Fundamentals and recent advances in the evaluation and management of medullary thyroid carcinoma.

Medullary thyroid carcinoma (MTC) is a rare primary neuroendocrine thyroid carcinoma that is distinct from other thyroid or neuroendocrine cancers. Most cases of MTC are sporadic, although MTC exhibits a high degree of heritability as part of the multiple endocrine neoplasia syndromes. REarranged during Transfection (RET) mutations are the primary oncogenic drivers and advances in molecular profiling have revealed that MTC is enriched in druggable alterations. Surgery at an early stage is the only chance for cure, but many patients present with or develop metastases. C-cell-specific calcitonin trajectory and structural doubling times are critical biomarkers to inform prognosis, extent of surgery, likelihood of residual disease, and need for additional therapy. Recent advances in the role of active surveillance, regionally directed therapies for localized disease, and systemic therapy with multi-kinase and RET-specific inhibitors for progressive/metastatic disease have significantly improved outcomes for patients with MTC.

Recurrent Laryngeal Nerve Invasion by Thyroid Cancer: Laryngeal Function and Survival Outcomes.

BACKGROUND: Recurrent laryngeal nerve (RLN) invasion by thyroid carcinoma represents an advanced disease status with potentially significant co-morbidity. METHODS: In a retrospective single-center study, we included patients with invaded RLNs operated on while using nerve monitoring techniques. We studied pre-, intra-, and postoperative parameters associated with postoperative vocal cord paralysis (VCP); 5-year recurrence-free survival (RFS); and 5-year overall survival (OS) in addition to two subgroup analyses of postoperative VCP in patients without preoperative VCP and based on source of RLN invasion. RESULTS: Of 65 patients with 66 nerves-at-risk, 39.3% reported preoperative voice complaints. Preoperative VCP was documented in 43.5%. The RLN was invaded by primary tumor in 59.3% and nodal metastasis in 30.5%. Papillary thyroid carcinoma was the most common pathologic subtype (80%). After 6 months, 81.8% had VCP. Complete tumor resection of the RLN was not associated with 5-year RFS (p = 0.24) or 5-year OS (p = 0.9). Resecting the RLN did not offer statistically significant benefit on 5-year RFS (p = 0.5) or 5-year OS (p = 0.38). Radioactive Iodine (RAI) administration was associated with improvement in 5-year RFS (p = 0.006) and 5-year OS (p = 0.004). Patients without preoperative VCP had higher IONM amplitude compared with patients with VCP. After a mean follow-up of 65.8 months, 35.9% of patients had distant metastases, whereas 36.4% had recurrence. CONCLUSION: Preoperative VCP accompanies less than half of patients with RLN invasion. Invaded RLNs may have existent electrophysiologic stimulability. Complete tumor resection and RLN resection were not associated with better 5-year RFS or OS, but postoperative RAI was. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2285-2292, 2022.

Pediatric intraoperative nerve monitoring during thyroid surgery: A review from the American Head and Neck Society Endocrine Surgery Section and the International Neural Monitoring Study Group.

Children are more likely to experience recurrent laryngeal nerve (RLN) injury during thyroid surgery. Intraoperative nerve monitoring (IONM) may assist in nerve identification and surgical decision making. A literature review of pediatric IONM was performed and used to inform a monitoring technique guide and expert opinion statements. Pediatric IONM is achieved using a variety of methods. When age-appropriate endotracheal tubes with integrated surface electrodes are not available, an alternative method should be used. Patient age and surgeon experience with laryngoscopy influence technique selection; four techniques are described in detail. Surgeons must be familiar with the nuances of monitoring technique and interpretation; opinion statements address optimizing this technology in children. Adult IONM guidelines may offer strategies for surgical decision making in children. In some cases, delay of second-sided surgery may reduce bilateral RLN injury risk.

Benign Intratracheal Thyroid: A Systematic Review of 43 Cases With Five New Case Reports.

OBJECTIVE/HYPOTHESIS: To examine the clinical features of benign intratracheal thyroid (ITT) and their management strategies and outcomes. STUDY DESIGN: Case series study. METHODS: This systemic review was conducted in two international academic centers. This review includes 43 patients: one new case from the Massachusetts Eye and Ear Infirmary, four new cases from Beijing Tongren Hospital, and 38 previously published cases. We analyzed these 43 cases and summarized the patients' epidemiological data, clinical features, and treatment regimens. RESULTS: ITTs were less common in men than in women (male:female ratio of 3:10). ITT was observed in patients as young as neonates and as old as 85 years. Orthotopic thyroid nodules were present in 55.8% of the patients with ITT. Malignancy was incidentally found in 4.6% of all ITTs. Imaging examinations showed that the ITTs were typically attached to the posterolateral/lateral tracheal wall of the first, second, or third tracheal rings. Tissue attachment between the ITT and normal thyroid lobes was seen in 59.5% of the patients. Thirty-seven patients underwent surgery: 30 underwent open surgery, and seven underwent endoscopic debulking resections. One neonate received thyroid suppression therapy. One patient with ITT and papillary thyroid cancer was treated with radiotherapy and ultimately died after recurrence. CONCLUSIONS: Surgical resection is an effective treatment for benign ITT. We hypothesized that abnormalities during the embryonic development of Berry's ligament might play a role in ITT pathogenesis. LEVEL OF EVIDENCE: NA Laryngoscope, 131:E2609-E2617, 2021.

