Al-Azhar Neurosurgery

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Al-Hussein university hospital , Al-Sayyed Galal hospital

+2 0102 9252247, +2 0111 1726597

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Case 2

Case 2 Chiari malformation type 2 (CM-II)

A close-up of a brain scan Description automatically generated

Figure 2: MRI axial T1.

Clinical presentation

  • A male baby who is 35 days old and born full term, the fourth child from a non-consanguineous marriage delivered by C-section.
  • The condition began one week after surgery to repair meningocele on January 14, 2024, at Al-Hussein University Hospital.
  • The patient has shown progressive increase in head circumference, as indicated by a CT scan revealing hydrocephalus changes.
  • Upon examination, the baby is conscious with spontaneous eye movement, a good cry, strong suckling power, and freely moving limbs. The anterior fontanelle measures 2×2 finger breadth and appears tense and bulging, while the head circumference measures 38 cm.
  • Q1: What is the optimal time for repair of meningomyelocele in Chiari type 2?
  • Early closure of the meningomyelocele (MM) defect is not associated with improvement in neurological function, but evidence supports a lower infection rate with early closure. The MM should be closed within 24 hours, regardless of whether the membrane is intact (after 36 hours, the back lesion becomes colonized, increasing the risk of postoperative infection).
  • Q2: What percentage of patients with MM will develop hydrocephalus HCP?
  • Hydrocephalus develops in 65-85% of patients with meningomyelocele (MM). Over 80% of MM patients who will develop hydrocephalus do so before age 6 months. Closure of the MM defect may convert latent hydrocephalus to active hydrocephalus by eliminating a route for cerebrospinal fluid (CSF) drainage, potentially leading to issues like CSF leakage from the wound site of the MM.
  • Q3: What is the underlying embryology behind the development of meningomyelocele (MM)?
  • It is due to failure of closure of the neural tube, specifically the posterior neuropore, which typically closes around 26 days after conception. There are several risk factors associated with this condition; certain drugs, such as Na valproate used for seizures, are implicated. Another significant factor is folic acid deficiency. Therefore, folic acid supplementation should be recommended to all couples planning to have a baby and should ideally be started before conception.