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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Year : 2009  |  Volume : 14  |  Issue : 4  |  Page : 226-227

Congenital duodenal diaphragm and enteroliths: A Unique complication

Department of Paediatric Surgery, Sheri Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India

Date of Web Publication4-Feb-2010

Correspondence Address:
Nisar Ahmad Bhat
Shehjar House, Noorani Colony, Srinagar Kashmir, J&K
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.59609

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We report an unusual case of duodenal diaphragm (DD) with "wind-sock" anomaly in a 6-year-old male. The child presented with an acute duodenal obstruction precipitated by multiple pigmented stones completely blocking the duodenum.

Keywords: Bile stones, duodenal diaphragm, intestinal obstruction

How to cite this article:
Bhat NA. Congenital duodenal diaphragm and enteroliths: A Unique complication. J Indian Assoc Pediatr Surg 2009;14:226-7

How to cite this URL:
Bhat NA. Congenital duodenal diaphragm and enteroliths: A Unique complication. J Indian Assoc Pediatr Surg [serial online] 2009 [cited 2022 Aug 11];14:226-7. Available from: https://www.jiaps.com/text.asp?2009/14/4/226/59609

   Introduction Top

Congenital duodenal obstruction (CDO) is a common cause of intestinal obstruction in the newborn. In delayed presentations, the duodenum is uninterrupted and the intrinsic obstruction is a membranous diaphragm web with a central aperture. Intra-operatively, duodenum appears normal in cases where the diaphragm has progressed on to a "wind-sock" abnormality. [1]

   Case Report Top

A 6-year-old male child was admitted with a history of bilious vomiting of 3 days duration. The past history was that of an intermittent vomiting (both bilious and non-bilious) since the age of 1 year. The episodes of vomiting usually followed upper abdominal distention with a frequency of 2-3 times per week; also, there was poor weight gain.

On examination, the child was of normal intelligence but malnourished, dehydrated, and with deranged serum electrolytes. The patient was evaluated after correcting his dehydration and electrolyte imbalance. Plain x-ray of the abdomen revealed typical "double-bubble sign" with distal intestinal gas in the abdomen. Barium meal follow-through study showed both stomach and duodenum dilated and a streak of contrast going down the duodenum into the small bowel. The size of the duodenum was twice the size of the stomach [Figure 1]. Blood screening for hemolytic disorders was negative. Laparotomy showed distended stomach and hugely distended duodenum filled with a mass of stones like structures [Figure 2]. Duodenotomy revealed the mass to be multiple black stones of varying sizes and shapes [Figure 3]. After the removal of stones, wind-sock abnormality of duodenal diaphragm with a small central aperture was seen arising from the second part of the duodenum. Duodenal diaphragm was excised and duodenoplasty was done by closing the longitudinal incision in the duodenum transversely. The biliary tree and liver were normal. The postoperative period was uneventful except for duodenal ileus that lasted for 8 days. The patient is on regular follow-up and continues to gain weight. Chemical analysis showed stones to be of pigment variety.

   Discussion Top

The diagnosis in CDO is usually easy and, in the majority of cases, this can be established with plain abdominal radiograph and upper gastrointestinal contrast studies. [2] The classical "double-bubble" appearance on plain abdominal radiograph is pathognomic of CDO. However, symptoms may not be specific sometimes and there may be appreciable distal gastrointestinal air in addition to double-bubble, and diagnosis in such patients may be delayed. The possibility of DD with a central hole must be considered in children with repeated attacks of vomiting even if it is non-bilious as was the case in our patient.

A variety of intrinsic and extrinsic congenital lesions may cause CDO. [3],[4],[5],[5] It may not be possible to differentiate between them clinically or radiologically. Our patient had a classic duodenal diaphragm arising from the second part of duodenum. The weight of stones probably aggravated the process further.

Nawaz et al. reported a case of duodenal obstruction secondary to a duodenal diaphragm with a central hole that was obstructed by date seeds. [6] In our case, more than 50 small, black stones of varying sizes impacted the small aperture of the diaphragm causing almost complete obturation. The source of pigment stones remains mysterious as the blood investigations did not reveal any evidence of hemolytic disease in the child or the family. However, a simple progressive sedimentation of bile in a commodius duodenum with dysmotility in addition to, Ascaris lumbricoides infestation, that is endemic in the area, should be assumed as the possible reason of stone formation and accumulation.

The important lesson to learn from this child's case is that, clinically, low frequency of bilious vomiting, lack of abdominal distention, and atypical abdominal x-ray may contribute to delayed diagnosis. However, better understanding of congenital duodenal membrane behavior may prevent such delay and its sequelae like growth retardation and prolonged hospitalization.

   References Top

1.Rowe MI, Buckner D, Clatworthy HW Jr. Windsock web of the duodenum. Am J Surg 1968;116:444-9.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Eustace S, Connolly B, Blake N. Congenital duodenal obstruction: An approach to diagnosis. Eur J Pediatr Surg 1993;3:267-70.  Back to cited text no. 2      
3.Lee FA, Mahour GH, Gwinn JL. Roentgenographic aspects of intrinsic duodenal obstruction. Ann Radiol (Paris) 1978;21:133-42.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Knechtle SJ, Filston HC. Anomalous biliary ducts associated with duodenal atresia. J Pediatr Surg 1990;25:1266-9.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Bickler SW, Harrison MW, Blank E, Campbell JR. Microperforation of a duodenal diaphragm as a cause of paradoxical gas in congenital duodenal obstruction. J Pediatr Surg 1992;27:747-9.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Nawaz A, Matta H, Jacobsz A, Trad O, Al Salem AH. Congenital duodenal diaphragm in eight children. Ann Saudi Med 2004;24:193-7.  Back to cited text no. 6  [PUBMED]  Medknow Journal  


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
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2 Abnormalities of intestinal rotation and congenital intrinsic duodenal obstruction
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Annals of Pediatric Surgery. 2013; 9(2): 61
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