Postoperative Disposition Following Pediatric Sistrunk Procedures: A National Database Query.

OBJECTIVE/HYPOTHESIS: Variability exists in the postoperative disposition of children following Sistrunk procedures. Management options include discharge home versus overnight observation, with the latter allowing monitoring for immediate postoperative complications, presumably reducing the need for subsequent readmission. This study investigates the association between overnight observation and ambulatory management of children undergoing Sistrunk procedures and relevant postoperative complication and revisit rates to clarify best practice for these patients. METHODS: This was a retrospective database review using the Pediatric Health Information System database from 2007 to 2016. RESULTS: The cited dataset identified 6,434 qualifying patients, categorized into ambulatory versus overnight observation cohorts. The overall 30-day revisit rate was 4.9%; the revisit rate with overnight observation (6.1%) was higher than for ambulatory patients (3.8%, adjusted odds ratio (OR) 1.60; 95% confidence interval (CI): 1.21, 2.12). Revisit rates were significantly higher in patients 2 years of age or younger compared to older patients (6.7% vs. 4.3%). The rates of return to the operating room for the observation versus ambulatory groups were 1.8% and 0.5%, respectively. Cervical fluid collection and neck swelling were among the most common revisit indications in both groups, with a mean time to presentation of 9 days. CONCLUSIONS: This study demonstrates that ambulatory management following a Sistrunk procedure is not associated with increased rates of common postoperative complications, readmission, or need for secondary surgical intervention. A Sistrunk procedure may be safely performed on an ambulatory basis in select cases. LEVEL OF EVIDENCE: IV Laryngoscope, 131:E2352-E2355, 2021.

Immediate and partial neural dysfunction after thyroid and parathyroid surgery: Need for recognition, laryngeal exam, and early treatment.

BACKGROUND: Laryngeal dysfunction after thyroid and parathyroid surgery requires early recognition and a standardized approach for patients that present with voice, swallowing, and breathing issues. The Endocrine Committee of the American Head and Neck Society (AHNS) convened a panel to define the terms "immediate vocal fold paralysis" and "partial neural dysfunction" and to provide clinical consensus statements based on review of the literature, integrated with expert opinion of the group. METHODS: A multidisciplinary expert panel constructed the manuscript and recommendations for laryngeal dysfunction after thyroid and parathyroid surgery. A meta-analysis was performed using the literature and published guidelines. Consensus was achieved using polling and a modified Delphi approach. RESULTS: Twenty-two panelists achieved consensus on five statements regarding the role of early identification and standardization of evaluation for patients with "immediate vocal fold paralysis" and "partial neural dysfunction" after thyroid and parathyroid surgery. CONCLUSION: After endorsement by the AHNS Endocrine Section and Quality of Care Committee, it received final approval from the AHNS Council.

Parathyroid 4D CT: What the Surgeon Wants to Know.

Parathyroid four-dimensional (4D) CT is an increasingly used and powerful tool for preoperative localization of abnormal parathyroid tissue in the setting of primary hyperparathyroidism. Accurate and precise localization of a single adenoma facilitates minimally invasive parathyroidectomy, and localization of multiglandular disease aids bilateral neck exploration. However, many radiologists find the interpretation of these examinations to be an intimidating challenge. The authors review parathyroid 4D CT findings of typical and atypical parathyroid lesions and provide illustrative examples. Relevant anatomy, embryology, and operative considerations with which the radiologist should be familiar to provide clinically useful image interpretations are also discussed. The most important 4D CT information to the surgeon includes the number, size, and specific location of candidate parathyroid lesions with respect to relevant surgical landmarks; the radiologist's opinion and confidence level regarding what each candidate lesion represents; and the presence or absence of ectopic or supernumerary parathyroid tissue, concurrent thyroid pathologic conditions, and arterial anomalies associated with a nonrecurrent laryngeal nerve. The authors provide the radiologist with an accessible and practical approach to performing and interpreting parathyroid 4D CT images, detail what the surgeon really wants to know from the radiologist and why, and provide an accompanying structured report outlining the key information to be addressed. By accurately reporting and concisely addressing the key information the surgeon desires from a parathyroid 4D CT examination, the radiologist substantially impacts patient care by enabling the surgeon to develop and execute the best possible operative plan for each patient. ©RSNA, 2020.

