient was discharged after 1 week with advice for regular follow-up. Keywords Omphalocele major . Amelia
The Case A 37-week-gestation male baby born by Caesarian section presents immediately after birth with a large abdominal wall defect. The defect was covered by a thin transparent
membrane under which intestinal loops and liver were visible (Fig. 1). The patient also had an absent whole left lower limb but a well-formed pelvis and abnormally fused right foot (Fig. 2). A thin band of membrane was passing from the lower aspect of the anterior abdominal wall to the lower back (Fig. 3). The patient’s general condition was average and hemodynamically stable. The anal opening was present normally. A normalsized phallus with both testes in the scrotum were seen. The patient passed meconium and urine immediately after birth. The patient was accepting and tolerating the mother’s feeding well. Echocardiography was normal. There was no significant antenatal history regarding drug or radiation exposure. A diagnosis of omphalocele major with amelia was made. No immediate surgical procedure was performed. Initial management of omphalocele major was done by application of topical povidone–iodine. It promotes eschar formation and epithelialisation of the sac. The patient was discharged after 7 days with instructions to apply povidone–iodine regularly and advised for followup after 4 weeks for definitive treatment.
Discussion V. K. Kundal (*) : M. Gajdhar : R. Ahmed : L. D. Agrawal Dept of Paediatric Surgery, SMS Medical College and Associated SPMCHI, Jaipur 302004, India e-mail: [email protected] R. Kundal Dept of Anaesthesia, SMS Medical College and Associated SPMCHI, Jaipur, India
Omphalocele major is a congenital defect of the anterior abdominal wall. The incidence of omphalocele is 17 per 100,000 [1]. The defect is covered by a sac consisting of the amnion, peritoneum and Wharton’s jelly. When the contents of the sac include liver, along with bowel loops and spleen, it is referred to as omphalocele major. Congenital absence of the limb/limbs is called amelia.
Indian J Surg
Fig. 1 The neonate with omphalocele and fused right foot
Fig. 3 The perineum of the neonate
The presence of abdominal wall defects associated with a variable spectrum of visceral and limb anomalies are called limb–body wall complex (LBWC). Van Allen et al. in 1987 [2] gave the diagnostic criteria for LBWC, i.e. presence of two of following three abnormalities: (1) encephalocele/exencephaly with facial clefts, (2) thoraco or abdominoschisis and (3) limb defects. Various theories proposed for LBWC include anomalies of early embryonic disc/ectodermal placodes, amniotic rupture, compressions, bands, vascular hypoperfusion and failure of amnion fusion [3]. The prognosis of LBWC is poor
when compared to isolated anterior abdominal wall defects [4]. LBWC associated with severe systemic anomalies has a high mortality. A pelvic–limb prosthesis can be used for management of amelia. Management of omphalocele includes proper evaluation followed by either primary closure or staged repair, i.e. direct closure, application of dura patch, Vicryl mesh and silo [5]. Non-operative treatment includes topical application of escharotic agents like 25 % aqueous mercurochrome, 70 % alcohol or povidone–iodine on the omphalocele sac which allows epithelisation of the sac and delayed closure.
References
Fig. 2 The omphalocele sac and absent right lower limb
1. Calzolari E, Bianchi F, Dolk H, Milan M (1995) Omphalocele and gastroschisis in Europe: a survey of 3 million births 1980–1990. Am J Med Gen 58:187–194 2. Van Allen MI, Curry C, Gallagher L (1987) Limb body wall complex: 1, pathogenesis. Am J Med Genet 28:529–548 3. Hunter AGW, Seaver LH, Stevenson RE (2011) Limb-body wall defect. Is there a defensible hypothesis and can it explain all the associated anomalies? Am J Med Genet Part A 155:2045–2059 4. Pumberger W, Schaller A, Bernaschek G (2001) Limb-body wall complex, a compound anomaly pattern in body-wall defects. Pediatr Surg Int 17:486–490 5. Henrich K, Huemmer HP, Reingruber B, Weber PG (2008) Gastroschisis and omphalocele: treatments and long term outcomes. Pediatr Surg Int 24:167–173
IMAGES IN SURGERY
Omphalocele Major with Absent Lower Limb Vijay Kumar Kundal & Mufique Gajdhar & Raksha Kundal & Reyaz Ahmed & Leela Dhar Agrawal
Received: 10 October 2012 / Accepted: 2 January 2013 # Association of Surgeons of India 2013
