Reoperation in an adult female with "right-sided" Hirschsprung's disease complicated by refractory hypertension and cough

Abstract

Hirschsprung’s disease (HD) is an intestinal malformation caused by the innate absence of ganglion cells in the neural plexus of the colorectal wall, and is most common in male infants. It is rare in adult, and is usually left-sided. Herein we reported based on the CARE guidelines a case of a 47-year-old adult female suffering from “right-sided” HD complicated by refractory hypertension and cough. The patient with a history of cesarean section and with digestive unfitness (abdominal pain, distention, and constipation) only since 20 years old had recurrence of HD after initial surgery due to the incomplete removal of the HD-affected bowel based on a diagnosis of “chronic ileus”, leading to the relapse of the digestive symptoms and the emergence of some intractable circulatory and respiratory complications which could be hardly controlled by conservative treatment. During the long interval before coming to our department for help, she had been re-hospitalized for several times with various misdiagnoses and supplied merely with symptomatic treatment which could only achieve temporary symptomatic relief. At her admission to our department, the imaging examinations strongly indicated recurrent HD which was further supported by pathological examinations, and right hemi-colectomy was performed to remove the remnant aganglionic intestinal segment. Intraoperative and postoperative pathology supported the completeness of the definitive resection. Post-operation, the patient’s bowel motility significantly improved, and interestingly, the complications disappeared. For adult patients with long-term constipation combined with cough and hypertension, rare diseases like HD which requires definite surgery and which could be “right-sided” should not be overlooked. It is vital to diagnose and cure HD patients in childhood. Through the comparison of the two surgeries, it is noteworthy that for diagnosed HD, sufficient removal of the non-functional intestine confirmed by intraoperative pathology is essential.

Keywords: Adult Hirschsprung’s disease, Reoperation, Ileus, Chronic constipation, Hypertension, Cough, CARE

Core tip: Hirschsprung’s disease (HD) is most common in male infant, and is usually left-sided. Herein we reported a case of a 47-year-old adult female suffering from “right-sided” recurrent HD complicated by refractory hypertension and cough and receiving reoperation. The patient with digestive unfitnesses only since 20 years old had recurrence of HD after the initial surgery due to the incomplete removal of the HD-affected bowel. At her admission to our department, right hemi-colectomy was performed to completely remove the remnant aganglionic intestinal segment, which was confirmed by intraoperative pathology. Post-operation, the patient’s bowel motility significantly improved, and interestingly, the complications disappeared.