Early postnatal cranial vault reduction and fixation surgery for severe hydrocephalic macrocephaly

Area of Science:

• Neurosurgery
• Pediatric Surgery
• Craniofacial Surgery

Background:

• Severe neonatal hydrocephalus with extreme macrocephaly often requires early cerebrospinal fluid (CSF) diversion.
• Early CSF diversion can lead to significant cranial deformities due to overdrainage and overlapping cranial plates.
• Subsequent extensive cranial vault remodeling is challenging, with high morbidity and mortality rates.

Purpose of the Study:

• To evaluate the institutional experience of managing extreme neonatal hydrocephalus with CSF diversion, with and without cranial vault reduction and fixation (CVRF).
• To assess the efficacy of early CVRF in improving cosmesis and simplifying management in infants with severe hydrocephalic macrocephaly.
• To compare outcomes between infants treated with shunting alone versus shunting combined with CVRF.

Main Methods:

• Retrospective review of infants (head circumference > 49 cm) treated between 2005 and 2017 with ventriculoperitoneal shunting, with or without CVRF.
• Data collected included patient demographics, hydrocephalus etiology, CVRF type, follow-up duration, craniometric measurements, operative details, and complications.
• Developmental and esthetic outcomes were assessed using standardized questionnaires and photographic imaging.

Main Results:

• Eleven infants underwent CSF shunting; 5 with shunting alone and 6 with shunting and CVRF.
• The mean calvarial vault volume reduction with CVRF was 44.5%.
• Shunting alone resulted in severe cranial deformities in 3 of 5 patients, whereas CVRF combined with shunting led to only 1 poor cosmetic outcome in 6 patients. Mortality occurred in 2 patients (shunting alone) and 1 patient (shunting and CVRF).

Conclusions:

• CVRF in the neonatal period, combined with CSF shunting, can simplify management for severe hydrocephalic macrocephaly.
• This combined approach leads to favorable cosmetic outcomes, as reported by families.
• Early CVRF may mitigate the severe cranial deformities often seen with CSF diversion alone in this patient population.