Gosavi Kundan S, Mundada Surbhi D
Department of Anaesthesia and Critical Care, Grant Medical College and Sir. J. J. Groups of Hospitals, Mumbai, Maharashtra, India.
Indian J Anaesth. 2012 Jul;56(4):394-6. doi: 10.4103/0019-5049.100833.
Gorlin-Goltz syndrome is a rare autosomal-dominant syndrome related to mutation in "Patched" tumour suppressor gene on chromosome 9. Basocellular carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri are its major features, along with more than 100 minor features. Odontogenic cysts, notorious for recurrence, can make endotracheal intubation difficult, requiring modification of the standard intubation technique. We report such a case managed successfully by awake fibreoptic intubation. Direct laryngoscopy under anaesthesia later confirmed that it was a good decision.
戈林-戈尔茨综合征是一种罕见的常染色体显性综合征，与9号染色体上“patched”肿瘤抑制基因的突变有关。基底细胞癌、牙源性角化囊肿、掌跖凹陷和大脑镰异位钙化是其主要特征，还有100多种次要特征。以复发而闻名的牙源性囊肿会使气管插管困难，需要对标准插管技术进行调整。我们报告了一例通过清醒纤维喉镜插管成功处理的病例。后来在麻醉下进行直接喉镜检查证实这是一个明智的决定。
