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The authors describe a technique for construction of a continent catheterizable stoma from distal defunctionalized colon in a patient with imperforate anus, urethral atresia, and sacral agenesis.
Twenty-nine children under 18 years of age underwent pyloroplasty alone or together with other abdominal operations during a 6-year period. The 21 males and 8 females ranged in age from 2 weeks to 17 years (mean, 54 months). Peptic ulcer disease was the indication for operation in only 6 patients, whereas 16 patients underwent pyloroplasty for functional or mechanical gastric outlet obstruction; 8 had the antral dysmotility syndrome. Other indications included colon interposition in five patients and gastric resection and esophagogastric devascularization in one patient each. Only five patients had concomitant vagotomy. Ten other patients with antral dysmotility syndrome were successfully managed medically. Follow-up ranged from 2 months to 6 years (mean, 30 months). Excellent catch-up weight gain occurred in over 90% of children with functional or mechanical gastric outlet obstruction, with the best results obtained in children with antral dysmotility syndrome. One patient had transitory dumping symptoms following colon interposition with pyloroplasty which remitted with diet changes. Two patients eventually died of the underlying disease (familial dysautonomia, gastric cancer). There were two major complications, respiratory arrest and wound dehiscence, each occurring following emergency operations for peptic ulcer disease. Pyloroplasty was effective in improving gastric emptying and produced minimal morbidity even in the absence of vagotomy. The indications for pyloroplasty in children are different than for adults.
Four groups of patients, 21 neonates with gastrointestinal anomalies, 8 with necrotizing enterocolitis, 5 with low anorectal anomalies and 15 with spina bifida were studied for the development of IgA and IgA subclass immunoglobulin levels. A highly significant increase in IgA levels (p less than 0.001) was seen in neonates with gastrointestinal anomalies and necrotizing enterocolitis while patients with low anorectal anomalies and spina bifida had normal or undetectable IgA levels. Estimation of IgA sub-class distribution in neonates with raised total serum IgA did not demonstrate any striking correlation between either IgA1 or IgA2 sub-classes and the clinical conditions but rather they reflected the level of total IgA. The levels if IgA, correlated closely with those of total IgA (r = 0.93) in all patients implying that in these patients total serum IgA is composed principally of IgA1 immunoglobulin.
PURPOSE: The authors devised computerized equipment for use in the biofeedback therapy in the management of fecal continence after surgery for anorectal malformations. METHODS: The therapy was used for two to eight sessions in 14 children (11 who had high-type anomalies and three who had intermediate-type anomalies). The ages ranged 5 to 14 years. A control group of 17 children, aged 5 to 11 years, who had encopresis, was also treated with the same biofeedback therapy. RESULTS: Clinical improvement was noted in 5 of the 14 (36%) children who had fecal incontinence, and in 15 of the 17 children (88%) who had encopresis. Both in patients who had fecal incontinence and in those who had encopresis, anal resting pressures were not affected by biofeedback therapy. Furthermore, the anal resting pressure in children who had fecal incontinence was significantly lower than that in children who had encopresis. However, anorectal manometry showed that the biofeedback therapy improved voluntary sphincter function and rectal sensation in both groups. CONCLUSION: Biofeedback therapy appears to be effective in most children who have encopresis whose sphincter function is intact, and in some children who have fecal incontinence after surgery for anorectal malformations.
A rare case of a gastrointestinal cystic duplication in a 7-day-old infant is described. The duplication diagnosed antenataly at 25 weeks of gestation was found during surgery to be separated from the gastrointestinal tract, hanging on a vascular pedicle, with no connection to the mesentery. The duplication was excised, and postoperative follow-up of 14 months was uneventful. The possible pathogenesis of this malformation is discussed.
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A population of 80 home-reared children with cri du chat syndrome was investigated to document the clinical heterogeneity of the syndrome and to analyze the factors influencing the severity of the phenotypic characteristics. When individuals with isolated deletions were compared with those possessing unbalanced translocations involving other chromosomes in addition to number 5, the latter group had a greater incidence of physical anomalies, more frequent hospitalizations, and a higher mortality. Chronic complaints in both groups included upper respiratory tract infection, otitis media, and a previously unrecognized association with gastrointestinal tract anomalies. In children with terminal deletions, there was a significant negative correlation between the size of the deletion and the individual's intelligence quotient. In addition, patients with larger deletions had more severe growth retardation, particularly with respect to the degree of microcephaly. The gradual progression with age of the characteristic facial features remained consistent regardless of differing racial backgrounds and the size of the deletion. Our findings delineate the variation in the clinical and karyotypic features of this syndrome.
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