Isolated Ileal Perforation in Infancy: A Lethal Initial Presentation of Hirschsprung's Disease
نویسندگان
چکیده
Arare case of ileal perforation, as a fatal initial presentation of total colonic aganglionosis (TCA) in infancy is reported. A 10-week-old boy, was brought to the emergency department with symptoms of complicated intestinal obstruction. He looked ill, was lethargic, markedly dehydrated and had a severely distended abdomen. An abdominal X-ray revealed multiple air fluid levels seen in a distended small intestine. During exploratory laparotomy the ileum was massively dilated with distal segment perforation. Ileal perforation repair was performed. A totally collapsed microcolon was identified. Biopsies were taken from the high rectum, sigmoid and hepatic flexure. Appendectomy and ileostomy were performed. All biopsies, as well as the appendix, showed absence of ganglion cells. Despite this procedure the patient progressively deteriorated and later died due to sepsis. Ileal perforation in infants is a rare, but potentially fatal initial presentation of TCA. Early detection is essential to prevent life-threatening complications.
منابع مشابه
Discordant monozygotic total colonic Hirschsprung's disease presenting with neonatal isolated ileal perforation.
To cite: Patel RV, Lawther S, Mccallion WA. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2013-200743 DESCRIPTION A full-term monozygotic twin, one presented with failure to pass meconium at 48 h of birth, refusal to feeds and abdominal distention. She was unwell with tachycardia and temperature 38.5° C. Abdomen was distended and tender with absent bowel sounds...
متن کامل[Hirschsprung's disease in adolescents and adults].
Megacolon must be considered as a diagnosis in any case of intractable constipation also in the adult. Besides the Hirschsprung's disease and the idiopathic megacolon, which have no apparent organic cause, many other pathologic conditions may produce a marked dilatation of the large bowel. The differential diagnosis depends, after a valuation of the clinical and radiologic features, also from e...
متن کاملAscaridial Volvulus: An Uncommon Cause of Ileal Perforation
Ascariasis is a common worm infestation in developing and under-developed countries. It is caused by the ingestion of food contaminated with Ascaris eggs from faeces and is more common in places with poor sanitation. Almost 25% of the world population is infested by Ascaris lumbricoides. Although ascariasis is a chronic disease, it can present as acute abdomen rarely. The wandering nature of As...
متن کاملCrohn's disease in the elderly.
The natural history of Crohn's disease in 47 patients, 60 years of age or older at the time of diagnosis has been defined, and their clinical management and long term prognosis reviewed. Distal colonic involvement is common in this group while extensive colonic and diffuse small bowel disease is rare. Distal colonic involvement usually carries a good prognosis except for those few patients who ...
متن کاملLaparoscopically detected and nonsurgically managed ileal perforation by an ingested fish bone: a case report
INTRODUCTION Ileal perforation due to fish bone is a rare event. The condition is difficult to diagnose due to lack of specific clinical features and low sensitivity of imaging techniques. We report a case of ileal perforation by a fish bone that was detected laparoscopically and managed nonsurgically. CASE PRESENTATION A 45-year-old Sinhalese man presented with acute onset right iliac fossa ...
متن کامل