Diagnosis: Neurofibromatosis type I, optic glioma,
renovascular hypertension
Treatment: Inpatient admission
The insurer denied the inpatient admission.
The denial is overturned.

The patient is a female child with complex medical history significant for neurofibromatosis type I, optic glioma status post chemotherapy, poorly controlled renovascular hypertension secondary to left renal artery stenosis that failed medical treatment status post left renal artery angioplasty.

The patient presented for renal artery angiogram, left renal artery angioplasty with stent placement and post stent dilatation for left renal artery ostial stenosis. She did well and was admitted post-procedure for monitoring and repeat renal sonogram the next day. She reported nausea treated with Zofran and mild incisional pain. Admission orders included holding home antihypertensive medications (enalapril, amlodipine), consideration for isradipine for sustained hypertension, initiation of treatment with Plavix, resume aspirin, Tylenol as needed for pain, Hematology consultation, repeat renal ultrasound with doppler the following morning, regular diet, and maintenance intravenous fluids. She did well overnight, with blood pressures within normal limits off antihypertensives.

Follow-up renal ultrasound revealed patent renal vasculature with no sonographic evidence of hemodynamically significant renal artery stenosis in the main or accessory renal arteries, improved flow within the left kidney compared to prior, mild bilateral renal pelvic fullness decreased on the left compared to prior without significant hydronephrosis, and a small amount of pelvic free fluid. She was deemed stable for discharge to continue both Plavix and aspirin and follow-up with the Renal Team.

Yes, post-procedural inpatient hospitalization was medically necessary and appropriate for this patient.

Neurofibromatosis is an autosomal dominant neurocutaneous syndrome with an incidence of one in 3000 live births. The diagnosis of neurofibromatosis type I is made when two of the following seven features are present: six or more café au lait spots greater than five millimeters in diameter (15 millimeters in post-pubertal patients), axillary or inguinal freckling, two or more iris Lisch nodules, two or more neurofibromas or one plexiform neurofibroma, sphenoid dysplasia or cortical thinning of the long bones without pseudoarthrosis, optic gliomas, and a first-degree relative with neurofibromatosis type I. Neurofibromas typically involve the skin but may be located along peripheral nerves and blood vessels and within viscera. Plexiform neurofibromas are present at birth and result from thickening of nerve trunks resulting in overgrowth and deformity. Patients with neurofibromatosis type I are susceptible to learning disabilities, seizures, moyamoya, and precocious puberty. Infrequently, there may be progression to malignancy.

Renovascular hypertension accounts for about 5% of pediatric hypertension, of which neurofibromatosis type I (NF1) is one cause, along with fibromuscular dysplasia, midaortic syndrome, Takayasu's arteritis, William's syndrome, Marfan's syndrome, congenital rubella syndrome, Kawasaki disease, and Crohn's disease. Renovascular hypertension results in decreased blood flow to the kidneys increasing production of renin. Angioplasty restores blood flow, decreasing renin production. Other treatment modalities include medications and surgery. About 15% of patients with neurofibromatosis type I develop renovascular hypertension associated with renal artery stenosis that is generally ostial in location. Revascularization is essential in these patients before permanent end-organ damage occurs.

This female child with neurofibromatosis type I had failed medical management and previous angioplasty with persistent and severe renovascular hypertension. She underwent outpatient intervention which she tolerated with only some nausea and vomiting after. She was admitted to the hospital for close monitoring off all home antihypertensives as well as renal sonogram with doppler to evaluate response to therapy. Given the extremely complicated nature of her condition and previous poor response to therapy, it was medically necessary and appropriate to monitor her in the hospital following her procedure. She could not have been appropriately managed in the outpatient setting.