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

Abstract

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.

Neonatal manifestation of 22q11.2 deletion syndrome – four case reports and a mini-literature review

“…If untreated, low blood calcium levels can cause seizures in the neonatal period. Calcium homeostasis often normalizes with age, although the recurrence of hypocalcaemia in later childhood has been reported during illness [14][15]. None of the presented patients demonstrated any symptoms of seizures due to hypocalcaemia.…”

Chromosome 22q11.2 Deletion Syndrome: A Comprehensive Review of Molecular Genetics in the Context of Multidisciplinary Clinical Approach

“…The presentation of hypocalcemia is more likely to occur at a young age and may vary considerably, ranging from transient neonatal silent hypocalcemia to hypocalcemic tetany and overt hypoparathyroidism throughout the lifespan. Affected patients may also experience fatigue and paresthesia, as well as more severe manifestations including low seizure threshold and prolongation of the QT interval, which significantly affect both early life neurodevelopment [69] and clinical outcome in cardiac and non-cardiac surgical procedures [70][71][72]. Thyroid dysfunction often presents later, in older childhood or adulthood, and is commonly due to autoimmune thyroiditis.…”

Chromosome 22q11.2 Deletion Syndrome: A Comprehensive Review of Molecular Genetics in the Context of Multidisciplinary Cinical Approach

“…The presentation of hypocalcemia may considerably vary ranging from transient neonatal silent hypocalcemia to hypocalcemic tetany and overt hypoparathyroidism throughout the lifespan. Affected patients may also experience fatigue, paresthesia, as well as more severe manifestations including low seizure threshold and prolongation of the QT interval, significantly affecting both early life neurodevelopment [69] and clinical outcome in cardiac and non-cardiac surgical procedures [70][71][72]. Thyroid dysfunction is commonly due to autoimmune thyroiditis and thyroid autoantibodies, particularly antithyroperoxidase antibodies have been found in up to 5% of children and in approximately 30% of adults affected with 22q11.2 DS [73].…”