Abstract A newborn delivered by Caesarian section presented with an absent anterior abdominal wall and visible bowel loops and liver. The defect was covered by a thin membrane. The patient had associated absent left lower limb and right foot fusion defect. The patient was haemodynamically stable; general condition was average. No genito-urinary abnormality was detected. The anal opening was present normally, and the patient passed meconium immediately after birth. A diagnosis of omphalocele major with amelia was made. The patient was initially managed by topical application of povidone–iodine for eschar formation and epithelisation of the sac. The patient was discharged after 1 week with advice for regular follow-up. Keywords Omphalocele major . Amelia
The Case A 37-week-gestation male baby born by Caesarian section presents immediately after birth with a large abdominal wall defect. The defect was covered by a thin transparent
membrane under which intestinal loops and liver were visible (Fig. 1). The patient also had an absent whole left lower limb but a well-formed pelvis and abnormally fused right foot (Fig. 2). A thin band of membrane was passing from the lower aspect of the anterior abdominal wall to the lower back (Fig. 3). The patient’s general condition was average and hemodynamically stable. The anal opening was present normally. A normalsized phallus with both testes in the scrotum were seen. The patient passed meconium and urine immediately after birth. The patient was accepting and tolerating the mother’s feeding well. Echocardiography was normal. There was no significant antenatal history regarding drug or radiation exposure. A diagnosis of omphalocele major with amelia was made. No immediate surgical procedure was performed. Initial management of omphalocele major was done by application of topical povidone–iodine. It promotes eschar formation and epithelialisation of the sac. The patient was discharged after 7 days with instructions to apply povidone–iodine regularly and advised for followup after 4 weeks for definitive treatment.
Discussion V. K. Kundal (*) : M. Gajdhar : R. Ahmed : L. D. Agrawal Dept of Paediatric Surgery, SMS Medical College and Associated SPMCHI, Jaipur 302004, India e-mail: [email protected] R. Kundal Dept of Anaesthesia, SMS Medical College and Associated SPMCHI, Jaipur, India
Omphalocele major is a congenital defect of the anterior abdominal wall. The incidence of omphalocele is 17 per 100,000 [1]. The defect is covered by a sac consisting of the amnion, peritoneum and Wharton’s jelly. When the contents of the sac include liver, along with bowel loops and spleen, it is referred to as omphalocele major. Congenital absence of the limb/limbs is called amelia.
Indian J Surg
Fig. 1 The neonate with omphalocele and fused right foot
Fig. 3 The perineum of the neonate
The presence of abdominal wall defects associated with a variable spectrum of visceral and limb anomalies are called limb–body wall complex (LBWC). Van Allen et al. in 1987 [2] gave the diagnostic criteria for LBWC, i.e. presence of two of following three abnormalities: (1) encephalocele/exencephaly with facial clefts, (2) thoraco or abdominoschisis and (3) limb defects. Various theories proposed for LBWC include anomalies of early embryonic disc/ectodermal placodes, amniotic rupture, compressions, bands, vascular hypoperfusion and failure of amnion fusion [3]. The prognosis of LBWC is poor
when compared to isolated anterior abdominal wall defects [4]. LBWC associated with severe systemic anomalies has a high mortality. A pelvic–limb prosthesis can be used for management of amelia. Management of omphalocele includes proper evaluation followed by either primary closure or staged repair, i.e. direct closure, application of dura patch, Vicryl mesh and silo [5]. Non-operative treatment includes topical application of escharotic agents like 25 % aqueous mercurochrome, 70 % alcohol or povidone–iodine on the omphalocele sac which allows epithelisation of the sac and delayed closure.
References
Fig. 2 The omphalocele sac and absent right lower limb
1. Calzolari E, Bianchi F, Dolk H, Milan M (1995) Omphalocele and gastroschisis in Europe: a survey of 3 million births 1980–1990. Am J Med Gen 58:187–194 2. Van Allen MI, Curry C, Gallagher L (1987) Limb body wall complex: 1, pathogenesis. Am J Med Genet 28:529–548 3. Hunter AGW, Seaver LH, Stevenson RE (2011) Limb-body wall defect. Is there a defensible hypothesis and can it explain all the associated anomalies? Am J Med Genet Part A 155:2045–2059 4. Pumberger W, Schaller A, Bernaschek G (2001) Limb-body wall complex, a compound anomaly pattern in body-wall defects. Pediatr Surg Int 17:486–490 5. Henrich K, Huemmer HP, Reingruber B, Weber PG (2008) Gastroschisis and omphalocele: treatments and long term outcomes. Pediatr Surg Int 24:167–173
