Abstract: SA-PO0665
Nutcracker Syndrome in an 11-Year-Old Girl with ADPKD
Session Information
- Pediatric Nephrology: Transplantation, Hypertension, AKI, Genetics, and Developmental Diseases
November 08, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1900 Pediatric Nephrology
Authors
- Van Cleve, Margaret Drew, University of South Florida Morsani College of Medicine, Tampa, Florida, United States
- Markwood, Jennifer, USF Health, Tampa, Florida, United States
- Ayestaran Cassani, Frank W., Johns Hopkins All Children's Hospital, St. Petersburg, Florida, United States
Introduction
With an estimated prevalence of 9.3 /10,000 in the US, Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common genetic renal disease and accounts for 5%-10% of kidney failure in the US and Europe. While individuals are often asymptomatic until adulthood, disease course is characterized by progressive kidney enlargement and eventual end stage renal disease. Entrapment of the left renal vein between the superior mesenteric artery and the abdominal aorta, known as Nutcracker Syndrome (NCS), is a rare occurrence in the pediatric population but is associated with significant morbidity. The incidence of NCS in individuals with ADPKD is not well documented.
Case Description
An 11-year-old female with history significant for ADPKD presented to her pediatrician for flank pain and hematuria. Urinalysis (UA) showed “large amounts of blood and some tissue” for which she was prescribed Cefdinir, and a renal US revealed new renal cysts and enlargement of previous cysts. She subsequently presented to the ED 6 days later for continued flank pain. UA at that time showed large blood, trace leukocyte esterase (LE), 6-10 WBC, and 3-5 RBC. She was again diagnosed with a UTI and discharged on Cephalexin. On follow up with nephrology, UA showed trace blood and trace protein with negative LE, but her flank pain persisted. She was admitted to the hospital for CT abdomen and pelvis. CT was significant for “numerous non obstructing variably sized cystic areas within bilateral kidneys.” Interventional Radiology was involved with her medical management and raised concern for NCS, which was confirmed on renal venogram. Her pain continued and she eventually underwent celiac plexus nerve block with significant improvement in pain.
Discussion
Early diagnosis and treatment of renal pathology in individuals with ADPKD is critical in preserving renal function for as long as possible. When other pathologies that mimic known diagnoses arise, there is risk of delayed or missed diagnosis. In this case, flank pain and hematuria were initially suspected to be caused by an enlarging, infected, or hemorrhagic cyst. Treatment of NCS in patients with ADPKD should involve an individualized approach and may require a lower threshold for more aggressive management, such as endovascular stenting or surgical intervention, as well as closer monitoring to preserve kidney function.