What is the management and treatment approach for a pediatric patient with Meningoencephalocoele (a congenital condition involving herniation of the meninges and brain tissue through a skull defect)?

Management of Meningoencephalocoele in Pediatric Patients

Surgical repair should be performed shortly after birth, ideally within 48 hours, using a combined intra-extracranial approach for anterior skull base defects or endoscopic endonasal techniques for smaller transethmoidal lesions, with immediate attention to associated hydrocephalus requiring shunt placement in approximately one-third of cases. 1, 2, 3

Initial Assessment and Diagnosis

Determine the anatomical location and contents of the defect immediately:

• Frontal/frontoethmoidal lesions (most common anterior location) present with visible midline nasal masses or intranasal protrusion 2, 4
• Occipital lesions (most common overall, representing 65% of cases) present as posterior skull defects 1
• Basal/transethmoidal defects may present subtly with recurrent meningitis or unilateral CSF rhinorrhea 5, 3

Obtain high-resolution imaging before surgery:

• CT scan with bone windows to identify skull base defects and bony anatomy 5, 3
• MRI with T1 and T2 sequences to differentiate meningeal tissue from brain parenchyma 5, 3
• Consider high-pressure subarachnoid cisternography with CT for occult defects presenting with recurrent meningitis 5

Surgical Management Algorithm

For anterior skull base (frontoethmoidal/basal) defects:

• Combined intra-extracranial approach is the gold standard, providing superior exposure and reliable skull base reconstruction with 91% success rate (10/11 cases) 2
• Endoscopic endonasal approach is acceptable for smaller transethmoidal lesions, offering less invasive access with shorter recovery and fewer complications 3, 4
• Simple frontal or lateral nasal approaches have unacceptably high failure rates and should be avoided 2

For occipital defects:

• Direct posterior approach with primary closure of the defect 1
• Repair dural defect with watertight closure to prevent CSF leak 1

Timing is critical:

• Operate within 48 hours of birth to minimize infection risk and neurological complications 1
• Early recognition prevents recurrent meningitis, brain abscess, and progressive neurological deterioration 5, 3

Management of Associated Hydrocephalus

Approximately 35% (18/52) of patients develop progressive hydrocephalus requiring shunt placement shortly after initial repair 1:

• Monitor for signs of increased intracranial pressure immediately postoperatively 1
• Place ventriculoperitoneal shunt promptly if hydrocephalus develops 1
• This parallels the 70% hydrocephalus rate seen in myelomeningocele, though meningoencephalocoele has lower incidence 6

Prognostic Factors

Outcome depends on three key variables 1:

1. Anatomical location: Occipital lesions generally have better outcomes than complex skull base defects 1, 2

2. Sac contents: Pure meningoceles (CSF and meninges only) have excellent prognosis, while lesions containing significant brain tissue (encephaloceles) carry higher morbidity 1

3. Associated congenital anomalies: Screen for additional CNS malformations that worsen prognosis 1

Expected outcomes with appropriate surgical management:

• 73% (38/52) achieve good outcomes with no major disabilities 1
• 17% (9/52) mortality rate 1
• 10% (5/52) severe disability 1

Critical Pitfalls to Avoid

Do not delay surgery - waiting increases infection risk and neurological complications 1, 5

Do not use inadequate surgical approaches - simple frontal or lateral nasal incisions have high failure rates; combined approaches or endoscopic techniques are mandatory 2

Do not miss associated hydrocephalus - one-third of patients require shunt placement; failure to recognize this leads to preventable neurological deterioration 1

Do not overlook recurrent meningitis as a presentation - this indicates an occult skull base defect requiring urgent imaging and repair 5, 3

Postoperative Surveillance

Monitor for recurrence during follow-up:

• No recurrence occurred in successfully repaired cases with appropriate surgical technique 2
• Watch for delayed CSF leak or meningitis suggesting incomplete repair 5, 3
• Long-term neurological follow-up for patients with brain tissue involvement 1