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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 53  |  Issue : 1  |  Page : 29-31

Duodenal atresia type III showing distal bowel gas and atypical malrotation in association with gastric and bile duct duplications


Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission03-Jun-2019
Date of Decision19-Aug-2019
Date of Acceptance20-Oct-2019
Date of Web Publication19-Feb-2020

Correspondence Address:
Dr. Rahul Gupta
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_47_19

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  Abstract 


Association of duodenal atresia (DA) with gastric duplication cyst is an extremely rare entity. Furthermore, DA type III with the presence of distal bowel gas is very rare. We report the case of a 13-day-old preterm male with neonatal intestinal obstruction. Radiological evaluation revealed duodenal obstruction at the second part with the presence of few distal bowel gas shadows. Laparotomy revealed dilated gastroduodenum, atypical malrotation, and a cystic structure (duplication cyst) identical to the stomach attached to its greater curvature along with type III atresia of the second part of the duodenum. Duodenotomies revealed the presence of bile along with air at either blind ends of the duodenum which could be explained by the presence of bile duct duplication at the distal end. We propose that this association is due to “defective mesenchyme formation during organogenesis,” which may explain multiple duplication abnormalities and DA.

Keywords: Atypical malrotation, distal bile duct duplication, distal bowel gas, duodenal atresia type III, gastric duplication cyst


How to cite this article:
Gupta R, Gupta AK. Duodenal atresia type III showing distal bowel gas and atypical malrotation in association with gastric and bile duct duplications. Formos J Surg 2020;53:29-31

How to cite this URL:
Gupta R, Gupta AK. Duodenal atresia type III showing distal bowel gas and atypical malrotation in association with gastric and bile duct duplications. Formos J Surg [serial online] 2020 [cited 2020 Apr 4];53:29-31. Available from: http://www.e-fjs.org/text.asp?2020/53/1/29/278671




  Introduction Top


Duodenal atresia (DA) is a common cause of neonatal intestinal obstruction, while gastric duplications are rare alimentary tract duplications.[1],[2] We present herein a neonate with DA type III with distal bowel gas due to bile duct duplication and atypical malrotation in association with gastric duplication cyst, which is extremely rare and not described in literature till date. We propose that this association in this index case is due to “defective mesenchyme formation during organogenesis,” which may explain multiple duplication abnormalities and intestinal atresia(s).


  Case Report Top


A 13-day-old preterm (32 weeks) male neonate weighing 1650 g presented with bilious vomiting and epigastric fullness since birth. Antenatal ultrasounds were not done. On examination, the neonate was dehydrated, jaundiced, and hemodynamically unstable; pulse rate was 142/min and respiratory rate was 48/min. The abdomen was soft, and epigastric fullness was present. Laboratory investigations revealed indirect hyperbilirubinemia (total bilirubin – 12 mg/dl) and C-reactive protein levels >6 mg/L. Abdominal ultrasound was suggestive of duodenal obstruction with the presence of sludge in the gallbladder. An abdominal X-ray revealed duodenal obstruction at the second part with the presence of few distal bowel gas shadows [Figure 1]. An upper gastrointestinal contrast series showed complete obstruction at the second part of duodenum [Figure 1]. Preoperative optimization was performed. Laparotomy revealed large, dilated gastroduodenum; atypical malrotation with the presence of Ladd's bands over the second part of the duodenum, ligament of Treitz to the left of the midline, relatively narrow mesenteric base, and cecum in the left hypochondrium. A cystic structure (duplication cyst) identical to the stomach wall, attached to its greater curvature, noncommunicating with the lumen of the stomach, and sharing common blood supply was appreciated [Figure 2]. Division of Ladd's bands and kocherization of the duodenum revealed type III atresia of the second part of duodenum. Noncollapsed small bowel (suggestive of air and meconium filled) loops were present. Duodenotomies revealed the presence of bile along with air at either (blind) ends of the duodenum. Widening of the mesenteric base and Kimura's duodeno-duodenostomy were performed [Figure 2]. Excision of large noncommunicating gastric duplication cyst was deferred as the neonate had an episode of intraoperative desaturation. Furthermore, the neonate was premature, was of low birth weight, and there was sepsis. The postoperative course was uneventful. The patient became jaundice free and is doing well, gaining weight at 2-month follow-up.
Figure 1: Abdominal X-ray (images on the left) showing gastric and duodenal gas shadows up to the second part of the duodenum along with the presence of few small distal bowel gas shadows (indicated by red stars); also seen are two separate gas shadows in the right upper abdomen (arrows). Upper gastrointestinal contrast study (images on the right) revealing dilated stomach and duodenum up to its second part with the absence of contrast distally

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Figure 2: Intraoperative images showing dilated stomach, cystic structure (indicated by yellow arrows) identical to the stomach wall, attached to its greater curvature, and sharing common blood supply from omental vessels; atypical malrotation with cecum present in the left hypochondrium (pointed by forceps) is appreciated. Noncollapsed small bowel (suggestive of air and meconium filled) loops in the left upper and right image (before duodenotomies) are also seen. The left lower image shows completed Kimura's duodeno-duodenostomy

