Retrosigmoid microvascular decompression as a treatment for trigeminal neuralgia in a patient with osteogenesis imperfecta.

Individuals with osteogenesis imperfecta develop pathologic bone due to genetic defects in collagen synthesis. These patients are prone to skull base abnormalities with resultant lower cranial nerve deficits, most common of which is trigeminal neuralgia. Typically, such patients are managed medically, and surgical options are not well explored for those patients, who become refractory to medication management. While microvascular decompression is often recommended for patients with classical trigeminal neuralgia, neurovascular compression by MRI, and normal skull base anatomy, ablative procedures have been described for patients with trigeminal neuralgia and osteogenesis imperfecta. MVD via a retrosigmoid approach has not been described in a patient with trigeminal neuralgia and skull base abnormalities secondary to osteogenesis imperfecta. A 23-year-old man with osteogenesis imperfecta was referred with right-sided classical trigeminal neuralgia. His trigeminal pain had become refractory to a number of medications. High-resolution MRI demonstrated compression of the trigeminal nerve by the superior cerebellar artery. Microvascular decompression of the trigeminal nerve via a retrosigmoid craniectomy was performed, and he remains pain-free 6 months after surgery. Microvascular decompression of the trigeminal nerve through a retrosigmoid approach can be an effective surgical treatment for young patients with trigeminal neuralgia secondary to osteogenesis imperfecta.