Clinical Manifestations of Hirschsprung’s Disease: A Six Year Course Review of Admitted Patients in Gilan, Northern Iran

Abstract

 Background

Hirschsprung’s disease (HD) is a congenital disease in which intes­tinal ganglionic cells are absent, leading to 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 six year course study in Gilan, a northern province of Iran.

Methods

We studied patients diagnosed with HD who were referred to the Gilan Surgery Referral Center in Poursina Hospital from 1995 until 2001. In this cross sectional descriptive-analytical study, all data were collected from patients’ files and questionnaires. Data included demographics, clinical presentations, diagnostic methods, involved intestinal segments, type of surgical procedure, and surgical complications.

Results

There were 58 cases of HD during this six year period that underwent surgery. Of these, 19 patients were female and 39 were male whose ages ranged from one day to 18 years old. A variety of clinical find­ings such as: constipation, abdominal distension, failure to pass me­conium, diarrhea, and other less common manifestations were pres­ent. We found three different pathological types: rectosigmoid, ultra short segment and total colonic involved segment. No differences between disease presentations were discovered among these three types. The following surgical procedures were performed: Swenson, Soave–Boley, and Lynn. Early complications, which occurred during the first month following surgery, were anastomotic leakage, wound infection, hemorrhage and anastomosis stenosis. Late complications, occurring at least one month after surgery, comprised the following: long-term con­stipation, enterocolitis, incontinence, and obstruction due to adhesion. No significant difference between the complications of these surgical procedures and any accompanying anomalies were found.

Conclusion

Most patients had chronic constipation and abdominal distension regardless of the types of involvement, age or clinical presentations. There were few associated anomalies amongst the patients. In some cases, surgical complications were less than those seen in other studies; therefore, it seemed that the performed procedures had fewer complica­tions or may have been performed at the appropriate time.