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 ::  Abstract
 ::  Introduction
 ::  Case report
 ::  Discussion
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Year : 1998  |  Volume : 44  |  Issue : 1  |  Page : 16-8

Jejunal angiomatoses causing small bowel obstruction in a patient with Down syndrome: a case report.

Department of Radiology, Seth G.S. Medical College, Mumbai, India., India

Correspondence Address:
T Patankar
Department of Radiology, Seth G.S. Medical College, Mumbai, India.
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Source of Support: None, Conflict of Interest: None

PMID: 0010703561

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 :: Abstract 

Gastrointestinal vascular anomalies are extremely uncommon. We describe a patient with Down syndrome who presented with acute abdominal pain due to a mixed capillary and venous vascular malformation involving the proximal jejunum.

Keywords: Adult, Angiomatosis, complications,Case Report, Down Syndrome, complications,Female, Human, Intestinal Obstruction, etiology,Intestine, Small, pathology,Jejunal Diseases, Tomography, X-Ray Computed,

How to cite this article:
Patankar T, Prasad S, Joshi A, Deshmukh H. Jejunal angiomatoses causing small bowel obstruction in a patient with Down syndrome: a case report. J Postgrad Med 1998;44:16

How to cite this URL:
Patankar T, Prasad S, Joshi A, Deshmukh H. Jejunal angiomatoses causing small bowel obstruction in a patient with Down syndrome: a case report. J Postgrad Med [serial online] 1998 [cited 2023 Mar 23];44:16. Available from:

  ::   Introduction Top

Gut vascular anomalies are poorly defined and imperfectly understood complex, heterogeneous lesions, which are rarely encountered in clinical practice. In general, two types of benign vascular lesions exist which have characteristic histomorphology, natural history and therapeutic implications. They are haemangiomas, which constitute benign endothelial neoplasms and vascular malformations, which represent errors in morphogenesis[1]. Recognition of these two entities is of paramount importance as it has considerable therapeutic implications.

  ::   Case report Top

A thirty-year-old woman, a diagnosed case of Down syndrome presented with a two days history of acute abdominal pain. Clinical examination was unremarkable. Laboratory investigations were normal. Plain abdominal radiograph demonstrated dilated duodenum. Barium upper gastrointestinal series showed dilated duodenum and concentric luminal narrowing involving the duodenojejunal flexure and proximal jejunum [Figure - 1].

Plain and contrast enhanced computerized tomographic (CT) scan of the abdomen demonstrated circumferential bowel wall thickening of the proximal jejunum, duodenal dilatation and collapsed distal small bowel loops [Figure - 2]. Exploratory laparotomy showed long segment stricture involving the duodenojejunal flexure and the proximal jejunum. Resection of the involved segment and end-to-end bowel anastomosis was performed. Histopathological examination revealed ectatic, congested capillaries, and venules scattered throughout the entire wall of the bowel [Figure - 3] thus confirming the diagnosis of a mixed capillary and venous vascular malformation. The patient made an uneventful recovery from the operation and was discharged on the fourth day.

  ::   Discussion Top

Gastrointestinal vascular anomalies are frequently asymptomatic[1],[2]. Symptomatic patients present with gastrointestinal bleeding, bowel obstruction and abdominal pain[2]. Infrequently encountered manifestations include diarrhoea, protein-losing enteropathy, and high-output congestive cardiac failure[1]. They may be associated with cutaneous and other musculoskeletal lesions.

Vascular malformations represent errors in vascular morphogenesis[1]. They are classified by the nature of predominant vascular channels within the lesion and rheologic characteristics into Pure Venous, Lymphatico-venous and Capillary-lymphatico-venous malformations[1]. Although all these lesions are congenital, they may manifest anytime during life and may be associated with the many syndromes. Rendu-Osler- Weber syndrome More Details is characterised by the classic triad of muco-cutaneous/ visceral telangiectasias, repeated haemorrhages and familial occurrences[3],[4]. These vascular lesions bleed easily and may cause severe anaemia. Pure venous type of submucosal vascular malformation of the small intestine which give rise to chronic anaemia may be associated with the phenotype of blue-rubber-bleb syndrome[1]. Klippel-Trenaunay syndrome[5] is associated with capillary-lymphatico-venous malformation and limb overgrowth. Resection and Endoscopic / surgical or angiographic vaso-obliteration constitute most appropriate therapy of these vascular lesions[1].

In contrast, haemangiomas are usually present at birth with a female preponderance (female to male ratio of 3:1). They constitute 7%-10% of all benign tumours of the small bowel[4] and may be associated with Kasabach - Merritt syndrome and Mafucci’s syndrome. Kasabach - Merritt syndrome is characterized by haemangiomas, thrombocytopenia and a haemorrhagic diathesis which is cured following tumour resection[6],[4]. Mucocutaneous haemangiomas with enchondromas characterize Mafucci’s syndrome[1].

Haemangiomas present either as polypoid masses projecting into the lumen or as diffusely infiltrating lesions involving long segment of the gut[2]. Majority of lesions are manifest during the first two years of life and charter a benign course once they enter the involution phase. They may present with internal bleeding which may be massive and life threatening[4]. Angiogenesis inhibition pharmacologic therapy (using steroids, interferons) is the mainstay of treatment of haemangiomas[1].

Patients with Down syndrome have genetic predisposition to gut anomalies like oesophageal atresia, duodenal obstruction and Hirschsprung disease. 33% of infants with duodenal obstruction and 3%-5% of infants with Hirschsprung disease have Down syndrome[7]. Majority of Down syndrome patients with these anomalies present in infancy[7]. We did not find mention of acute abdomen due to jejunal angiomatoses occurring in an adult patient with Down syndrome, in English literature. In conclusion, symptomatic gastrointestinal vascular anomalies are extremely uncommon. Vascular anomalies of the gut may occur in patients with Down syndrome and have to be considered in patients presenting with acute abdomen.

 :: References Top

1. Fishman SJ, Burrows PE, Leichtner AM, Mulliken JB. Gastrointestinal manifestations of vascular anomalies in Childhood: Varied Etiologies require multiple therapeutic modalities. J Ped Surg 1998; 33(7):1163-1167.  Back to cited text no. 1    
2.Boyle L, Lack BE. Solitary cavernous haemangioma of small intestine: Case report & literature review. Arch Pathol Lab Med 1993; 117:939-941.  Back to cited text no. 2    
3.Smith CR Jr, Bartholomew LO, Cain JC. Hereditary haemorrhagic telangiectasia and gastrointestinal haemorrhage. Gastroenterology 1963; 44:1-6.  Back to cited text no. 3    
4.Eenoglio-Preiser CM, Lantz PE, Listrom MB, Davis M, Rilke FO. Gastrointestinal Pathology. An Atlas and Text. NewYork: Raven Press; Chapter 16. 1989, pp 569-571.  Back to cited text no. 4    
5.Ghahremani GG, Kangaraloo H. Volberg F. Meyers M. Diffuse cavernous haemangioma of the colon in Klippel-Trenauny syndrome. Radiology 1976; 11S:673.  Back to cited text no. 5    
6.Kasabach HH, Merritt KK. Capillary haemangioma with extensive purpura. Am J Dis Child 1940; 59:1063.  Back to cited text no. 6    
7.Sabiston Jr. DC, Lyerly HK. Textbook of Surgery. The biological basis of modem surgical practice. Philadelphia: WB Saunders Co., 15th Edition, Vol: 2, 1997; pp 1234-1245.   Back to cited text no. 7    


[Figure - 1], [Figure - 2], [Figure - 3]

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