Author Affiliations: Department of Pediatrics, Kinki University School of Medicine, Osaka, Japan (Koshimichi M, Sugimoto K, Yanagida H, Fujita S, Miyazawa T, Sakata N, Okada M, Takemura T)

Corresponding Author: Tsukasa Takemura, MD, PhD, Department of Pediatrics, Kinki University School of Medicine 377-2 Ohno-higashi, Osaka-Sayama 589-8511, Japan (Tel: +11-81-72-366-0221; Fax: +11-81-72-368-1566; Email: tsukasa@med.kindai.ac.jp)

doi: 10.1007/s12519-012-0349-1

Background: In addition to the urinary abnormalities, symptoms of left renal vein entrapment between the aorta and superior mesenteric artery (left renal vein entrapment syndrome, LRVES) may include abdominal and flank pain as well as chronic fatigue. We investigated various LRVES symptoms in this study.

Methods: In 53 pediatric LRVES patients treated at our department, 22 had a score of 5 points or higher on orthostasis. Initial evaluation of LRVES by abdominal ultrasonography showed a stenotic-to-prestenotic vein diameter ratio of 0.2 or less. Definitive diagnosis was made by computed tomography and magnetic resonance angiography. Cortisol, catecholamine (CA), and brain natriuretic peptide (BNP) were also measured.

Results: The frequency of LRVES was 2.5 times higher in girls than in boys. Low or very low body mass indexes were seen in both sexes. The most common initial finding was urine abnormalities, followed by dizziness and malaise. In 6 patients, orthostasis precluded school attendance. Ten patients had orthostasis scores above 12. Patients unable to attend school had either low levels of plasma or urinary cortisol. Midodrine significantly decreased orthostasis scores. Some patients required treatment with fludrocortisone. Plasma CA, renin, and BNP levels were all normal.

Conclusions: Locally excessive venous pressure may cause reversible adrenal dysfunction with transitory Addisonian symptoms. Children with cryptogenic malaise or severe orthostasis should be evaluated for LRVES.

Key words: abdominal and flank pain; dizziness; hematuria; nutcracker syndrome; pre-stenotic dilation; proteinuria

                   World J Pediatr 2012;8(2):116-122