Supernumerary Intestinal Muscle Coat in a Patient with Hirschsprung Disease/Mowat-Wilson Syndrome
Supernumerary Intestinal Muscle Coat in a Patient with Hirschsprung Disease/Mowat-Wilson Syndrome
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DOI：
10.2350/09-09-0715-cr.1
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发表时间：
2010-09-01
影响因子：
1.9
通讯作者：
Ramani, Pramila
中科院分区：
文献类型：
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作者：
Leong, May;Verey, Frances;Ramani, Pramila
我们提出了第一个关于患有 Mowat-Wilson 综合征和先天性巨结肠症的患者出现额外肠平滑肌层的病例报告。患者5个月大时切除无神经节结肠后，最初出现间歇性便秘，随后到5岁时出现持续性腹泻，需要治疗十多年。尽管该病例中肠道运动异常的确切机制尚不清楚，但我们推测，多余的肌肉及其相关的神经丛可能是导致该患者在先天性巨结肠治疗中出现不寻常的晚期并发症的原因。
We present the 1st case report of an additional enteric smooth muscle layer in a patient with Mowat-Wilson syndrome and Hirschsprung disease. after resection of the aganglionic colon at the age of 5 months, our patient initially suffered from intermittent constipation, and subsequently by the age of 5 years, he developed ongoing diarrhea requiring medical treatment for more than a decade. although the exact mechanism of abnormal gut motility in this case is unknown, we postulate that the supernumerary muscle and its associated neural plexus may be responsible for the patient's unusual late complication in treated Hirschsprung disease.