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Year : 2020  |  Volume : 9  |  Issue : 1  |  Page : 89-91

Duodenal atresia associated with the complete absence of small bowel

Department of Pediatric Surgery, PGIMER, Chandigarh, India

Date of Submission28-Sep-2019
Date of Decision21-Nov-2019
Date of Acceptance24-Nov-2019
Date of Web Publication29-Jan-2020

Correspondence Address:
Dr. Shailesh Solanki
Block 3A, Room No. 3103, Advance Pediatric Centre, PGIMER, Sector 12, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_105_19

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Duodenal atresia (DA) is one of the most common sites of neonatal intestinal obstruction and is frequently associated with various other anomalies. An isolated variant of DA has a better prognosis. Some associated intestinal abnormalities are not compatible with survival and definitive surgery could not be offered; however, these associated anomalies can be identified during laparotomy only. We present the case of a neonate with DA associated with the absence of the entire small bowel and also discuss its relevant embryology and pathology.

Keywords: Duodenal atresia, intestinal atresia, neonatal intestinal obstruction

How to cite this article:
Pai D, Solanki S, Menon P. Duodenal atresia associated with the complete absence of small bowel. J Clin Neonatol 2020;9:89-91

How to cite this URL:
Pai D, Solanki S, Menon P. Duodenal atresia associated with the complete absence of small bowel. J Clin Neonatol [serial online] 2020 [cited 2020 Jul 14];9:89-91. Available from: http://www.jcnonweb.com/text.asp?2020/9/1/89/277222

  Introduction Top

Duodenal atresia (DA) is one of the most common sites of neonatal intestinal obstruction. It has been commonly associated with trisomy 21, annular pancreas, and congenital heart disease. Bowel atresia has also been reported in association with DA, but is uncommon.[1],[2] The outcomes are different when there is associated intestinal abnormality, as internal anatomy decides the definitive surgical procedure. If associated intestinal atresia is multiple and/or causing short-bowel syndrome, it is difficult to decide for the surgical procedure, as the survival of these newborns is difficult. However, at the other end, if the anatomy is so aberrant that it would not compatible with survival, one should not attempt the surgery. We present the case of a neonate with DA associated with the absence of the whole small bowel and also discuss the presentation, management, and possible embryological explanation.

  Case Report Top

A 5-day-old female neonate presented in emergency with the complaint of not tolerating feed and bilious vomiting since birth. The child was full-term, born to primigravida nondiabetic mother by normal vaginal delivery at home. There was no history of consanguineous marriage. The antenatal period was not supervised and no ultrasonography (USG) was done.

On examination, the child was dehydrated and the abdomen was distended. There was no meconium stain over the infant feeding tube during per rectal insertion. The abdominal radiograph on anteroposterior view [Figure 1]a showed a single bubble whereas on lateral X-ray [Figure 1]b showed double-bubble appearance and absence of gas in the lower abdomen. Abdominal USG was done which was suggestive of the perigastric collection (size 6.4 cm × 5.3 cm) with internal septations and which was compressing the stomach. Computerized tomography (CT) scan of the abdomen [Figure 1]c was done, which showed a large (6.5 cm × 6 cm) multiseptated cystic lesion in the lesser sac showing enhancing septa within and no calcification, suggesting the possibility of meconium pseudocyst or venolymphatic malformation.
Figure 1: Plain X-ray abdomen in anteroposterior view (a) showing single bubble (black arrow), lateral X-ray (b) showing double bubble (black arrows), computerized tomography abdomen (c) showing large cyst with septations, occupying almost the whole abdomen and compressing stomach, intraoperative picture (d) showing thick wall opened up cyst (black arrow) with area of ulceration and hemorrhage. Blue arrow showing cranial and caudal end

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The child was taken up for laparotomy and on exploration there was a large cyst, occupying almost the whole of the abdomen with blind-ending duodenum and absence of the whole small bowel. Large bowel was present, from the cecum (appendix was not present) till the rectum. The posterior wall of the cyst was densely adhered, the cyst was opened [Figure 1]d, and necrotic fluid was drained out. The anterior wall of the cyst was excised and sent for histology. The abdomen was closed and no definitive surgery was performed. Poor prognosis and nature of the disease were explained to the parents and they had decided to discontinue further treatment. The histopathology of the cyst wall was suggestive of intestinal lining including all layers, ulcerated mucosa with luminal calcification, transmural inflammation reaching up to serosa, myocytolysis of muscularis propria, and vessel wall showed hyalinization.

