Appeal type: Medical necessity
Decision: Upheld

Diagnosis: Neurofibromatosis Type 1  
Treatment: Inpatient Level of Care Admission
The insurer denied the Inpatient Level of Care Admission.
The health plan's determination is upheld.

The patient is a female who initially presented to the emergency department with concerns for left sided facial swelling and erythema. Her pertinent history included neurofibromatosis type 1 and depression. The initial vital signs revealed a temperature of 36.8, heart rate (HR) 97, blood pressure (BP) 158/106, respiratory rate (RR) 18 and her peripheral capillary oxygen saturation (SpO2) was 97 percent (%) on room air. The initial examination documented a left temporal tender fluctuant mass without significant skin changes. The initial laboratory testing revealed a white blood cell count (WBC) 9.54, hemoglobin 14.8, platelets (Plt) 276, sodium 140, blood urea nitrogen (BUN) 15, creatinine 0.58 and her glucose was 85.

The maxillofacial computed tomography (CT) revealed a lobulated hyperdense soft tissue mass near facial nerve and external ear canal. The ear, nose, and throat (ENT) specialist and plastic surgeon were consulted and recommended corticosteroids as well as a magnetic resonance imaging (MRI). The patient was admitted to the hospital thereafter with an impression of left-sided localized facial swelling possibly secondary to infectious etiology versus malignancy versus less likely neurofibroma. The treatment plan included empiric antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, MRI, and consultations. The patient was afebrile and hemodynamically stable. The MRI of the face was performed with findings consistent with a plexiform neurofibroma. Infectious disease was consulted and recommended holding antibiotics if aspiration was not to be performed at a later date. The ENT consultant reviewed the findings and cleared the patient for discharge with outpatient follow-up. 

Based on the clinical documentation provided, evidence based literature and standards of care, the acute inpatient level of care was not indicated as medically necessary for the entire admission. Evaluation and management could have been performed at an alternate level of care with transition to outpatient services.
Gutmann et al. notes neurofibromatosis type 1 (NF1) is a complex autosomal dominant disorder caused by germline mutations in the NF1 tumor suppressor gene. Nearly all individuals with neurofibromatosis type 1 develop pigmentary lesions (cafe-au-lait macules, skinfold freckling and Lisch nodules) and dermal neurofibromas. Some individuals develop skeletal abnormalities (scoliosis, tibial pseudarthrosis and orbital dysplasia), brain tumors (optic pathway gliomas and glioblastoma), peripheral nerve tumors (spinal neurofibromas, plexiform neurofibromas and malignant peripheral nerve sheath tumors), learning disabilities, attention deficits, and social and behavioral problems, which can negatively affect quality of life.[1]
The patient had a history of neurofibromatosis who presented with a fluctuant mass over the left temporal bone. The initial evaluation found the patient to be afebrile, hemodynamically stable, and with unremarkable laboratory testing. The CT imaging revealed a lobulated soft tissue mass near the facial nerve. Surgical services were consulted and recommended corticosteroids, empiric antibiotics, and an MRI. The MRI revealed findings suggestive of a plexiform neurofibroma. The ENT consultant cleared the patient for discharge with outpatient follow-up. Infectious disease recommended to discontinue empiric antibiotics. No other issues or complications were documented. Clinical indications for admission to inpatient care were not met. The patient did not have severe pain unresponsive to analgesia, leukemia, lymphoma, tumor lysis syndrome, erythema multiforme, or a need for surgical intervention.[2]
 
The health plan's determination of medical necessity is upheld in whole.