Neuromuscular and vascular hamartoma of the small intestine.

Neuromuscular and vascular hamartoma is an extremely rare stricturing condition of the small bowel. It consists of abnormal mixtures of intestinal tissues: disorganized fascicles of smooth muscle derived from the submucosa, bundles of nonmyelinated nerve fibers with scattered abnormal ganglion cells and hemangiomatous vessels, occurring focally and causing recurrent obstructive symptoms or occult chronic gastrointestinal bleeding. In this paper we report our experience with this tumor.

[Neuromuscular and vascular hamartoma of the small intestine. Report of a clinical case and review of the literature]. / L'amartoma neuromuscolare e vascolare dell'intestino tenue. Presentazione di un caso clinico e revisione della letteratura.

We present the case of a 50-year old patient, operated on for three adjoining annular strictures in the jejunum with proximal dilatation. He presented with a one-year history of recurrent cramping in the upper abdomen and vomiting, with two episodes of intestinal obstruction. Microscopic examination of the stricture revealed, in the submucosa, disorganised fascicles of smooth muscle derived from the muscularis mucosae, bundles of non-myelinated nerve fibres with scattered abnormal ganglion cells and haemangiomatous vessels. The pathological findings observed were similar to those described as neuromuscular and vascular hamartoma, a rare stricturing condition of the small intestine. Many authors have questioned the hamartomatous nature of this disorder, since identical features may be seen in Crohn's disease, in ischaemic enteritis, in radiation enteritis and in non-steroidal antiinflammatory drug-induced small intestinal strictures. On the basis of a review of the 5 previously described cases and of our own experience, we believe that neuro muscular and vascular hamartoma of the small bowel should be considered as a distinct entity if histological hallmarks of Crohn's disease are absent, in patients with no history of gastrointestinal disease, or of chronic ingestion of non-steroidal antiinflammatory drugs.

Congenital hepatic dysplasia from cytomegalovirus.

The Authors report on a case of liver dysplasia in a four-month-old infant affected by prenatal cytomegalovirus infection. Immunologic, histologic and ultrastructural studies suggest an embryopathic origin of the lesions.

Mollaret's meningitis: an unusual disease with a characteristic presentation.

Mollaret's meningitis (benign recurrent aseptic meningitis) is a rare syndrome with characteristic features. Patients present with recurrent attacks of meningismus which are sudden in onset and last from one to seven days. During the attacks the spinal fluid shows pleocytosis with lymphocyte predominance. Large endothelial cells with indistinct cytoplasm (Mollaret's cells) are typically present in the CSF. The attacks resolve spontaneously and without sequellae. Symptom-free intervals can be as brief as a few days or as long as several years. Though specific treatment is not available, Colchicine has been reported to decrease the severity and frequency of attacks. While acute episodes may cause significant symptoms, the long-term prognosis is excellent.