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   <subfield code="a">Improvements in the management of total colonic aganglionosis</subfield>
   <subfield code="h">[Elektronische Daten]</subfield>
   <subfield code="c">[B. Ratta, E. Kiely, L. Spitz, R. Brereton]</subfield>
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   <subfield code="a">Children with total colonic aganglionosis pass through three critical phases, the first extending from birth to the establishment of the correct diagnosis with construction of a ganglionic stoma. The second phase lasts from the raising of the stoma to its closure, including the definitive surgical procedure. The third phase begins with closure of the stoma and ends in a gradual decrease in the frequency of defaecation together with the acquisition of continence. The three phases have been analysed in two groups of patients; group I presented between 1978 and 1982 (n = 23) and group II between 1983 and 1987 (n = 17). In the first phase, 9 children in each group underwent inappropriate surgery. The mortality in the second phase was 26% in group I and 6% in group II and in the third phase 6% and 0% respectively. Lack of awareness of the condition and a reluctance to transfer neonates with intestinal obstruction to specialised centres led to delay in diagnosis and inappropriate treatment in both groups. In the second phase, improvements in results were due to increased use of parenteral nutrition, careful monitoring and replacement of stomal electrolyte losses, and improved management of enterocolitis. During the third phase, nocturnal incontinence remained a problem.</subfield>
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   <subfield code="u">The Hospitals for Sick Children, Great Ormond Street, London, UK</subfield>
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