TY - JOUR
T1 - Preoperative multiple endocrine neoplasia type 1 diagnosis improves the surgical outcomes of pediatric patients with primary hyperparathyroidism
AU - Romero Arenas, Minerva A.
AU - Morris, Lilah F.
AU - Rich, Thereasa A.
AU - Cote, Gilbert J.
AU - Grubbs, Elizabeth G.
AU - Waguespack, Steven G.
AU - Perrier, Nancy D.
PY - 2014/4
Y1 - 2014/4
N2 - Background Primary hyperparathyroidism (PHPT) is uncommon in children. The surgical management of PHPT in children has evolved over the past two decades. Methods A retrospective study of patients who underwent parathyroidectomy for PHPT diagnosed at age < 18 years and managed at a tertiary referral center for endocrine and familial disorders. Results Thirty-eight patients met eligibility criteria (1981-2012). Median age at PHPT diagnosis was 15 years. Two-thirds of patients were symptomatic (68%, n = 26), most commonly from nephrolithiasis. Twenty-six (68%) patients underwent a standard cervical exploration while 32% underwent a focused unilateral parathyroidectomy. Multiple endocrine neoplasia type 1 (MEN1) was diagnosed preoperatively in 22/26 patients. Patients with a preoperative diagnosis of MEN1 were more likely to undergo a complete initial operation (> 3 gland parathyroidectomy with transcervical thymectomy, 13/22, 59% vs. 0/4, 0%; P = 0.03) and less likely to have recurrent disease (10/22, 45% vs. 3/4, 75%; P < 0.001) during follow up than patients diagnosed postoperatively. Conclusions Children with PHPT should raise suspicion for MEN1. Preoperative MEN1 evaluation helped guide the extent of initial parathyroidectomy and was associated with lower rates of recurrence in sporadic and familial PHPT in pediatric patients. Management should occur at a high volume center with experienced clinicians and genetic counseling services.
AB - Background Primary hyperparathyroidism (PHPT) is uncommon in children. The surgical management of PHPT in children has evolved over the past two decades. Methods A retrospective study of patients who underwent parathyroidectomy for PHPT diagnosed at age < 18 years and managed at a tertiary referral center for endocrine and familial disorders. Results Thirty-eight patients met eligibility criteria (1981-2012). Median age at PHPT diagnosis was 15 years. Two-thirds of patients were symptomatic (68%, n = 26), most commonly from nephrolithiasis. Twenty-six (68%) patients underwent a standard cervical exploration while 32% underwent a focused unilateral parathyroidectomy. Multiple endocrine neoplasia type 1 (MEN1) was diagnosed preoperatively in 22/26 patients. Patients with a preoperative diagnosis of MEN1 were more likely to undergo a complete initial operation (> 3 gland parathyroidectomy with transcervical thymectomy, 13/22, 59% vs. 0/4, 0%; P = 0.03) and less likely to have recurrent disease (10/22, 45% vs. 3/4, 75%; P < 0.001) during follow up than patients diagnosed postoperatively. Conclusions Children with PHPT should raise suspicion for MEN1. Preoperative MEN1 evaluation helped guide the extent of initial parathyroidectomy and was associated with lower rates of recurrence in sporadic and familial PHPT in pediatric patients. Management should occur at a high volume center with experienced clinicians and genetic counseling services.
KW - Children
KW - Genetic testing
KW - Hereditary disorders
KW - Hypercalcemia
KW - Multiple endocrine neoplasia
KW - Primary hyperparathyroidism
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U2 - 10.1016/j.jpedsurg.2013.11.059
DO - 10.1016/j.jpedsurg.2013.11.059
M3 - Article
C2 - 24726110
AN - SCOPUS:84921957173
SN - 0022-3468
VL - 49
SP - 546
EP - 550
JO - Journal of pediatric surgery
JF - Journal of pediatric surgery
IS - 4
ER -