Cystic Progression of a Cavernous Malformation at the Level of the Trigeminal Root Entry Zone Presenting With Sudden Onset of Trigeminal Neuralgia

Abstract

Background: Cavernous malformations (CM) in the cerebellopontine angle (CPA) are rare, and most of them are solid and extend from the internal auditory canal into the CPA. In contrast, cystic CM arising in the CPA and not involving the internal auditory canal and dura of the skull base are extremely rare. The authors present an uncommon large cystic progression of a cavernous malformation at the level of the trigeminal root entry zone evolving to severe trigeminal neuralgia and brainstem compression.

Methods: A 62-year-old female presented a sudden onset of left trigeminal neuralgia, caused by a large cystic lesion at the level of the root entry zone of the left 5th nerve. On neurological examination, she showed slight gait ataxia and hypoesthesia on the left hemiface (on the first and second trigeminal branches). Other cranial nerves were in order. Magnetic resonance imaging showed a large cystic intracranial mass, with a small solid portion, leading to brainstem compression.

Results: Microsurgical removal of the lesion was performed via retrosigmoid approach, with intraoperative monitoring of somato-sensory evoked potentials, facial, and cochlear nerves. The posterior-medial portion of the lesion was solid, whereas the main portion was cystic, containing xanthochromic fluid. The small solid lesion continued with a thin capsule of a large cyst adherent to brainstem, cerebellar hemisphere, and trigeminal nerve entry zone. A big draining vein arising from the solid part of the lesion runned parallel to brainstem. The mass was piecemeal totally removed.After surgery the patient recovered both left trigeminal neuralgia and hypoesthesia; ataxia was significantly relieved too. Postoperative magnetic resonance imaging confirmed the total removal. Histopathological features were consistent with a CM. At 6-month follow-up, patient's symptoms at the presentation had resolved.

Conclusion: The authors present a very rare patient of large cystic cavernous malformation at the level of the trigeminal root entry zone presenting with sudden onset of trigeminal neuralgia. Even if it has not established imaging features, a cystic cavernoma of the cerebello-pontine angle may be suspected when a cystic mass is present, not involving the internal acoustic meatus nor the skull base dura mater. Careful microneurosurgical technique and monitoring of cranial nerves allow good long-term results.