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.

译文

Hirschsprung病 (HD) 是由于结肠直肠壁神经丛中神经节细胞先天缺失而引起的肠道畸形,最常见于男婴。在成人中很少见,通常为左侧。在此,我们根据护理指南报告了一例47岁的成年女性患有 “右侧” HD并伴有难治性高血压和咳嗽的病例。自20岁起才有剖宫产史且消化功能不健康 (腹痛,扩张和便秘) 的患者,由于诊断为 “慢性肠梗阻”,HD受影响的肠道未完全切除,因此在初次手术后复发了HD。导致消化系统症状复发,并出现一些顽固性循环和呼吸系统并发症,保守治疗难以控制。在来我们部门寻求帮助之前的很长一段时间内,她因各种误诊而再次住院了几次,仅提供对症治疗,只能暂时缓解症状。在她入院时,影像学检查强烈提示复发性HD,并得到病理检查的进一步支持,并进行了右半结肠切除术以去除残留的神经节肠段。术中和术后病理支持确定性切除的完整性。术后,患者的肠蠕动明显改善,有趣的是,并发症消失了。对于长期便秘合并咳嗽和高血压的成人患者,HD等罕见疾病需要明确手术且可能 “右侧” 的疾病不容忽视。诊断和治疗儿童时期的HD患者至关重要。通过两种手术的比较,值得注意的是,对于确诊的HD,术中病理证实的无功能肠的充分切除至关重要。

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