Spina bifida repair

Alternative names
Meningocele repair; Myelomeningocele repair; Myelomeningocele closure

Definition

A meningocele is a protrusion of cerebrospinal fluid and meninges (covering layers of the spinal cord and brain) into the tissues beneath the skin. Meningocele repair is a surgical procedure to drain the cerebrospinal fluid and repair and close birth defects of the spine and spinal membranes. It is used to treat the conditions listed below:

     
  • Spina bifida: a birth defect which involves an abnormal opening of the spinal area.  
  • Spina bifida occulta: the congenital absence of spinal bones with no exposure of the internal structures such as meninges or neural tissue.  
  • Meningocele: a congenital defect in the bony structures with distension (swelling) of the meninges, but no abnormality of neural tissue.  
  • Myelomeningocele: a congenital defect in the bones of the spine with distention of the meninges and structural or functional abnormality of the spinal cord or nerve roots.

Description
Surgery is usually done within 24 hours after birth. While the baby is anesthetized, an incision is made in the sac around the spinal cord, and excess fluid is drained off. The spinal cord is then covered with the membranes (meninges). The skin is closed over the meninges, spinal cord, and nerves.

Indications
Surgery is recommended if a meningocele or meningomyelocele are present.

Risks
Risks for any anesthesia include the following:

     
  • Reactions to medications  
  • Problems breathing

Risks for any surgery include the following:

     
  • Bleeding  
  • Infection

Additional risks include the following:

     
  • Excessive fluid and pressure in the brain (hydrocephalus)  
  • Infection or inflammation of the spinal cord (Meningitis)  
  • Paralysis, weakness or sensory changes related to loss of nerve function  
  • Increased chance of developing Urinary tract infections

Expectations after surgery

The long-term results depend on the initial condition of the spinal cord and nerves. Outcomes range from normal development to various types of paralysis (paraplegia), depending on the level of spinal cord involvement. With modern treatments, greater than 85% of infants survive, and about 50% will be able to walk.

Hydrocephalus (“water on the brain”) develops in about 70% of patients with myelomeningocele and it is already present in 5-10% of patients at birth.

In patients with myelomeningocele and developing hydrocephalus, most surgeons will wait for a few days after repairing the myleomeningocele to treat the hydrocephalus with a shunt to relieve raised intracranial pressure. If hydrocephalus is present at birth and is already compromising the infant’s neurologic function, then myelomeningocele repair and shunting for hydrocephalus are done at the same time.

Postoperative antibiotics are routinely administered to prevent infection.

Convalescence
Patients usually spend about 2 weeks in the hospital after surgery. During this time the patient is kept flat without lying on the incision as it heals. Bladder catheterization is usually required.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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