Management of Tracheobronchial Stenosis in Chondrodysplasia Punctata.

Chondrodysplasia punctata (CDP) is a rare congenital syndrome characterized by aberrant, punctate deposition of calcium during endochondral bone formation, resulting in the characteristic finding of epiphyseal stippling on radiographs. While otolaryngologic manifestations such as nasomaxillary hypoplasia and mixed hearing loss are common, tracheobronchial calcification occurs rarely in neonates with CDP. The management of CDP-related airway stenosis is complex and there is limited literature pertaining to outcomes of airway interventions. Herein, we describe the clinical course and outcome of tracheal dilation for a newborn patient with CDP. Laryngoscope, 134:1464-1468, 2024.

Surgical and Biochemical Outcomes in Nerve Monitored Reoperation Surgery for Recurrent Papillary Thyroid Carcinoma.

OBJECTIVE: To study the surgical and biochemical outcomes in nerve-monitored reoperation or revision surgery for recurrent thyroid cancers. STUDY DESIGN: A single-center retrospective study. SETTING: Tertiary center. METHODS: We identified patients with recurrent papillary thyroid carcinoma (PTC) who underwent reoperation/revision surgery. Study outcomes were surgical complications frequency, recurrence, distant metastasis, and biological complete response (BCR) by comparing preoperative and postoperative thyroglobulin (Tg) levels. RESULTS: Out of 227 patients, 33.9% presented for ≥2 reoperation surgeries. Nineteen (8.4%) had permanent preoperative hypoparathyroidism while 22 patients (9.7%) had preoperative vocal cord paralysis (VCP). Following reoperation surgery, there were 12 cases (5.3%) of permanent hypocalcemia and no cases of unexpected postoperative VCP. BCR was achieved in 31 patients (35.2%) with complete Tg data. Mean preoperative Tg was 47.7 ng/mL and was 19.7 ng/mL postoperatively (p = .003). The cervical nodal recurrence rate after final surgery was 7.0% (n = 16). CONCLUSION: Reoperation surgery for recurrent PTC may help achieve biochemical remission regardless of age or the number of prior surgeries.

Development of an interdisciplinary microtia-atresia care model: A single-center 20-year experience.

Objectives: Microtia and aural atresia are congenital ear anomalies with a wide-ranging spectrum of phenotypes and varied functional and psychosocial consequences for patients. This study seeks to analyze the management of microtia-atresia patients at our center over a 20-year period and to propose recommendations for advancing microtia-atresia care at a national level. Methods: We performed a retrospective analysis of data from patients presenting to the Massachusetts Eye and Ear (Boston, MA) for initial otolaryngology consultation for congenital microtia and/or aural atresia between 1999 and 2018. Results: Over the 20-year study period, 229 patients presented to our microtia-atresia center at a median age of 7 years. The severity of microtia was most commonly classified as grade III (n = 87, 38%), 61% (n = 140) of patients had complete atresia, the median Jahrsdoerfer grading scale score was 6 (range 0-10), and 81 patients (35%) underwent surgery for microtia repair. For hearing rehabilitation, 30 patients (64%) underwent bone conduction device implantation and 17 patients (36%) underwent atresiaplasty. The implementation of an interdisciplinary, longitudinal care model resulted in an increase in patient (r = 0.819, p < .001) and surgical volume (microtia surgeries, r = 0.521, p = .019; otologic surgeries, r = 0.767, p < .001) at our center over time. Conclusion: An interdisciplinary team approach to microtia-atresia patient care may result in increased patient volume, which could improve aesthetic and hearing outcomes over time by concentrating care and surgical expertise. Future work should aim to establish standardized clinical consensus recommendations to guide the creation of high-quality microtia-atresia care programs. Level of Evidence: 4.

Emerging trends in rhinoplasty education: accelerated adoption of digital tools and virtual learning platforms.

PURPOSE OF REVIEW: The COVID-19 pandemic catalyzed the rapid adoption of digital tools and virtual learning platforms by rhinoplasty educators and trainees alike. This review provides an overview of the variety of digital software and web-based tools rhinoplasty educators have adopted and highlights the advantages and potential drawbacks of virtual learning via e-content. RECENT FINDINGS: Medical education including subspecialty surgical training has recently undergone a dramatic digital transformation. Rhinoplasty surgeon-educators have been forced to embrace new digital tools, including videoconferencing, podcasts, virtual simulation and social media to reach and teach trainees. Recognizing the advantages of this new, limitless digital space, rhinoplasty surgeons are also engaging in virtual transcontinental collaboration and distance mentorship. SUMMARY: The dramatic evolution in how clinical educational materials are now digitally created, curated, disseminated and consumed is likely to far outlast the COVID-19 pandemic itself. Rapid, exponential growth of this digital library, however, places increased responsibility on educators to guide trainees towards evidence-based and state-of-the-art content.

Prolapsed Nasal Polyp Causing Acute Airway Obstruction: An Exceptional Presentation.

Although nasal polyposis is a common clinical entity, there is limited literature describing the rare presentation of sudden prolapse of a massive nasal polyp resulting in an airway emergency in an adult. We present the first case report to our knowledge of a patient without any preceding sinonasal symptoms or history of anticoagulation who experienced acute upper airway obstruction due to sudden hemorrhage and prolapse of a large nasal polyp. Based on our experience treating this patient, we discuss special considerations in all phases of care to ensure safe and effective management of such an exceptional clinical scenario.

