Uremic leontiasis ossea due to secondary hyperparathyroidism complicated by vitamin C deficiency in a non-adherent chronic hemodialysis patient: A case report

Abstract

Non-adherence to medical therapy in patients with end-stage kidney disease (ESKD) can lead to severe metabolic derangements rarely seen in the current medical era. Such complications may take the form of secondary hyperparathyroidism (HPT) leading to rare manifestations of bone mineral disease, and profound vitamin C deficiency from poor nutrition combined with removal of water-soluble vitamins during dialysis. Secondary HPT causes renal osteodystrophy which can lead to diffuse enlargement of the facial skeleton and morphological changes suggestive of leontiasis ossea. We report a 36-year-old, non-adherent woman on chronic dialysis for over 10 years who developed progressive, diffuse facial bone enlargement in the context of years of extreme HPT and newly diagnosed severe vitamin C deficiency. Imaging revealed diffuse hypertrophy of the maxillary and mandibular bones. Histopathology showed extensive fibro-osseous proliferation without evidence of Brown tumor, suggestive of uremic leontiasis ossea. In this report, we discuss the orofacial manifestations of secondary HPT and the possible potentiating role of vitamin C deficiency on the development of renal osteodystrophy through altered vitamin D metabolism. Non-adherent patients on chronic dialysis should be evaluated for vitamin C deficiency, and the development of uremic leontiasis ossea should be considered when such patients present with distortion of facial features in the context of severe secondary HPT.

Uremic Leontiasis Ossea in a Patient With End-Stage Renal Disease in Hemodialysis

“…In addition, decreased activation of Vitamin D in the kidney leads to increased PTH secretion. Excess PTH leads to unbalanced increased activity of osteoclasts and osteoblasts which results in increased unmineralized bone contributing to the ULO; the same pathogenesis is seen in renal osteodystrophy [2,4,5].…”

“…ESRD patient's non-adherent to the hemodialysis regimen and medical treatment are likely most susceptible to uremic leontiasis ossea. Such non-compliance to hemodialysis is also seen in our ESRD patient, leading up to his ULO presentation due to possible chronic metabolic derangements and secondary hyperparathyroidism [3][4][5].…”

Uremic leontiasis ossea: distinctive imaging features allow differentiation from other clinical causes of leontiasis ossea

“…Massicotte-Azarniouch et al. describe the morphological changes in ULO not limited to osseous changes but include soft tissue changes which eventually gives the lion-like facial appearance [15] . In 2004, Sagliker et al.…”

“…Hence, the diagnosis of ULO in patients with craniofacial changes depends on clinical findings of ESRD, biochemical results of SHPT, and classical imaging findings. ULO presents clinically with progressive painless massive overgrowth of the craniofacial bones in particular the jaw, widening of the nares, flattening of the nasal bridge, and increased interdental spacing [15 , 24] . Beside its cosmetic effects, the involvement of the craniofacial bones in ULO causes progressive narrowing of the skull base foramina which may results in compressive cranial neuropathy [3 , 25] .…”

Effect of combined vitamin D and microwave ablation of parathyroid glands on blood pressure and cardiac function in maintenance-hemodialysis patients with uremic secondary hyperparathyroidism

“…The technology of microwave ablation involves using a microwave needle to puncture into the tumor tissues, resulting in tissue dehydration and necrosis due to frictional heating. Thus, it results in therapeutic effect [8][9][10][11]. The present research was carried out to study the influence of microwave ablation of parathyroid glands when used in combination with active vitamin D on blood pressure and cardiac function in 120 maintenancehemodialysis patients with uremic secondary hyperparathyroidism.…”