[Renovascular hypertension in a child with type 1 neurofibromatosis]
Amira Peco-Antić1, Zoran Krstić, Zivko Borić
1University Children's Hospital, Belgrade. amipecob@eunet.yu
Insights
Renal autotransplantation successfully treated severe bilateral renal artery stenosis in a pediatric patient with neurofibromatosis type 1 when other methods failed. This procedure preserved kidney function and improved blood pressure control.
Area of Science:
- Pediatric Nephrology
- Vascular Surgery
- Genetics
Background:
- Neurofibromatosis type 1 (NF 1) can cause arterial hypertension in children, often due to renal artery stenosis (RAS).
- RAS in NF 1 typically affects the proximal renal artery and can be challenging to treat with standard methods like medication or angioplasty.
- Severe or bilateral RAS may necessitate advanced surgical interventions when conservative and interventional treatments are insufficient.
Observation:
- A 5-year-old girl with NF 1 presented with hyponatremic hypertensive syndrome due to severe bilateral RAS.
- The patient had an occluded right proximal renal artery and 80% stenosis of the left ostial renal artery.
- Standard antihypertensive drugs and percutaneous transluminal angioplasty (PTA) were ineffective; ACE inhibitor therapy induced acute renal failure.
Findings:
- Renal autotransplantation was performed on the right kidney, successfully preserving its function.
- The procedure led to improved blood pressure control in the patient.
- This case highlights the potential of renal autotransplantation for complex renovascular hypertension in pediatric NF 1.
Implications:
- Renal autotransplantation offers a viable option for managing refractory renovascular hypertension in children with NF 1.
- This case underscores the importance of considering surgical solutions beyond PTA and medical management for specific pediatric vascular conditions.
- The successful outcome suggests that autotransplantation can be a life-saving intervention, particularly when dealing with challenging anatomical and pathological features of RAS in NF 1.
Abstract:
Arterial hypertension in pediatric patients with neurofibromatosis type 1 (NF 1) is usually due to renal artery stenosis (RAS) mainly involving the proximal part of the vessel. The treatment modalities are highly individualized. In severe and/or bilateral RAS, antihypertensive drugs are either ineffective or have the potential risk for acute renal failure, while percutaneous transluminal angioplasty (PTA) has limited success due to the ostial localization of RAS and the tough fibrotic tissue involved that is refractory to dilatation Renal autotransplantation has potential advantages when medical control and PTA/or bypass techniques failed. Here we report 5 year-old girl with NF 1 and hyponatremic hypertensive syndrome due to severe bilateral disease, occluded proximal part of the right artery and ostial stenosis (80%) of the left one. Only left kidney was identified on 99 m Tc DTPA, but the right one was visualized on the renal ultrasonography and in the late phase of arterial renography due to well developed collateral circulation. Multiple antihypertensive drugs (nifedipine, labetolol and minoxidil) in maximal doses and PTA failed to normalize BP while short term therapy with ACEI with NF1 and hyponatremic hypertensive syndrome due to severe bilateral renovascular disease; occluded proximal part of the right renal artery and ostial stenosis (80%) of the left one. Only left kidney was identified on 99 m Tc DTPA, but the right one was visualized on the renal ultrasonography and in the late phase of arterial renography due to well developed collateral circulation. Multiple antiphypertensive drugs (nifedipine, labetolol and minoxidil) in maximal doses and PTA failed to normalize BP while. short term therapy with ACEI, captopril induced transient acute renal failure. Autotransplantation of right kidney saved its function and improved BP control. Our current case Autotransplantation of right kidney saved its function and improved BP control. Our current case is illustrative for a difficult management of renovascular hypertension in children with NF1. This is the first and up to now the only case of autotransplantation performed in Yugoslavia.
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