Foundations of Myelomeningocele Management: History, Embryology, Diagnosis, and Treatment of Myelomeningoceles Through the Sheffield Study.

Abstract

Myelomeningocele is a common neural tube defect caused by failure of the closure of the posterior neuropore during neurulation. This defect, described as a neural placode (flattened neural tissue), manifests as an open spinal lesion most frequently found in the thoracolumbar region. Throughout history, clinicians have worked to better understand this condition, leading to a stepwise increase in understanding of the disease. Complications associated with myelomeningocele include cognitive and functional neurological deficits, hydrocephalus, Chiari II malformation, bladder dysfunction, meningitis, and often death. Historically, management of myelomeningocele evolved from a largely palliative approach to more aggressive surgical interventions aimed at improving quality of life and reducing morbidity. Early surgical repair within 24-48 hours after delivery remains the standard of care, although in utero fetal surgery is becoming increasingly commonplace. This paper reviews the origins of the early management of myelomeningocele and the advances that have shaped our modern day understanding of this disease.