An 18‐year‐old man with neurofibromatosis‐1 was admitted after surgery to correct a right cavus foot deformity and was noted to have postoperative hypertension. He was found to have systolic blood pressures above the 99th percentile, ranging from 151 to 167 systolic (from 54 to 72 diastolic) during the admission and a single recording of 152/64 in orthopedics clinic 2 months prior. In addition, he had multiple documented previous elevated blood pressures at his primary care physician's office, but no further evaluation was performed. He denied headache, chest pain, flushing, sweating, or hematuria. There was no known family history of hypertension, cardiac disease, stroke, or renal disease. There was no correlation between his pain and blood pressure levels. Abdominal ultrasound and follow‐up CT‐angiogram revealed a small right kidney and high‐grade stenosis of the right renal artery as well high‐grade stenosis of the proximal SMA. EKG and echocardiogram showed left ventricular hypertrophy. The vascular and renal teams were consulted, and medical management of hypertension was recommended. He was started on amlodipine and later switched to benazepril with improvement of blood pressure to the 140s/60s. He was discharged home with close follow‐up. His blood pressures have been improved on subsequent outpatient visits.
Hypertension in adolescents has increased over the past decade due to increased prevalence of primary hypertension from the obesity epidemic. However, unlike in older adults, a significant portion of hypertension in the adolescent is caused by a secondary etiology such as renal disease and therefore requires further evaluation. NF‐1 in particular is associated with both primary hypertension (5%–16% of NF‐1 adults) and secondary hypertension due to vascular lesions such as renal artery stenosis (2% prevalence). In children with renal artery stenosis, 11%–26% have NF‐1, suggesting a need to screen for NF‐1 in this population. In addition, patients with NF1 are at increased risk for pheochromocytomas (0.1%–5.7% prevalence), which could lead to hypertension.
The hospitalist should be aware of secondary causes of hypertension in young adults. Key components to the workup and management of an adolescent with hypertension include: (1) a detailed history to evaluate severity and possible secondary causes, lifestyle, family history of cardiac risk factors, and medication or illicit drug use; (2) physical exam focused on the cardiovascular system and signs of secondary causes such as abdominal bruits, thyromegaly, or café au lait spots; (3) laboratory/imaging evaluation including urinalysis, renal ultrasound, and echocardiography; and (4) management including dietary and exercise changes and pharmacological treatment for patients with stage 1 or 2 hypertension. NF‐1 renal lesions respond poorly to surgical therapy or angioplasty, thus medical management is preferred.
To cite this abstract:Le P, Peebles C, Nair A. Eighteen‐Year‐Old Man with Neurofibromatosis and Hypertension. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 486. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/eighteenyearold-man-with-neurofibromatosis-and-hypertension/. Accessed March 29, 2020.