Clinical and economic burden of neurofibromatosis type 2 in the United States.

OBJECTIVES: We aimed to investigate the prevalence and cost-associated risk factors for hospital stays for Neurofibromatosis Type 2 (NF2) patients in the past decade. PATIENTS AND METHODS: A multi-year cross-sectional study was performed using the National Inpatient Sample. Patients with a diagnosis code of NF2 according to the International Classification of Diseases, 9th Revision, Clinical Modification coding system were queried from 2006-2014. Sampling discharge weights were used to calculate trend estimates for national demographics, hospital characteristics, comorbidities, and surgical interventions. Regression analysis was performed to determine significant independent associations between comorbidities and admission cost. RESULTS: From 2006-2014, there were 5,078 discharges for patients diagnosed with NF2. Patient demographics, comorbidities, and procedures performed were overall consistent over time. The most common admission diagnoses were hearing loss (28.2 %), acoustic schwannoma (14.3 %), cranial meningioma (11.8 %) and epilepsy (10.8 %). The most common procedures performed were craniotomy and meningioma resection (10.2 %) and acoustic neuroma open resection (7.9 %). The median inflation-adjusted cost of admission did not change over time, with an admission cost value of $12,387 [6,042 - 26,051]. On regression analysis, obstructive hydrocephalus, craniotomy and meningioma resection, acoustic neuroma open resection, and spine tumor resection were all independent predictors of increased cost. CONCLUSION: The care for NF2 patients continues to evolve over time. We report the prevalence of patient demographics, comorbidities, and treatments in the NF2 inpatient population. Further studies are warranted to better understand the risk factors for higher costs, so that patients with NF2 may continue to receive life-long quality care in a cost-effective manner.

Neurofibromatosis.

Neurofibromatosis is a heritable disease characterized by disordered growth of ectodermal tissues.

Magnetic resonance imaging: a cost-effective first line investigation in the detection of vestibular schwannomas.

Magnetic resonance imaging (MRI) is currently the 'gold-standard' investigation in patients with a unilateral sensorineural hearing loss. The procedure, however, is expensive and of limited availability. Instead, such patients often undergo a series of audiovestibular tests and computed tomography in an attempt to exclude or diagnose a vestibular schwannoma. We describe seven cases of unilateral vestibular schwannoma in which conventional assessment was either equivocal or failed to demonstrate a tumour subsequently diagnosed by magnetic resonance imaging. Two patients with neurofibromatosis type 2 are also reported to show how magnetic resonance imaging confirmed the presence of a second vestibular schwannoma despite CT that showed only a unilateral lesion. We also illustrate how limited protocol MRI of patients is slightly more expensive yet much more cost effective than the usual battery of tests and propose that it should be the first line investigation for patients in whom the clinical picture requires exclusion of a retrocochlear lesion. Not all of these early diagnosed tumours have been immediately removed. In some of the more elderly or infirm patients a 'wait and rescan' policy has been adopted. Nevertheless, the early establishment of the correct diagnosis facilitates the subsequent management of these patients.