López Ruiz José Antonio, Tallón Aguilar Luis, Sánchez Moreno Laura, López Pérez José, Pareja Ciuró Felipe, Oliva Mompeán Fernando, Padillo Ruiz F Javier
Cirugía General y del Aparato Digestivo, HU Virgen Macarena, España.
UGC Cirugía General y Aparato Digestivo, H. U. Virgen Macarena, España.
Rev Esp Enferm Dig. 2016 Nov;108(11):742-746. doi: 10.17235/reed.2016.3841/2015.
Hirschsprung's disease is characterized by absence of ganglion cells in submucosal and myenteric plexus of distal bowel. Most cases become manifest during the neonatal period, but in rare instances, this disease is initially diagnosed in adult age. It usually presents as severe constipation with colonic dilatation proximal to the aganglionic segment. The treatment is surgical, removing the aganglionic segment and restoring continuity of digestive tract. The disease rarely presents as an acute intestinal obstruction. We report a case not previously diagnosed, which presented as a massive colonic dilatation with a maximum diameter of 44 cm, with imminent risk of drilling that forced to perform an emergency surgery. We include a review of existing literature.
先天性巨结肠的特征是远端肠段黏膜下和肌间神经丛中缺乏神经节细胞。大多数病例在新生儿期出现，但在罕见情况下，这种疾病最初在成年期被诊断出来。它通常表现为严重便秘，伴有无神经节段近端的结肠扩张。治疗方法是手术，切除无神经节段并恢复消化道的连续性。这种疾病很少表现为急性肠梗阻。我们报告一例此前未被诊断的病例，该病例表现为最大直径达44厘米的巨大结肠扩张，存在即将穿孔的风险，因此被迫进行急诊手术。我们还纳入了对现有文献的综述。
