Fatal Cerebral Swelling Associated with Scoliosis Surgery in Two Patients with Neuromuscular Scoliosis and Congenital Hydrocephalus: A Report of Two Cases.

Abstract

Patients with progressive neuromuscular scoliosis often undergo spinal fusion to prevent progression, improve sitting balance, and preserve pulmonary function1,2. These patients often have abnormalities in cerebrospinal fluid outflow, resulting in hydrocephalus and requiring ventricular shunting3. They also can have tethering of the spinal cord, which can restrict spinal cord excursion during correction of sagittal plane deformities4-6. These factors can predispose patients to cerebral swelling and brain injury during scoliosis surgery. We present two patients with neuromuscular scoliosis and ventriculoperitoneal (VP) shunts who developed acute, fatal cerebral swelling following spinal surgery. The Hospital for Special Surgery institutional review board approved waivers for both consent and HIPAA privacy rules since the patients met the appropriate criteria. Case 1. An eleven-year-old girl presented with progressive neuromuscular scoliosis. The patient’s congenital hydrocephalus had been managed with a VP shunt when she was two years old. A tethered cord release had been performed when she was five years old. At the age of eleven years, she presented with limited walking ability, inability to hold a standing position, and diminishing pulmonary function. A right thoracolumbar prominence was grossly evident on examination (Figs. 1-A and 1-B). Progressive lower-extremity weakness correlated with the increasing sagittal plane spinal deformity and may have been related to increasing compression of the conus medullaris or spinal cord over the apex of the thoracolumbar kyphosis. Fig. 1 Clinical photographs ( Figs. 1-A and 1-B) demonstrating spinal deformity (Case 1). Radiographs demonstrated a long right thoracolumbar scoliosis extending from T4 to L4 with a Cobb angle of 40° (Fig. 2-A). There was reversal of both normal thoracic kyphosis and lumbar lordosis with Cobb angle measurements of 20° of lordosis from T2 to T12, 28° of kyphosis from L1 to S1, and 57° of kyphosis at the …