Abstract
Background:
We present a case of an 8-year-old female presenting with chronic headaches and elevated intracranial pressure (ICP) secondary to craniocerebral disproportion (CCD), in the absence of classic craniosynostosis. Although medical management, including acetazolamide and multiple lumbar punctures, provided temporary relief, imaging consistently revealed signs of restricted intracranial volume.
Case presentation:
The patient underwent posterior vault distraction osteogenesis (PVDO) to increase intracranial volume. The procedure involved a biparieto-occipital craniotomy and placement of distractors, ultimately achieving a 30 mm expansion. Although she experienced recurrent distractor-site infections, these were successfully managed with local wound care and antibiotics, ultimately resolving completely.
Outcome:
Following PVDO and hardware removal, the patient experienced a significant symptomatic improvement. Estimated intracranial volume, calculated using the ellipsoid approximation method, increased from 1387 cm3 preoperatively to 1627 cm3 postoperatively, representing a 17.3% increase. Imaging confirmed interval healing of the cranial vault, ventricular expansion, and increased overall intracranial volume.
Conclusion:
This case suggests that PVDO may represent a viable treatment option for chronic headaches caused by CCD in the absence of overt craniosynostosis. While limited to a single patient, this report adds to a growing body of literature supporting the consideration of calvarial volume expansion in select patients with atypical presentations of elevated ICP. It also emphasizes the importance of multidisciplinary collaboration for accurate diagnosis, surgical planning, and long-term follow-up.
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