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Key messages

  • A meningomyelocele is a sac-like structure protruding through a defect in the vertebral arches.
  • A meningomyelocele is the most common neural tube defect (NTD).
  • Infants with a meningomyelocele need early transport to a tertiary neonatal centre for assessment.
  • In term infants without other complicating features, transport by local ambulance services may be appropriate.

Please note that all guidance is currently under review and some may be out of date. We recommend that you also refer to more contemporaneous evidence in the interim.

The neural tube normally closes between 15 and 28 days after conception, failure of closure results in a neural tube defect (NTD). A meningomyelocele is the most common NTD. It is a sac-like structure protruding through a defect in the vertebral arches, containing meninges, CSF and spinal cord +/- nerve roots.

Detection and referral

Some points to note about detection and referral for neural tube defects:

  • The detection of neural tube defects frequently occurs before birth as a result of maternal alpha-fetoprotein measurement or ultrasound examination.
  • Referral to a multidisciplinary team for family counselling and management plan development is then appropriate.
  • Genetics Health Services Victoria provides services throughout Victoria and can be contacted via (03) 8341 6201.
  • Occasionally affected infants will deliver unexpectedly.

Prognosis

Prognosis for survival and extent of the disability depends on:

  • the level of the lesion
  • the degree of involvement of the spinal cord fibres
  • presence of infection
  • the presence of associated anomalies:
    • central nervous system (such as hydrocephalus and Arnold-Chiari malformation)
    • cardiac
    • oesophageal
    • intestinal
    • genitourinary.

Differential diagnosis

Meningocele

Differential diagnosis includes:

  • Herniation of meninges without associated neural tissue through the bony defect.
  • Following surgical repair there is a good prognosis.
  • Diagnosis is made by appropriate imaging studies (for example, ultrasound).

Investigation

Detailed clinical examination is required to assess:

  • site and level of lesion
  • motor and sensory level involvement
  • presence of clinical hydrocephalus:
    • palpate the fontanelle/sutures for evidence of raised intracranial pressure
  • symptoms of hindbrain herniation:
  • presence of musculoskeletal deformity or anomalies of other organ systems:
    • assess anal tone
    • urine output
    • dribbling
    • palpable bladder.

Further investigations (undertaken at tertiary centre)

  • Neurosurgical assessment
    • MRI brain and spine
    • Sequential OFC measurements and cranial USS
  • Renal tract assessment
    • Renal tract USS
    • Urine MCS
    • Urology consultation, need for clean intermittent catheterisation
  • Orthopedic assessment
    • Hip USS
  • Microarray

Management

Management issues include:

  • Infants need referral and transport to a tertiary neonatal centre for assessment by a coordinated team of specialists experienced in dealing with these lesions.
  • A treatment policy can be formulated and discussed with the parents.
  • Before and during transport:
    • the lesion, especially if ruptured, should be covered with a sterile non-adherent dressing such as cling wrap
    • the infant should be nursed in the prone position and the defect protected, for example, by foam rubber cut into a doughnut
  • IV access is required to provide antibiotics such as penicillin and gentamicin (preferably after blood is taken for culture).
  • IV fluids are required if an excessive delay before oral feeds can commence is anticipated, respiratory difficulty or hypoglycaemia is present.
  • In some infants, intermittent urinary catheterisations may be required.
  • Multidisciplinary follow up is required.

Areas of uncertainty in clinical practice

Intrauterine surgery to close the defect has been associated with reduction of shunt dependent hydrocephalus and long-term neurological complications.

Caesarean section before the onset of labour is often the desired mode of birth since this has been associated with improved neurological outcomes.

More information

Consumer

References

Get in touch

Centre of Clinical Excellence - Women and Children
Safer Care Victoria

Version history

First published: February 2015

Last web update: October 2018

Review by: December 2020

Uncontrolled when downloaded

Page last updated: 17 Feb 2021

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