Search

Importance: The incidence of Graves disease (GD) is rising in children, and adequate care of these patients requires a multidisciplinary approach. Whether patients are seen in the context of endocrinology, nuclear medicine, or surgery, it is important to know the nuances of the therapeutic options in children., Observations: Given the rarity of GD in children, it is important to recognize its various clinical presenting signs and symptoms, as well as the tests that may be important for diagnosis. The diagnosis is typically suspected clinically and then confirmed biochemically. Imaging tests, including thyroid ultrasonography and/or nuclear scintigraphy, may also be used as indicated during care. It is important to understand the indications for and interpretation of laboratory and imaging tools so that a diagnosis is made efficiently and unnecessary tests are not ordered. Clinicians should be well-versed in treatment options to appropriately counsel families. There are specific scenarios in which medical therapy, radioactive iodine therapy, or surgery should be offered., Conclusions and Relevance: The diagnosis and treatment of pediatric patients with GD requires a multidisciplinary approach, involving pediatric specialists in the fields of endocrinology, ophthalmology, radiology, nuclear medicine, and surgery/otolaryngology. Antithyroid drugs are typically the first-line treatment, but sustained remission rates with medical management are low in the pediatric population. Consequently, definitive treatment is often necessary, either with radioactive iodine or with surgery, ideally performed by experienced, high-volume pediatric experts. Specific clinical characteristics, such as patients younger than 5 years or the presence of a thyroid nodule, may make surgery the optimal treatment for certain patients.

Importance: Current guidelines recommend total thyroidectomy for the majority of pediatric thyroid cancer owing to an increased prevalence of multifocality. However, there is a paucity of information on the exact prevalence and risk factors for multifocal disease-knowledge that is critical to improving pediatric thyroid cancer management and outcomes., Objective: To determine the prevalence and risk factors for multifocal disease in pediatric patients with papillary thyroid carcinoma (PTC)., Design, Setting, and Participants: This multicenter retrospective cohort study included patients 18 years or younger who underwent thyroidectomy for PTC from 2010 to 2020 at 3 tertiary pediatric hospitals and 2 tertiary adult and pediatric hospitals in the US., Main Outcomes and Measures: Demographic and clinical variables, including age, family history of thyroid cancer, autoimmune thyroiditis, prior radiation exposure, cancer predisposition syndrome, tumor size, tumor and nodal stage, PTC pathologic variant, and preoperative imaging, were assessed for association with presence of any multifocal, unilateral multifocal, and bilateral multifocal disease using multiple logistic regression analyses. Least absolute shrinkage and selection operator analysis was performed to develop a model of variables that may predict multifocal disease., Results: Of 212 patients, the mean age was 14.1 years, with 23 patients 10 years or younger; 173 (82%) patients were female. Any multifocal disease was present in 98 (46%) patients, with bilateral multifocal disease in 73 (34%). Bilateral multifocal disease was more accurately predicted on preoperative imaging than unilateral multifocal disease (48 of 73 [66%] patients vs 9 of 25 [36%] patients). Being 10 years or younger, T3 tumor stage, and N1b nodal stage were identified as predictors for multifocal and bilateral multifocal disease., Conclusions and Relevance: This large, multicenter cohort study demonstrated a high prevalence of multifocal disease in pediatric patients with PTC. Additionally, several potential predictors of multifocal disease, including age and advanced T and N stages, were identified. These risk factors and the high prevalence of multifocal disease should be considered when weighing the risks and benefits of surgical management options in pediatric patients with PTC.

Importance: Initial data suggest the effectiveness of oncogene-specific targeted therapies in inducing tumor regression of diverse cancers in children and adults, with minimal adverse effects., Observations: In this review, preliminary data suggest that systemic therapy may be effective in inducing tumor regression in pediatric patients with unresectable invasive thyroid cancer. Although most pediatric patients with thyroid cancer initially present with operable disease, some children have extensive disease that poses substantial surgical challenges and exposes them to higher than usual risk of operative complications. Extensive disease includes thyroid cancer that invades the trachea or esophagus or encases vascular or neural structures. Previous efforts to manage extensive thyroid cancer focused on surgery with near-curative intent. With the recent development of oncogene-specific targeted therapies that are effective in inducing tumor regression, with minimal drug-associated adverse effects, there is an opportunity to consider incorporating these agents as neoadjuvant therapy. In patients with morbidly invasive regional metastasis or with hypoxia associated with extensive pulmonary metastasis, neoadjuvant therapy can be incorporated to induce tumor regression before surgery and radioactive iodine therapy. For patients with widely invasive medullary thyroid cancer, in whom the risk of surgical complications is high and the likelihood of surgical remission is low, these agents may replace surgery depending on the response to therapy and long-term tolerance., Conclusions and Relevance: With oncogene-specific targeted therapy that is associated with substantial tumor regression and low risk of adverse reactions, there appears to be an opportunity to include children with advanced invasive thyroid cancer in clinical trials exploring neoadjuvant targeted oncogene therapy before or instead of surgery.

