Congenital Spina Bifida

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Spina Bifida (SB) is one of the most common complex congenital birth defects in developing countries.  Children with SB are faced with multiple medical and psychosocial issues that require a  comprehensive multidisciplinary care plan. 

Clinical presentation 

  • Swelling on the back
  • CSF leak

Investigations 
Prenatal diagnosis 

  • Ultrasound in second trimester of pregnancy
  • Amniotic fluid analysis

Postnatal diagnosis 

  • Cranial Ultrasound—evaluate for associated hydrocephalus
  • Neuraxial MRI-is indicated in complex cases to plan surgical management and evaluate for associated defects—Hydrocephalus, chiari malformations, tethered cord
  • Preoperative screening echocardiogram (ECHO)

Pharmacological Treatment 

Initiate antibiotics in children with open infected spinal bifida lesions 

B: ceftriaxone (IV) 100mg/kg 12hourly for 2 weeks 

OR 

B: amoxicillin +clavulanate (FDC) (PO) 15mg/kg 8hourly for 2weeks 

OR 

C: amoxicillin +clavulanate (FDC) (IV)15mg/kg 8hourly for 2weeks 

AND 

A: metronidazole (PO) 7.5mg/kg/day 8hourly for 2weeks 

OR 

B: metronidazole (IV) 7.5mg/kg/day 8hourly for 2weeks 

Non-pharmacological Treatment 

Surgical management of SB 

  • Refer for expert neurosurgical evaluation for surgical closure of the spinal defect and subsequent evaluation for the need to treat associated hydrocephalus with CSF shunts or ETV+CPC.

Supportive and preventive and measures 

  • Provide routine screenings and testing for specific secondary conditions (HDC, skeletal limb deformities, UTIs) to minimize medical complication rates and help control cost of care.
    • Provide lower limb orthoses (splints, calipers)
    • Provide wheelchair for ambulation
  • Track and counsel women about recurrence risk and prescribe 5mg of folic acid beginning at least one month (preferably 3 months) before another conception and continue through the first 3 months of pregnancy.