Maximizing the functional benefits of posterior calvarial vault distraction in syndromic craniosynostoses: a nuanced approach to volume, vein, vector, and the vexed challenge of functional outcome in craniosynostoses.

PURPOSE

To evaluate indications, techniques, nuances, and outcomes of posterior cranial vault distraction (PCVD) in children with craniosynostoses.

METHODS

We performed clinical assessments, multidimensional CT, MRI brain rapid protocol, ophthalmological evaluation, sleep study, and nasal endoscopy (if indicated). Detailed data was collected in Excel. Customized craniotomy (supratorcular or subtorcular), distraction vectors, strategic barrel staving on stenosed bones (other than lambdoid), and ~ 2 cm relief craniectomy for venous decompression were employed. Additional procedures were performed for the frontal and midface aspects during distractor removal based on functional needs.

RESULTS

Thirty-seven patients (ages 4-204 months, mean 32.94 months) underwent PCVD. Supratorcular PCVD in 8, subtorcular in 29. The distraction vector was posterior-horizontal in 28 cases and posterior-inferior in 9. Strategic barrel staving was used in 8 cases, and venous decompression in 24. Initial assessments showed satisfactory clinical and radiological outcomes. Long-term follow-up indicated seven of 11 patients with hydrocephalus required a ventriculoperitoneal shunt, and two needed additional PCVD procedures due to symptom recurrence. Average intracranial volume increased by 186 ± 42.67 cm (18 patients), and the average distraction achieved was 21 ± 2.64 mm (37 patients). Additional procedures at the time of distractor removal included fronto-facial or monobloc advancement (n = 3), isolated fronto-orbital advancement and remodeling (n = 13), and midface distraction for airway issues (monobloc advancement, n = 3; isolated midface, n = 9). Nine patients underwent all three procedures in sequence.

CONCLUSION

PCVD is an accepted surgical strategy for craniosynostosis with posterior calvarial involvement. Our technical modifications aim to enhance functional and aesthetic outcomes without increasing morbidity.