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  Discussion Top


DA is a common cause of neonatal intestinal obstruction with an estimated incidence of between 1 in 6000 and 1 in 10,000 births.[2] Type III DA is two blind ends of the duodenum separated by a gap with a V-shaped mesenteric defect.[2] Usually, all types of DA are limited to the second (postampullary) part of the duodenum, and bilious vomiting is the main presenting symptom.[2] Plain radiography shows “Double Bubble” sign and is the most valuable diagnostic tool in all cases of DA. Exceptions from this sign are those with incomplete obstruction due to duodenal web (type I) or distally located duodenal webs.[2] In our case, radiological evaluation revealed duodenal obstruction at the second part with the presence of few distal bowel gas shadows. A differential diagnosis of DA was made. Furthermore, DA type III with the presence of distal bowel gas is very rare, as seen in our case. This unusual presence of distal bowel gas could be explained by the presence of partial duplication of distal end of the bile duct associated with DA as reported in some series.[3],[4] Distal end of the bile duct with partial duplication may be “T” or “Y” shaped.[3] Anomalous bifurcated bile duct conduit with distal bowel gas was demonstrated by preoperative upper gastrointestinal series in only 8.77% of patients in a large series of DA.[4] In our index case, partial duplication of bile duct could not be demonstrated by upper gastrointestinal series, which may be due to smaller lumen of the bile duct, allowing only passage of air through the bifid duct into the distal bowel. This was confirmed intraoperatively by noncollapsed small bowel (suggestive of air and meconium filled) loops, as shown in [Figure 2].

Gastric duplications are rare, constituting about 2%–7% of all alimentary tract duplications.[1] Majority of them are noncommunicating cysts, arise at the greater curvature, and present as large lump in an early age. Delayed presentation with complications such as acute abdomen, peritonitis and pancreatitis due to hemorrhage, infection, perforation, volvulus, and intussusception and compression effects of the cyst is reported.[1] Intestinal duplications have sporadically been reported with intestinal atresia,[5] but the association of gastric duplication with DA is extremely rare, with only one case reported in literature till date.[6] The recommended treatment of gastric duplication cyst is surgical resection or partial resection with the mucosal stripping of the remaining part of the cyst.[1],[6]

“Atypical malrotation” is diagnosed when intestinal anatomy is somewhere between completely normal and classically malrotated.[7] Malrotation is defined as “typical” if the ligament of Treitz is to the right of the midline or absent, whereas in atypical malrotation, it is at or left of the midline.[7],[8] Cecal position is abnormal if it is somewhere other than the right lower quadrant.[7] In our case, the intraoperative findings were suggestive of atypical malrotation.

To the best of our knowledge, the presence of DA type III with distal bowel gas due to distal bile duct duplication and atypical malrotation in association with gastric duplication cyst has not been reported previously and thus, it is an index case. In this neonate, cyst excision was deferred due to intraoperative desaturation and the presence of sepsis in our premature (32 weeks), low-birth-weight (1650 g) neonate.

Association of gastric duplications (and other duplications) with atresia (especially DA) has been previously explained because of “vascular accident theory,” which fails to explain the presence of both entities in different sites.[6] We propose that this association is due to “defective mesenchyme formation during organogenesis,” which may explain multiple (complete and partial) duplication abnormalities and DA in our case.[9] As a consequence to abnormality in the mesenchymal precursor cells, there is defective mesenchymal formation at multiple sites.[9] Furthermore, neural tube closure defects have been associated with abnormalities in mesenchymal cells and mesenchyme.[10] This theory may also explain intestinal duplication abnormalities and associated intestinal atresias.[5] It may also explicate the association of DA with duplication in the biliary system including gallbladder duplication.[3],[4]

In conclusion, DA type III with distal bowel gas due to distal bile duct duplication and atypical malrotation in association with gastric duplication cyst is extremely rare. We propose that the association of complete and partial duplication abnormalities and associated intestinal atresias is due to “defective mesenchyme formation during organogenesis.”

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kayastha K, Sheikh A. Gastric duplication cyst presenting as acute abdomen: A case report. APSP J Case Rep 2010;1:6.  Back to cited text no. 1
    
2.
Gupta R, Mathur P, Gubbi S, Gupta PK, Shukla R, Bhandari A. More distally located duodenal webs: A case series. J Neonatal Surg 2016;5:56.  Back to cited text no. 2
    
3.
Reid IS. Biliary tract abnormalities associated with duodenal atresia. Arch Dis Child 1973;48:952-7.  Back to cited text no. 3
    
4.
Komuro H, Ono K, Hoshino N, Urita Y, Gotoh C, Fujishiro J, et al. Bile duct duplication as a cause of distal bowel gas in neonatal duodenal obstruction. J Pediatr Surg 2011;46:2301-4.  Back to cited text no. 4
    
5.
Soon OC, Woo HP. Duodenal duplication cyst associated with duodenal atresia. Pediatr Surg Int 1995;10:167-8.  Back to cited text no. 5
    
6.
Ghoroubi J, Mirshemirani A, Roshanzamir F, Razavi S, Sarafi M. Gastric duplication cyst in association with duodenal atresia in a neonate. APSP J Case Rep 2016;7:6.  Back to cited text no. 6
    
7.
Mehall JR, Chandler JC, Mehall RL, Jackson RJ, Wagner CW, Smith SD. Management of typical and atypical intestinal malrotation. J Pediatr Surg 2002;37:1169-72.  Back to cited text no. 7
    
8.
McVay MR, Kokoska ER, Jackson RJ, Smith SD. Jack barney award. The changing spectrum of intestinal malrotation: Diagnosis and management. Am J Surg 2007;194:712-7.  Back to cited text no. 8
    
9.
Gupta R. Type 5 congenital pouch colon and its embryopathogenesis. Med J DY Patil Vidyapeeth 2018;11:554-8.  Back to cited text no. 9
  [Full text]  
10.
O'Shea KS. Ultrastructural analysis of chromosome translocation-induced neural tube defects. Brain Res Bull 1986;16:833-44.  Back to cited text no. 10
    


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