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Journal of Indian Association of Pediatric Surgeons
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Year : 2021  |  Volume : 26  |  Issue : 4  |  Page : 271-273

Omphalomesenteric duct remnant: A rare and unusual cause of intestinal atresia

Department of Paediatric Surgery, Children's Health Ireland at Crumlin, Dublin, Ireland

Date of Submission12-May-2020
Date of Decision15-Jul-2020
Date of Acceptance16-Sep-2020
Date of Web Publication12-Jul-2021

Correspondence Address:
Prof. Brice Antao
Department of Paediatric Surgery, Children's Health Ireland at Crumlin, Cooley Road, Crumlin, Dublin
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_158_20

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Intestinal atresia is generally considered to result from mesenteric vascular disruptions during fetal life. This report describes an unusual case of ileal atresia, resulting from an omphalic ring closure anomaly and an omphalomesenteric duct (OMD) remnant. A 2-day-old male neonate presented with bilious vomiting and abdominal distension. At laparotomy, the distal portion of the atretic ileum was entrapped within the umbilical ring, causing volvulus of the proximal dilated atretic bowel around the fixed distal bowel at the omphalic ring. This case report supports OMD remnants as a rare, potential cause of jejunoileal atresia.

Keywords: Intestinal atresia, omphalic ring, omphalomesenteric duct remnant, segmental volvulus

How to cite this article:
Kane G, Zaidan H, Antao B. Omphalomesenteric duct remnant: A rare and unusual cause of intestinal atresia. J Indian Assoc Pediatr Surg 2021;26:271-3

How to cite this URL:
Kane G, Zaidan H, Antao B. Omphalomesenteric duct remnant: A rare and unusual cause of intestinal atresia. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2023 Jan 28];26:271-3. Available from: https://www.jiaps.com/text.asp?2021/26/4/271/321194

   Introduction Top

Intestinal atresia (IA) is one of the common causes of neonatal intestinal obstruction, with an incidence ranging from 1.3 to 2.8 out of 10,000 live births.[1] It is postulated that the etiology of distal small bowel atresia is secondary to an intravascular accident in the late intrauterine life.[2] It is thought that this causes ischemic necrosis and resorption of the affected bowel segment or segments, resulting in different types of atresia, ranging from intestinal wall contracture by a scar and subsequent stenosis to a complete intestinal and mesenteric defect.[2] The most common vascular accidents causing IA are volvulus, intussusception, internal hernia, congenital constriction bands, omphalocele, and gastroschisis.[3] However, an omphalomesenteric duct (OMD) remnant causing IA is extremely rare.[4],[5],[6],[7],[8] We report a rare and unusual case of IA secondary to a vascular insult from entrapment of the bowel within a partially closed omphalic ring secondary to an OMD remnant.

   Case Report Top

A preterm male neonate, born at 36 weeks gestation, presented on day 2 of life with poor feeding, bilious vomiting, and failure to pass meconium. He was born by spontaneous vaginal delivery with a birth weight of 2.92 kg. There was a history of maternal gestational diabetes mellitus, and antenatal ultrasound scans were normal. He was stable at birth, and clinical examination revealed a soft, distended abdomen with a normally positioned patent anus and bilious nasogastric aspirates. Abdominal plain film showed dilated bowel loops, and a contrast enema revealed a small caliber colon with no reflux of contrast into dilated small bowel.

At laparotomy, on the 2nd day of life, a type 3b ileal atresia was identified. The distal atretic segment was a 2 cm stump of the terminal ileum along with the cecum and appendix entrapped within a tight omphalic ring with a fibrotic attachment to the under surface of the umbilical cicatrix [Figure 1]. The proximal ileal atretic bowel was 165 cm from the duodenojejunal flexure, which was in a normal position with no evidence of malrotation. There was a segmental volvulus of 15 cm of ischemic, dilated, proximal terminal atretic bowel around the distal atretic bowel loop that was fixed at the omphalic ring. The proximal atretic segment was derotated, and the tethered distal atretic segment was carefully dissected from the umbilical ring [Figure 2]. This 15 cm of proximal segmental ischemic dilated bowel [Figure 3] and the distal atretic stump of the terminal ileum along with the appendix and cecum [Figure 4] were excised, and a primary end-to-end ileocolic anastomosis was carried out. The large mesenteric defect was closed, and the umbilical ring was then repaired internally. The baby made a good recovery from surgery and was discharged on the postoperative day 19 on full oral feeds. He continues to thrive well at 6 months of age with no concerns. Histological analysis of resected bowel specimens confirmed a diagnosis of ileal atresia.
Figure 1: This distal atretic small stump of terminal ileum, along with the cecum and appendix, was entrapped within the omphalic ring with a fibrous band (dashed arrow). Segmental volvulus of 15 cm of ischemic, dilated, proximal atretic bowel around the distal atretic bowel loop that was fixed at the omphalic ring (solid arrow)

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Figure 2: Distal atretic segment of small bowel after detachment from the umbilical ring (dashed arrow) and proximal dilated ischemic atretic segment of small bowel (solid arrow)

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Figure 3: Histopathology specimen of excised proximal atretic dilated small bowel, showing a blind ending ileal stump (arrow)

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Figure 4: Histopathology specimen of excised distal atretic bowel, showing a blind ending terminal ileum (solid black arrow) with appendix (solid dashed black arrow) and cecum (solid white arrow)

