PEDIATRIC NEPHROLOGY, 2023 (SCI-Expanded)
Nutcracker syndrome in pediatrics: initial findings and long‑term
follow‑up results
Iryna Akdemir1 · Ece Mekik Akar2 · Songül Yılmaz2 · Nilgün Çakar2 · Suat Fitöz3 · Zeynep Birsin Özçakar2
Received: 12 June 2023 / Revised: 24 August 2023 / Accepted: 25 August 2023
© The Author(s), under exclusive licence to International Pediatric Nephrology Association 2023
Abstract
Background Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal
vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein,
we present our single-center experience with a large number of patients who have long-term follow-up results.
Methods All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were
obtained retrospectively.
Results A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10–14)
years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were
asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria
(16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination.
All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%)
within 35.8 ± 25.8 months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3 months.
The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly
when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation.
Conclusions In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents.
Our results support conservative management in children as the first-line treatment approach.