Burckhardt-Bravo Valentina, Bañados-Alarcón Antonia, Funes-Ferrada Rodrigo, Caviedes-Rivera César
Universidad de los Andes, Santiago de Chile, Chile.
Departamento de Nefrología, Clínica Dávila, Santiago, Chile.
Rev Med Chil. 2024 May;152(5):634-639. doi: 10.4067/s0034-98872024000500634.
Uremic leontiasis ossia (ULO) is a rare manifestation of renal osteodystrophy in) patients with end-stage chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPTH). It occurs due to increased osteoclastic activity secondary to high plasmatic parathyroid hormone (PTH) levels. This leads to bone deformation with thickening and massive enlargement of the cranial vault, resulting in a leonine face appearance. Imaging such as CT scans of the head and neck can reveal bone remodeling, which aids in diagnosing ULO in patients with elevated PTH. Treatment requires a multidisciplinary approach, including medical management of SHPTH, parathyroidectomy, and osteoplasty under a maxillofacial surgery specialist to correct anatomical abnormalities.
Herein, we present a case report of a patient with ULO, demonstrating valuable insights into early recognition and multidisciplinary management of the disease.
A 39-year-old female patient with a past medical history of CKD on hemodialysis, hypertension, and SHPTH. The patient was admitted for total parathyroidectomy due to non-toxic multinodular goiter. Physical exam shows a nonpainful increasing bilateral growth of maxillary and mandibular bone, loss of nasolabial folds, and widening of the nares, resulting in a leonine appearance. Relevant laboratory exams showed a plasmatic PTH level of 4557 pg/ml. CT scan of the head and neck shows tunnel-like bones in the cranial vault consistent with ULO. The patient underwent total thyroidectomy and subtotal parathyroidectomy without complications. Laboratory exams on the first day post-surgery showed improvement in PTH plasmatic levels.
ULO is a rare disease and poses a diagnostic challenge. Early recognition of clinical features of ULO and elevated PTH levels in end-stage CKD patients is essential for a prompt diagnosis and appropriate treatment to avoid the consequences of ULO.
尿毒症性狮面骨病（ULO）是终末期慢性肾脏病（CKD）和继发性甲状旁腺功能亢进（SHPTH）患者肾性骨营养不良的一种罕见表现。它是由于血浆甲状旁腺激素（PTH）水平升高继发破骨细胞活性增加所致。这会导致颅骨增厚和大量增大，从而引起骨骼变形，导致面部呈现狮面样外观。头部和颈部的CT扫描等影像学检查可显示骨重塑，有助于诊断PTH升高患者的ULO。治疗需要多学科方法，包括SHPTH的药物治疗、甲状旁腺切除术以及在颌面外科专家指导下进行骨成形术以纠正解剖异常。
在此，我们报告一例ULO患者的病例，展示对该疾病早期识别和多学科管理的宝贵见解。
一名39岁女性患者，有CKD、高血压和SHPTH病史，正在接受血液透析。该患者因非毒性多结节性甲状腺肿入院接受甲状旁腺全切术。体格检查显示上颌骨和下颌骨双侧无痛性增大、鼻唇沟消失、鼻孔增宽，导致面部呈现狮面样外观。相关实验室检查显示血浆PTH水平为4557 pg/ml。头部和颈部CT扫描显示颅骨内有隧道样骨质，符合ULO表现。患者接受了甲状腺全切术和甲状旁腺次全切除术，无并发症发生。术后第一天的实验室检查显示PTH血浆水平有所改善。
ULO是一种罕见疾病，具有诊断挑战性。早期识别终末期CKD患者的ULO临床特征和PTH水平升高对于及时诊断和适当治疗至关重要，以避免ULO的后果。
