Neurofibromatosis Type 2 (NF2)

The airway is usually the easy part - Cutaneous features in NF2 are subtle and less common than in NF1: plaque-like lesions, subcutaneous nodules and cutaneous schwannomas rather than neurofibromas. Do not import the NF1 airway concerns; the risk in NF2 is intracranial and spinal.

Hearing loss and communication - Sensorineural hearing loss, tinnitus and balance problems are the presenting triad, and hearing loss correlates poorly with tumor size, so do not judge it from the scan. Work out before induction how this patient actually communicates: leave hearing aids in until the last moment, face them so they can lip-read, and write things down. Warn them they may wake hearing less than they did, particularly after vestibular schwannoma surgery.

Raised intracranial pressure - A cerebellopontine angle mass compresses cranial nerves and brainstem and raises ICP, and large tumors cause hydrocephalus. Take headache seriously and read gait abnormality as brainstem compression rather than clumsiness; meningiomas add seizures and focal deficits by location. On induction, ICP is the hemodynamic goal — smooth induction, no coughing, no hypertensive surge, no hypercarbia. Bradycardia with a widening pulse pressure is the Cushing reflex, not vagal tone.

Posterior fossa positioning - The cerebellopontine angle cistern holds cranial nerves V, VI, VII and VIII alongside the anterior inferior cerebellar artery in a narrow corridor, and access is usually head-up. Any time the field sits above the heart, venous air embolism is on the table: watch for a sudden fall in end-tidal CO2, and consider end-tidal nitrogen and a precordial Doppler.

Facial nerve monitoring gates your relaxant - Facial nerve involvement by the vestibular schwannoma is what makes the surgery difficult, and intraoperative nerve monitoring is how the surgeon protects it. Agree the neuromuscular blockade plan before you dose — full paralysis blinds the monitor. At the end, the 2023 ASA guidance is quantitative monitoring at the adductor pollicis to a train-of-four ratio of at least 0.9 before extubation, with sugammadex preferred over neostigmine for rocuronium or vecuronium. A fresh posterior fossa resection cannot afford residual weakness.

Neuraxial anesthesia is not a free choice - NF2 seeds the spine: schwannomas on the dorsal root, meningiomas in the cervical and thoracic region, and intramedullary ependymomas, presenting as pain, weakness and paresthesia. Contrast MRI of the entire spine is part of routine surveillance — read it before offering a neuraxial technique, and document baseline deficits either way.

Lower cranial nerves and aspiration - Brainstem compression produces ataxia and dysphagia alongside the hearing loss. A patient who cannot protect their airway before surgery will not protect it after; check swallow and cough preoperatively, and keep them intubated until both return.

Vision compounds the problem - Ophthalmic examination in NF2 looks specifically for cataracts and optic nerve involvement. Between the eyes and the ears, assume this patient's sensory access to the room is limited and orient them by touch.

The surveillance workup answers your questions - Contrast MRI of brain and entire spine with thin cuts through the internal auditory canals, plus audiology and brainstem evoked potentials, are already done annually. Read them rather than repeating the interview.

Expect them back - Vestibular schwannoma recurs after surgery in about 44% of cases, radiation carries a risk of malignant transformation, and bevacizumab is used medically after shrinking tumors and improving hearing in some series. Document your airway findings, positioning and any evoked-potential changes for the next team.

Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder caused by loss-of-function mutation of the NF2 gene at 22q12.2, which encodes merlin — a tumor suppressor found in Schwann cells. It is far rarer than type 1, about 1 in 25,000 to 40,000, and makes up roughly 3% of neurofibromatosis. It is a central nervous system tumor disease, not a skin disease.

Bilateral vestibular schwannomas are the hallmark and are present in 90% to 95% of patients; meningiomas occur in about 50%, and schwannomas, meningiomas and ependymomas also seed the spine. Symptoms usually begin around age 20. Perioperatively the syndrome presents as a mass in the cerebellopontine angle — sensorineural hearing loss, cranial nerve palsies, brainstem compression and raised intracranial pressure — with repeated posterior fossa and spinal surgery over a lifetime.