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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 6  |  Issue : 2  |  Page : 120-121
Duodenal atresia with 'apple-peel configuration' of the ileum and absent superior mesenteric artery: A rare presentation

Department of Pediatric Surgery, Lokmanya Tilak Muncipal General Hospital, Sion, Mumbai, India

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Date of Web Publication29-Jul-2009


According to the current understanding, duodenal atresia is considered to be a primary malformation resulting from the errors in recanalisation in early gestation. We report a rare case of duodenal atresia with apple-peel configuration of remaining small bowel with absent superior mesenteric artery in a preterm child, which indicates that in rare circumstances vascular accidents may be the underlying cause for duodenal atresia, and jejuno-ileal atresia; only second such case to be reported in the English literature.

Keywords: Apple-peel configuration of the ileum, duodenal atresia, vascular accident

How to cite this article:
Ahmad A, Sarda D, Joshi P, Kothari P. Duodenal atresia with 'apple-peel configuration' of the ileum and absent superior mesenteric artery: A rare presentation. Afr J Paediatr Surg 2009;6:120-1

How to cite this URL:
Ahmad A, Sarda D, Joshi P, Kothari P. Duodenal atresia with 'apple-peel configuration' of the ileum and absent superior mesenteric artery: A rare presentation. Afr J Paediatr Surg [serial online] 2009 [cited 2021 Oct 27];6:120-1. Available from:

   Introduction Top

The duodenum is the most common site of neonatal intestinal obstruction, accounting for nearly half of all cases. [1] Incidence of duodenal atresia has been estimated at 1 in 6,000 to 1 in 10,000 births. [2] It is considered to result from errors in recanalisation of embryonic foregut in early gestation. [3] This aetiology differs from that of intestinal atresias in other parts of the bowel, which are assumed to result from vascular accidents during later phases of gestation. [4] Due to the significantly different aetiology, the association of duodenal atresia with apple-peel, small bowel atresia is very rare. We present one such rare association, which raises questions on the aetiology of duodenal atresia. To the best of our knowledge, ours is the second such case to be reported in the English literature. [5]

   Case Report Top

An 8-day-old boy, 1.2 kg preterm (34 weeks) neonate, was referred from neonatal intensive care unit (NICU) to us with complaints of persistent high bilious aspirates. The child had passed stools in scanty quantities. Antenatal ultrasonography (USG) showed maternal polyhydromnios. On examination, the baby was alert, active, and had no other external congenital anomalies. Abdominal examination revealed soft, nontender abdomen, with fullness of the epigasrium [Figure 1]. There was normally situated anal opening. Plain radiograph of the abdomen showed the classic "double-bubble" sign [Figure 2] of duodenal atresia with total absence of distal bowel gas. After optimisation of general condition, the patient had exploratory laparotomy, which revealed loss of third and fourth part of the duodenum with an apple-peel configuration of the remaining small bowel with absence of superior mesenteric artery [Figure 3]a and b. The precarious blood supply of apple- peel small bowel depended entirely on the marginal artery that was fed retrogradely through the left colic artery. Duodenum was anastomosed end-to-end to the hypoplastic apple-peel jejunum. Postoperatively, patient was kept on ventilator support. However, he succumbed on second day due to very low birth weight with prematurity.

   Discussion Top

The cause of duodenal and small bowel atresias may vary according to the site of the lesion. Embryologically, the lumen of the gut in a five-week-old embryo is well-defined throughout its length. After the formation of a primary intestinal canal, the developing embryonic foregut goes through a complete solid core stage that subsequently vacuolises. [5] Duodenal and upper jejunal atresias are believed to be related to failure of recanalisation of the solid stage of developing embryonic foregut, as proposed by Tandler in 1900. [6] Atresia of rest of the small bowel is considered to result from interruption of vascular supply to the atretic bowel segment. [7]

In 1961, Santulli and Blanc used the term apple-peel atresia for the first time. [8] Apple-peel atresia consists of a high jejunal atresia with a discontinuity of the small bowel and a wide gap in the mesentery. The distal segment of ileum is shortened and assumes a helical configuration around a retrograde perfusing vessel, which compensates for the partially absent superior mesenteric artery. An intrauterine vascular accident in late gestation has been accepted as the cause of apple-peel atresia and presents with a spectrum of occlusions of one or more branches of the superior mesenteric artery. [9]

The present patient had an atretic third and fourth part of duodenum and proximal jejunum with typical apple-peel configuration of the ileum with total absence of superior mesenteric artery (SMA). The totally missing SMA probably caused duodeno-jejunal atresia due to interruption of pancreatico-duodenal arcade, in addition to apple-peel atresia. Although, an unrelated occurrence of duodenal atresia and apple-peel atresia in this child cannot be ruled out; it seems to be very unlikely.

The current concept favours the recanalisation theory to explain the duodenal atresia. Our case suggests, however, that in rare circumstances vascular accidents may be the underlying cause of duodenal atresia.

   References Top

1.Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: A 27 year experience with 277 cases. Arch Surg 1998;133:490-6.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Fonkalsrud EW, DeLorimier AA, Hays DM. Congenital atresia and stenosis of the duodenum: A review compiled from the members of the Surgical Section of the American Academy of Pediatrics. Pediatrics 1969;43:79-83.  Back to cited text no. 2  [PUBMED]  
3.Gross E, Armon Y, Abu-Dalu K, Gale R, Schiller M. Familial combined duodenal and jejunal atresia. J Pediatr Surg 1996;31:1573.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Lambrecht W, Kluth D. Hereditary multiple atresias of the gastrointestinal tract: Report of a case and review of the literature. J Pediatr Surg 1998;33:794-7.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Weber DM, Freeman NV. Duodenojejunal atresia with apple-peel configuration of the ileum and absent superior mesenteric artery: Observations on pathogenesis. J Pediatr Surg 1999;34:1427-9.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Tandler J. Zur Entwickelungsgeschichte des menschlichen duodenum in fruhen Embryonalstadien. Morph Jahrb 1900;29:187-216.  Back to cited text no. 6    
7.Louw JH, Barnard CN. Congenital intestinal atresia. Lancet 1955;2:1065-67.  Back to cited text no. 7    
8.Santulli TV, Blanc WA. Congenital atresia of the intestine: Pathogenesis and treatment. Ann Surg 1961;154:939-48.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Lister J. Intestinal atresia and stenosis, excluding the duodenum. In: Lister J, Irving I, Rickham PP, editors. Neonatal Surgery. London, England: Butterworth and Co; 1990. p. 453-73.  Back to cited text no. 9    

Correspondence Address:
Paras Kothari
Department of Paediatric Surgery, L.T.M. Medical College and General Hospital, Sion, Bombay - 400 022
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-6725.54780

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  [Figure 1], [Figure 2], [Figure 3]

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