Volume 6, Issue 1 , Pages 39-41, January 2011
Hyperparathyroid crisis caused by ectopic parathyroid adenoma in a pediatric patient
Article Outline
Abstract
Hyperparathyroid crisis is a lifethreatening endocrine emergency, rarely found in the pediatric population. Herein, we report a 12-year-old girl who presenting with Hyperparathyroid crisis due to an ectopic parathyroid adenoma. The unusual patient presentation and management are described and illustrated. The pertinent literature is reviewed.
Keywords: Hyperparathyroid crisis, Ectopic parathyroid adenoma, Pediatric patient
1. Introduction
Primary hyperparathyroidism occurs rarely in children and is uncommon in adolescents [1]. Although most of pediatric patients with primary hyperparathyroidism were symptomatic at initial presentation and nearly half of them had end organ damage [2], they rarely presented with Hyperparathyroid crisis. We report a pediatric patient harboring an ectopic parathyroid adenoma in supraclavicular fossa who on presentation had clinical and biochemical evidence of Hyperparathyroid crisis.
2. Case reports
A 12-year-old girl was seen for her complaints of progressive pain in her left lower extremity, polydipsia, fatigue, decreased appetite and emesis for the preceding 2 months. In her physical examination, weight was 22
kg, height was 132cm, and blood pressure was 118/83mmHg. Examination of the neck revealed a soft, nontender mass in suprasternal fossa, measuring 2.8cm×2cm. Palpitations, polyuria, sweating, and headache were not present. The past medical history of the patient and her family was unremarkable. There was no familial history of primary hyperparathyroidism and no evidence of multiple endocrine neoplasia.
Laboratory data on admission were as follows: hemoglobin: 135g/l, hematocrit: 39.7%, Sodium: 136.7mmol/l, creatinine: 52μmol/l, urea nitrogen: 3.6mmol/l, chloride: 105.8mmol/l, white blood cell: 12230/mm3 (normal: 4000–10000/mm3), potassium: 2.85mmol/l (normal: 3.5–5.5mmol/l), calcium: 4.01mmol/l (normal: 2.1–2.6mmol/l), phosphorus: 0.64mmol/l (normal: 0.81–1.55mmol/l), intact parathyroid hormone (PTH): 441.74ng/dl (normal: 0–20ng/dl), uric acid: 467μmol/l (normal: 155–428μmol/l), alkaline phosphatase (ALP): 2727U/l (normal: 34–114U/l). Her 24h urinary tests revealed calcium: 10.23mmol/day (normal: 0–6.25mmol/day), phosphorus: 9.7mmol/day (normal: 12.9–42mmol/day). Liver function tests and urine analysis were normal. Free triiodothyronine, free thyroxine and thyroid-stimulating hormone were normal. Ultrasonography of thyroid was normal. Ultrasonography of suprasternal fossa revealed a hypoechoic, regularly nodular lesion, measuring 2.53cm×1.57cm. Technetium-99m sestamibi (MIBI) scanning showed an area of significantly increased uptake in suprasternal fossa both on the early and delayed image (Fig. 1). Bone densitometry suggested severe osteoporosis. Abdominal ultrasound showed no renal stone.
Fig. 1.
Tc-99
m scintigraphy of the patient showing focal accumulation in the suprasternal fossa.
A diagnosis of Hyperparathyroid crisis was thus established. Treatment was initiated for hydration with saline infusion, diuresis with furosemide and intramuscular calcitonin. The response was not good and calcium still maintained high levels (3.38–4.22mmol/l). She underwent ectopic parathyroid adenomectomy 4 days after drug treatment. The ectopic parathyroid gland (3.2cm×2.5cm×1.5cm) was found in the suprasternal fossa (Fig. 2). Macroscopically, a well-circumscribed soft reddish brown nodule was seen (Fig. 3). Microscopically, a tumor composed of uniform, polygonal chief cells was observed, and the lesion was diagnosed as ectopic parathyroid adenoma in the suprasternal fossa (Fig. 4).
Fig. 2.
Perioperative view demonstrating the ectopic parathyroid adenomas in the suprasternal fossa.
Fig. 3.
Gross specimen after surgery showing ectopic parathyroid adenomas.
Fig. 4.
Pathological examination of the specimen. Hematoxylin and eosin (H&E) stain, 200×.
During the postoperative period, paresthesia and clinical findings of hypocalcemia (Chvostek's sign and Trousseaus's phenomenon) were observed. Serum calcium concentration decreased to 1.93mmol/l on the postoperative 6 day (Table 1). She required oral calcium and calcitriol substitution and was discharged on the postoperative 8 day. As her follow-up Ca levels were normal, oral Ca and calcitriol supplementation has been decreased gradually and was stopped at the 5th month. Until the 5th month, serum calcium, phosphate, PTH, ALP, and bone mineral density values were completely normal. Her height was found to be increased 3.9cm; it was 135.9cm, and weight was found to be increased 3kg; it was 25kg.
