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Year : 2000  |  Volume : 10  |  Issue : 3  |  Page : 153-154
Isolated segmental duodenal aganglionosis

1 Dept of Radiodiagnosis, GB Pant Hospital, New delhi - 110 002, India
2 Dept of Gastroenterology, GB Pant Hospital, New delhi - 110 002, India
3 Dept of Gastro-Intestinal Surgery, GB Pant Hospital, New delhi - 110 002, India

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Keywords: duodenum, aganglionosis

How to cite this article:
Puri SK, Panicker H, Panigrahi P, Kumar N, Chaudhary A. Isolated segmental duodenal aganglionosis. Indian J Radiol Imaging 2000;10:153-4

How to cite this URL:
Puri SK, Panicker H, Panigrahi P, Kumar N, Chaudhary A. Isolated segmental duodenal aganglionosis. Indian J Radiol Imaging [serial online] 2000 [cited 2020 Aug 13];10:153-4. Available from:
Aganglionosis of the bowel is commonly seen in the rectum and rectosigmoid as in Hirschsprung's disease. Total colonic involvement and contiguous involvement of the small bowel extending even till the duodenum has been reported in literature [1]. However, isolated segmental duodenal aganglionosis is extremely rare [2] and we would like to report one such case.

   Case Report Top

A twenty-six-years old man presented with a history of recurrent vomiting for six years. On examination he was very thin, malnourished and had a palpable distended stomach. Routine blood examinations were normal. The Mantoux test was negative. The chest radiograph was unremarkable.

US examination revealed a dilated stomach and duodenum with no evidence of an intraluminal mass. No lymph nodes could be detected in the epigastrium. The pancreas was normal. A barium study revealed a large distended stomach. The duodenum was massively dilated till the distal third part, beyond which there was a gradually tapering, smooth, tight stricture [Figure - 1]. The exact length and extent of stricture could not be demonstrated as the barium just trickled through the stricture. Hyperperistalsis was noticed in the proximal duodenum. The jejunal and ileal loops were seen in the right half of the abdomen. Delayed films and subsequently barium enema examination demonstrated the cecum and ascending colon in the left abdomen [Figure - 2] suggestive of small and large gut malrotation. The possibility of a congenital stricture or tuberculous stricture of the duodenum was considered.

The patient was subjected to surgery. No evidence of any inflammation or scarring was revealed in the narrowed segment. The narrowed segment was approximately 5 cms long with involvement from the distal third part of duodenum to the duodeno-jejunal flexure, which was on the right side of the spine. No peri-duodenal adhesions or lymph nodes could be seen. No intraluminal mass was seen and the mucosa appeared healthy. Multiple full thickness biopsies were taken from the narrowed segment. A posterior gastro-jejunostomy was performed. The patient recovered uneventfully and was relieved of his symptoms.

Histopathology revealed that the muscularis was atrophic and there was near complete absence of ganglion cells.

   Discussion Top

Aganglionosis of the bowel is the absence of ganglionic cells in the myenteric plexus. It belongs to a group of intestinal neuromuscular disorders of which Hirschsprung's disease is the most common. Various theories proposed include congenital disorders of migration of neuroblasts and acquired etiology because of ischemia of the bowel [3].

Most of the patients present in infancy and childhood but in 7-8% cases diagnosis is delayed till adulthood [4]. Occasionally aganglionosis is associated with intestinal malrotation and ileal atresia [5].

A zone of transition separates the normal ganglionic from the aganglionic segment. This zone is most often located in the rectosigmoid (65%), distal colon (14%), proximal colon (10%), rectum (8%) and small bowel (3%) [6]. Total aganglionosis is rare but is often associated with small bowel involvement (70%) [5].

Total intestinal aganglionosis with involvement of entire colon as well as small bowel till the duodenum has also been reported [7]. Abnormal motility of duodenum and esophagus has been reported in Hirschsprung's disease [3] and there have also been reports of megaduodenum and megacolon in the same patient [8].

Isolated duodenal, segmental aganglionosis is extremely rare. Barnett and Wall reported a case of megaduodenum in an adult with a history dating back to infancy. Although the histopathology was not conclusive, they suspected that diminution or absence of ganglion cells could result in failure of orderly progressive peristaltic waves and thereby produce changes characteristic of megaduodenum [8]. W J Mayo, Downes and Melchior have all noticed a striking similarity between the dilated hypertrophied duodenum in some cases of megaduodenum with that of the colon in Hirschsprung's disease and were of the opinion that the abnormality is a congenital neurogenic or neuromuscular derangement [8].

However, the only authentic report of isolated duodenal aganglionosis proven by histopathology is by Sulaiman et al [2]. The patient was an adult and had complained of vomiting for four years. He had a 6.0 cms long aganglionic segment immediately beyond the pylorus. Considering the short history an acquired etiology because of segmental ischemia was considered. Our patient was similar to this one with a similar short duration of illness but the aganglionic segment was more distal, in the third and fourth parts of duodenum. Associated bowel malrotation lead us to presume that the etiology might be congenital rather than acquired.

We emphasize that aganglionosis, though rare, should be considered a possible etiology in cases of megaduodenum with no discontinuity of lumen or mechanical obstruction. Conclusive proof is always provided by histopathology.

   References Top

1.Walker A W, Kempson RL, Ternberg JL. Aganglionosis of the small intestine. Surgery 1966; 60: 449-57.   Back to cited text no. 1    
2.Sulaimani SH, Alam MK, Soliman AAA, Sabah DM. Segmental duodenal aganglionosis in an adult. Br J Surg 1986; 73: 43-44.   Back to cited text no. 2    
3.Faure Ch, Ategbo S, Ferruia GC et al . Duodenal and esophageal manometry in total colonic aganglionosis. J Pediatr Gastroenterol Nutr 1994; 18: 193-99.   Back to cited text no. 3    
4.Todd IP. Adult Hirschsprungs disease. Br J Surg 1977; 64: 311-12.   Back to cited text no. 4    
5.Ikeda K, Goto S. Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients. J Pediatr Surg 1986; 21: 319-22.   Back to cited text no. 5    
6.Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon; an analysis of 501 patients. J Pediatr Surg 1973; 8: 587.   Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Bodian M, Carter Co. A family study of Hirschsprung's disease. Ann Hum Genet 1963; 26: 261.   Back to cited text no. 7    
8.Barnett WO, Wall L. Megaduodenum resulting from absence of the parasympathetic ganglion cells in Auerbach's plexus.  Back to cited text no. 8    

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Sunil Kumar Puri
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