Cases reported • Gastrointestinal Diseases; Cholera Infantum

11/28. Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.

We report on two children who were admitted with chronic ileus without mechanical obstruction. In the 4-month-old female newborn, high-dose radiation was applied after extirpation of a sympathicoblastoma. Within a few years a metaplasia of the muscle coat of the small intestine developed with a resulting malabsorption syndrome. Although the damaged part of the intestine was resected, the process progressed and the child died. In the second case, a chronic ileus developed at the age of 10 years as a result of fibrosis of the intestinal tract. Repeated laparotomies were performed, and no mechanical obstruction could be found. The most probable diagnosis is a form of scleroderma affecting mainly the alimentary tract without any skin involvement. The patient died in a severe cachexia.

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12/28. Gastrointestinal manifestations of Sipple syndrome in children.

The diagnosis and management of patients with multiple endocrine neoplasia (men) type IIA and type IIB are of special challenge to pediatric surgeons. patients characteristically present early in life with significant intestinal symptoms at a time when the characteristic phenotypic features of men IIB are frequently absent. We are reporting 12 patients with men type II (9 with type IIA and 3 type IIB or Sipple's syndrome), all of whom presented with gastrointestinal manifestations. All 12 patients had signs and symptoms of bowel obstruction during the neonatal period. An unusual association of Hirschsprung's disease and men IIA was noted in our nine patients found among a kindred of 92 individuals. All three patients with Sipple's syndrome (men IIB) had severe gastrointestinal symptoms since birth, including recurrent pseudoobstruction. The possibility of men type II should be considered in all cases of bowel obstruction in the newborn period. Screening for medullary carcinoma of the thyroid must be carried out from infancy. A detailed family history is very important to avoid unnecessary surgery for bowel obstruction in Sipple's syndrome.

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13/28. Gastrointestinal histoplasmosis.

Three cases are reported of gastrointestinal histoplasmosis in patients who came from the Caribbean or south america and had lymphoma, acquired immunodeficiency syndrome, and prior local radiation therapy. The patients had small-bowel obstruction with ileal involvement, mucosal erythema, and friability on colonoscopy with colonic involvement and an exophytic rectal mass with rectal involvement. Review of the 77 reported cases of gastrointestinal histoplasmosis shows that this is a clinical subset of disseminated histoplasmosis. With gastrointestinal involvement, pulmonary symptoms are uncommon and gastrointestinal symptoms predominate. fever is less common than in other forms of dissemination. The most common lesions are a mass or ulcers, which often mimic inflammatory bowel disease or carcinoma. Terminal ileal involvement predominates in one third. The complement fixation test was positive in about three quarters of cases tested, but the skin test is not diagnostically useful. In one quarter of patients there is other evidence of immunosuppression. In the immunosuppressed, gastrointestinal histoplasmosis must be considered, even in a patient from a nonendemic area, who presents with lesions appearing like carcinoma or inflammatory bowel disease. When feasible, endoscopic examination and biopsy with stains and culture for histoplasmosis is recommended for diagnosis. Medical management is recommended, with surgery reserved for acute emergencies or when mandatory for diagnosis.

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14/28. cryptosporidiosis of the stomach.

A patient with acquired immune deficiency syndrome who developed symptoms of partial gastric outlet obstruction during the course of a severe diarrheal illness due to cryptosporidiosis is presented. Endoscopic evaluation of the stomach revealed an inflamed pyloric ring and channel with clinical evidence suggestive of partial outlet obstruction. The finding of cryptosporidial organisms on biopsy of the pylorus highlights the potential for diffuse gastrointestinal involvement by this parasite and offers an alternative explanation for the symptoms of nausea and vomiting associated with the diarrheal illness.

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15/28. A familial visceral myopathy with dilatation of the entire gastrointestinal tract.

