1型神经纤维瘤病患儿的小肠系膜神经纤维瘤病

Neurofibromatosis of the small intestine mesentery in a child with neurofibromatosis type 1.

作者信息

Frawley K J, Cohen R, O'Loughlin E V, North K, Arbuckle S

机构信息

Department of Radiology, Royal Alexandra Hospital for Children, Parramatta, New South Wales, Australia.

出版信息

J Pediatr Surg. 1997 Dec;32(12):1783-5. doi: 10.1016/s0022-3468(97)90535-2.

DOI:10.1016/s0022-3468(97)90535-2

PMID:9434028

Abstract

The authors present a case of mesenteric neurofibromatosis in a child, which is a rare complication of neurofibromatosis type 1 (NF1). This case is presented to illustrate the clinical features and the radiological appearance of mesenteric neurofibromatosis, which, in the appropriate clinical setting of NF1, are diagnostic. The authors also discuss the long-term complications of this condition, which includes the potential risk of malignant degeneration.

摘要

作者报告了一例儿童肠系膜神经纤维瘤病，这是1型神经纤维瘤病（NF1）的一种罕见并发症。呈现该病例是为了说明肠系膜神经纤维瘤病的临床特征和影像学表现，在NF1的适当临床背景下，这些特征具有诊断意义。作者还讨论了这种疾病的长期并发症，包括恶性变的潜在风险。