Sagliker Syndrome: A Case Series and Literature Review With Clinical Characteristics, Post Parathyroidectomy Biochemical Trajectories and Hungry Bone Syndrome
Siddharth Madhad, Puneet Bhardwaj, Amal Shaharyar, Vivek Jha, Soham Mukherjee, Divya Dahiya, Uma Nahar Saikia, Rimesh Pal, Sanjay Kumar BhadadaABSTRACT
Objective
To describe the clinical, biochemical, radiological, histopathological, and postoperative characteristics of patients with Sagliker syndrome.
Methods
Three patients with dialysis‐dependent end‐stage renal disease and Sagliker syndrome who underwent parathyroidectomy at our institution were retrospectively analysed. A literature review identified six published reports with detailed clinical, biochemical, radiological, surgical, and postoperative data suitable for comparative analysis.
Results
All three patients presented with progressive diffuse craniofacial skeletal remodelling characterised by maxillomandibular enlargement, facial widening, and severe secondary hyperparathyroidism. Preoperative intact parathyroid hormone concentrations ranged from 2258 to 4750 pg/mL, accompanied by marked hyperphosphatemia and elevated alkaline phosphatase levels consistent with advanced high‐turnover bone disease. Sequential clinical imaging demonstrated progression from preserved facial morphology to severe craniofacial deformity following end‐stage renal disease. Radiologic findings included osteitis fibrosa cystica, acro‐osteolysis, and salt‐and‐pepper skull appearance. All patients developed severe postoperative hungry bone syndrome requiring intensive calcium and calcitriol supplementation following parathyroidectomy. Published reports demonstrated a highly concordant results with prolonged dialysis exposure, female predominance, severe biochemical hyperparathyroidism, diffuse craniofacial involvement, and frequent postoperative hypocalcemia.
Conclusions
Early recognition and optimal management of CKD‐MBD is pivotal in preventing severe, irreversible complications, including craniofacial deformities.