Open spinal dysraphism with a posterior meningocele

Open spinal dysraphism with a posterior meningocele is a rare congenital neural tube defect in which the spinal canal fails to close properly during embryonic development, resulting in a sac-like protrusion (meningocele) through a defect in the posterior vertebral arches. The meningocele contains cerebrospinal fluid and meninges (the protective membranes surrounding the spinal cord) but typically does not contain neural tissue (spinal cord or nerve roots) within the herniated sac. Because this is classified as an open dysraphism, the defect is exposed or covered only by a thin membrane rather than intact skin, which distinguishes it from closed spinal dysraphisms. The condition is apparent at birth and primarily affects the central nervous system and musculoskeletal system. Clinical features may include a visible cystic mass along the posterior midline of the back, and depending on the level and extent of the defect, patients may experience varying degrees of neurological impairment. Because neural elements are generally not displaced into the sac, neurological deficits tend to be less severe than in myelomeningocele; however, complications such as cerebrospinal fluid leakage, risk of meningitis, tethered cord, and hydrocephalus may still occur. Orthopedic abnormalities and bladder or bowel dysfunction can also be present in some cases. Treatment is primarily surgical, with early postnatal repair of the defect to prevent infection, protect neural structures, and restore the integrity of the spinal covering. Surgical closure is typically performed within the first days of life, especially in open defects where the risk of central nervous system infection is high. Long-term management may involve multidisciplinary follow-up including neurosurgery, urology, orthopedics, and rehabilitation to monitor for complications such as tethered cord syndrome or hydrocephalus. Prenatal folic acid supplementation is a well-established preventive measure that reduces the overall risk of neural tube defects.

Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

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