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Journal of Indian Association of Pediatric Surgeons
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Year : 2022  |  Volume : 27  |  Issue : 3  |  Page : 345-347

Delayed presentation of jejunal atresia with multiple enteric fistula: A case report and review of literature

Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India

Date of Submission29-Jan-2021
Date of Decision23-May-2021
Date of Acceptance15-Jun-2021
Date of Web Publication12-May-2022

Correspondence Address:
Dr. Devendra Kumar Yadav
Room No-4002, Teaching Block, Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_17_21

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A 6-month-old boy presented with features of intestinal obstruction. Laparotomy revealed Type IIIa jejunal atresia. The proximal and distal bowel loops were in continuity through multiple fistulae between the adjoining bowel loops. To the best of our knowledge, it is the first report of congenital Type IIIa intestinal atresia surviving beyond the neonatal age without surgery.

Keywords: Delayed presentation, entero-enteric fistula, jejunal atresia

How to cite this article:
Nandan R, Dhua AK, Yadav DK, Jain V, Goel P. Delayed presentation of jejunal atresia with multiple enteric fistula: A case report and review of literature. J Indian Assoc Pediatr Surg 2022;27:345-7

How to cite this URL:
Nandan R, Dhua AK, Yadav DK, Jain V, Goel P. Delayed presentation of jejunal atresia with multiple enteric fistula: A case report and review of literature. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Jun 25];27:345-7. Available from: https://www.jiaps.com/text.asp?2022/27/3/345/345118

   Introduction Top

Congenital intestinal atresia is a common cause of neonatal intestinal obstruction.[1] Delayed presentation of Type IIIa jejunal atresia beyond the neonatal period has not been reported. This patient presented at 6 months of age. Multiple entero-enteric fistulae (EEFs) were present between the jejunal segments proximal and distal to the site of atresia. Similar cases of acquired intestinal atresia have been described in necrotizing enterocolitis (NEC), wherein intestinal continuity had been restored by fistulization.[2]

   Case Report Top

A 6-month-old boy (weight: 6.8 kg) presented with recurrent bilious vomiting and associated abdominal distension for 3 days. There was no history of recurrent fever, constipation, diarrhea, jaundice, bleeding per rectum, frequent hospital admission for abdominal distension. He was born at full term by normal vaginal delivery (birth weight – 2.6 kg) and passed meconium on the 1st day of life. Antenatal sonography was not suggestive of any anomaly. No polyhydramnios was present. The child had been on exclusive breastfeed until 2 weeks back when weaning was initiated. On examination, the child was irritated, crying incessantly and had gross abdominal distension with visible bowel loops. There were no features of peritonitis. Hemogram, liver function test, and kidney function tests were normal. Abdominal radiograph was suggestive of intestinal obstruction. We kept the differential diagnosis of malrotation, intussusception, Hirschsprung's disease, obstruction due to internal bands, mesocolic hernia, enteric duplication, jejunoileitis, unnoticed intestinal perforation, delayed presentation of NEC due to strictures.

The child was resuscitated and monitored for hemodynamic stability before exploratory laparotomy. Supracolic compartment showed grossly dilated duodenum and infracolic compartment had dense adhesions between the bowel loops. Bowel loops were separated. Proximal jejunum was grossly dilated till 40 cm from the duodenojejunal junction [Figure 1]a and then connected with distal bowel at multiple sites extending over a length of 30 cms. Upon further separation of the bowel, the atretic end of the distal bowel loop was found embedded within the inter-bowel adhesion mass [Figure 1]b. These findings were suggestive of multiple EEFs connecting the bowel segments proximal and distal to the site of atresia [Figure 1]c.
Figure 1: Intraoperative images: (a) Dilated duodenum (DU), duodenojejunal flexure (DJ), and proximal jejunum, (b) Type IIIa jejunal atresia with proximal (PJL) and distal (DJL) jejunal loop and mesenteric defect (block arrow), (c) Proximal (PJL) and distal (DJL) jejunal loop interconnected through multiple entero-enteric fistula (arrow), (d) Duodenum (DU) in close proximity with cecum (*-Appendix)

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The duodenojejunal flexure was to the right of midline in close proximity with cecum, suggestive of malrotation [Figure 1]d. The duodenojejunal loop was straightened and placed on the right side. Distal 10 cm of proximal jejunum and 30 cm of distal bowel interconnected with proximal jejunum at multiple sites were excised. Excision tapering of grossly dilated proximal jejunum followed by jejuno-jejunal anastomosis was done in single layers. Gastrostomy was done to ensure proximal diversion. The remaining small bowel length was 120 cm.

