The Laryngoscope Lippincott Williams & Wilkins, Inc. © 2007 The American Laryngological, Rhinological and Otological Society, Inc. Solitary Parathyroid Adenoma: A Rare Cause of Primary Hyperparathyroidism in Children Frederic Venail, MD; Richard Nicollas, MD, PhD; Denis Morin, MD, PhD; Tara Mackle, MD; J. M. Garnier, MD; Jean-Michel Triglia, MD, PhD; Michel Mondain, MD, PhD Solitary parathyroid adenoma is a rare cause of primary hyperparathyroidism in children. We report the cases of four children, aged 7 to 14 years old, who presented with a variety of symptoms (growth retar- dation, glomerulonephritis, pathological fracture, mood swings). Diagnosis was based on clinical exam- ination, imaging, and biochemical analysis. Treat- ment consisted of surgical excision of the adenoma. As diagnosis was delayed in all four patients, we rec- ommend systematically checking serum calcium lev- els in children with certain nonspecific symptoms. Adenoma resection usually restores normal serum calcium levels and, hence, avoids further complica- tions. Key Words: Parathyroid adenoma, hypercalce- mia, primary hyperparathyroidism, hungry bone syn- drome, nephrocalcinosis. Laryngoscope, 117:946 –949, 2007 INTRODUCTION Primary hyperparathyroidism (PHPT) is the third most common endocrine disorder in adults. Conversely, this pathology is rarely observed in children. Solitary parathyroid adenoma is the main etiology of PHPT both in the adult and the pediatric populations. Clinical features are well established in adults but not in children, in whom clinical presentation tends to be unusual and complications are often severe. Surgical removal of the adenoma is the treatment of choice and generally leads to a return of normal serum calcium levels. In children, atypical presentation of- ten results in delayed diagnosis with increased morbidity. We report four cases of children with solitary parathyroid adenoma who presented with unusual symptoms. Clinical presentation, biological profile, imaging data, surgical find- ings, and postoperative follow-up are described to help phy- sicians with diagnosis and management. CASE REPORTS Patient 1 Patient 1 was referred with suspected appendicitis in July 2004. This 7-year-old girl had a ventriculoperitoneal (VP) shunt after a ventricular hemorrhage at birth. Symptoms did not im- prove after appendicectomy, and she remained confused with fever and headache. The VP shunt was found to be functioning normally. Pyelonephritis with nephrocalcinosis (Fig. 1) and acute renal failure were diagnosed. Serum calcium was elevated (4.34 mmol/L to 17 mg/dL) with hypophosphatemia (0.86 mmol/liter to 8.2 mg/dL). Urea and creatinine levels were increased (9 mmol/L to 54 mg/dL and 108 mol/L to1.2 mg/dL, respectively). Intact parathyroid hormone (iPTH) was elevated (1135 pg/mL; normal range 10 –55 pg/mL) with a normal level of parathyroid hormone- related peptide (PTH-RP). Neck ultrasonography and 99mTc- MIBI scintigraphy were suspect for a left inferior parathyroid adenoma (Fig. 2). After medical management (rehydration, pam- idronate, and furosemide), this patient was operated on, and the left solitary inferior parathyroid adenoma was removed. During the postoperative period, she developed hypocalcemia (1.47 mmol/L to 5.9 mg/dL) with normal PTH levels and hungry bone syndrome requiring further treatment with calcium and 1--cholecalciferol for 6 months. There was no familial history of PHPT and no evidence of multiple endocrine neoplasia (MEN). Osteodensitometry displayed a low bone mineral density (Z score -4.1 standard deviation [SD]). On retrospective questioning, her parents revealed that she had been polyuric during the 6 months prior to admission. Her serum calcium (2.25 mmol/L to 9 mg/dL) remains normal 22 months post- operatively. Renal function is normal, but calcium deposits can be observed on renal ultrasonographic examination. Patient 2 Patient 2 is a 14-year-old boy admitted in July 2005 with bilateral lower limb fractures as the result of a bicycle accident. While an inpatient, he developed mild abdominal pain and vom- iting. Following discharge, his parents noted that he was polyuric From the Pediatric ENT Department (F.V., T.M., M.M.), University Hospital Guide de Chauliac, Montpellier, France; the Department of Pe- diatric Otolaryngology/Head and Neck Surgery (R.N., J.M.T.), La Timone Children’s Hospital, Marseille, France; the Pediatric Nephrology and En- docrinology Department (D.M.), University Hospital Arnaud de Villeneuve, Montpellier, France; and the Pediatric Department (J.M.G.), Hopital Nord, Marseille, France. Editor’s Note: This Manuscript was accepted for publication January 5, 2007. Send correspondence to Michel Mondain, MD, PhD, Pediatric ENT Department, Service ORL B, Centre Hospitalier Universitaire Gui de Chauliac, 80 Avenue Augustin Fliche, 34295 Montpellier, France. E-mail: michel.mondain@free.fr DOI: 10.1097/MLG.0b013e3180337d95 Laryngoscope 117: May 2007 Venail et al.: Solitary Parathyroid Adenoma 946