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Posterior nutcracker phenomenon with Down syndrome
Kidney Res Clin Pract xxx (2015) 1e2
Kidney Research and Clinical Practice journal homepage: http://www.krcp-ksn.com Contents lists available at ScienceDirect
Images in Practice
Posterior nutcracker phenomenon with Down syndrome Eun Sil Koh 1, Min Young Kim 2, Yoon Sik Chang 2, Sungjin Chung 2, * 1 2
Department of Cellular & Integrative Physiology, College of Medicine, University of Nebraska Medical Center, Omaha, NE, USA Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
Article history: Received 2 September 2015 Received in revised form 30 September 2015 Accepted 1 October 2015 Available online xxx Keywords: Anemia Down syndrome Hematuria Nutcracker syndrome
The patient was a 24-year-old man with a history of Down syndrome who presented himself with a 9-year history of intermittent gross painless hematuria with anemia. There were no external signs of trauma on physical examination except a
pale appearance. Laboratory investigations revealed the hemoglobin and hematocrit levels were 6.2 g/dL and 22.5%, respectively. Although his family witnessed another episode of gross hematuria few weeks before admission, urinalysis on admission showed no evidence of hematuria. An initial abdominal ultrasonography showed normal size and echogenicity of both kidneys without definite focal lesion. However, computed tomography of the abdomen revealed entrapment of a retroaortic left renal vein between the aorta and the vertebral column (Fig. 1), indicating posterior nutcracker syndrome. After consultation, it was decided that he should undergo conservative follow-up. The retroaortic left renal vein, also known as posterior nutcracker syndrome, is a rare congenital phenomenon. Because patients with Down syndrome usually have a decreased risk for vascular anomalies because of increased vascular endothelial growth factor inhibitors from an extra copy of chromosome 21, posterior nutcracker syndr-
Figure 1. Abdominal CT of the patient. (A) The arrowheads indicate dilated left renal vein at the renal hilum. (B) The arrowheads indicate the passing of the left renal vein between the aorta and the vertebral body. CT, computed tomography.
* Corresponding author. Division of Nephrology, The Catholic University of Korea Yeouido St. Mary’s Hospital, 10, 63-ro, Yeongdeungpo-gu, Seoul 07345, Korea. E-mail address: [email protected] (S Chung). http://dx.doi.org/10.1016/j.krcp.2015.10.001 2211-9132/Copyright © 2015. The Korean Society of Nephrology. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Koh ES, et al., Posterior nutcracker phenomenon with Down syndrome, Kidney Research and Clinical Practice (2015), http://dx.doi.org/10.1016/j.krcp.2015.10.001
2
Kidney Res Clin Pract xxx (2015) 1e2
ome comorbid with Down syndrome is especially a rare diagnosis.
review. E.S.K. and S.C. were major contributors in writing the manuscript. All authors read and approved the final manuscript.
Conflicts of interest Acknowledgments All authors have no conflicts of interest to declare.
Authors' contributions
This report was approved by The Catholic University of Korea Yeouido St. Mary's Hospital Institutional Review Board (No. SC13ZISE0084).
E.S.K., M.Y.K., Y.S.C., and S.C. were involved in the day-to-day patient care. E.S.K., M.Y.K., and S.C. performed the literature
Please cite this article in press as: Koh ES, et al., Posterior nutcracker phenomenon with Down syndrome, Kidney Research and Clinical Practice (2015), http://dx.doi.org/10.1016/j.krcp.2015.10.001
Kidney Research and Clinical Practice journal homepage: http://www.krcp-ksn.com Contents lists available at ScienceDirect
Images in Practice
Posterior nutcracker phenomenon with Down syndrome Eun Sil Koh 1, Min Young Kim 2, Yoon Sik Chang 2, Sungjin Chung 2, * 1 2
Department of Cellular & Integrative Physiology, College of Medicine, University of Nebraska Medical Center, Omaha, NE, USA Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
Article history: Received 2 September 2015 Received in revised form 30 September 2015 Accepted 1 October 2015 Available online xxx Keywords: Anemia Down syndrome Hematuria Nutcracker syndrome
The patient was a 24-year-old man with a history of Down syndrome who presented himself with a 9-year history of intermittent gross painless hematuria with anemia. There were no external signs of trauma on physical examination except a
pale appearance. Laboratory investigations revealed the hemoglobin and hematocrit levels were 6.2 g/dL and 22.5%, respectively. Although his family witnessed another episode of gross hematuria few weeks before admission, urinalysis on admission showed no evidence of hematuria. An initial abdominal ultrasonography showed normal size and echogenicity of both kidneys without definite focal lesion. However, computed tomography of the abdomen revealed entrapment of a retroaortic left renal vein between the aorta and the vertebral column (Fig. 1), indicating posterior nutcracker syndrome. After consultation, it was decided that he should undergo conservative follow-up. The retroaortic left renal vein, also known as posterior nutcracker syndrome, is a rare congenital phenomenon. Because patients with Down syndrome usually have a decreased risk for vascular anomalies because of increased vascular endothelial growth factor inhibitors from an extra copy of chromosome 21, posterior nutcracker syndr-
Figure 1. Abdominal CT of the patient. (A) The arrowheads indicate dilated left renal vein at the renal hilum. (B) The arrowheads indicate the passing of the left renal vein between the aorta and the vertebral body. CT, computed tomography.
* Corresponding author. Division of Nephrology, The Catholic University of Korea Yeouido St. Mary’s Hospital, 10, 63-ro, Yeongdeungpo-gu, Seoul 07345, Korea. E-mail address: [email protected] (S Chung). http://dx.doi.org/10.1016/j.krcp.2015.10.001 2211-9132/Copyright © 2015. The Korean Society of Nephrology. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Koh ES, et al., Posterior nutcracker phenomenon with Down syndrome, Kidney Research and Clinical Practice (2015), http://dx.doi.org/10.1016/j.krcp.2015.10.001
2
Kidney Res Clin Pract xxx (2015) 1e2
ome comorbid with Down syndrome is especially a rare diagnosis.
review. E.S.K. and S.C. were major contributors in writing the manuscript. All authors read and approved the final manuscript.
Conflicts of interest Acknowledgments All authors have no conflicts of interest to declare.
Authors' contributions
This report was approved by The Catholic University of Korea Yeouido St. Mary's Hospital Institutional Review Board (No. SC13ZISE0084).
E.S.K., M.Y.K., Y.S.C., and S.C. were involved in the day-to-day patient care. E.S.K., M.Y.K., and S.C. performed the literature
Please cite this article in press as: Koh ES, et al., Posterior nutcracker phenomenon with Down syndrome, Kidney Research and Clinical Practice (2015), http://dx.doi.org/10.1016/j.krcp.2015.10.001