[Currarino syndrome. A rare cause of severe constipation. Case report and literature review]. / Síndrome de Currarino. Una causa poco frecuente de estreñimiento grave. Presentación de un caso y revisión de la literatura.

The association of congenital anorectal malformation, sacral defect and a presacral mass is known as the Currarino syndrome described for the first time in 1981. Currarino et al. proposed that abnormal endoectodermal adhesions and notochordal defects in early fetal life may result in a fistula between the gut and the spinal ca- nal with enteric elements ventrally and neural elements dorsally. In over 80% of cases, the syndrome is diagnosed during the first decade of life. Intractable constipation since birth is the leading symptom of this triad, which follows an autosomal dominant mode of heredity. Rectal examination, plain radiographs and magnetic resonance imaging are the main tools for the diagnosis. The medical therapy is poorly successful and, therefore, combined medical and neurosurgical assessment and management for all cases of Currarino syndrome are recommended. The authors present a case of a patient with the classic features of this syndrome and briefly review the relevant literature.

Transanal endorectal pull-through for Hirschsprung's disease.

PURPOSE: A new endorectal pull-through technique using a transanal approach is presented in this report. METHODS: Mucosectomy, colectomy, and pull-through are performed transanally, and neither laparotomy or laparoscopy are required. Five patients affected with Hirschsprung's disease have been operated on with this technique. CONCLUSION: During the 6- to 15-month follow-up period, all of them have had postoperative normal bowel movements.

Gastric triplication and peritoneal melanosis.

Duplications of the intestinal tract are rare malformations, and triplications are even less frequent; only two cases are found in the literature. Peritoneal melanosis, a diffuse, black pigmentation of the peritoneum is also rare; only six cases have been reported; five in young women and one in a 2-year-old girl. The authors report on a 6-month-old boy with a gastric triplication and peritoneal melanosis. The diagnostic criteria and the possible embryological origin are discussed.

Angiodysplasia of the colon in children.

Angiodysplasia of the colon as a cause of lower intestinal bleeding is diagnosed frequently in the elderly, with defined clinical characteristics. In the pediatric population there is little experience; only six cases have been reported. The present study evaluates the clinical, radiological, and surgical aspects of nine children with angiodysplasia of the colon who were treated at the National Institute of Pediatrics in Mexico City between 1970 and 1993. The mean age of clinical onset was 2.3 years. In six patients, symptoms appeared before the first year of life. Lower intestinal bleeding and severe anemia were present in all cases. The diagnosis and localization of the lesions were established by selective visceral angiography. Six patients were treated by operative resection of the affected colonic segments. For four patients with lesions in the rectum and sigmoid, a Swenson pull-through was performed. In one patient the lesion was recurrent because of incomplete resection. Follow-up ranges from 8 months to 4 years; all patients have had normal hemoglobin levels and negative stool results. Unlike in the elderly and the cases reported in the literature, the left hemicolon was the most frequently involved area.

Central nervous system tumors in Mexican children.

Five hundred and seventy primary central nervous system (CNS) tumors from the Department of Pathology at the National Institute of Pediatrics in Mexico City, collected from 1970 to 1989, were histologically reclassified in order to find out their relative incidence as well as their outstanding features. With this, we could establish a frame of reference for our local population, contributing to the epidemiological analysis of these entities. All the tumors were examined independently by two pathologists (C.R. and M.R.), using the classification of Rorke et al. Histological type, patient age and sex, and tumor location were analyzed. CNS tumors were the secondmost frequently encountered solid tumors, after lymphomas, and were increasing in incidence at a rate of 2.2 annually. Children in the age group 0-9 years were most often affected, and there was a predominance of male patients. Astrocytoma and medulloblastoma were the most common tumor types. The infratentorial region was the most frequent tumor location in the 2- to 9-year age group. By contrast, in the under 2-year-olds a supratentorial location was more frequent, and the incidence of germ cell tumors was proportionally high. In general, some histological types seemed to be associated with particular age groups. Although we found primitive neuroectodermal tumors to be the fifth most common at all ages (except for medulloblastoma), many other authors do not report a similar finding.