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Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea

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Abstract

Summary

We described six uremic leontiasis ossea (ULO) patients who underwent total parathyroidectomy with autotransplantation. ULO demonstrated more a systemic disease than a simple craniofacial deformation. The surgery seemed an effective treatment to alleviate secondary hyperparathyroidism and to improve patients’ quality of life. ULO may have a high postoperative recurrence tendency.

Introduction

ULO is a rare disease derived from uremic secondary hyperparathyroidism (SHPT). Previous studies mostly focused on the craniofacial deformations. This study aims to investigate the systemic features of the disease and the surgical outcomes.

Methods

The present study retrospectively assessed six ULO patients who underwent total parathyroidectomy (TPTX) with autotransplantation (AT). Follow-up data were recorded. The follow-up status was considered as “effectiveness” if serum intact parathyroid hormone (iPTH) levels were <150 pg/mL in the first 3 days after surgery, or as “recurrence” if serum iPTH gradually increased >300 pg/mL during follow-up in patients whose status was initially considered as “effectiveness”.

Results

Craniofacial deformations, short stature, thoracocyllosis, spine malformations, osteodynia, and muscle weakness were observed in all patients. Abnormal pulmonary functions were observed in five patients. After surgery, one patient died from respiratory failure. Surgery was effective in the remaining five patients with relieved osteodynia and stopped craniofacial deformation. A mean follow-up of 7.6 (4 to 12) months was available. Three patients suffered from recurrence of hyperparathyroidism originating from autografts.

Conclusions

Our data suggests that ULO is not only a simple disease with craniofacial malformations but is a severe systemic disease leading to increased surgical risks. TPTX with AT seems an effective treatment to relieve SHPT and to improve quality of life. ULO may have a high postoperative recurrence tendency.

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Acknowledgments

This work was funded by the National Natural Science Foundation of China (Grant No. 81170660) and the Priority Academic Program Development (PAPD) of Jiangsu Higher Education Institutions.

Conflicts of interest

All the authors declared no competing interests.

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Authors and Affiliations

  1. Department of Nephrology, First Affiliated Hospital of Nanjing Medical University, 300# Guangzhou Road, Nanjing, Jiangsu, 210029, China

    G. Yang, B. Zhang, N-N. Wang & C-Y. Xing

  2. Department of General Surgery, First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China

    X-M. Zha

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  1. G. Yang
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  2. B. Zhang
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  3. X-M. Zha
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  4. N-N. Wang
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Corresponding author

Correspondence to N-N. Wang.

Additional information

N-N. Wang and C-Y. Xing contributed equally to this paper.

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Yang, G., Zhang, B., Zha, XM. et al. Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea. Osteoporos Int 25, 1115–1121 (2014). https://doi.org/10.1007/s00198-013-2488-1

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  • DOI: https://doi.org/10.1007/s00198-013-2488-1

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