Postoperative Hypocalcemia following Non-Cardiac Surgical Procedures in Children with 22q11.2 Deletion Syndrome.

The guidelines for management of children with 22q11.2 deletion syndrome (22q11DS) highlight the risk for developing hypocalcemia after surgery and recommend monitoring calcium perioperatively. Despite this guidance, little has been published on postoperative hypocalcemia and 22q11DS. Our goals were to evaluate the frequency of perioperative calcium monitoring and examine how often postoperative hypocalcemia was identified. This is a retrospective chart review of patients in our 22q Center's repository. Inclusion criteria were a diagnosis of 22q11DS and a history of a non-cardiac surgical procedure. Data collected included all non-cardiac surgeries and perioperative calcium labs. In total, 68 patients were included and underwent 305 on-cardiac surgeries. Patients in only 17% of these surgeries had postoperative calcium testing, but of those tested, 58% showed hypocalcemia. Patients with history of hypocalcemia at the time of chart review undergoing non-cardiac surgeries were tested postoperatively 40% of the time; however, 67% of these had hypocalcemia. Similarly, for patients without history of hypocalcemia, postoperative testing occurred 60% of the time, with 52% of these having hypocalcemia. This study demonstrates that postoperative hypocalcemia in children with 22q11DS following non-cardiac surgeries is common and affects patients both with and without prior history of hypocalcemia. These data support establishing a protocol for perioperative testing/management of hypocalcemia for patients with 22q11DS.

More than one way to skin a thyroid. Managing pediatric hypothyroidism with weekly intramuscular levothyroxine.

BACKGROUND: Oral levothyroxine (L-T4) supplementation is usually an effective therapy in pediatric hypothyroidism, except in patients with malabsorption or pseudomalabsorption. In these cases, parenteral L-T4 may be required, but there is a paucity of information about this delivery method in the pediatric population. CASE PRESENTATION: We present three cases of pediatric patients with primary hypothryoidism unresponsive to oral L-T4 that were successfully treated with intramuscular (IM) L-T4. CONCLUSIONS: This is the first pediatric case series of successful IM L-T4 therapy in refractory primary hypothyroidism. Our case series demonstrates that once weekly IM L-T4 dosing may be a safe and well tolerated treatment regimen for pediatric patients.