Renal Arteriovenous Malformation, Cogenital

Inborn

Step 1: View clinicals

A 32 year old man presents following the passage of dark red urine with blood clots, soon after his regular morning jog. He also complains of a feeling of fullness in the left flank. There is no history of trauma, or of other urinary symptoms. Nor is there a family history of bleeding disorders. His smoking history amounts to 5 pack years. He only drinks socially, and does not use recreational drugs. When a urine sample is requested, he complains of difficulty voiding, and can only partially empty his bladder. Urinalysis demonstrates a field full of red blood cells, with no cell casts or pus cells. A full blood count is significant for a hemoglobin level of 10.1 g/dL. A renal function assay is within normal parameters.


Step 2: Order all relevant investigations

Coagulation Profile

aPTT: 32 sec (30-40) PT: 10 sec (10-12) INR: 1.0 (0.9-1.1)

Renal + Bladder Ultrasound

There is an echogenic, mobile, round mass approximately 2cm in diameter in the bladder, along with a large amount of debris. The kidneys appear normal, and there is no evidence of urinary calculi.

Flexible Cystoscopy

Cystoscopy reveals a large blood clot within the bladder lumen. Small fragments of blood clots are also seen emanating from the left ureteric orifice. The bladder walls appear normal. Ureteropyeloscopy of the left side demonstrates similar fragments throughout the ureter, and a large organized blood clot in the lower moiety of the left renal pelvis, overlying and erythematous and vascular urothelium. Cytology of the urine and washings from left ureter and renal pelvis is negative for tumor cells. Histological examination of biopsy specimens from the blood clots reveal no evidence of malignancy.

CT Angiography

CT Angiography demonstrates multiple localized tortuous vascular channels in the lower pole of the left kidney, in association with early filling of the left renal vein.


Step 3: Select appropriate management

Urgent Catheterization
Antibiotic Prophylaxis
L/s Partial Nephrectomy
Transcatheter Arterial Embolization


Score: ★★☆