RETROSPECTIVE STUDY OF 53 CHILDREN WITH HIRSCHSPRUNGS DISEASE: CLINICAL AND LABORATORIAL FINDINGS

Abstract

Objective:

to analyze some clinical and laboratory characteristics in children with Hirschsprung.s disease. Methods: a retrospective study of all 53 children with Hirschsprung.s disease attended at the University Hospital, Faculty of Medicine of Ribeirão Preto, USP, from January 1981 to December 1999. Some clinical parameters were investigated at the first visit (patients distribution by gender, age at onset of signs and symptoms, at the first visit and at the time of diagnosis, main signs and symptoms, and nutritional status), as well as laboratory tests that permitted the diagnosis (some characteristics observed by barium enema, anorectal manometry and rectal biopsy) and the evolution of the patients after surgical treatment. Results: 42 children (79.2%) were boys. The first signs and symptoms occurred during the first month of life in 43 cases (81.1%), the average age at the first visit was 7 months and age at the time of diagnosis was 8.8 months. The main clinical manifestations were: abdominal distension in 83.0%, intestinal constipation in 73.6%, delayed passage of meconium in 60.7%. No important deficit in weight and height was observed. A barium enema showed a transition zone in 47.2% (17/36) and anorectal manometry revealed the absence of the relaxation reflex of the internal anal sphincter in 73.7% (14/19). All anorectal biopsies demonstrated aganglionosis. One child was submitted to clinical treatment and 52 to surgical treatment. Colostomy was performed in 22/52 children (42.3%) and definitive surgical treatment in 49 (Duhamel Haddad surgery in 77.6%, Soave surgery in 20.4% and Lestar Martin surgery in 2.0%). The most frequent complications before surgery were intestinal obstruction in 28.3% and enterocolitis in 11.3%. The major complications after colostomy were eventration and/or prolapse and/or stenosis in 22.7%, and the major complications after definitive surgical treatment were soiling and/ or fecal incontinence in 28.6%, infection and/or dehiscence of the surgical wound in 22.4%, and enterocolitis and/or diarrhea in 20.0%.Three children were not submitted to surgical treatment. Ten (18.9%) received ambulatory discharge, 14 (26.0%) are still being followed up and 27 (50.9%) abandoned the service. There was a death (1.9%) by enterocolitis some time after definitive surgical treatment and one child (1.9%) is still being followed up in another service, after reconstruction of the transit. Conclusions: there was a discrepancy between the age at onset of symptoms and the age at diagnosis, demonstrating a delay in patient referral for diagnostic confirmation. The late diagnosis probably determined the high percentage of presurgical complications of Hirschsprung.s disease, i.e.,: intestinal obstruction (28.3%) and enterocolitis (11.3%) which are the most frequent and continue to be the major causes of morbidity and mortality in this disease.