Congenital Renal Arteriovenous Malformation: Diagnostic Clues and Methods

Background and objectives: Renal arteriovenous malformation (AVM) is a rare disease and is difficult to be diagnosed by conventional methods because of its rarity. In this study, we investigated the diagnostic clues, and made an algorithm for the better diagnosis of renal AVM. Materials and Methods: We reviewed 13 patients who were diagnosed with AVM by using renal angiography from 1986 to 2020 at our institutes. We evaluated clinical features, diagnostic tools, treatment modalities, and outcomes after the treatment of patients. Results: All patients were female, and the mean age was 36.9 years (range 19 to 54 years). Twelve (92.3%) patients complained of gross hematuria. Four (30.8%) patients showed symptoms in relation with pregnancy and delivery. Angiographic findings demonstrated cirsoid type in 10 patients and aneurysmal type in 3 patients. Among the 11 patients who underwent computed tomography, AVMs were detected in 3 (27.3%) patients. Renal duplex Doppler was performed in 6 patients, and all of these patients were diagnosed with AVM, demonstrating a vascular turbulence or blood-rich area. Twelve patients were initially treated with transarterial embolization. Nephrectomy was performed in two patients due to persistent bleeding with hypovolemic shock. Conclusions: We should consider possible AVMs in patients who were not detected by conventional work up for hematuria, especially in mid-aged, pregnant, or recently delivered women. Renal duplex Doppler might be the optimal diagnostic modality in these patients. Our diagnostic algorithm could be aid to diagnosis and treatment for renal AVM patients.

Renal arteriovenous malformation and venous thrombosis: a tumour-like presentation

Renal arteriovenous malformation is a primarily congenital renal vascular abnormality. It is usually diagnosed incidentally on imaging, and the most common subtype is ‘cirsoid’, consisting of multiple, enlarged arterial feeders interconnecting with draining veins. We present a 74-year-old woman with an incidental finding of what was at first considered a hypervascularised kidney tumour but turned out to be a left intrarenal arteriovenous malformation associated with a left renal vein thrombosis. Selective endovascular embolisation was performed. The cause-consequence relationship between the arteriovenous malformation and the thrombosis is unique. To our knowledge, no such case has ever been reported.

Congenital renal arteriovenous malformation with cirsoid and cavernosal-type characteristics: a case report

Renal arteriovenous malformations (AVMs) are infrequent vascular morphological anomalies. About 20% of AVMs are congenital renal AVMs (CRAVMs). A 53-year-old female patient presented with a 5-day history of gross hematuria and right flank pain. The patient underwent the selective renal arteriography and embolization under local anesthesia. Renal computed tomography angiography (CTA) and digital subtraction angiography (DSA) results showed bleeding of the right renal arteriovenous malformation, both nidus and aneurysm, which indicated that the patient had both cirsoid and cavernosal types of CRAVM. Endovascular management was chosen to treat the patient. The patient was cured and discharged, then followed-up for 3 months. These results show that early identification using radiologic tests is important for diagnosis and treatment of CRAVM.

Congenital renal arteriovenous malformation: a rare cause of visible haematuria

We report a case of renal arteriovenous malformation (AVM) and describe its angioarchitecture and endovascular management. A 28-year-old male patient presented with visible painless haematuria. CT of the abdomen showed a right renal AVM. Digital subtraction angiography of the right renal vessels showed an AVM of middle and lower pole segmental arteries with communication to a large saccular aneurysm, which was arising from the right main renal vein. Complete occlusion of the AVM was done by using glue (a mixture of n-butyl-cyanoacrylate and lipiodol), resulting in nonvisualisation of the aneurysm on angiography. His vital signs were stable during the procedure. Follow-up CT after 12 months showed no residual flow in the aneurysm, normal upper pole renal parenchyma and nonvisualisation of AVM. Early diagnosis of this clinical entity is of paramount importance for proper management as it can cause massive blood loss and rapid clinical deterioration.