We are reporting a new kindred in which familial visceral myopathy was identified in four siblings. There was no other symptomatic case identified in this 75-member, four-generation family, suggesting an autosomal recessive inheritance. All patients had recurrent symptoms of intestinal obstruction, and 3 died from malnutrition. Gastrointestinal lesions showed marked dilatation of the entire digestive tract from the esophagus to the rectum, which is different from other types of familial visceral myopathies. Microscopic examination of the gastrointestinal smooth muscle showed degeneration and increased fibrosis indistinguishable from other types of familial visceral myopathy.

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16/28. Pneumomediastinum: an unusual complication of acute gastrointestinal disease.

Atraumatic or primary pneumomediastinum is an uncommon complication of severe retching and vomiting associated with acute gastrointestinal disorders. In two cases pneumomediastinum was the presenting radiographic abnormality; one patient presented with acute gastroenteritis, and the other presented with duodenal obstruction due to hematoma. The pathogenesis of pneumomediastinum in these patients is presented.

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17/28. Oculogastrointestinal muscular dystrophy.

The author reports on four patients (one male, three females) from the same kindred with a newly recognized autosomal recessive condition involving striated and smooth muscle that has been designated oculogastrointestinal muscular dystrophy. It is characterized by ptosis, ophthalmoplegia, and progressive intestinal pseudo-obstruction leading to malnutrition and death before 30 y. autopsy studies in two cases showed a severe primary myopathy of smooth muscles of the stomach and intestine with intact myenteric plexus and vagus nerves. The proposita notably had myopathic changes of striated muscles but also involvement of the peripheral nerves and central nervous system characterized by demyelinating and axonal neuropathy and focal spongiform degeneration of the posterior columns.

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18/28. A familial visceral myopathy.

A kindred contained at least 18 members with visceral myopathy. Sixteen had symptoms of chronic obstruction of the gastrointestinal or urinary tracts. Of six patients with megaduodenum on contrast roentgenograms, two were asymptomatic. Four patients had redundant colon on barium enema, and four had megacystis. Specimens from duodenum, jejunum, ileum, colon, or urinary bladder from five patients showed thinning and extensive collagen replacement of the longitudinal muscle layer; ganglion cells were normal by light and electron microscopy. Esophageal manometry in three patients showed decreased gastroesophageal sphincter pressures and no contractions in the smooth muscle segment of the esophagus; duodenal manometry showed a low frequency and amplitude of contractions. Three patients developed fever and signs of peritonitis after operations to bypass dilated segments. This seems to be a generalized smooth muscle disease with variable clinical manifestations and with an autosomal dominant or sex-linked dominant mode of inheritance.

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19/28. Ultrasound patterns of disorders affecting the gastrointestinal tract.

A mass associated with the gastrointestinal tract was detected by sonography in 33 patients. Etiologies included primary or metastatic tumor; intussusception; inflammation secondary to bowel infarction, pancreatitis, or irradiation; and a dilated, fluid-filled gut related to retained gastric contents, obstruction, ileus, or an ileal bypass. Mesenteric or omental changes were identified with inflammation and frequently with metastatic disease. The diagnosis was confirmed by repeat sonography, abdominal radiography, barium examination of the small bowel, computed tomography, surgery, or autopsy. Ultrasound patterns are characteristic in tumor, intussusception, and inflammation; specific features allowing differentiation between tumor and inflammation are described. Colonic haustra, valvulae conniventes, or bowel contours and peristalsis on real-time sonography are helpful in identifying fluid-filled bowel loops.

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20/28. The many faces of Meckel's diverticulum.

Meckel's diverticulum usually presents as a diagnostic problem. Its most common clinical manifestations--lower GI bleeding, small bowel obstruction, and features suggesting acute appendicitis--are fairly well known. Other clinical presentations include a Meckel's diverticulum in an omphalocele, lower GI bleeding followed by perforation, perforation secondary to blunt trauma, and presentation as iron deficiency anemia with and without episodes of overt hemorrhage. Illustrative cases emphasize indications for and usefulness of abdominal scanning as a diagnostic aid.

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