Purpose: To describe the clinical, radiologic, and pathologic findings in patients with uremic leontiasis ossea (ULO).
Materials And Methods: Five patients with renal osteodystrophy developed marked hyperostosis of the facial and cranial bones. Radiologic studies included plain radiography of the skull (n = 5), computed tomography with three-dimensional reconstruction (n = 4), magnetic resonance imaging (n = 3), and fluorine-18 sodium fluoride positron emission tomography (PET) (n = 1). Specimens from bone biopsies (three patients) were examined.
Results: Skull and facial alterations were remarkably similar. Numerous nodules of varying attenuation and signal intensity in the widened diploic space suggested brown tumors in different stages of evolution. Biochemical data and PET findings enabled confirmation of markedly increased bone turnover. Bone specimens demonstrated severe osteitis fibrosa. After parathyroidectomy, facial changes in all patients stabilized or improved mildly.
Conclusion: A similar entity in animals, "bighead" disease, which results from nutritional and uremic secondary hyperparathyroidism, may provide a useful animal model for ULO in humans. Mild forms of this entity may be more common than the scarcity of previous reports suggests.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1148/radiology.199.1.8633151 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
[Uremic Leontiasis Ossea: A Challenge in End-Stage Chronic Kidney Disease. About a Case].
Rev Med Chil
May 2024
Departamento de Nefrología, Clínica Dávila, Santiago, Chile.
Article Synopsis
- Uremic leontiasis ossia (ULO) is a rare condition linked to renal osteodystrophy in patients with end-stage chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPTH), causing bone deformities that can lead to a 'leonine' facial appearance.
- The case study focuses on a 39-year-old female patient undergoing treatment, who exhibited significant symptoms and had elevated parathyroid hormone (PTH) levels, confirmed by CT scans.
- Treatment involves a multidisciplinary approach including surgery and management of SHPTH to correct anatomical issues and prevent further complications.
Leontiasis ossea in the setting of chronic kidney disease and limited dialysis access: A case report with discussion of therapeutic implications.
Radiol Case Rep
March 2025
Department of Family Medicine, University of South Florida, Morsani College of Medicine, Tampa, FL, USA.
Article Synopsis
- Leontiasis ossea, or craniofacial fibrous dysplasia, is a rare condition that causes abnormal growth of facial bones, often linked to chronic kidney disease and high parathyroid hormone levels.
- A case of a 30-year-old male with severe facial swelling and bone changes highlights the rapid progression of symptoms after poor dialysis access and the persistence of hyperparathyroidism despite treatment efforts.
- The article also reviews the underlying causes, imaging techniques used for diagnosis, and management strategies for this complex disease.
Uremic Leontiasis Ossea.
Radiology
May 2024
From the Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, 600 N Wolfe St, Phipps B-100, Baltimore, MD 21287.
Uremic Leontiasis Ossea: Theoretical Concepts and Practical Considerations.
Saudi J Kidney Dis Transpl
September 2022
Department of Nephrology, General Hospital of Livadia, Livadia, Greece.
Leontiasis ossea (LO) in chronic kidney disease patients, also known as Sagliker syndrome, is an exceptionally uncommon uremic complication of long-lasting and severe secondary hyperparathyroidism. The prominent features of uremic LO (ULO) encompass the characteristic clinical trial of massive thickening of maxillary and mandibular bones, widening of interdental spaces, and flattening of nasal bridges and nares. Moreover, during the transformation of craniofacial architecture, significant structural and functional consequences may appear, including upper airway patency, visual and hearing acuity, oral phase of swallowing as well as various neurological and psychiatric disorders.
View Article and Find Full Text PDFUremic Leontiasis Ossea in a Pediatric Patient.
Cureus
March 2022
Radiology, Buraidah Central Hospital., Buraidah, SAU.
Uremic leontiasis ossea (ULO) is a rare disease characterized by extensive thickening of the cranium, resulting in a characteristic, lion-like facial appearance. It is considered the most severe osseous complication of renal dystrophy. Although rare, ULO can occur even in young patients, which can be catastrophic, as it can not only lead to life-threatening conditions but also multiple complications that cause severe determent to the quality of life.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!