Staged posterior fossa decompression and microvascular decompression for trigeminal neuralgia in autosomal dominant osteopetrosis type I: A case report.

RATIONALE: Osteopetrosis is a rare hereditary skeletal disorder characterized by increased bone density resulting from defective osteoclast-mediated bone resorption. This condition may lead to cranial nerve compression syndromes, with trigeminal neuralgia (TN) occurring infrequently.

PATIENT CONCERNS: We report the case of a 26-year-old woman with autosomal dominant osteopetrosis who presented with intractable, electric shock-like facial pain consistent with TN that was refractory to medical therapy.

DIAGNOSES: Imaging revealed marked posterior fossa crowding with evidence of neurovascular compression of the left trigeminal nerve root entry zone, further compounded by cranial hyperostosis.

INTERVENTIONS: A staged surgical approach was undertaken. The first stage involved suboccipital craniectomy with C1 laminectomy to decompress the posterior fossa and restore cerebrospinal fluid dynamics. The second stage comprised microvascular decompression via a retrosigmoid approach after optimization of the intracranial space.

OUTCOMES: Following completion of the staged surgical intervention, the patient achieved complete resolution of facial pain without neurological deficits. Symptom relief was sustained over a 5-year follow-up period, with no evidence of recurrence.

LESSONS: A staged decompressive approach represents an effective and durable treatment option for TN secondary to osteopetrosis, particularly in cases with markedly compromised posterior fossa anatomy. Initial posterior fossa decompression facilitates a safer and more effective subsequent microvascular decompression by improving the surgical corridor.