We searched the Cochrane Library and Medline with the search terms: “renovascular hypertension”, “renovascular disease”, “renal artery stenosis”, and “infants”, “children”, and “adolescents”. We selected all publications that we judged relevant to our topic but gave priority to those published in the past 10 years. We also searched our own files and the reference lists of articles that were recovered. Searches were not restricted by language.
SeminarRenovascular hypertension in children
Introduction
Renovascular disease is an uncommon but important cause of childhood hypertension because it is potentially amenable to curative treatment. In this Seminar, we review current knowledge of renovascular disease in children; its cause, pathogenesis, and associations; diagnostic methods; and medical, interventional, and surgical treatment.
Section snippets
Prevalence
Renovascular disease causes 5–10% of all childhood hypertension.1, 2 The causes and management of renovascular hypertension in children differ greatly from those in adults, in whom the disorder is mostly related to atherosclerosis.3 Before the recent epidemic of childhood obesity, most cases of confirmed childhood hypertension were secondary to other diseases, with most being renal in origin.1, 2, 4
Causes of renovascular hypertension
Various diseases are associated with childhood renovascular hypertension (panel 1). Findings of international reports indicate strikingly different pathologies with varying diagnostic patterns, which not only could suggest genuine differences between causes of renovascular disease throughout the world but also highlights the major difficulties in defining diagnostic criteria (see section How to diagnose renovascular hypertension).
Clinical spectrum
Renal artery stenosis is bilateral in 53–78% of childhood cases of renovascular disease.10, 16, 18 Deal and colleagues10 reported paediatric renovascular hypertension associated with intrarenal disease alone in 44% and renovascular hypertension associated with a combination of intrarenal disease and main artery stenosis in 31%. Most children without any of the above-mentioned comorbid disorders have a single, focal, branch artery stenosis.42, 43 Concomitant stenoses of other arteries are also
How to diagnose renovascular hypertension
Diagnosis of childhood hypertension is usually delayed because children infrequently undergo routine blood-pressure measurement owing to the scarcity of screening programmes and difficulties in measuring and interpreting their blood-pressure findings. Assessment of blood pressure requires consideration of the child's age, sex, and height centile.44 Unfortunately, some children are referred with a history of very high blood-pressure readings recorded over several years without any clinical
When should renovascular hypertension be suspected?
Panel 2 shows symptoms and clinical signs that increase the chance that diagnosed hypertension is renovascular in origin. In 26–70% of paediatric cases of renovascular disease, hypertension is an incidental finding in an asymptomatic child.10, 17, 19, 45, 46 However, children with renovascular hypertension can present with severe symptoms attributable to target-organ damage, such as an acute cerebral incident (10–15%) or heart failure (7%).10, 16, 18, 19 Bell's palsy is a recognised
Investigations
Guidelines have been published for investigation of children with established hypertension.44 We have summarised our suggestions in a clinical pathway (figure 2). Hyperaldosteronism with excessive amounts of renin activity in the blood and hypokalaemia (or low to low-normal potassium concentrations) can increase the likelihood of renovascular hypertension. Findings in adults have also suggested that stimulation of renin response by treatment with an angiotensin-converting-enzyme inhibitor
Imaging
The diagnosis of renovascular hypertension can be established with several different imaging modalities. The precise role of non-invasive imaging in children with suspected renovascular hypertension is unclear. At present, no technique exists that can reliably exclude every potentially treatable cause of renovascular hypertension.43 For this reason, these investigations are usually restricted to three aims: for patients in whom the need for angiography is uncertain; for follow-up; and to
Renal vein renin sampling
Measurement of renal vein renin concentrations seems to be a useful technique in children, especially those with renal artery stenosis that is bilateral, segmental, or both. However, a general anaesthetic is sometimes needed for renal vein renin sampling, which is usually undertaken at the same time as diagnostic angiography with a femoral vein approach.48, 74 Blood samples are taken from the infrarenal inferior vena cava and the main renal veins and their larger intrarenal tributaries. Renal
Treatment
Figure 5 summarises our suggested treatment pathway for renovascular hypertension. After confirmation of hypertension, all children should be treated with antihypertensive drugs. These patients generally present with very high blood pressure, of which slow reduction is important. As in adults, treatment typically includes a calcium-channel blocker, a β blocker, or both. Details of such treatment regimens are outside the scope of this Seminar. As mentioned above, one of the characteristics of
Renal angioplasty
When blood-pressure control is inadequate or is associated with significant adverse effects, endovascular treatment can be attempted (figure 5). Percutaneous transluminal renal angioplasty (PTRA), with or without stenting, is a valuable treatment option in paediatric renal artery stenosis.80 This method is usually undertaken from a femoral approach, with either a long vascular sheath or a guiding catheter.79 Angioplasty equipment designed for use in adult coronary arteries is ideal for the
Surgical procedures
Primary indications for surgical intervention are refractory hypertension for which medical treatment and angioplasty have failed. The surgical treatment can be revascularisation or nephrectomy.
Outcome of interventional angiography treatment
PTRA in children has given rise to very different results, with 28–94% of treated children achieving cure or improvement in studies.26, 46, 103, 104, 105, 106, 107, 108, 109 This range was noted in an overview of ten published studies with a total of 163 children.19
Many reasons exist for these very divergent results. Different diagnostic patterns were reported in these studies, with most children assumed to have not only fibromuscular dysplasia but also inflammatory disease, such as Takayasu's
Conclusions
Renovascular hypertension in children is a severe disease, usually with complicated comorbidity. Combined treatment with angioplasty, surgery, and drugs offers many patients hope for a good long-term outcome.
