Pediatric renovascular hypertension due to fibromuscular dysplasia presenting with refractory hypertension and status epilepticus.   A case study.

Gowda   Amruthraj G; R Vijayakumar; Dharwadkar   Sachin; Ansari  Adeel; A Vivek; Ghai  Gunjot  Singh

doi:10.51168/sjhrafrica.v7i2.2704

Authors

Gowda Amruthraj G Department of Urology, JSS Academy of Higher Education and Research (JSSAHER), JSS Medical College and Hospital, Mysuru, Karnataka, India.
R Vijayakumar Department of Urology, JSS Academy of Higher Education and Research (JSSAHER), JSS Medical College and Hospital, Mysuru, Karnataka, India.
Dharwadkar Sachin Department of Urology, JSS Academy of Higher Education and Research (JSSAHER), JSS Medical College and Hospital, Mysuru, Karnataka, India.
Ansari Adeel Department of Urology, JSS Academy of Higher Education and Research (JSSAHER), JSS Medical College and Hospital, Mysuru, Karnataka, India.
A Vivek Department of Urology, JSS Academy of Higher Education and Research (JSSAHER), JSS Medical College and Hospital, Mysuru, Karnataka, India.
Ghai Gunjot Singh Department of Urology, JSS Academy of Higher Education and Research (JSSAHER), JSS Medical College and Hospital, Mysuru, Karnataka, India.

DOI:

https://doi.org/10.51168/sjhrafrica.v7i2.2704

Keywords:

Refractoy hypertension, status epilepticus, fibromuscular dysplasia, renovascular hypertension

Abstract

Introduction

Renovascular hypertension is an uncommon but important cause of secondary hypertension in children and may result in severe neurological complications if diagnosis is delayed. Fibromuscular dysplasia is a recognized cause of renal artery stenosis in the pediatric population.

Case Presentation

A 5-year-old girl presented with recurrent episodes of status epilepticus and refractory hypertension despite treatment with three antihypertensive medications. Magnetic resonance angiography demonstrated left renal artery stenosis with juxta-ostial occlusion and distal recanalization. A radionuclide scan revealed a non-functioning hypoplastic left kidney contributing only 0.9% of total renal function. Balloon angioplasty was attempted but was unsuccessful. The patient subsequently underwent laparoscopic left nephrectomy. Histopathological examination demonstrated fibromuscular dysplasia involving the renal artery. The postoperative course was uneventful, with gradual improvement in blood pressure control and reduction in antihypertensive requirements during follow-up.

Conclusion

This case highlights the importance of investigating secondary causes of hypertension in children presenting with refractory hypertension and neurological manifestations. Laparoscopic nephrectomy may be an effective treatment option in selected patients with a non-functioning kidney due to renovascular disease.

Take-away Lessons

Early recognition of renovascular hypertension in children is essential to prevent complications. Comprehensive imaging and functional assessment aid diagnosis, while timely surgical intervention can achieve favorable outcomes in appropriately selected patients.

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