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Cephaloceles: treatment, outcome, and antenatal diagnosis
Insights
Infants with cranial meningoceles generally fare well, while those with meningoencephaloceles often have poor outcomes. Early antenatal diagnosis and neurosurgical involvement are crucial for managing these diverse conditions.
Area of Science:
- Pediatric Neurosurgery
- Developmental Biology
- Craniofacial Surgery
Background:
- Cephaloceles are congenital abnormalities involving protrusion of intracranial contents through skull defects.
- Meningoencephaloceles, containing brain tissue, present greater challenges than cranial meningoceles, which involve only meninges.
Purpose of the Study:
- To analyze outcomes and etiological diversity in a series of 74 cephaloceles.
- To highlight differences in prognosis between cranial meningoceles and meningoencephaloceles.
- To emphasize the importance of early diagnosis and management strategies.
Main Methods:
- Retrospective review of 74 cases of cephalocele.
- Analysis of clinical data, including type of cephalocele, associated conditions, and treatment interventions.
- Evaluation of patient outcomes and etiological factors.
Main Results:
- Infants with cranial meningoceles had favorable outcomes, even with hydrocephalus.
- Infants with meningoencephaloceles, particularly those with brain tissue, had poor prognoses despite surgical interventions.
- Frontoethmoidal meningoencephaloceles require specific genetic counseling and surgical approaches, including craniofacial reconstruction for associated facial deformities.
- Antenatal diagnosis via ultrasound was successful in 4 cases.
Conclusions:
- Prognosis for cephaloceles is highly dependent on the type and content of the protrusion.
- Early antenatal diagnosis and multidisciplinary management, including neurosurgical input, are vital.
- Frontoethmoidal meningoencephaloceles present unique challenges requiring specialized care and surgical correction of craniofacial anomalies.
Abstract:
A series of 74 cephaloceles (17 cranial meningoceles and 57 meningoencephaloceles) is reported. Infants born with large meningoencephaloceles containing recognizable cerebral tissue usually did badly despite endeavors to conserve brain function by expanding the cranial capacity (5 cases) or decompressing hydrocephalic ventricles (9 cases). Infants with cranial meningoceles almost all did well, even when there was associated hydrocephalus. The etiological diversity of cephaloceles is emphasized. Frontoethmoidal meningoencephaloceles, which occur with noteworthy frequency in South and Southeast Asia, require separate consideration in both genetic counseling and treatment; the associated facial deformities (hypertelorism and orbital dystopia) can be corrected with a one-stage craniofacial reconstruction. Antenatal diagnosis by ultrasound is now often possible and was achieved in 4 cases; we suggest that neurosurgeons should participate in such antenatal evaluations.