Search strategy and selection criteria
References (110)
- et al.
Hypertension in pediatric patients
Am J Kidney Dis
(1996) - et al.
Multivisceral fibromuscular dysplasia in childhood: case report and review of the literature
Ann Vasc Surg
(2000) - et al.
Abdominal aortic coarctation, renovascular, hypertension, and neurofibromatosis
Ann Vasc Surg
(2002) - et al.
Renovascular disease in childhood
J Pediatr
(1992) - et al.
Diagnosis and management of stenotic aorto-arteriopathy in childhood
J Pediatr
(1998) Neonatal hypertension
Pediatr Clin North Am
(1978)- et al.
Renal transplantation in children
Transplant Proc
(2006) - et al.
Renovascular hypertension in children
Pediatr Clin North Am
(1993) - et al.
Renovascular hypertension in childhood: a changing perspective in management
J Pediatr
(1985) - et al.
Doppler sonography in renal artery stenosis: an evaluation of intrarenal and extrarenal imaging parameters
Clin Imaging
(2003)
Color duplex ultrasonography in detecting renal artery abnormalities in a patient with neurofibromatosis 1: a case report
Clin Imaging
Evaluation of duplex ultrasound and captopril renography for detection of renovascular hypertension
Kidney Int
Coarctations of descending and abdominal aorta: long-term results of surgical therapy
J Vasc Surg
[Coarctation of the abdominal aorta: treatment by stent placement]
Rev Esp Cardiol
Segmental renal artery embolization for treatment of pediatric renovascular hypertension
J Vasc Interv Radiol
Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children
J Vasc Surg
Interventional radiology for renovascular hypertension in children
Tech Vasc Interv Radiol
Percutaneous renal revascularization in children and adolescents with renovascular hypertension
Tech Vasc Interv Radiol
Midaortic syndrome associated with fetal alcohol syndrome
J Vasc Interv Radiol
Cutting balloon angioplasty in children with resistant renal artery stenosis
J Vasc Interv Radiol
Coarctation of the abdominal aorta
Ann Vasc Surg
Staged reconstruction for middle aortic syndrome
J Pediatr Surg
Analysis of 100 children with severe and persistent hypertension
Arch Dis Child
A single pediatric center experience with 1025 children with hypertension
Acta Paediatr
Etiology of sustained hypertension in children in the southwestern United States
Pediatr Nephrol
Ruptured superior cerebellar artery aneurysm in a child with bilateral fibromuscular hyperplasia of the renal arteries: case report and review of the literature
J Neurosurg
Fibromuscular dysplasia
N Engl J Med
Renovascular hypertension in Marfan syndrome
Pediatr Nephrol
Hypertension secondary to progressive vascular neurofibromatosis
Arch Dis Child
Mid-aortic syndrome presenting in childhood
Br J Surg
Renal artery stenosis and aneurysmatic dilatation of arteria carotis interna in tuberous sclerosis complex
Pediatr Nephrol
[Klippel-Trenaunay syndrome with systemic hypertension and chronic renal failure]
Ann Pediatr (Paris)
Management of severe hypertension in a child with tuberous sclerosis-related major vascular abnormalities
J Hypertens
Clinical spectrum of intrinsic renovascular hypertension in children
Pediatrics
Renovascular hypertension in children
Scand J Urol Nephrol
Evaluation and long-term outcome of pediatric renovascular hypertension
Pediatr Nephrol
Angioplasty for renovascular hypertension in children: 20-year experience
Pediatrics
Renal vascular disease in neurofibromatosis type 2: association or coincidence?
Dev Med Child Neurol
Renovascular disease and hypertension in children with neurofibromatosis
Pediatr Nephrol
Kawasaki disease complicated by renal artery stenosis
Arch Dis Child
Sustained hypertension in children
Indian Pediatr
Malignant hypertension in children in India
Nephrol Dial Transplant
Angiographic features of 26 children with Takayasu's arteritis
Pediatr Radiol
Percutaneous transluminal angioplasty for renovascular hypertension in children: initial and long-term results
Pediatrics
The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis
Arthritis Rheum
EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides
Ann Rheum Dis
Pheochromocytoma and renovascular hypertension: a case report on a review of the literature
Acta Med Scand
Phaeochromocytoma: investigation and management of 10 cases
Arch Dis Child
Arterial hypertension developing 10 years after radiotherapy for Wilms's tumour
Br Med J (Clin Res Ed)
Renovascular hypertension in the pediatric patient
Arch Surg
Cited by (202)
Malignant arterial hypertension in a 2-month-old girl: Etiological diagnosis and treatment
2022, Archives de PediatrieHistologic and morphologic character of pediatric abdominal aortic developmental coarctation and hypoplasia
2022, Journal of Vascular SurgeryPediatric Renovascular Hypertension: Not Just a “String of Beads”
2022, Journal of Vascular and Interventional RadiologyMidterm Outcomes of Angioplasty for Pediatric Renovascular Hypertension
2022, Journal of Vascular and Interventional RadiologyNeonatal Hypertension
2022, Clinics in Perinatology