Meningomyelocele

Summary
Introduction
Differential Diagnosis
Investigation
Management
Areas of Uncertainty in Clinical Practice

Summary

infants need early transport to a neonatal centre for assessment
for term infants, without other complicating features, transport by local ambulance services is often appropriate

Introduction

The detection of neural tube defects frequently occurs before delivery as a result of maternal alphafetoprotein 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 for survival and extent of the disability depend on

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

Differential Diagnosis

Meningocele is a 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 (eg ultrasound).

Investigation

Detailed clinical examination is required to assess

site and level of lesion
motor and sensory level
presence of clinical hydrocephalus
presence of hindbrain herniation e.g. stridor, apnoea, swallowing difficulty
presence of musculoskeletal deformity or anomalies of other organ systems

Management

Infants need referral and transport to a neonatal centre for assessment by a co-ordinated team of specialists experienced in dealing with these lesions so that a treatment policy can be discussed with the parents.

Before and during transport

the lesion, especially if ruptured, should be covered with a sterile non-adherent dressing
the infant should be nursed in the prone position and the defect protected e.g. by foam rubber cut into a doughnut

IV access is required to provide antibiotics e.g. 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 hypoglycemia is present.

Areas of Uncertainty in Clinical Practice

Fetal surgery to close the defect and thus possibly reduce the incidence of hydrocephalus and long term neurological complications remains experimental.

Cesarean section before the onset of labour is usually the desired mode of delivery since this has been associated with improved neurological outcomes.

Web links

http://www.spinabifida.org This is the Home page of the Spina Bifida Hydrocephalus Association of Queensland. Its great. Plenty of information and good diagrams.

http://www.sbaa.org/index.shtml This is the Home page of the Spina Bifida Association of America. Its very good.

Spina Bifida Foundation of Victoria.

Neural Tube Defects in the Neonatal Period Ellenborgen R.G. eMedicine Journal, July32001, Vol.2, No. 7 http://www.emedicine.com/ped/topic2805.htm

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