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   Discussion Top

The OMD is the normal tubular embryological structure that links the primitive gut to the yolk sac of the embryo. During normal development, at the 6th week of embryogenesis, the mid-gut loop elongates and herniates into the umbilical cord. At the same time, as the mid-gut elongates, the lumen of the OMD begins a process of obliteration. By the 10th week of early fetal development, the mid-gut returns to the abdominal cavity and the OMD becomes a thin fibrous band, which undergoes resorption. Persistence of the duct leads to a spectrum of anomalies in the new-born period, infancy, or later childhood years, such as Meckel's diverticulum, umbilical fistula, umbilical sinus, umbilical cyst, umbilical polyp, or a fibrous cord connecting the ileum to the umbilicus. OMD remnants often cause intestinal obstruction from a duct-associated closed loop, volvulus, direct bowel compression, kinking of the bowel, or intussusception with a Meckel's diverticulum or inflammatory mass.[9]

OMD remnants as a cause for IA are extremely rare. There are only five previously reported cases of OMD remnants and IA in the literature, with varied etiopathogenesis.[4],[5],[6],[7],[8] In three of these reports, IA has been secondary to congenital bands caused by an OMD remnant.[4],[5],[6] Tineo et al. reported a case of an umbilical fistula with a fibrotic cord connected to a distal ileal atresia.[7] Hasegawa et al. reported a case of ileal atresia due to mesenteric vascular insult from strangulation around an OMD remnant at the umbilicus with necrotic bowel.[8] There is another reported case of ileal atresia as a result of entrapment of the ileum and vascular accident, secondary to an omphalic ring closure anomaly without evidence of OMD remnant.[10]

We believe that the IA in our case was secondary to mesenteric vascular insufficiency as a result of a combination of an OMD fibrotic band at the umbilicus and ileal entrapment within omphalic ring. The distal portion of the ileum was connected to the umbilicus with a fibrous band and formed the axis of strangulation. The atresia could have resulted from either necrosis of the entrapped bowel or vascular compression of the intestinal mesentery, which was evident by the widely separated proximal atretic bowel, which was within the abdominal cavity and the large mesentery defect. The segmental entrapment of small bowel could lead to necrosis of the entrapped bowel within the omphalic ring. Once this segment resorbs, the proximal atretic segment may drop back into the abdominal cavity. This mechanism is postulated in some cases of IA without rotational anomalies.[10] However, given the presence of an OMD remnant in our case, the distal atretic segment was still attached to the umbilicus within a partially closed omphalic ring.

Embryologically, a physiological intestinal hernia occurs during the 6th week and returns back into the coelom by the 9th–11th week. Premature closure of an omphalic ring or failure of part of the small bowel to return into the abdominal cavity may lead to its entrapment within the omphalic ring similar to what was seen in our case and another case previously reported.[10] We believe that the persistence of the OMD remnant contributed to the entrapment of the bowel within a partially closed omphalic ring. The segmental volvulus that was seen at the time of surgery was a postnatal event rather than the cause of the atresia, whereby the mobile proximal atretic segment with a large mesenteric defect had twisted around the fixed distal atretic segment at the omphalic ring.

Our case supports the evidence of OMD remnant and an omphalic ring closure anomaly as potential mechanisms of IA. As surgeons, we need to remain cognizant of this rare and unusual cause of IA, while planning surgical incision/port placement around the umbilicus and during mobilization and inspection of the bowel at the time of surgery, especially in the era of neonatal minimally invasive surgery.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Hemming V, Rankin J. Small intestinal atresia in a defined population: Occurrence, prenatal diagnosis and survival. Prenatal Diagnosis 2007;27:1205-11.  Back to cited text no. 1
Louw JH, Barnard CN. Congenital intestinal atresia; observations on its origin. Lancet 1955;269:1065-7.  Back to cited text no. 2
Nehra D, Goldstein AM. Congenital intestinal obstruction. In: Elzouki AY, Harfi HA, Nazer HM, Stapleton FB, Oh W, Whitley RJ, editors. Textbook of Clinical Pediatrics. Publishers location is Berlin, Heidelberg: Springer, Berlin, Heidelberg; 2012. p. 4011-26.  Back to cited text no. 3
Santulli TV, Blanc WA. Congenital atresia of the intestine: pathogenesis and treatment. Ann Surg 1961;154:939-48.  Back to cited text no. 4
Mercado MG, Bulas DI, Chandra R. Prenatal diagnosis and management of congenital volvulus. Pediatr Radiol 1993;23:601-2.  Back to cited text no. 5
Petrikovsky BM, Nochimson DJ, Campbell WA, Vintzileos AM. Fetal jejunoileal atresia with persistent omphalomesenteric duct. Am J Obstet Gynecol 1988;158:173-5.  Back to cited text no. 6
Tineo N, Nunez N, Rodríguez L, Rodríguez A. Distal ileal atresia with whole umbilical fistula in Newborn, on the basis of a case pediatrics service-SAHUAPA. EC Paediat 2018;7:1131-7.  Back to cited text no. 7
Hasegawa T, Sakurai T, Tazuke Y, Ueda S, Kitano N, Dezawa T. Ileal atresia associated with an omphalomesenteric duct remnant. Pediatr Surg Int 1998;13:182-3.  Back to cited text no. 8
Gaisie G, Curnes JT, Scatliff JH, Croom RD, Vanderzalm T. Neonatal intestinal obstruction from omphalomesenteric duct remnants. AJR Am J Roentgenol 1985;144:109-12.  Back to cited text no. 9
Pratap A, Yadav RP, Bajracharya A, Agrawal A, Bhatta N, Agrawal CS. Intrauterine omphalic ileal entrapment as an unusual cause of ileal atresia: Report of a case. Surg Today 2008;38:141-3.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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