Sagliker syndrome: A case report on a rare case of severe renal osteodystrophy due to untreated secondary hyperparathyroidism

Abstract

Aim: To describe a rare clinical syndrome related to secondary hyperparathyroidism (SHP). Background: Sagliker syndrome is a rare entity associated with long-term untreated SHP that results in severe renal osteodystrophy with typical skeletal deformities (mostly craniofacial), high levels of parathormone (PTH), and difficult medical control of the disease. Adequate treatment of the secondary hyperparathyroidism generally includes a surgical approach, and is essential to stop the progression of the disease. Case description: We present the case of a 26-year-old African male with chronic kidney failure on dialysis since the age of 15 and severe SHP (PTH > 5,000 pg/L). He presented with craniofacial dystrophy, benign facial bone tumors, depression, and long-bone fractures. After failure of medical treatment, he underwent total parathyroidectomy with parathyroid autotransplant with normalization of the serum parathormone. “Hungry bone syndrome” was verified postoperatively. After 2 years of follow-up, the skeletal changes were stabilized without regression of the clinical spectrum. No recurrence of secondary hyperparathyroidism was identified at 1 year of follow-up. Conclusion: Identification of this syndrome is of clinical significance, allowing the identification of a subgroup of patients with predicable failure of the medical treatment of secondary hyperparathyroidism and who benefit from surgical treatment to stop the progression of the skeletal changes. Clinical significance: The early identification of this clinical syndrome may allow for better outcomes with reduced morbidity.