Sixty-Two-Year-Old Male Suffering From Uremic Leontiasis Ossea Caused by Severe Secondary Hyperparathyroidism.

Yogendranath Purrunsing,Yi Xu,Wei Liu,Changying Xing,Ying Cui,Xiaoming Zha,Jingjing Zhang,Xunning Hong,Ningning Wang

doi:10.1002/jbm4.10038

Yogendranath Purrunsing, Yi Xu + Show 7 more

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https://doi.org/10.1002/jbm4.10038

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Journal: JBMR plus	Publication Date: Mar 30, 2018
Citations: 4	License type: CC BY 4.0

Affiliation: Nanjing Medical University

Abstract

ABSTRACTSecondary hyperparathyroidism (SHPT) is a long‐term complication of chronic kidney disease–mineral and bone disorder (CKD‐MBD). SHPT is characterized by hyperplasia of the parathyroid glands and abnormal secretion of parathyroid hormones (PTH), calcium and phosphorous metabolic disorders, renal osteodystrophy, vascular and soft tissue calcification, malnutrition, and other multiple system complications, which can seriously affect the quality of life of the patient and increase the risk of cardiovascular disease and mortality rate. Uremic leontiasis ossea (ULO) is a medical condition only rarely encountered clinically. SHPT causes craniofacial bone deformity accompanied by lesions of the nerve, cardiovascular, respiratory, bone, or other systems within the body. The case discussed here is related to severe SHPT. A 62‐year‐old male patient was suffering from leontiasis ossea, pectus excavatum, vascular calcification, spontaneous bone fractures, and lower limb deformities. He was undergoing hemodialysis and given total parathyroidectomy (TPTX) with autotransplantation (AT). We further analyzed the multivariate therapeutic effects of TPTX on this patient in order to provide clinical data for standardized treatment of individuals with CKD‐MBD. © 2018 The Authors JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.

Highlights

Leontiasis ossea, known as leontiasis or lion face, is a form of severe bone remodeling that prevails in patients with chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPT), renal osteodystrophy, Paget diseases, and fibrous dysplasia characterized by craniofacial, ribs, long bones, and spine deformations.[1,2] Virchow in 1864 first described leontiasis in a patient as the thickening of facial bones.[3]. In 1953, Cohen reported uremic leontiasis ossea (ULO) to be correlated with SHPT.[4]. This malady increases the size of maxillary bones, which expand into the skull sinuses, affecting the ocular, auditory, nasal, and oral areas, causing acute visual deterioration, bilateral optic nerve palsy, bilateral deafness, dysphagia, and dyspnea.[5]
Once the patient was referred to our hospital, we evaluated his case and proposed PTX as according to the Kidney Disease Improving Global Outcomes (KDIGO) guidelines; when a CKD patient with SHPT is experiencing biochemical, radiological, and cardiovascular irregularities and does not respond to medical/pharmacological therapy, parathyroidectomy is recommended.[14]
Uncontrolled SHPT led to renal osteodystrophy, the CT scan (Fig. 4) revealed the compression fractures of the thoracolumbar vertebras and Fig. 1C showed the affected lower limbs, all these combined factors led to the patient’s decreased of height

Summary

Introduction

Leontiasis ossea, known as leontiasis or lion face, is a form of severe bone remodeling that prevails in patients with chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPT), renal osteodystrophy, Paget diseases, and fibrous dysplasia characterized by craniofacial, ribs, long bones, and spine deformations.[1,2] Virchow in 1864 first described leontiasis in a patient as the thickening of facial bones.[3]. The present case, a male patient suffering from ULO, is a rare form of severe bone remodeling due to uncontrolled SHPT underwent total parathyroidectomy (TPTX) with autotransplantation (AT).(6)

Results

Conclusion