Experience with a cholinesterase histochemical technique for rectal suction biopsies in the diagnosis of Hirschsprung's disease.

Cryostat sections from 160 rectal suction biopsies were stained for cholinesterases by the method of Karnovsky and Roots (1964) in an attempt to facilitate the diagnosis of Hirschsprung's disease. The method proved at least as reliable as experienced assessment of paraffin haematoxylin-eosin sections, and appeared to offer the advantages of reduced scanning fatigue and superior demonstration of the increased cholinesterase-positive nerves in Hirschprung's disease. Contrary to the findings of Meier-Ruge (1971) it was not possible to base a diagnosis on mucosal cholinesterase activity.

Hirschsprung's disease: an appraisal of histochemically demonstrated acetylcholinesterase activity in suction rectal biopsy specimens as an aid to diagnosis.

Suction rectal biopsy specimens, from a series of 168 infants and children with constipation and other gastrointestinal problems, were stained with a sensitive acetylcholinesterase method, and the results were compared with routine histology, radiology, anorectal manometry, and the final diagnosis. In all cases of Hirschsprung's disease, there was an increase in numbers and sizes of cholinergic nerves in the lamina propria and muscularis mucosae. No false-positive or false-negative results were found. The test was found to be more reliable and consistent than other methods available.

Levatorplasty: A secondary operation for fecal incontinence following primary operation for anorectal agenesis.

A procedure is described which combines the posterior liberation of the levator with the levator plication. This operation was performed in 15 children, born with anorectal agenesis, all of whom were incontinent of feces after primary operation. Four of the 15 children became fully continent following levatorplasty, seven patients now have only occasional soiling, and four patients continue to soil persistently. In all the cases, a good correlation was found between bowel control after levatorplasty and anal canal pressures. Normal resting anal canal pressure was as significant as the voluntary squeeze pressure in relation to continence.

Bilateral retractile testes--subsequent effects on fertility.

A group of 43 adults who as children had been diagnosed as having bilateral retractile testes were traced. Seventy-four per cent of the married patients had children. The testicular volume was normal in all the 19 patients examined.

The internal anal sphincter in translevator (low) anal anomalies.

Internal sphincteric function was studied manometrically in 19 children with translevator anal anomalies. The normal internal sphincter inhibitory response to rectal distension was present in ten pateints, attenuated in six, and absent in three. Our findings suggest that a functional internal sphincter is usually present in translevator anal anomalies.

The results of treatment of anorectal anomalies: a thirteen to twenty year follow-up.

The long-term results of low anomalies are excellent, as expected, whether primarily treated by cutback or transplantation. Those of the high anomalies are perhaps better than expected but only achieved after an unacceptably long period of incontinence. Better techniques are still needed for these supralevator deformities. Permanent colostomy also had its problems.

Spontaneous anastomosis of the esophagus in esophageal atresia.

An operative technique is described for the management of babies with esophageal atresia, where primary anastomosis is either impossible or unsuccessful. The procedure has been performed on six patients. One infant subsequently required resection of a resultant stricture, and one developed a recurrent tracheoesophageal fistula. All six infants required several esophageal dilatations. No patient required dilatation beyond the age of 5 months and all are now swallowing normally and thriving.

Relationship between functional and histologic appearances of developing ganglion cells in the guinea pig rectum.

Time dated fetal guinea-pigs between 47 and 67 days gestation were studied for segmental propulsive activity in the rectosigmoid under fluoroscopy. Large bowel from the sacrificed fetus was studied histologically to determine distribution and morphology of ganglion cells. Contractions were first observed on day 54 and these became more frequent and vigorous after day 63. Between 47-53 days gestation circular muscle is thin and neurons consists of cluster of neuroblastic cells. Near the end of gestation the neurons arrange themselves into discrete groups and have an appearance like mature ganglion cells.

Enterocolitis in Hirschsprung's disease: a controlled study of the etiologic role of Clostridium difficile.

Cytopathic toxin neutralized by Clostridium sordellii antiserum was found in the feces of seven out of 13 children with Hirschsprung's disease complicated by enterocolitis (54%). Clostridium difficile was isolated from ten of these 13 children (77%). The frequency of fecal toxin positivity, the magnitude of toxin titers, and the isolation rate of C difficile were all significantly greater in children with Hirschsprung's enterocolitis than in children whose Hirschsprung's disease was not complicated by enterocolitis or in children without Hirschsprung's disease. It is suggested that C difficile may be causally related to enterocolitis in Hirschsprung's disease, but the age distribution of positive results indicates that the major etiologic role is confined to children under three years of age. Vancomycin was found to be an effective form of treatment in the children with enterocolitis in whom it was employed.

Neuronal colonic dysplasia: an unusual association of Hirschsprung's disease.

A case of neuronal colonic dysplasia in association with Hirschsprung's disease is described. It is suggested that this condition be borne in mind when investigating operated Hirschsprung's cases having residual symptoms.

Megacystis-microcolon-intestinal hypoperistalsis syndrome: a visceral myopathy.

Two newborn infants with megacystis-microcolon-intestinal hypoperistalsis syndrome are described. Review of the literature revealed twenty previously reported cases of this syndrome. Electron microscopic examination of the ileum and urinary bladder showed vacuolar degenerative changes in the smooth muscle cells with abundant amount of connective tissue between the muscle cells. These ultrastructural findings suggest that a degenerative disease of smooth muscle may be the cause of megacystis microcolon intestinal hypoperistalsis syndrome.