Atypical Parathyroid Adenoma Complicated with Protracted Hungry Bone Syndrome after Surgery: A Case Report and Literature Review

Abstract

Hungry Bone Syndrome refers to the severe and prolonged hypocalcemia and hypophosphatemia, following parathyroidectomy in patients with hyperparathyroidism. We present the case of an eighteen-year-old woman with a four-year history of hyporexia, polydipsia, weight loss, growth retardation, and poor academic performance. The diagnostic work-up demonstrated primary hyperparathyroidism with hypercalcemia of 13.36 mg/dL, a PTH level of 2551 pg/mL, bone brown tumors, and microcalcifications within pancreas and kidneys. Neck ultrasonography revealed a parathyroid adenoma of 33 × 14 × 14 mm, also identified on 99Tc-sestamibi scan. Bone densitometry showed decreased Z-Score values (total lumbar Z-Score of −4.2). A right hemithyroidectomy and right lower parathyroidectomy were performed. Pathological examination showed an atypical parathyroid adenoma, of 3.8 g of weight and 2.8 cm in diameter. After surgery she developed hypocalcemia with tetany and QTc interval prolongation. The patient required 3 months of oral and intravenous calcium supplementation due to Hungry Bone Syndrome (HBS). After 42 months, she is still under oral calcium. Usually HBS lasts less than 12 months. Therefore we propose the term “Protracted HBS” in patients with particularly long recovery of 1 year. We present a literature review of the diagnosis, pathophysiology, and treatment of HBS.

Leczenie ciężkiej, zagrażającej życiu hipokalcemii ludzkim rekombinowanym teryparatydem pacjentów z pooperacyjną niedoczynnością przytarczyc — seria przypadków

“…Hungry bone syndrome is observed in 13-30% of cases following parathyroid surgery [32]; in patients with bone changes caused by hyperparathyroidism the incidence of HBS is increased to 25-90%, while the absence of bone changes is as- Total Ca -total calcium; Ionised Ca -ionised calcium; P -phosphates; PTH -parathormone; Mg -magnesium; na -not assayed sociated with a risk reduction to 0-6% [28]. Most HBS definitions include the presence of hypocalcaemia in a patient subjected to surgery for hyperparathyroidism, as well as hypophosphatemia and hypomagnesaemia as an effect of bone remineralisation [28,33]. Some authors define HBS-related hypocalcaemia as the values < 8.5 mg/dL and hypophosphatemia as the values < 3.0 mg/dL [27] with hypocalcaemia persisting for over four days despite calcium ion supplementation, with normal vitamin D levels in serum [31].…”

“…Literature reports consistently claim that preoperative bisphosphonate treatment may prevent hungry bone syndrome after parathyroidectomy, both in primary and secondary hyperparathyroidism, although in the long perspective, this treatment may potentially delay bone remodelling [37][38][39]. HBS treatment is long-lasting and usually requires high doses of calcium and vitamin D preparations [33,35]. Prolonged hypocalcaemia despite treatment is the reasons for long hospitalisations.…”

“…Araya et al [40] reported that mean duration of hospital treatment was 19 days. On the other hand, Juarez-Leon et al [33] described a female patient with HBS, who required oral and intravenous supplementation of calcium for up to three months. Our patient no.…”

Fine needle cytology pre‐surgical differentiation of parathyroid neoplasms: Is it reliable?

“…PAA are large nodules with some typical PC features (banding fibrosis, mitotic activity, trabecular growth, adherence of neoplasm to adjacent soft tissues or thyroid and focal capsule infiltration), without a clear invasion of surrounding tissues. These pathological features are usually associated with a benign course in terms of survival . P‐HPT is caused by a single adenoma (PA) in approximately 95% of cases, in 3%‐4% of cases by enlarged, overactive glands (four‐gland hyperplasia), rarely by multiple adenomas (0.5%) and by parathyroid carcinoma (PC) in <1% .…”

Síndrome Da Fome Óssea Como Uma Complicação Da Paratireoidectomia: Uma Revisão Integrativa.

“…O rápido declínio em seus níveis circulantes no pós-operatório resulta em aumento do influxo de cálcio para os ossos ocasionando maior formação e aumento da densidade mineral óssea, secundária a uma atividade excessiva osteoblástica e parada da atividade osteoclástica, o que acredita ser o principal mecanismo por trás da rápida perda de cálcio sérico observada na HBS (WONG et al, 2020;FLORAKIS et al 2019). Desse modo, os pacientes submetidos à tal cirurgia podem se apresentar com sintomas correspondentes à hipocalcemia de variada gravidade, desde parestesias à convulsões (JUÁREZ-LEÓN et al, 2015; WITTEVEEN el al. 2013; CARTWRIGHT, ANASTASOPOULOU, 2022) .A duração da HBS se relaciona com a duração da hipocalcemia no pós-operatório (PO) ou o tempo necessário para a normalização dos níveis séricos de cálcio, espelhados pela melhora dos marcadores do turnover ósseo e das características ósseas observadas na radiografia (WITTEVEEN el al.…”