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Abstract：
Adult Hirschsprung\'s disease (AHD) is a rare condition characterized by a shortened aganglionic segment in the distal colon or rectum that is diagnosed after the age of 10. Diagnostic challenges stem from its rarity, nonspecific presentation, and often delayed consideration following emergent interventions. This report details the case of a 33-year-old male who presented with chronic constipation and abdominal pain, leading to a severe bowel obstruction attributed to self-reported Hirschsprung\'s disease (HD). Clinical, radiological, and historical aspects were suggestive of AHD, but definitive diagnostic procedures, including manometry and biopsy, were hindered by the patient\'s deteriorating condition. Exploratory laparotomy unveiled a secondary small bowel obstruction due to volvulus, necessitating immediate intervention, resulting in the removal of 4000 cc of fecal material. A comprehensive resection involving mid-to-distal transverse colon, left colon, sigmoid colon, and proximal rectum with the creation of Hartman\'s colostomy was performed due to the patient\'s worsening clinical status. We present a case of possible ultrashort-segment Hirschsprung\'s disease (USHD) and sketch a classic presentation of AHD. This endeavor aims to enhance awareness and consideration of AHD and/or USHD within the spectrum of potential diagnoses for chronic constipation when relevant and demonstrate the effectiveness of surgical intervention in this population.
摘要：
成人先天性巨结肠病（AHD）是一种罕见的疾病，其特征是在10岁后诊断为远端结肠或直肠的神经节段缩短。诊断挑战源于它的稀有性，非特异性演示，并经常在紧急干预后推迟考虑。本报告详述了一名33岁男性慢性便秘和腹痛的病例，导致严重的肠梗阻归因于自我报告的先天性巨结肠病（HD）。临床,放射学,历史方面暗示了AHD，但明确的诊断程序,包括测压和活检,受到病人病情恶化的阻碍。剖腹探查术揭示了由于肠扭转引起的继发性小肠梗阻，需要立即干预，导致去除4000cc的粪便。涉及中到远端横结肠的全面切除，左结肠,乙状结肠,由于患者的临床状况恶化，进行了Hartman结肠造口术的近端直肠。我们介绍了可能的超短节段Hirschsprung病（USHD）的病例，并概述了AHD的经典表现。这项努力旨在提高对AHD和/或USHD在慢性便秘的潜在诊断范围内的认识和考虑，并证明手术干预在该人群中的有效性。
