Congenital epigastric evisceration: a case report

Congenital Epigastric Evisceration: A Case Report By Montserrat Castan˜o´n, Luciano Guimara˜es, Xavier Tarrado, Begon˜a San Vicente, Asteria Albert, and Luis Morales Barcelona, Spain

A rare case of abdominal wall defect at the epigastric midline is presented. This newborn boy had only the greater omentum eviscerated, and no other abnormalities could be detected. This case does not seem to relate to the abdominal wall defects reported so far in the literature. This singular case contributes to enlarge the spectrum of congenital defects of the abdominal wall.

J Pediatr Surg 38:1253-1254. © 2003 Elsevier Inc. All rights reserved.

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uneventful postoperative course and was discharged 7 days after surgery.

BDOMINAL WALL defects (AWD) often are seen in association with other malformations or clinical features. There is not yet agreement about etiopathogenesis, nomenclature, and classification of AWD. Several investigators have attempted to make a classification, but none has so far been able to cover all known types of AWD. This singular case enlarges the spectrum of congenital defects of the abdominal wall. CASE REPORT

A 4-hour-old newborn was transferred to our hospital with the diagnosis of gastroschisis made at birth. He was the second child of a healthy 38-year-old mother. First trimester amniocentesis had been performed because of maternal age. No previous disease had been reported during prenatal examinations. He was delivered by elective cesarean section at 38 gestational weeks because of breech presentation and the mother’s history of 2 previous uterine procedures— one myomectomy and one cesarean section. His birth weight was 3.400 kg, and Apgar score was 5/10 at 1 and 5 minutes. An uncovered epigastric defect was identified at birth, through which, a mass of tissue was eviscerated. Physical examination on admission showed greater omentum protruding through the abdominal wall in the midline, 2 cm below the xiphoid process in an otherwise normal boy. The defect measured 1.5 ⫻ 1.5 cm. The omental mass measured 3.8 ⫻ 2.2 ⫻ 0.8 cm and was edematous and congested but showed no signs of necrosis; it was adherent all round the abdominal wall defect (Fig 1). The umbilical cord was normally implanted onto the abdominal wall and showed no abnormality. Neither clinical examination nor laboratory and radiologic tests showed any other type of abnormality. A transverse laparotomy was performed through the epigastric defect. The eviscerated content, which consisted of edematous greater omentum, was resected. After a normal inspection of the abdominal cavity the abdomen was closed in layers. The skin edges of the defect were fused to the parietal peritoneum, as seen in gastroschisis. On pathologic examination, the resected specimen was identified as omentum, showing nothing but edema, congestion, and enlarged vessels, compatible with chronic inflammation. The baby had an

Journal of Pediatric Surgery, Vol 38, No 8 (August), 2003: pp 1253-1254

INDEX WORDS: Abdominal wall defect, evisceration, congenital.

DISCUSSION

None of the proposed classifications and pathogenetic explanations of AWD from Duhamel’s first scientific contributions on this subject1 up to the most recent, can be considered either complete or sufficient to cover all known malformations.2-4 In 1953, Moore and Stokes5 established criteria for classifying gastroschisis and omphalocele. In 1968 Daudet et al6 published the first case of gastroschisis located in the lower left quadrant of the abdomen. De Vries,2 Blair,7 Irving,8 and Toth and Kimura,9 have all presented cases of gastroschisis with defects on the left side of the umbilical cord. More recently, Melikoglu et al10 reported a new type of malformation consisting of a gastroschisis-like defect in the upper left abdominal quadrant. Ciftci et al,11 in 1997, described an epigastric defect and suggested the need to adopt a new nomenclature for what they considered a new finding: the epigastric cleft: a congenital midline abdominal defect. Our case resembles that particular case, only in the site of the defect other features, such as associated malformations and an epithelial covering, were absent. Omental herniation through the abdominal wall has From the Division of Pediatric Surgery, Unitat Integrada Hospital Sant Joan de De´u-Clı´nic, Universitat de Barcelona, Barcelona, Spain. Address reprint requests to M. Castan˜on Garcia-Alix, MD, Servicio de Cirugia, Hospital Sant Joan de Deu, Passeig Sant Joan de De´u, 2, Esplugues 08950 Barcelona, Spain. © 2003 Elsevier Inc. All rights reserved. 0022-3468/03/3808-0028$30.00/0 doi:10.1016/S0022-3468(03)00282-3

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Fig 1. Newborn boy with an epigastric abdominal wall defect through which only the greater omentun is eviscerated.

been seen in newborns that have had intrauterine blood transfusions during the third trimester, before the real-time ultrasonography.12,13 Although amniocentesis had been done in our case, it had been an ultrasound-guided, uneventful, first trimester procedure, performed only for screening and karyotype, during which no attempt was made at cord puncture. The sample obtained was described as clear amniotic fluid. It is our belief that the case reported here can be seen as a smaller variant of gastroschisis, despite the absence of major evisceration of abdominal contents. The observed defect could be the consequence of a deficient closure of the abdominal wall caused by an abnormality in the migration process of the cephalic plate or the result of a vascular accident that occurred during the period of fetal development. It can even be assumed that a combination of different factors could explain this type of occurrence. A satisfactory outcome can be expected in these cases of small AWD because of the small volume of herniated tissue, the absence of intestine in it, and the fact that it is not associated with other malformations. The current case, together with other rare forms of AWD reported show that no single explanation for all AWD cases is yet possible.

REFERENCES 1. Duhamel B: Embryology of exomphalos and allied malformations. Arch Dis Child 38:142-147, 1963 2. De Vries PA: The pathogenesis of gastroschisis and omphalocele. J Pedatr Surg 15:245-248, 1980 3. Hoyme HE, Jones MC, Lyons-Jones K: Gastroschisis: Abdominal wall disruption secondary to early gestational interruption of the omphalomesenteric artery. Semin Perinntol 7:294-299, 1983 4. Vermeij-Keers C, Hartwing NG, van der Werff JF: Embriogenic development of the ventral body wall and its congenital malformations. Semin Pediatr Surg 5:82-87, 1996 5. Moore TC, Strokes GE: Gastroschisis. Surgery 33:112-117, 1953 6. Daudet M, Cappuis JP, Carron JJ: Le laparoschisis: Reflexions a propos de differents aspects anatomiques et des malformations intestinales associes. Ann Chir Infant 9:303-316, 1968

7. Blair GK: Letter to the editor. J Pediatr Surg 23:989-991, 1988 8. Irving IM: Umbilical abnormalities in neonatal surgery (ed 3). London, England, Buterworths, 1990, pp 376-402 (chap 27) 9. Toth PP, Kimura K: Left-side gastroschisis. J Pediatr Surg 28: 1543-1544, 1993 10. Melikoglu M, Karaguzel G, Ogus M, et al: A gastroschisis-like abdominal wall defect in the left hypocondrium: Case report and literature review. Eur J Pediatr Surg 8:52-54, 1998 11. Ciftci AO, Tanyel FC, Hicsonmez A: Epigastric cleft: A previously unreported entity. J Pediatr Surg 32:1247-1249, 1997 12. Low RAL, Grant JC: Intrauterine transfusion complicated by omental herniation of the newborn. J Pediatr Surg 7:62-63, 1972 13. Chaim W, Mares AJ, Leiberman J, et al: Omental herniation. Obstet Gynecol 47:621-622, 1976