Myelomeningocele – Assoc anomalies


A Myelomeningocele results from an abnormality in fusion of the embryologic neural tube during the first month of gestation. The incidence is approximately 1 per 1000. Failure of neural tube closure results in a sac-like herniation of the meninges (meningocele) or a herniation of neural elements (myelomeningocele). Myelomeningoceles most commonly occur in the lumbosacral area, but can occur at any level resulting in loss of motor and sensory function below the level of lesion, bladder, bowel, and sexual dysfunction. High thoracic defects will result in a severe neurological deficit. Repair of myelomeningocele is a surgical emergency, and neonates should be operated on in the first few days after birth. These children will usually need multiple surgeries over the course of their lives, thus latex precautions should be initiated. Virtually all patients are considered latex allergic. Nearly all infants born with a myelomeningocele have supraspinal neurologic manifestations of the disease process. Arnold–Chiari II malformation is frequently present and includes a small posterior fossa, brain stem abnormalities, and hindbrain herniation (downward displacement of the medulla, cerebellum, and fourth ventricle into the spinal canal), resulting in hydrocephalus. Ventriculoperitoneal shunting is required in 85–90% of these children, and despite shunting, some children will have permanent developmental deficits from the Chiari malformation. Children with Chiari II malformation may suffer from central hypoventilation/apnea, bradycardia, stridor, vocal cord dysfunction, swallowing difficulties and aspiration. Thus, children with associated Chiari malformation and/or hydrocephalus need special attention during the perioperative period. Other associated anomalies include scoliosis, restrictive lung disease from scoliosis, renal anomalies, tethered cord, and syringomyelia.



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