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Year : 2016  |  Volume : 21  |  Issue : 4  |  Page : 190-192

Inflammatory duodenal necrosis complicating gastroschisis

1 Department of Paediatric Surgery, Evelina London Children's Hospital, London SE1 7EH, United Kingdom
2 Department of Neonatology, St Thomas' Hospital, London SE1 7EH, United Kingdom

Date of Web Publication19-Jul-2016

Correspondence Address:
Dina Fouad
Department of Paediatric Surgery, Evelina London Children's Hospital, St Thomas' Hospital, London SE1 7EH
United Kingdom
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.186551

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Babies with gastroschisis have an increased risk of necrotizing enterocolitis (NEC) that can lead to short bowel syndrome, a long-term parenteral nutrition requirement, and its associated complications. To our knowledge, this is the first case report of recurrent duodenal ischemia and necrosis associated with gastroschisis in the absence of NEC totalis.

Keywords: Gastroschisis, necrotizing enterocolitis, short bowel

How to cite this article:
Fouad D, Lee GJ, Upadhyaya M, Drake D. Inflammatory duodenal necrosis complicating gastroschisis. J Indian Assoc Pediatr Surg 2016;21:190-2

How to cite this URL:
Fouad D, Lee GJ, Upadhyaya M, Drake D. Inflammatory duodenal necrosis complicating gastroschisis. J Indian Assoc Pediatr Surg [serial online] 2016 [cited 2023 Sep 23];21:190-2. Available from: https://www.jiaps.com/text.asp?2016/21/4/190/186551

   Introduction Top

Gastroschisis is an abdominal wall defect with a rising incidence of five in every 10,000 live births. [1] The majority are diagnosed by antenatal ultrasound. Gastroschisis can be simple or complex (associated atresia, necrosis, or perforation). The main aim of the treatment is the safe return of the exteriorized bowel to the abdomen.

   Case report Top

A male baby was born at 36 + 4 weeks gestation, weighing 2.6 kg, by emergency cesarean section for fetal distress. Antenatal ultrasound confirmed dilated bowel loops. The bowel was wrapped in cling film, and the baby positioned to avoid kinking of the mesentery. Bowel loops appeared pink and healthy; however, the duodenum was notably dilated. A sudden deterioration required intubation and ventilation. A size four preformed silo was then placed. On day 1, the bowel within the preformed silo appeared pink and healthy, and two tucks were performed. On day 2, the bowel within the preformed silo appeared blue. The silo was removed and abdominal wound extended laterally. Subsequently, the majority of the bowel appeared to be well-vascularized; however, a few small ischemic patches were still visible. A size five silo was then placed. On day 3, the bowel loops became more distended. To preempt further vascular compromise and as the bowel had become more distended, a prolene mesh surgical silo was placed.

On day 5, extensive necrotic segments of bowel were identified, and laparotomy was performed. The most significant findings included: A very dilated duodenum with multiple necrotic patches affecting more than half of the circumference of the third and fourth parts, nonrotation of a very narrow-based mesentery, a thick, edematous ileum with three further necrotic perforations, 10 cm of ischemic jejunum, and an ischemic segment of sigmoid colon. No intrinsic cause of duodenal dilatation was ever identified. The ischemic jejunum was resected and a surgical silo applied with a defunctioning duodenal stoma and jejunal/ileal mucus fistula brought out through this [Figure 1] due to previous incisions and lack of remaining abdominal domain. Four further laparotomies were required. On day 12, three new patches of necrosis in the duodenum were resected. On day 15, the duodenostomy was refashioned and a further 9 cm of small bowel required resection due to new areas of necrosis. On day 27, a perforation was found in the defunctioned small bowel. Final laparotomy was on day 37: Five small bowel anastomoses, one large bowel resection and anastomosis, a repair of a further duodenal perforation, and appendectomy were completed followed by the closure of the stoma and the abdominal wall. With the preservation of the ileocecal valve 45 cm of the small bowel remained.

