Renal Artery Stenosis and Mid-Aortic Syndrome in Children-A Review

Abstract

Background: Renal artery stenosis (RAS) and mid-aortic syndrome (MAS) are significant yet under-recognized causes of pediatric hypertension. RAS is characterized by the narrowing of the renal arteries, while MAS involves the stenosis of the abdominal aorta along with its associated vessels. The etiologies of RAS and MAS often involve genetic factors and acquired conditions such as fibromuscular dysplasia and Takayasu arteritis, contributing to their complex clinical presentations. Despite advancements in diagnostic imaging, challenges remain in effectively identifying these conditions. Pharmacological treatment can achieve partial blood pressure control, but it usually does not lead to complete recovery. Treatment options range from angioplasty to more definitive surgical interventions such as renal artery reimplantation and aorto-aortic bypass, tailored according to the specific pathology and extent of the disease. Methods: This review explores the diagnosis and management of RAS and MAS in children, highlighting the necessity for early detection and showcasing the evolving landscape of treatment. Conclusions: We advocate for a multidisciplinary approach that includes advanced imaging for effective diagnosis and tailored therapy. By integrating the latest research and clinical practices, this article provides valuable insights into managing complex vascular conditions in the pediatric population, ultimately aiming to enhance the quality of life for affected individuals.

Keywords: Takayasu arteritis; fibromuscular dysplasia; kidney disease; renal artery stenosis; renovascular hypertension.

Figure 1

Suggested diagnostic approaches to a pediatric patient with suspected renovascular hypertension.

Figure 2

Increased maximum flow velocity (~400 cm/s) in the place of abdominal aorta stenosis; analysis of an eight-year-old ♂ patient with MAS by spectral Doppler ultrasound.

Figure 3

Parvus et tardus waveform patterns in renal artery trunk; analysis of an eight-year-old ♂ patient with MAS by spectral Doppler ultrasound.

Figure 4

Increased maximum flow velocity (~200 cm/s) in the place of renal artery stenosis; analysis of an eleven-year-old ♂ patient with FMD by spectral Doppler ultrasound.

Figure 5

Parvus et tardus waveform patterns in intrarenal branches; analysis of an eleven-year-old ♂ patient with FMD by spectral Doppler ultrasound.

Figure 6

Stenosis of the abdominal aorta between the celiac trunk and renal arteries in a patient with MAS, analyzed by computed tomographic angiography.

Figure 7

Renal artery stenosis in a patient with FMD, analyzed by computed tomographic angiography.