Harlianto Netanja I, Mohamed Hoesein Firdaus A A, de Jong Pim A, Verlaan Jorrit-Jan, Westerink Jan
Department of Orthopedic Surgery, University Medical Centre Utrecht, Utrecht, the Netherlands.
Department of Radiology, University Medical Centre Utrecht, Utrecht, the Netherlands.
Bone Rep. 2021 Jul 27;15:101111. doi: 10.1016/j.bonr.2021.101111. eCollection 2021 Dec.
Dysphagia due to extensive ossification at anterior segments of the cervical spine is a rare occurrence and is usually attributable to diffuse idiopathic skeletal hyperostosis (DISH). We present the case of a 74-year-old female with dysphagia most likely due to ossification in pseudohypoparathyroidism type 1a (PHP1a). PHP1a is a rare, autosomal dominant disorder caused by mutations in the GNAS1 gene. Our patient had characteristic phenotype features of PHP1a, also known as Albright's hereditary osteodystrophy (AHO), which was diagnosed without genetic confirmation. She was conservatively treated with dietary measures and observation, and reported persisting symptoms of dysphagia at six-month follow-up. This is the first case to describe dysphagia in PHP1a with a similar presentation to DISH.
颈椎前段广泛骨化导致的吞咽困难较为罕见,通常归因于弥漫性特发性骨肥厚(DISH)。我们报告了一例74岁女性,其吞咽困难很可能是由1a型假性甲状旁腺功能减退症(PHP1a)中的骨化所致。PHP1a是一种罕见的常染色体显性疾病,由GNAS1基因突变引起。我们的患者具有PHP1a的典型表型特征,也称为奥尔布赖特遗传性骨营养不良(AHO),在未进行基因确认的情况下被诊断出来。她接受了饮食措施和观察的保守治疗,在六个月的随访中报告吞咽困难症状持续存在。这是第一例描述PHP1a中吞咽困难且表现与DISH相似的病例。