تظاهرات باليني هيرشپرونگ در يك دوره شش ساله در شهر رشت

Clinical manifestations of Hirschsprungʹs disease:A 6-year course review in Rasht

Background&Objective: Hirschsprungʹs 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 length of bowel may be involved. Our aim was to study Hirschsprungʹs clinical presentations and its rate of intestinal involvement in hospitalized patients in a 6-year course study in Rasht. The capital city of Gillan province in north of Iran. Materials&Methods: We studied the Hirschsprungʹs patients referred to Poursina Referal Hospital between 1995-2001.A cross sectional descriptive- analytic study whose data was collected from patientʹs files and questionnaires including demographic data, disease presentations, diagnostic methods, involved segment, surgical procedure types, surgical complications. Finally, the data was analyzed in SPSS10 software. Results: We detected 58 H.D in these 6 years, whome underwent surgery. There were 19 females and 39 males. Age of patients differed from one day to 18 year. Clinical findings were variable as follow: constipation (79.31%), abdominal distention (67.24%), inability to pass mecunium (17.24%), diarrhea (5.17%) and other less common manifestations. Pathologically, we divided our patients into 3 kinds: rectosigmoid, ultra short - segment, total - colonic. Surgeries were performed in three ways as follow Swenson - Soave - Lynn. Early complications, which come within one month after operation, contain anastomotic leakage (10.2%), prolonged constipation (10.2%). There were not any significant differences between these three types of surgical procedures. Conclusion: Majority of patients presented with a chronic course of constipation and abdominal distention. There wasnʹt any difference between kinds of involvement and age and type of presentations. There are few associated anomalies in our patients. In some cases, surgical complications were less than other studies; so, it is probable that our procedures had fewer complications or they had performed in appropriate time.