The surgical silo and stoma were extremely challenging to manage. This required a balance of reducing the silo contents, caring for the high-output stoma, avoiding excoriation of underlying skin, and preventing the narrow-based mesentery from kinking on the elevation of the silo. Full intravenous total parenteral nutrition (100 mL/kg/day) from the 1 st week of life to 2 months was administered. Management and prevention of sepsis were with repeated courses of intravenous antibiotics, initially with benzylpenicillin, gentamicin, and metronidazole. Subsequent choices of antibiotics (e.g., vancomycin and meropenem) were guided by blood and surface cultures.
Figure 1: Surgical silo with stoma and mucous fistula

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Histopathology of the small bowel revealed necrosis, inflammation, and hemorrhage most likely secondary to ischemia.

Following a concerted multidisciplinary team effort, the baby was discharged, aged 4 months and 12 days. Despite multiple small and large bowel resections and a total of 134 days of parenteral nutrition, the child was successfully discharged on full enteral feeds. At 1 year, weight and height were between 25 th and 50 th centiles, and stool frequency controlled with loperamide.

   Discussion Top

Gastroschisis is most commonly diagnosed by antenatal ultrasound in the first trimester, as occurred in our case, and can be seen as early as at 11 weeks of gestation. [2] When the bowel becomes exposed to the amniotic fluid, it results in changes ranging from mild inflammation to bowel necrosis and atresia due to a sterile inflammatory reaction developing characterized by the development of a fibrous coat of the bowel wall. Babies with antenatally dilated bowel loops, as was present in our case, have been reported to have a poorer outcome. Goetzinger et al. [3] found that babies with antenatal bowel dilatation of >14 mm were more likely to have bowel atresia postnatally and longer stay in the Neonatal Intensive Care Unit.

The aim of treatment in gastroschisis is to safely return the bowel into the abdominal cavity. Management options include primary surgical closure or staged abdominal closure using a preformed or surgical silo. Debate still remains as to the optimal course of management.

Necrotizing enterocolitis (NEC) affects 4-10% of babies with gastroschisis, [4] and there are no other reported cases of extensive and recurrent duodenal necrosis associated with gastroschisis as described in this case. A 30-year study of 185 patients with gastroschisis found that eight patients developed NEC. These babies had a 5.8-fold increased risk of sepsis, and a six-fold increased the risk of mortality. [5] Histopathological analysis cannot differentiate the etiology of ischemia and necrosis of the bowel; however, we postulate that NEC may have been the cause of the recurrent bowel necrosis in our case.

   Conclusion Top

We describe a complex and challenging case of gastroschisis with recurrent duodenal necrosis and perforation. To our knowledge, this is the first case report of recurrent duodenal ischemia and necrosis associated with gastroschisis in the absence of NEC totalis. This case was successfully managed with a concerted multidisciplinary team effort that resulted in the closure of the abdominal wall and the baby being discharged without a parenteral nutrition requirement.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Mastroiacovo P, Lisi A, Castilla EE. The incidence of gastroschisis: Research urgently needs resources. BMJ 2006;332:423-4.  Back to cited text no. 1
Cullen MT, Green J, Whetham J, Salafia C, Gabrielli S, Hobbins JC. Transvaginal ultrasonographic detection of congenital anomalies in the first trimester. Am J Obstet Gynecol 1990;163:466-76.  Back to cited text no. 2
Goetzinger KR, Tuuli MG, Longman RE, Huster KM, Odibo AO, Cahill AG. Sonographic predictors of postnatal bowel atresia in fetal gastroschisis. Ultrasound Obstet Gynecol 2014;43:420-5.  Back to cited text no. 3
David AL, Tan A, Curry J. Gastroschisis: Sonographic diagnosis, associations, management and outcome. Prenat Diagn 2008;28:633-44.  Back to cited text no. 4
Snyder CL. Outcome analysis for gastroschisis. J Pediatr Surg 1999;34:1253-6.  Back to cited text no. 5


  [Figure 1]

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