Postoperatively recovery was uneventful. Total parenteral nutrition was started on postoperative day 1 which was gradually transitioned to oral feed starting on postoperative day 7. The child was discharged in a stable condition after gastrostomy removal on postoperative day 12. Gross examination of the surgical specimen was suggestive of Type IIIa atresia with multiple EEFs. Histopathology was suggestive of mild chronic inflammatory changes and mild fibrosis at the site of fistulae. At a follow-up of 1 year, the child has been doing well.

   Discussion Top

Intestinal atresia is the most common congenital anomaly of small intestine with an incidence of 1.3–2.9 cases per 10,000 live births. Different theories have been described regarding the etiology. The most accepted etiology for jejunoileal atresias was proposed by Louw and Barnard which relates to late intrauterine mesenteric vascular accidents including volvulus, intussusceptions, snaring at umbilical ring, kinks, and bands.[1]

Children with intestinal atresia typically present on day 1 of life with abdominal distention, bilious vomiting, and failure to pass meconium. Cases of intestinal atresia with delayed presentation have also been reported.[2],[3] Delayed presentation is seen in Type I atresia with a defect in the diaphragm. The degree of obstruction in Type I jejunal atresia depends on the size of the defect. Smaller the defect size, earlier is the presentation.[2] Delayed presentation of intestinal atresia has also been seen when it is caused by intrauterine intussusception. Usually, these cases present within 48 h because of complete intestinal obstruction. Differentiation from sequelae NEC may be difficult. Uncertainty in diagnosis may lead to a delay in confirming the diagnosis. Considering that NEC sometimes merits conservative management while intussusception with intestinal atresia will need surgical intervention, a delay in diagnosis may affect the prognosis.[3]

Our patient was born at full term by normal vaginal delivery having a birth weight of 2.6 kg. He was started on breastfeed on day 1 of life which he tolerated well and passed meconium on the same day. The child remained asymptomatic till 6 months of age. The clinical features of underlying pathology manifested only after weaning was initiated. At no point in time, he had clinical features suggestive of NEC. To the best of our knowledge, the communication of blind loops of congenital intestinal atresia through EEF has not been documented previously. The cause of congenital atresia may be any of the late intrauterine mesenteric vascular accidents including volvulus, intussusceptions, snaring at umbilical ring, kinks, and bands.[1] However, in the presence of malrotation, volvulus is common.

Cases of acquired intestinal atresia in NEC with spontaneous reconstitution of intestinal continuity through fistulization have been described. Ischemia and bowel necrosis can stir up inflammatory response which may result in affected bowel to adhere to adjacent bowel and eventually lead to fistula formation.[4] The other cause acquired atresia described is adhesive band entrapping the mesentery and bowel segment but without any EEF.[5] The cause for EEF in our patient may be antenatal perforation leading to peritonitis. The intrauterine inflammation between the bowel loops might have eventually lead to EEF. The findings in the index case and the underlying etiologic mechanisms are likely to have been antenatal and may have important implications in understanding the pathophysiology of intestinal atresia and the antenatal healing processes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Louw JH, Barnard CN. Congenital intestinal atresia; observations on its origin. Lancet 1955;269:1065-7.  Back to cited text no. 1
Chen HD, Jiang H, Kan A, Huang LE, Zhong ZH, Zhang ZC, et al. Intestinal obstruction due to dual gastrointestinal atresia in infants: Diagnosis and management of 3 cases. BMC Gastroenterol 2014;14:108.  Back to cited text no. 2
Lewis MP, Emberton M, Owen ER, Singh MP. Delayed presentation of intestinal atresia and intussusception--A case report and literature review. Eur J Pediatr Surg 1993;3:296-8.  Back to cited text no. 3
Kalane S, Suryawanshi P, Vaidya U, Shrotriya S. Gastro colic fistula in a neonate – Case report of a rare complication of necrotizing enterocolitis. Journal of Pediatric Surgery Case Reports 2015;3:10-2.  Back to cited text no. 4
Mandelia A, Agarwala S. Acquired jejunal atresia in a 2-month-old infant. J Clin Diagn Res 2015;9:D01-2.  Back to cited text no. 5


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