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Revista Cubana de Pediatría
On-line version ISSN 1561-3119
Abstract
DURAN ALVAREZ, Sandalio; ABIJANA RODRIGUEZ, María Isabel; PENA MORALES, Andrea Carolina and DIAZ ZAYAS, Niurka. Nutcracker Syndrome and Right Renal Agenesis. A Rare Association. Rev Cubana Pediatr [online]. 2023, vol.95 Epub Mar 01, 2023. ISSN 1561-3119.
Introduction:
Nutcracker syndrome is considered an infrequent and poorly thought out anomaly in medical practice; its incidence is not well established due to its varied symptomatology. Unilateral renal agenesis is estimated to be between 1/2500 and 1/4000 live births.
Objectives:
To describe an 18-year-old female patient with suspected pre-natal imaging-confirmed right renal agenesis after birth, who was admitted due to proteinuria.
Case presentation:
An 18-year-old female patient with known right renal agenesis, in whom a urine test detected proteinuria from the age of 13. In the study it was classified as orthostatic proteinuria and evolutionarily she referred mild and transient left low back pain. Renal ultrasound, performed to assess single kidney growth, showed dilation of the left renal vein. The ultrasound study was repeated to specify aorto-mesenteric angle and left renal vein dilation, and nutcracker syndrome was confirmed.
Conclusions:
The association between right renal agenesis and nutcracker syndrome is extremely rare, and, when it presents with orthostatic proteinuria and occasional low back pain, it should be followed expectantly, but surgical treatment is not needed in most cases.
Keywords : nutcracker syndrome; nutcracker phenomenon; orthostatic proteinuria; unilateral renal agenesis; single kidney.