Giant Omphalocele, Intrinsic Duodenal Obstruction

TE Herman and MJ Siegel Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO, USA

Journal of Perinatology (2011) 31, 293–295; doi:10.1038/jp.2010.163 Denouement and discussion Patient 1 demonstrated duodenal atresia with a complete Case obstruction and a double bubble sign on the UGI. The patient was Case 1 taken to the operating room, where duodenal atresia with an A 2290-g infant girl was born at 34 weeks gestation by C-section annular pancreas was found and she was treated with a section for pre-term labor, footling breech presentation and large duodenoduodenostomy. Unfortunately, she had a prolonged omphalocele. The pregnancy had been complicated by antenatal hospitalization complicated by continued poor feeding, prolonged diagnosis of polyhydramnios and omphalocele containing both parenteral nutrition, pulmonary hypoplasia, episodes of sepsis and bowel and liver. Chromosomes obtained at amniocentesis were died at 7 months of age. normal; 46 XX. At birth the baby was confirmed to have a giant Patient 2 was found to have a duodenal web on the UGI series. omphalocele (Figure 1). Apgars were 5 at 1 min and 8 at 5 min. The patient was taken to the operating room, where the web There was no evidence of macroglossia, hemihypertrophy or was divided anteriorly to avoid injury to the sphincter of Oddi. macrosomia. The patient was taken to the operating room on the This patient is alive and tolerating oral feedings at 3 months of first day of life, where the omphalocele was closed with skin age. The patient is treated with Phenobarbital for seizures without cardiovascular compromise. The child lost weight and fed probably related to hypoxic ischemic encephalopathy, but is poorly, so on the fifteenth day of life an upper gastrointestinal hemodynamically stable, not requiring surgical intervention (UGI) series was performed (Figure 2). for Tetralogy of Fallot up to the current date. Case 2 A 2640-g infant boy was born at 38 weeks gestation by cesarean section. The pregnancy had been complicated by a diagnosis of large omphalocele, Tetralogy of Fallot and intrauterine growth retardation. Chromosomes obtained at amniocentesis were normal 46 XY. After delivery, Apgars were 9 at 1 min and 9 at 5 min. Initial radiographs demonstrated an apparent double bubble (Figure 3). On the first day of life, omphalocele closure was performed with gortex. No intestinal atresia was detected at the time of omphalocele repair. Adhesiolysis was performed around the stomach and proximal bowel. A small anterior diaphragmatic defect was present, an almost midline small Morgagni hernia. This was closed. Postoperatively, the infant remained well oxygenated, with sonographic evidence of good flow through the right ventricular outflow tract. However, feeding difficulties developed, with worsening emesis with feedings. Therefore, at 3 weeks of life an UGI series was performed (Figure 4).

Correspondence: Dr TE Herman, Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 South Kingshighway Boulevard, St Louis, MO 63110, USA. Figure 1 Frontal supine radiograph of chest and abdomen. There is a large soft E-mail: [email protected] tissue mass overlying the right upper quadrant, a giant omphalocele. Relative Received 17 September 2010; accepted 14 October 2010 cardiomegaly is present secondary to the small thoracic cavity. Giant omphalocele TE Herman and MJ Siegel 294

Figure 3 (a) Frontal supine film of chest and abdomen R. (b) Lateral decubitus film of chest and abdomen. A giant omphalocele is present. On the frontal film there is an indication of a ‘double bubble’ sign with air in the stomach (S) and slightly dilated duodenum (D). On the left lateral radiograph (L), air has passed into the distal bowel loops and the soft tissue density of the liver (LIV) is appreciated along the superior aspect of the omphalocele.

Figure 2 Frontal supine radiograph during upper gastrointestinal series. Complete duodenal obstruction is present with a dilated duodenal bulb (D) consistent with duodenal atresia.

An omphalocele is a congenital defect of closure of the 1,2 Figure 4 (a) Frontal supine film of the abdomen during upper gastrointestinal umbilical ring usually occurring at 4 weeks of gestation. series (UGI). (b) Coned-down film of the proximal duodenum during UGI. There The eviscerated abdominal contents are covered by a is a high-grade obstruction appearing as a dilated stomach (ST) and duodenal three-layered membrane of peritoneum, Wharton jelly and bulb (DB). There is no bowel gas distal to the stomach. On the coned-down amnion.1 Omphaloceles are divided into minor and giant. A minor image, this obstruction is seen to be due to a thick membrane (arrow) at the omphalocele has a defect measuring less than 4 mm and does not transition from dilated duodenum to collapsed duodenum (open arrow). have the liver eviscerated, and is also called omphalocele with intracorporeal liver.1,3 Minor omphalocele can be closed almost consequence of the underdevelopment of the thoracic cavity due to always primarily. Giant omphalocele has a defect ring of greater the eviscerated liver.2 than 5 cm and contains liver, and is also called omphalocele with To the best of our knowledge, intrinsic duodenal obstruction extracorporeal liver. Associated anomalies among omphalocele had not been reported in omphalocele. The prior duodenal patients are very common, including Beckwith Wiedemann obstructions reported in giant omphaloceles have been extrinsic syndrome, pentalogy of Cantrell, trisomy 13, trisomy 18 and obstructions due to a malformed liver.5,6 This type of obstruction is trisomy 21.1 Chromosomal abnormalities are somewhat more presumably caused by an abnormal growth of the caudate lobe of frequent in patients with minor omphaloceles.3 the liver around the antropyloric region, allowed by the Giant omphalocele is complicated by abdominal cavity and extracorporeal location of both. This complication may be thoracic underdevelopment. This can lead to two significant corrected by either gastrojejunostomy or, in some cases, complications. First, because of the abdominal cavity conservative management with gastric decompression by an underdevelopment and the large size of eviscerated abdominal indwelling feeding tube.5,6 Our cases show that intrinsic contents, these lesions cannot be repaired initially, but require obstruction of the duodenumFatresia or webFcan also occur in initial coverage and delayed closure often by enlarging the patients with giant omphaloceles. Differentiation of intrinsic from abdominal cavity using intraperitoneal tissue expanders.2–4 The extrinsic duodenal obstruction is important so that appropriate other complication is pulmonary hypoplasia, believed to be a treatment can be started early.1

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Conﬂict of interest 3 St Vil D, Shaw KS, Lallier M, Yazbeck S, DiLorenzo M, Grignon A et al. The authors declare no conﬂict of interest. Chromosomal anomalies in newborns with omphalocele. J Pediatr Surg 1996; 31: 831–834. 4 Foglia R, Kane A, Becker D, Asz-Sigall J, Mychaliska G. Management of giant omphalocele with rapid creation of abdominal domain. J Pediatr Surg 2006; 41: References 704–709. 1 Van Eijck FC, Hoogeveen YL, van Weel C, Rieu PNMF, Wijnen RMH. Minor and 5 Oh KS, Strife JL, Fischer KC, Teele R. Pyloroduodenal deformity due to liver giant omphalocele: long term outcomes and quality of life. J Pediatr Surg 2009; 44: malformation associated with omphalocele. Am J Roentgenol 1977; 128: 135–1359. 957–960. 2 Martin AE, Khan A, Kim DS, Muratore CS, Luks FI. The use of intraabdominal tissue 6 Bruce J, Afshani E, Karp MP, Jewett TC. Omphalocele with pyloroduodenal expanders as a primary strategy for closure of giant omphaloceles. J Pediatr Surg 2009; obstruction by extrinsic hepatic compression: a case report. J Pediatr Surg 1988; 23: 44: 178–182. 1018–1020.

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