Sethi Priyanka
Department of Anaesthesia and Critical Care, SNMC, Jodhpur, India.
J Coll Physicians Surg Pak. 2015 Oct;25 Suppl 2:S73-5. doi: 10.2015/JCPSP.S73S75.
Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome which is characterized by formation of neurofibromas all over the skin and various other body systems. The anaesthetic management of these patients requires attention to all possible abnormalities and associated disturbances to prevent any peri-operative complication. NF-1-associated complications of the musculoskeletal, respiratory, cardiovascular, central nervous, and gastrointestinal and genitourinary systems present with various degrees of considerations for anaesthesiologists. While evaluating and managing these patients for surgical procedures. NF-1 or von Recklinghausen disease, is an autosomal dominant disease with incidence of approximately 1/3000 births. Gastrointestinal (GI) neoplasms are present in about 2 - 25% of patients of NF-1. In these patients, neurofibromas are the most frequently (52%) diagnosed benign neoplasms. Patients with NF-1 and GI symptoms are at risk for gastrointestinal neoplasms; symptomatic patients are likely to experience significant morbidity. This report describes the anaesthetic of a patient with NF-1 and gastric outlet obstruction.
1型神经纤维瘤病（NF-1）是一种常染色体显性遗传的神经皮肤综合征，其特征是在全身皮肤和各种其他身体系统中形成神经纤维瘤。对这些患者的麻醉管理需要关注所有可能的异常情况和相关紊乱，以预防任何围手术期并发症。NF-1相关的肌肉骨骼、呼吸、心血管、中枢神经以及胃肠道和泌尿生殖系统并发症给麻醉医生带来了不同程度的考虑因素。在评估和管理这些患者进行外科手术时。NF-1或冯·雷克林豪森病是一种常染色体显性疾病，发病率约为1/3000活产儿。胃肠道（GI）肿瘤在约2%-25%的NF-1患者中存在。在这些患者中，神经纤维瘤是最常被诊断出的良性肿瘤（52%）。患有NF-1和胃肠道症状的患者有患胃肠道肿瘤的风险；有症状的患者可能会出现严重的发病率。本报告描述了一名患有NF-1和胃出口梗阻患者的麻醉情况。
