Intraspinal anomalies: presentation, assessment, treatment and association with the development of scoliosis

Abstract The most common congenital intraspinal abnormalities presenting to medical attention is either a Chiari malformation with or without associated syringomyelia or caudal anomalies, such as tethered cord and fatty filum terminale. These intraspinal defects are usually diagnosed at birth or in early childhood, but they may also be identified in adult life due to newly presented neurological symptoms. Availability and use of appropriate imaging modalities, such as MRI and regular fetal screening has reduced the incidence of delayed diagnosis of spinal dysraphism and other accompanying congenital systemic anomalies. In view of the risk for neurological complications, prophylactic surgery is often recommended in spinal dysraphism; hence knowledge of various intraspinal abnormalities, their clinical presentation and natural history will allow early diagnosis and treatment in order to prevent neurological deterioration and irreversible morbidity. This review summarizes the embryological aspects and clinical manifestations of intraspinal abnormalities and provides a rational approach for evaluation and management in this group of patients.