ABSTRACT
Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III.
Subject(s)
Child , Female , Humans , Infant , Arnold-Chiari Malformation , Blindness, Cortical , Brain , Cerebellum , Deglutition , Developmental Disabilities , Encephalocele , Head , Prognosis , Rehabilitation , Twins , Visual PerceptionABSTRACT
Chiari malformations are brain stem anomalies with or without spinal canal involvement. Type III is the rarest of the Chiari malformations and is characterized by a low occipital or high cervical encephalocele in combination with multiple cerebellar and brain stem anomalies. We report a case of Chiari III malformation of a newborn, who underwent repair of occipital meningoencephalocele and endoscopic third ventriculostomy. We present the case with a review of related literature.