Sagliker syndrome in patients with secondary hyperparathyroidism and chronic renal failure: Case report

• Sagliker syndrome is assumed to result from insufficient treatment of secondary hyperparathyroidism in early stages of chronic renal failure.
• Sagliker syndrome should be added to current surgical indications for parathyroidectomy.

IntroductionSagliker described craniofacial, skeletal, neurologic and soft tissue abnormalities in patients with secondary hyperparathyroidism with end-stage chronic renal disease. This unique entity has been attributed to delay in treatment of renal insufficiency. As of 2012 sixty cases have been reported. The aim of the study is to report this entity in five patients and to propose this condition as an indication for parathyroidectomy.MethodsWe report five patients. All patients had chronic renal failure (CRF) and secondary hyperparathyroidism that fulfilled the criteria for Sagliker syndrome and underwent parathyroid surgery. We collected detailed information, including clinical history, laboratory data, and old/current photographs for comparison.ResultsThe five patients, four women and one man mean age 21 years, had severe secondary hyperparathyroidism (mean serum preoperative PTH = 3779.6 pg/ml, mean preoperative Ca2+ = 8.83 mg/dl and P3– = 5.66 mg/dl); maxillary and mandibular bone changes; teeth/dental abnormalities; and soft and benign tumors in the mouth. Three had severe uglifying facial appearance. All patients underwent total parathyroidectomy without postoperative complications. After surgery mean serum PTH was 17.16 pg/ml (p = 0.006), P3− = 3.38 mg/dl (p = 0.0068) and Ca2+ = 6.97 mg/dl (p = 0.345). At surgery, average height was 149.8 cm, a mean of 6 cm (p = 0.003) less than at the beginning of CRF. Preoperative Beck Depression Inventory test mean score was 37 (severe depression); 6 months after surgery mean score was 15.8 (mild mood disturbance)(p = 0.0001). Clinical follow-up was satisfactory with a mean follow up time of 14 months.ConclusionWe consider that Sagliker syndrome should be added to current surgical indications for parathyroidectomy.