Kamal Geeta, Gupta Anju, Batla Sapna, Gupta Nishkarsh
Department of Anaesthesia, Chacha Nehru Bal Chikitsalaya, New Delhi, India.
Department of Onco.Anaesthesia and Palliative Medicine, Dr. BRAIRCH, AIIMS, New Delhi, India.
Indian J Anaesth. 2017 Mar;61(3):266-268. doi: 10.4103/0019-5049.202168.
Stone Man syndrome or fibrodysplasia ossificans progressiva (FOP) is an extremely rare (1 in 2 million) genetic disorder characterised by ectopic ossification of the skeletal and connective tissues leading to progressive fusion of axial and appendicular skeleton. Surgery and anaesthesia-induced trauma can lead to disease flare-up if due precautions are not taken and disable the patient further. However, rarity of the disease may lead to its common misdiagnosis and anaesthesiologist may be caught unaware. There is relative paucity of literature regarding anaesthetic management of children with FOP. Videolaryngoscopes (VLs) provide a non-line-of-sight view and require less anterior force to visualise the glottis, may provide an alternative to fibreoptic intubation for airway management in such cases. Use of VL has only been reported once in an adult with FOP for nasotracheal intubation. We describe the successful anaesthetic management of an 11-year-old child with FOP and anticipated difficult airway.
石人综合征或进行性骨化性纤维发育不良(FOP)是一种极其罕见(200万分之一)的遗传性疾病,其特征是骨骼和结缔组织异位骨化,导致中轴骨和附属骨骼逐渐融合。如果不采取适当的预防措施,手术和麻醉引起的创伤会导致疾病发作,并使患者进一步致残。然而,这种疾病的罕见性可能导致其被误诊,麻醉医生可能会毫无防备。关于FOP患儿麻醉管理的文献相对较少。视频喉镜(VLs)提供非直视视野,可视化声门所需的向前用力较小,在这种情况下可能为气道管理提供纤维支气管镜插管的替代方法。VL在成人FOP患者中行鼻气管插管的应用仅有一次报道。我们描述了一名11岁FOP患儿并预计气道困难的成功麻醉管理。