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An unusual and difficult diagnosis of intestinal obstruction: The abdominal cocoon. Case report and review of the literature.

ABSTRACT: Abdominal cocoon is a rare cause of intestinal obstruction. The abdominal cocoon is probably a developmental abnormality, largely asymptomatic, and is found incidentally at laparotomy or autopsy. Pre-operative diagnosis cannot be often made correctly. This rare entity of intestinal obstruction has been described in the whole literature as a thick fibrotic sac covering the small bowel partially or completely. The etiology of abdominal cocoon is unknown and most often it is found in adolescent girls from tropical or subtropical countries. Complete recovery is generally expected after the removal of the membrane surgically. This paper reports a male patient who has had intestinal obstruction symptoms and has per-operatively been diagnosed as abdominal cocoon.

SUBMITTER: Devay AO

PROVIDER: S-EPMC1459267 | biostudies-literature | 2006 Mar

REPOSITORIES: biostudies-literature

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An unusual and difficult diagnosis of intestinal obstruction: The abdominal cocoon. Case report and review of the literature.

Devay Ali O AO Gomceli Ismail I Korukluoglu Birol B Kusdemir Ahmet A

World journal of emergency surgery : WJES 20060324

Abdominal cocoon is a rare cause of intestinal obstruction. The abdominal cocoon is probably a developmental abnormality, largely asymptomatic, and is found incidentally at laparotomy or autopsy. Pre-operative diagnosis cannot be often made correctly. This rare entity of intestinal obstruction has been described in the whole literature as a thick fibrotic sac covering the small bowel partially or completely. The etiology of abdominal cocoon is unknown and most often it is found in adolescent gir ...[more]

PMID: 16759404

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Project description:BackgroundIntestinal Ganglioneuromatosis (IG) is a rare disorder of the enteric nervous system. In pediatric age it is often associated with genetic syndromes such as Neurofibromatosis 1 (NF1), multiple endocrine neoplasia type 2B (MEN2B) and Cowden syndrome (PTEN mutation), and ganglioneuromas (GNs) may be sometimes the first sign of the disease. Isolated GNs are rare and sporadic. Clinical symptom vary and depend on the size and on the location of the GNs. This disorder affects intestinal motility and it, consequently, causes changes in bowel habits, abdominal pain, occlusive symptoms and rarely lower gastrointestinal bleeding secondary to ulceration of the intestinal mucosa. On the other hand, patients can remain asymptomatic for many years.Case presentationWe describe a 9-year-old boy referred to our emergency department for right lower quadrant abdominal pain. No familial history for gastrointestinal disorders. No history of fever or weight loss. At physical examination, he had diffused abdominal pain. Abdominal ultrasonography showed a hypoechoic formation measuring 41.8 mm by 35 mm in the right lower quadrant of the abdomen. Routine blood tests were normal, but fecal occult blood test was positive. Abdominal TC confirmed the hypodense formation, of about 5 cm in transverse diameter, in the right hypochondrium that apparently invaginated in the caecum-last ileal loop. Colonoscopy showed in the cecum an invaginated polypoid lesion of the terminal ileal loop. Laparoscopic resection of the polypoid lesion was performed. Histological diagnosis of the large neoplasm observed in the terminal ileum was diffuse ganglioneuromatosis. NF1, RET and PTEN gene tests resulted negative for specific mutations. At the 1 year follow-up, the patient presented good general condition and blood tests, fecal occult blood test, esophagogastroduodenoscopy, colonoscopy and MR-enterography were negative.ConclusionsOnly few cases are reported in literature of IG in pediatric age. Although rare, the present case suggests that this disorder must be taken in consideration in every patient with GI symptoms such as abdominal pain, constipation, lower intestinal bleeding, in order to avoid a delayed diagnosis.

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An unusual and difficult diagnosis of intestinal obstruction: The abdominal cocoon. Case report and review of the literature.

Publications

An unusual and difficult diagnosis of intestinal obstruction: The abdominal cocoon. Case report and review of the literature.

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