  Discussion Top

The etiology of small intestinal atresia varies with its site of origin. For DA, it is thought to be caused by errors in recanalization of intestine contrary to atresia in other parts of the bowel, which are caused by vascular accidents. The phase of proliferation and recanalization occurs before the eleventh week of intrauterine life. Failure of recanalization seems the best explanation for localized DA or stenosis. In jejunoileal atresia, however, bile, squamous cells, and lanugo, all of which are produced after the stage of epithelial proliferation, may be found distal to the site of obstruction. The presence of these elements in the intestinal lumen distal to any level of congenital atresia would indicate that occlusion occurred sometime after the eleventh or twelfth week of fetal life, well beyond the period of development of the intestinal tube.[3],[4] There are reports of DA associated with apple peel atresia of the small intestine and they concluded that, in rare circumstances, vascular accidents may be the underlying cause for DA.[5] Louw and Barnard experimentally produced the entire spectrum of neonatal intestinal obstruction by ligating the mesenteric vasculature of fetal puppies. More proximal the mesenteric vascular compromise greater the resulting severity of the intestinal atresia. The extent of these changes depends on the time of development of the obstruction as well as the site of involvement.[6]

The histological features such as mucosal edema, congestion, ulceration, flattening of villi, submucosal edema, prominent vascular proliferation, fibrosis, hemorrhage, gangrene, intramural bowel calcification, and perforation, all suggest the spectrum of bowel ischemia coupled with features of repair and regeneration. The histological features depend on the extent, severity, and duration of an ischemic event. The mucosa and submucosa are more susceptible to ischemic injury than the muscularis layer. Injury to the full thickness of the bowel may result in meconium peritonitis and perforation. If the ischemic segment undergoes healing rather than perforation, there may be scarring of mucosa, submucosa, and muscularis leading to stenosis or atresia.[7]

The index case had a combination of DA along with the absence of the entire small bowel. There is no reported such case in English literature to the best of our knowledge that the whole of the small intestine was converted into the large cyst. Embryologically, it could not be explained unambiguously. We hypothesize that intrauterine volvulus of the whole small intestine (from duodenojejunal flexure to ileocecal junction) with serosal adhesions and mucosal ischemia in a sterile in utero environment preceded to the mentioned condition. Furthermore, possibly the event was happened after the completion of the intestinal rotation, as cecum was present at its normal location. Usually, when volvulus happened around the superior mesenteric vessels, it involves the whole small intestine along with the large bowel till the mid-transverse colon. In index case, the large bowel was not involved at all, it could be because the volvulus was centered around a peritoneal band or happened at the time of normal rotation and fixation in a way that entangle small intestine only. The exact etiopathogenesis for this condition cannot be explained. We did not consider meconium pseudocyst as a differential because histopathology was suggestive of all the layers of the intestine.

Normally, cases of DA are investigated with plain abdominal radiographs, and if it shows a classic double-bubble sign, no further investigation is needed for diagnosis. In index case, plain X-ray [Figure 1]a showed single bubble whereas lateral X-ray [Figure 1]b showed double bubble and clinically also the abdomen was distended and not soft as usually present in DA, that is why ultrasound of the abdomen was done. Once USG was suggestive of the cystic lesion with internal septation in lesser sac region, CT scan of the abdomen was done to delineate anatomy more clearly. However, even after the CT scan, our provisional diagnosis was a venolymphatic malformation or meconium pseudocyst compressing on the stomach and duodenum. After laparotomy only, the anatomy became clear and we found that definitive surgery (duodenal-cecal anastomosis) was not compatible with life. The histological changes observed in the present study suggest that the affected infant was suffering from intrauterine intestinal ischemia contributing to the development of the particular condition.

  Conclusion Top

Conversion of the whole small bowel into a cyst is an unusual event and intrauterine intestinal ischemia due to vascular pathology followed by resorption of the bowel is the possible explanation of cyst formation. If clinical finding and radiological finding in suspected DA are not corroborating, USG and CT scan can give more insight information.

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

Sweed Y. Duodenal obstruction. In: Puri P, Höllwarth M, editors. Pediatric Surgery. Berlin, Heidelberg: Springer; 2009.  Back to cited text no. 1
Escobar MA, Ladd AP, Grosfeld JL, West KW, Rescorla FJ, Scherer LR 3rd, et al. Duodenal atresia and stenosis: Long-term follow-up over 30 years. J Pediatr Surg 2004;39:867-71.  Back to cited text no. 2
Martin LW, Zerella JT. Jejunoileal atresia: A proposed classification. J Pediatr Surg 1976;11:399-403.  Back to cited text no. 3
Todani T, Tabuchi K, Tanaka S. Intestinal atresia due to intrauterine intussusception: Analysis of 24 cases in Japan. J Pediatr Surg 1975;10:445-51.  Back to cited text no. 4
Saša RV, Ranko L, Snezana C, Lidija B, Djordje S. Duodenal atresia with apple-peel configuration of the ileum and absent superior mesenteric artery. BMC Pediatr 2016;16:150.  Back to cited text no. 5
Louw JH, Barnard CN. Congenital intestinal atresia; observations on its origin. Lancet 1955;269:1065-7.  Back to cited text no. 6
Subbarayan D, Singh M, Khurana N, Sathish A. Histomorphological features of intestinal atresia and its clinical correlation. J Clin Diagn Res 2015;9:EC26-9.  Back to cited text no. 7


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