AUTOTRANSPLANTATION WITH EXTRACORPORAL RECONSTRUCTION OF RENAL VESSELS: A CLINICAL CASE OF “THE NUTCRACKER SYNDROME” AND LEFT RENAL ARTERIES HYPOPLASIA COMBINATION

Abstract

Ex vivo kidney autotransplantation with renal artery reconstruction is an effective method of surgical correction of vascular abnormalities (fibromuscular dysplasia, hypoplasia, and renal artery aneurysms) showing good long-term results. The combination of renal artery abnormalities with aorto-mesenteric compression syndrome (“the nutcracker” syndrome) is extremely rare and complicates the diagnosis making greatly. Renal artery hypoplasia is an abnormality that is rarely diagnosed and may not manifest clinically in the absence of contralateral kidney pathology as well as its vascular structures. Renal hypoplasia may be caused by mutations in the kidney development genes (HNF1B, PAX2, PBX1) and/or several environmental factors such as intrauterine growth retardation, maternal diseases (diabetes, hypertension), medication taken by the mother (renin-angiotensin system inhibitors or nonsteroidal anti-inflammatory drugs) and intoxication (smoking and alcohol). Premature delivery (before the 36th week) is also a risk factor due to incomplete nephrogenesis. Renal arteries hypoplasia is associated with fibromuscular dysplasia in most cases. The disease is characterized by non-inflammatory non-uniform focal hyperplasia of the renal artery wall with damage to small and medium arteries. Simultaneous damage to two or more vascular segments is detected in 25% of patients.
 A clinical case of successful treatment of “the nutcracker” syndrome (annular renal vein) combined with hypoplasia of the left main and accessory renal arteries associated with fibromuscular dysplasia is described. A 27-year-old female was admitted to the Department of Vascular Surgery of Municipal Noncommercial Enterprise of the Lviv Regional Council “Lviv Regional Clinical Hospital” on October 4, 2021, with complaints of pain in the left lumbar region and the left half of the abdomen, hematuria, proteinuria, a persistent increase in blood pressure. Ultrasound of the renal veins: the left renal vein in the aorto-mesenteric segment was not visualized, the posterior portion of the left renal vein was determined. Peak systolic velocity was up to 230 cm/s at the point of confluence with the inferior vena cava and 28 cm/s more distally. The vein was up to 12.0 mm in the renal hilum and up to 11.0 mm in the middle third. MSCT of the abdominal cavity, retroperitoneal space and pelvic organs: CT signs of pyelonephritis of the left kidney. Hypoplasia of the left kidney. Reactive para-aortic lymphadenopathy on the left. Hypoplastic left renal artery (3,8 mm) and presense of accessory ones, annular left renal vein. Angiomyolipoma of the right kidney (2,6 cm). Varicose veins of the small pelvis on the left. The patient underwent surgical treatment, namely ex vivo autovenous prosthetic repair of the left renal artery with autotransplantation of the left kidney. The course of the postoperative period was without complications, the reconstruction was functioning. Ultrasound of the renal vessels (1 week after the surgery): the blood flow was located in the renal hilum in the course of Doppler sonography of intrarenal branches. Venous outflow was unobstructed. Corticomedullary differentiation was marked satisfactorily.
 The study presented by us demonstrates the effectiveness of kidney autotransplantation for the correction of renal vascular abnormalities.
 Though of a solitary nature, the combination of renal vessels abnormalities complicates significantly the course of the disease and the possibilities of this pathology diagnostics.