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Uremic Leontiasis Ossea

2024; Radiological Society of North America; Volume: 311; Issue: 2 Linguagem: Inglês

10.1148/radiol.233506

1527-1315

Dhairya A. Lakhani, Francis Deng,

Cerebrospinal fluid and hydrocephalus

HomeRadiologyVol. 311, No. 2 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyUremic Leontiasis OsseaDhairya A. Lakhani , Francis DengDhairya A. Lakhani , Francis DengAuthor AffiliationsFrom the Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University, 600 N Wolfe St, Phipps B-100, Baltimore, MD 21287.Address correspondence to D.A.L. (email: [email protected]).Dhairya A. Lakhani Francis DengPublished Online:May 28 2024https://doi.org/10.1148/radiol.233506MoreSectionsPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookXLinked In Supplemental material is available for this article.A 34-year-old patient with end-stage renal disease and hyperparathyroidism (serum parathyroid hormone level, 4485 ng/L) presented with nasal congestion and facial hypertrophy for 6 months. CT of the face showed marked maxillomandibular macrognathia, a pattern classically termed leontiasis ossea because of the lion-like physiognomy (Fig 1, Movie). Typical etiologies include fibrous dysplasia, Paget disease, renal osteodystrophy, and chronic hemolytic anemia (eg, due to thalassemia or sickle cell disease), which can be distinguished based on clinicoradiologic features (1).Figure 1: Facial bone CT cinematic volume renderings in (A) frontal and (B) oblique views show marked, symmetric maxillary and mandibular hypertrophy, characteristic of leontiasis ossea. The incisors are splayed apart (white arrow in A). The partly imaged frontal bone (black arrow in A) is affected by similar bone architecture changes without as much hypertrophy.Figure 1:Download as PowerPoint Movie: Rotational video of facial bone CT cinematic volume rendering shows marked, symmetric maxillary and mandibular hypertrophy, characteristic of leontiasis ossea.Download Original Video (8.8 MB)The bone architecture in this case showed internal serpiginous tunneling and cortical resorption (Fig 2), which are characteristic of uremic leontiasis ossea, consistent with this patient's risk factors (2). Moreover, symmetric, diffuse maxillofacial osseous involvement would be atypical for fibrous dysplasia or Paget disease, and the presence of cortical resorption would be atypical for chronic hemolytic anemia (1,2).Figure 2: Noncontrast facial CT multiplanar reformats with bone window settings in (A) axial and (B) coronal planes show the enlarged maxillae and mandible with a serpiginous, striated pattern of alternating high and low attenuation replacing the usual trabeculations, as is characteristic in uremic leontiasis ossea. The cortical bone is fully resorbed, resulting in lack of corticomedullary distinction. Similar changes affect the nasal septum (visible in B).Figure 2:Download as PowerPointSecondary hyperparathyroidism in chronic kidney disease results in a pathology of high bone turnover called osteitis fibrosa cystica. While mild craniofacial involvement appears as "salt-and-pepper" granular osteolysis, severe cases can be expansile, with various radiographic patterns including fibrous dysplasia–like ground-glass attenuation, coarse heterogeneous sclerosis and lucency, focal brown tumors, and, rarely, the trabecular tunneling seen in this case (2).Disclosures of conflicts of interest: D.A.L. Associate editor on the Radiology In Training Editorial Board. F.D. Deputy editor for Images in Radiology.References1. Alamer A. Uremic leontiasis ossea: distinctive imaging features allow differentiation from other clinical causes of leontiasis ossea. Radiol Case Rep 2021;17(3):553–557. MedlineGoogle Scholar2. Chang JI, Som PM, Lawson W. Unique imaging findings in the facial bones of renal osteodystrophy. AJNR Am J Neuroradiol 2007;28(4):608–609. MedlineGoogle ScholarArticle HistoryReceived: Dec 26 2023Revision requested: Feb 13 2024Revision received: Feb 15 2024Accepted: Mar 1 2024Published online: May 28 2024 FiguresReferencesRelatedDetailsRecommended Articles Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging ApproachRadioGraphics2018Volume: 38Issue: 3pp. 890-911Practical Approach to Radiopaque Jaw LesionsRadioGraphics2021Volume: 41Issue: 4pp. 1164-1185Imaging Spectrum of Calvarial AbnormalitiesRadioGraphics2021Volume: 41Issue: 4pp. 1144-1163Pediatric Panoramic Radiography: Techniques, Artifacts, and InterpretationRadioGraphics2021Volume: 41Issue: 2pp. 595-608Masses of the Nose, Nasal Cavity, and Nasopharynx in ChildrenRadioGraphics2017Volume: 37Issue: 6pp. 1704-1730See More RSNA Education Exhibits A Multimodality Review Of The Musculoskeletal Manifestations Of Hyperparathyroidism And What The Radiologist Needs To KnowDigital Posters2021From Fractures, Osteonecrosis and Beyond: A Multimodality Pictorial Review of Paget's Disease ComplicationsDigital Posters2020Paget Disease Of The Temporal Bone And Skull: A Pictorial Review.Digital Posters2021 RSNA Case Collection Brown tumour of the hard palateRSNA Case Collection2020Paget's DiseaseRSNA Case Collection2021Osteoporosis Circumscripta CraniiRSNA Case Collection2022 Vol. 311, No. 2 Metrics Altmetric Score PDF download