PDF(Pubmed)
Abstract：
Hirschprung\'s disease is a congenital disorder characterized by aganglionic bowel, usually diagnosed in infancy. Here, we present a unique case of Hirschprung\'s disease diagnosed in a 29-year-old female with acute on chronic constipation. As part of her work up, a computerized tomography of her abdomen and pelvis revealed large, bilateral dermoid cysts. A diagnostic and therapeutic colonoscopy allowed manual disimpaction and decompression of her bowel, as well as biopsy attainment. Histopathology revealed absence of ganglionic cells on haematoxylin and eosin stain and calretinin immunostaining. This case underscores the diagnostic challenges of Adult Hirschprung\'s disease and how this impacts patient quality of life, as well as the work up and management of concurrent causes abdominopelvic conditions.
摘要：
Hirschprung病是一种先天性疾病，其特征是神经节肠，通常在婴儿期被诊断。这里,我们介绍了一个独特的Hirschprung病病例，诊断为一名29岁女性急性慢性便秘。作为她工作的一部分，她腹部和骨盆的计算机断层扫描显示很大，双侧皮样囊肿。诊断和治疗性结肠镜检查允许对她的肠道进行手动减压和减压，以及活检。组织病理学显示苏木精和曙红染色和钙视网膜素免疫染色上没有神经节细胞。该病例强调了成人赫希普伦病的诊断挑战，以及这如何影响患者的生活质量，以及并发原因腹骨盆疾病的工作和管理。