Importance: Limited information exists about the short-term morbidity of parotidectomy in children. This information is important when counseling parents and planning treatment for children with parotid masses., Objective: To examine 30-day perioperative outcomes after parotidectomy in the pediatric population., Design, Setting, and Participants: A prospective case series of 87 pediatric patients who underwent parotidectomy from January 1, 2008, to June 30, 2015, performed by 2 pediatric head and neck specialists in a tertiary care pediatric hospital., Main Outcomes and Measures: Thirty-day perioperative complications, postoperative facial nerve function, reoperation rates, and readmission rates were the main outcome measures. Secondary outcome measures were operative time and length of stay., Results: Ninety parotidectomies (71 superficial and 19 total) were performed in 87 patients (48 male and 39 female). Mean age at operation was 8.3 years. Eighty-five of the cases (94%) were performed for benign disease. Mean operative time was 4.09 hours (range, 1.58-10.43 hours) and mean length of stay was 2.24 days (range, 0.97-4.33 days). Immediate postoperative facial weakness was observed in 32 cases (36%); 10 of these cases (31%) involved the upper branch and 29 (90%) involved the lower branch. Immediate postoperative paresis occurred more often after total parotidectomy vs superficial parotidectomy (11 of 19 [58%] vs 21 of 71 [30%]; P = .03) but was unrelated to the etiologic cause of the patients' parotid disease. Thirty of 32 cases (94%) of facial nerve weakness were transient, and the mean time to resolution was 61 days. Eleven complications (12%) other than facial nerve weakness occurred during the 30-day postoperative period. Three patients (3%) required a return to the operating room for hematoma or wound dehiscence. One patient (1%) required readmission in the 30-day postoperative period for contralateral parotitis., Conclusions and Relevance: We observed a low rate of 30-day perioperative complications as a result of superficial or total parotidectomy for pediatric parotid disease. Major adverse events included return to the operating room and 1 wound infection. Only 1 patient required readmission. Transient weakness of the facial nerve is relatively common, observed more often in the lower division, and can be expected to resolve in most patients by an average of 2 months.

Importance: There is a perceived increase in the number of microdirect laryngoscopies and bronchoscopies (MLBs) required on premature infants, infants with syndromic conditions, and those with complex congenital heart defects. Determining which neonates with certain underlying conditions require more aggressive interventions like tracheostomy, intubation, or supraglottoplasty might be useful for future preoperative planning and counseling of the families of newborns with complex medical conditions involving the airway., Objectives: To evaluate features and findings in neonates undergoing MLB in the first 28 days of life over a 10-year period and compare these findings with past publications., Design, Setting, and Participants: Retrospective case series of 162 consecutive patients 28 days or younger undergoing MLB between January 1, 2002, and December 31, 2012, at a tertiary care children's hospital., Intervention: Microdirect laryngoscopy and bronchoscopy., Main Outcomes and Measures: Frequencies of common indications and findings in neonates undergoing MLB. To determine if findings on MLB had significant associations with gestational age, neonates with cardiac defects or syndromic conditions, and neonates who had at least 1 other comorbid condition, χ2 and Fisher exact tests were performed. Similar associations were analyzed between neonates with other comorbid conditions and need for interventions such as supraglottoplasty, tracheostomy, and intubation., Results: Of the 162 patients, 101 were male (55.5%). The mean age at their procedure was 14.1 days. The mean weight of patients at MLB was 3.31 kg, and 32 (17.6%) were premature. Congenital conditions were noted in 114 patients (62.6%), and of these, 55 (30.2%) had congenital cardiac disease and 30 (16.4%) had syndromic conditions. Common indications for surgery were respiratory distress (n = 145 [79.7%]), stridor (n = 102 [56.0%]), and cyanosis or an acute life-threatening event (n = 67 [36.8%]). The most common findings at the time of surgery were laryngomalacia (n = 71 [39.0%]), subglottic stenosis (n = 58 [31.9%]), and tracheomalacia (n = 47 [25.8%]). Seventeen neonates (9.3%) required a tracheostomy, and 11 (6.0%) required a supraglottoplasty. Neonates with congenital heart defects were statistically significantly more likely to require long-term intubation (n = 9 [16.4%]; P = .03). Those with syndromic conditions were more likely to require long-term intubation and tracheostomy (n = 7 [23.3%] [P = .004] and n = 7 [23.3%] [P = .01], respectively). Premature infants who required MLB had a decreased incidence of laryngomalacia (n = 7 [21.9%]; P = .03), and those with comorbid conditions, an increased incidence (n = 43 [33.3%]; P = .01). There was minimal morbidity directly associated with the procedure., Conclusions and Relevance: Neonates undergoing MLB most commonly presented with respiratory distress and stridor and were most commonly found to have laryngomalacia and subglottic stenosis. More than half of the patients studied had other comorbid conditions. Those with cardiac defects and syndromic conditions were more likely to require intubation, and those with syndromic conditions were more likely to eventually undergo tracheostomy.