LAUSR

We present the clinical history of a 20-year-old woman who developed calvarial hyperostosis and hemihyperplasia-induced scoliosis since her infancy. By the age of 7 she was found to have ipselateral ocular hamartoma and otomastoid cholesteatoma; and at the age of 10 she became symptomatic for hydrocephalus secondary to non-tumoural choroid plexus cysts, requiring endoscopic exploration and insertion of a ventriculo-peritoneal shunt. The successful management of her hydrocephalus unfortunately aggravated the progression of her calvarial hyperostosis, leading to a deterioration of her already impaired visual acuity due to a persistently raised intracranial pressure, as noted during a 48-h trial of ICP (intracranial pressure) monitoring. After discussion in the craniofacial MDT, a fronto-orbital advancement was performed. This intervention stabilized the intracranial pressure, improved the visual disturbances and was accompanied by satisfactory cosmetic results. Despite a degree of mild mental retardation, the patient never suffered epilepsy, developed normally and attended mainstream school. She had yearly follow ups with neurosurgery, neurology, psychology, ENT and orthopaedic surgeons till her transition to adult care Fig. 1.