Table 1. Laboratory data before and after the ectopic parathyroid adenomectomy.
| Laboratory findings | Initial | Preoperative | Postoperative | |||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Day 1 | Day 2 | Day 3 | Day 4 | Day 6 | Day 8 | Day 20 | Fifth month | |||
| Serum calcium (N: 2.1–2.6mmol/l) | 4.01 | 3.96 | 3.15 | 2.64 | 2.15 | 2.17 | 1.93 | 2.16 | 2.44 | 2.37 |
| Serum phosphorus (N: 0.81–1.55mmol/l) | 0.64 | 0.80 | 0.48 | NM | NM | 0.44 | NM | NM | NM | 1.90 |
| PTH (N: 0–20ng/dl) | 441.74 | NM | 39.66 | NM | NM | 26.36 | NM | NM | NM | 19.90 |
3. Discussion
Primary hyperparathyroidism is the third most common endocrine disorder in adults, but it is rarely observed in children. The incidence is estimated from 2 to 5 in 100,000 [2]. Consequently, physicians frequently fail to check serum calcium and parathyroid hormone levels when evaluating children with nonspecific complaints such as polydipsia, polyuria, fatigue, poor appetite, weight loss, abdominal pain, nausea, and emesis. Diagnosis may be confirmed by calcium and PTH levels test in peripheral blood. In contrast to adult patients, where women are more often affected, the children with primary hyperparathyroidism are reported to be without gender predominance [2], [3].
Hyperparathyroid crisis is a lifethreatening endocrine emergency, first described by Hanes in 1939 [4]. It is a rare complication characterized by a profound increase in serum calcium, mental status changes, nausea and emesis, and disturbances in cardiac and renal function caused by marked elevation of PTH and calcium. Fewer than 350 patients with Hyperparathyroid crisis have been described in the literature [5]. Currently, Hyperparathyroid crisis is defined as a syndrome characterized by a serum calcium level greater than 3.75mmol/l from marked elevation of PTH, with severe signs and symptoms of hypercalcemia that are reversible with correction of the hypercalcemia [6]. The prevalence of Hyperparathyroid crisis varies from 1.6% to 6% of patients with hyperparathyroidism [5], [7], [8]. It is invariably fatal unless there is prompt surgical intervention, and the death rate of the crisis varies from 2.8% to 60% [9], [10].
Eighty-five percent of all primary hyperparathyroidism is caused by parathyroid adenomas, whereas parathyroid hyperplasia and parathyroid carcinoma account for 15% and less than 1%, respectively. Ectopic parathyroid adenomas occur in 20% of patients with primary hyperparathyroidism [11].
Ectopic parathyroid glands have been described in a number of different anatomic locations, which can occur from the angle of the mandible and carotid bifurcation to the mediastinum. The superior parathyroids arise from the fourth branchial arch. They are fairly reliably positioned in the posterior perithyroidal fat, behind the superior pole of the thyroid and near the path of the recurrent laryngeal nerve as it enters the cricothyroid muscle. Although they usually are behind the upper pole of the thyroid, they can descend posteriorly in the retroesophageal space and can even be found in the posterior mediastinum, occasionally. The inferior parathyroids arise from the third branchial arch, and descend with the thymus into the neck. Their anatomy is much less constant than the superior parathyroids. Although many rest adjacent to the inferior pole of the thyroid gland, the presence of a parathyroid gland within the suprasternal fossa, thyrothymic ligament, thymic parenchyma, or perithymic fat should be considered an expected ectopic location for an inferior gland.
The success of surgical treatment of primary hyperparathyroidism is based upon clear diagnosis and preoperative localization methods. In children, preoperative localization methods are essential due to significantly increased prevalence of failure of primary parathyroidectomies (20% in children vs. 1% in adults) [12]. According to the results of this, we recommend the combination of neck sonography and MIBI-scintigraphy. CT and/or MRI should be done if the diagnosis is uncertain.
In conclusion, to our knowledge, we report the first pediatric patient presenting with Hyperparathyroid crisis due to an ectopic parathyroid adenoma. Hyperparathyroid crisis is fairly rarely observed in children, but it is invariably fatal unless there is prompt surgical intervention. In addition, ectopic parathyroid adenomas occur in 20% of patients with primary hyperparathyroidism, therefore, we recommend the combination of neck sonography and MIBI-scintigraphy for preoperative localization, and CT and/or MRI should be done if the diagnosis is uncertain.
References
- . Primary hyperparathyroidism in children, adolescents, and young adults. World J. Surg. 1982;6:771–776
- Primary hyperparathyroidism in pediatric patients. Pediatrics. 2005;115:974–980
- Surgical treatment of primary hyperparathyroidism in children: report of 10 cases. Int. J. Pediatr. Otorhinolaryngol. 2008;72:1177–1182
- . Hyperparathyroidism due to parathyroid adenoma with death from para-hormone intoxication. Am. J. Med. Sci. 1939;197:85–89
- . Hyperparathyroid crisis: use of bisphosphonates as a bridge to parathyroidectomy. J. Am. Coll. Surg. 2008;206:1106–1115
- . Hypercalcaemic crisis: immediate parathyroidectomy and intraoperative intravenous calcium infusion improves outcome. Asian J. Surg. 2007;30:173–177
- . Hyperparathyroid crisis: clinical and pathologic studies of 14 patients. Ann. Surg. 1979;190:782–790
- . Acute primary hyperparathyroidism: experience of 59 cases. Br. J. Surg. 1989;76:979–981
- . Acute hyperparathyroidism: our experience with 36 cases. Ann. Ital. Chir. 2004;75:321–324
- . Hyperparathyroid crisis. Ann. Surg. 1967;166:1012–1015
- . Diagnosis of ectopic middle mediastinal parathyroid adenoma using endoscopic ultrasonography-guided fine-needle aspiration with real-time rapid parathyroid hormone assay. J. Am. Coll. Surg. 2009;209:e1–4
- . Clinical profile of primary hyperparathyroidism in adolescents and young adults. Clin. Endocrinol. (Oxf.). 1998;48:435–443
PII: S1871-4048(10)00007-9
doi:10.1016/j.pedex.2010.01.005
© 2010 Published by Elsevier Inc.
Volume 6, Issue 1 , Pages 39-41, January 2011
