Neal Matthew D, Deslouches Berthony, Ogilvie Jennifer
Division of Endocrine Surgery, Department of Surgery, University of Pittsburgh Medical Center 200 Lothrop Street, Pittsburgh, PA 15213 USA.
Cases J. 2009 Sep 10;2:7572. doi: 10.4076/1757-1626-2-7572.
To describe the use of combined preoperative imaging and intraoperative parathyroid hormone as a novel approach in the surgical management of a patient with tertiary hyperparathyroidism associated with X-linked hypophosphatemic rickets.
We present the first documented description of combined preoperative imaging and intraoperative parathyroid hormone as well as a review of the literature surrounding the surgical management of tertiary hyperparathyroidism in the setting of X-linked hypophosphatemic rickets. A 23 year-old female with X-linked hypophosphatemic rickets and renal impairment presented with symptomatic hypercalcemia and tertiary hyperparathyroidism. She had failed medical management and presented for surgical evaluation. Technitium-99 m Sestamibi SPECT imaging and parathyroid ultrasound were used to localize the enlarged parathyroid glands preoperatively. Intraoperative findings correlated well with pre-operative imaging. She underwent successful subtotal parathyroidectomy for four-gland hyperplasia, using intraoperative parathyroid hormone guidance. Despite severe post-operative bone hunger, her serum calcium normalized and she experienced resolution of her preoperative symptoms.
X-linked hypophosphatemic rickets is an uncommon disorder of phosphate metabolism resulting in bone deformity. Patients are predisposed to the development of secondary hyperparathyroidism due to chronic vitamin D supplementation which may progress to tertiary hyperparathyroidism with autonomous parathyroid function. Preoperative evaluation with Technitium-99 m Sestamibi SPECT and ultrasound imaging, as well as the use of intraoperative parathyroid hormone are effective in guiding surgical resection. Subtotal parathyroidectomy with cryopreservation is indicated to produce operative cure and limit the risk of recurrence. Although these patients are susceptible to severe postoperative bone hunger, appropriate supplementation with intravenous and oral calcium can minimize hypocalcemic symptoms.
描述联合术前成像和术中甲状旁腺激素在与X连锁低磷性佝偻病相关的三发性甲状旁腺功能亢进患者手术治疗中的应用。
我们首次记录了联合术前成像和术中甲状旁腺激素的情况，并回顾了X连锁低磷性佝偻病背景下三发性甲状旁腺功能亢进手术治疗的相关文献。一名患有X连锁低磷性佝偻病和肾功能损害的23岁女性出现有症状的高钙血症和三发性甲状旁腺功能亢进。她的药物治疗失败，前来接受手术评估。术前使用锝-99m甲氧基异丁基异腈单光子发射计算机断层扫描（Technitium-99 m Sestamibi SPECT）成像和甲状旁腺超声定位增大的甲状旁腺。术中发现与术前成像结果良好相关。在术中甲状旁腺激素引导下，她成功接受了甲状旁腺次全切除术以治疗四腺增生。尽管术后出现严重的骨饥饿，但她的血清钙恢复正常，术前症状也得到缓解。
X连锁低磷性佝偻病是一种罕见的磷代谢紊乱疾病，可导致骨骼畸形。由于长期补充维生素D，患者易发生继发性甲状旁腺功能亢进，可能进展为具有自主甲状旁腺功能的三发性甲状旁腺功能亢进。术前使用锝-99m甲氧基异丁基异腈单光子发射计算机断层扫描和超声成像评估，以及术中使用甲状旁腺激素，对指导手术切除有效。甲状旁腺次全切除术加冷冻保存可实现手术治愈并降低复发风险。尽管这些患者术后易出现严重的骨饥饿，但适当补充静脉和口服钙剂可将低钙血症症状降至最低。