To drain or not to drain following a Sistrunk procedure: A dual institutional experience.

INTRODUCTION: A Sistrunk procedure is the standard operative management of patients with thyroglossal duct cysts. Drain placement is commonly employed with the goal of reducing postoperative complications. This study investigates the association between drain use and relevant postoperative complications following pediatric Sistrunk procedures. METHODS: Retrospective cohort study evaluating 295 pediatric patients treated between 2007 and 2016 at two tertiary care children's hospitals. RESULTS: The mean age of the study population was 5.6 years (SD 4.0). A drain was utilized in 234 cases (79.3%). The mean procedural duration was 108 min (SD 48), and significantly longer in patients receiving a drain. Early postoperative complications included seroma (5.8%), secondary infection (3.4%), wound breakdown (2.0%) and hematoma (0.3%). The risk of such complications did not significantly differ between patients without drain placement (9.8%) versus those who underwent surgical drain placement (12.0%) after accounting for age and history of preoperative infection (adjusted RR = 0.86; 95% CI: 0.37, 1.98; p = 0.72). In the subgroup analysis, findings were consistent across institutions, age category, history of infection, and primary versus secondary procedure. CONCLUSION: This dual institutional study found drain placement during a Sistrunk procedure may not reduce rates of common postoperative complications, even in longer duration cases in which a drain is more frequently placed. This data suggests a Sistrunk procedure may be safely performed without drain placement in select cases.

Intraoperative Ultrasound for Localization and Removal of an Oropharyngeal Wire Grill-Brush Bristle.

OBJECTIVES: This paper reports the utilization of intraoperative ultrasound in the removal of an accidentally ingested wire grill-brush bristle from the pharyngeal space of a child. METHODS: Standard procedures for obtaining imaging of an accidentally ingested foreign body were performed, including radiograph, computed tomography (CT) imaging, and preoperative ultrasonography. Despite preoperative imaging, the object could neither be located nor removed. Ultrasonography was performed intraoperatively for real-time localization. RESULTS: Intraoperative ultrasonography was required to successfully locate and remove the wire grill-brush bristle from the patient's oropharyngeal space. CONCLUSIONS: Intraoperative ultrasound serves as a useful tool to guide surgical removal of aerodigestive foreign bodies and may offer an opportunity to reduce the need for CT imaging.

International neuromonitoring study group guidelines 2018: Part II: Optimal recurrent laryngeal nerve management for invasive thyroid cancer-incorporation of surgical, laryngeal, and neural electrophysiologic data.

The purpose of this publication was to inform surgeons as to the modern state-of-the-art evidence-based guidelines for management of the recurrent laryngeal nerve invaded by malignancy through blending the domains of 1) surgical intraoperative information, 2) preoperative glottic function, and 3) intraoperative real-time electrophysiologic information. These guidelines generated by the International Neural Monitoring Study Group (INMSG) are envisioned to assist the clinical decision-making process involved in recurrent laryngeal nerve management during thyroid surgery by incorporating the important information domains of not only gross surgical findings but also intraoperative recurrent laryngeal nerve functional status and preoperative laryngoscopy findings. These guidelines are presented mainly through algorithmic workflow diagrams for convenience and the ease of application. These guidelines are published in conjunction with the INMSG Guidelines Part I: Staging Bilateral Thyroid Surgery With Monitoring Loss of Signal. Level of Evidence: 5 Laryngoscope, 128:S18-S27, 2018.

Octopuses (Octopus bimaculoides) and cuttlefishes (Sepia pharaonis, S. officinalis) can conditionally discriminate.

In complex navigation using landmarks, an animal must discriminate between potential cues and show context (condition) sensitivity. Such conditional discrimination is considered a form of complex learning and has been associated primarily with vertebrates. We tested the hypothesis that octopuses and cuttlefish are capable of conditional discrimination. Subjects were trained in two maze configurations (the conditions) in which they were required to select one of two particular escape routes within each maze (the discrimination). Conditional discrimination could be demonstrated by selecting the correct escape route in each maze. Six of ten mud-flat octopuses (Octopus bimaculoides), 6 of 13 pharaoh cuttlefish (Sepia pharaonis), and one of four common cuttlefish (S. officinalis) demonstrated conditional discrimination by successfully solving both mazes. These experiments demonstrate that cephalopods are capable of conditional discrimination and extend the limits of invertebrate complex learning.