Clinical manifestations of Hirschsprung＊s disease: A 6-year course review on admitted patients in Guilan, north Province of Iran

作者：M Izadi; F Mansour Ghanaei; R Jafarshad; F Joukar

来源：Iranian Cardiovascular Research Journal, 2007.

• Hirschsprung&rsquo
• s disease
• Abdominal distension
• Anomalies

Background: Hirschsprung＊s disease (H.D) is a congenital disease in which intestinal ganglionic cells are absent and can cause intestinal obstruction. The disease has various clinical manifestations and different lengths of bowel may be involved. Our aim was to study Hirschsprung＊s clinical presentations and its rate of intestinal involvement in hospitalized patients in a 6-year course study in Guilan, north Province of Iran.Methods: We studied the patients with Hirschsprung＊s referred to Guilan surgery referral center, Poursina Hospital, from 1995 to 2001. In this cross sectional descriptive- analytic study all data were collected from patients＊ files and questionnaires including demographic data, clinical presentations, diagnostic methods, involved segments, surgical procedure techniques, surgical complicationsResults: We detected 58 cases of HD during these 6 years, who underwent surgery. 19 patients were female and 39 were male with age range from one day to 18 years old. Clinical findings were variable such as: constipation,abdominal distension, failure to pass mecunium, diarrhea and other less common manifestations. We found 3 different pathological types: rectosigmoid, ultra short-segment and total每colonic involved segment.There wasn＊t any difference between presentations of disease in these 3 groups. Surgical procedures which were performed were: Swenson, Soave每Boley and Lynn. Early complications, which occurred duringthe first month after operation, contain anastomotic leakage, wound infection, hemorrhage and stenosis of anastomosis. Late complications, occurring at least one month after operation, comprise long-term constipation,enterocolitis, incontinence, obstruction due to adhesion. We didn＊t find any significant difference between the complications of these surgical procedures and any accompanying anomalies except 3 ones.Conclusions: most of patients had a chronic course of constipation and abdominal distension. without any difference between types of involvements and age and clinical presentations. There are few associated anomalies in the patients. In some cases, surgical complications were less than those in other studies; so, it seems that the performed procedures had fewer complications or they may have been performed in appropriatetime.