FP1068 : Horizontal gaze palsy with progressive scoliosis (HGPPS) : Clinical features and surgical management

Dr.Aaditi Anilkumar, A21030

Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disorder believed to result from cranial nuclear maldevelopment. It is characterized by the absence of conjugate horizontal eye movements, normal vertical gaze and convergence and progressive scoliosis. The MRI findings show midline pontine cleft, butterfly configuration of the medulla, brain stem hypoplasia, and absence of facial colliculi. Simultaneous adduction occurs when there is adduction of both eyes on attempted lateral gaze. We present a case of HGPPS with simultaneous adduction with hyperopic refractive error and esotropia in primary position. She underwent bimedial recession for the esotropia and is doing well post operatively. She also had dental hypoplasia, regressing haemangiomas on her forearms and hyperextensibility of hip and knee joints. The above systemic associations have not been documented so far with HGPPS.