Newborn with a leaking lumbosacral myelomeningocele and ventriculomegaly
A male newborn is delivered at term and transferred at 6 hours of age with a 4 × 3 cm lumbosacral myelomeningocele. The sac is thin and leaking cerebrospinal fluid. He moves both legs but with weak ankle dorsiflexion and plantar flexion, and the anus is patulous. Head circumference is 37 cm, above the 97th centile, and the anterior fontanelle is tense. Cranial ultrasound shows moderate ventriculomegaly without haemorrhage. He is breathing spontaneously, feeding poorly, and has passed meconium. There is no prenatal repair. The parents are asking about urgency of closure, leg function, and whether a shunt will be needed. The neonatal unit has started antibiotics and is keeping him prone.
1. Outline the immediate management and the timing of surgical closure. What specific precautions would you take before and during surgery?
Read the worked answer
The immediate priorities are to protect the neural placode, prevent infection, and assess associated anomalies. Keep the infant prone, cover the defect with sterile saline-soaked non-adherent gauze and a plastic film, and change the dressing regularly. Continue intravenous antibiotics covering skin flora and gram-negative organisms. Use latex-free gloves and equipment because children with myelomeningocele have a high risk of latex sensitization. Monitor head circumference daily, check lower limb movement and bladder function, and obtain cranial ultrasound and spinal imaging. Close the defect within 24–48 hours, ideally as soon as the baby is stable, because leaking CSF increases the risk of meningitis and ascending infection. Do not delay closure to treat ventriculomegaly unless the infant is unstable. During surgery, handle the placode gently, preserve neural tissue, close the dura watertight, and reconstruct the fascia and skin without tension. Send CSF or sac fluid for culture if there is leakage.
2. How would you manage the ventriculomegaly, and when would you place a shunt or an EVD?
Read the worked answer
Ventriculomegaly is common in myelomeningocele and may be stable, slowly progressive, or symptomatic. Measure head circumference daily and repeat cranial ultrasound. If the infant is stable, feeding, and without signs of raised intracranial pressure, observe rather than shunt immediately. If there is progressive ventricular enlargement with a tense fontanelle, vomiting, lethargy, or poor feeding, place a temporary external ventricular drain at the time of closure or soon after. A permanent ventriculoperitoneal shunt is considered if hydrocephalus persists after the neonatal period, typically in 80–90% of children with myelomeningocele. Endoscopic third ventriculostomy is less reliable in this population because of associated Chiari malformation and altered CSF pathways. Discuss with the parents that shunt dependence is likely and that signs of shunt malfunction include irritability, vomiting, drowsiness, and bulging fontanelle. Avoid rapid CSF removal that can cause overdrainage and subdural haemorrhage.
3. The parents ask about long-term leg function, bladder control, and follow-up. How would you counsel them and what follow-up would you arrange?
Read the worked answer
Counsel them that the final motor level is best judged after closure and that some improvement may occur, but permanent weakness is common. Document baseline lower limb power and refer to orthopaedics and physiotherapy for splinting, contracture prevention, and mobility aids. Bladder and bowel dysfunction are likely; arrange urology review, clean intermittent catheterisation teaching, renal ultrasound, and urodynamic assessment. Check for hydrocephalus, tethered cord, and Chiari symptoms at follow-up. Late deterioration may indicate tethered cord, syringomyelia, or shunt malfunction. Provide latex avoidance advice and an emergency plan for shunt problems. Neurodevelopmental follow-up should include developmental assessment and school planning. Explain that multidisciplinary care improves outcomes but does not restore normal function. Document the discussion sensitively and offer genetic counselling about future pregnancies and folic acid supplementation.