Leveraging telemedicine to preserve pediatric global health missions in the era of COVID-19.

This paper outlines the use of a global telehealth program to leverage the potential of telehealth to not only 1) preserve the previous progress of our pediatric surgical airway global teaching mission, but also: 2) to provide rapid, international dissemination of information related to care of pediatric COVID-19 patients; 3) to virtually support the attainment of self-sufficiency of our host countries in relation to our teaching mission; and 4) to inspire host countries to be local champions for each other during the COVID-19 crisis.

COVID-19 associated parotitis.

As the 2019 coronavirus pandemic has unfolded, an increasing number of atypical presentations of COVID-19 have been reported. As patients with COVID-19 often present to emergency departments for initial care, it is important that emergency clinicians are familiar with these atypical presentations in order to prevent disease transmission. We present a case of a 21-year-old woman diagnosed in our ED with COVID-19 associated parotitis and review the epidemiology and management of parotitis. We discuss the importance of considering COVID-19 in the differential of parotitis and other viral-associated syndromes and emphasize the importance of donning personal protective equipment during the initial evaluation.

Carotid artery mobilization prior to pharyngeal flap inset for patients with 22q11.2 deletion syndrome.

The management of velopharyngeal insufficiency (VPI) in patients with 22q11.2 deletion syndrome (22q11DS) poses a significant clinical challenge due to presence of a large velopharyngeal gap and a relatively high rate of internal carotid artery (ICA) medialization. To our knowledge, we are the first group to have successfully managed VPI in a series of seven pediatric patients with 22q11DS with medialized ICAs via a novel surgical technique involving carotid artery mobilization followed by pharyngeal flap insertion. Thus far, we have found this technique to be reliably safe with no significant morbidity and caregivers have reported postoperative improvement in speech, swallowing and nasal regurgitation symptoms. Herein, we provide a detailed description of our novel surgical approach, including an instructional video, for correction of VPI in patients with medialized ICAs, who have previously had limited management options.

p53: An Attractive Therapeutic Target for Cancer.

Cancer is a leading cause of death worldwide. It initiates when cell cycle regulatory genes lose their function either by environmental and/or by internal factors. Tumor suppressor protein p53, known as "Guardian of genome", plays a central role in maintaining genomic stability of the cell. Mutation of TP53 is documented in more than 50% of human cancers, usually by overexpression of negative regulator protein MDM2. Hence, reactivation of p53 by blocking the protein-protein interaction between the murine double minute 2 (MDM2) and the tumor suppressor protein p53 has become the most promising therapeutic strategy in oncology. Several classes of small molecules have been identified as potent, selective and efficient p53-MDM2 inhibitors. Herein, we review the druggability of p53-MDM2 inhibitors and their optimization approaches as well as clinical candidates categorized by scaffold type.

Extended Survival After Surgical Resection for Pituitary Metastases: Clinical Features, Management, and Outcomes of Metastatic Disease to the Sella.

BACKGROUND: Patients with pituitary metastasis (PM) have a relatively poor prognosis. We describe the presentation, management, and outcomes of patients with PM. SUBJECTS, MATERIALS, AND METHODS: We performed a retrospective review of patients diagnosed with PM at a single institution from 1996 to 2015. Eighty-five patients diagnosed with metastasis to the pituitary or sella turcica by pathology or based on a combination of neuroimaging and clinical findings were included. Univariate and multivariable Cox regressions evaluated associations between clinical factors and overall survival. RESULTS: The most frequent sites of primary malignancies resulting in PM were lung (26%) and breast (26%). Median age at diagnosis was 60 years (range, 18-95). The most common complaints at diagnosis included visual deficits (62%), headache (47%), and cranial nerve palsy (31%). Seventy percent of patients had pituitary insufficiency-adrenal insufficiency (59%), hypothyroidism (59%), or diabetes insipidus (28%). Management of PM included radiation therapy (76%), chemotherapy (68%), surgical resection (21%), or combination therapy (71%). Fifty percent and 52% of patients who received surgical treatment and irradiation, respectively, reported symptomatic improvement. Median overall survival (OS) was 16.5 months (95% confidence interval: 10.7-25.4). On multivariable analysis, a primary cancer site other than lung or breast (p = .020), age <60 years (p = .030), and surgical resection (p = .016) were associated with longer OS. CONCLUSION: Patients <60 years of age, those with primary tumor sites other than lung or breast, and those who undergo surgical resection of the pituitary lesion may have prolonged survival. Surgical resection and radiation treatment resulted in symptomatic improvement in ~50% of patients. IMPLICATIONS FOR PRACTICE: This study is the largest original series of patients with metastatic disease to the sella. In patients with pituitary metastasis, younger age, primary site other than lung or breast, and metastatic resection may prolong survival. Resection and radiation led to symptomatic improvement in ∼50% of patients. Seventy percent of patients had hypopituitarism. These hormonal deficiencies can be life threatening and can result in substantial morbidity if left untreated. Patients should be treated using a multimodality approach-including a potential role for surgery, radiation, chemotherapy, and hormone replacement-with the goal of improving survival and quality of life.

Value within otolaryngology: Assessment of the cost-utility analysis literature.

OBJECTIVE: To assess the characteristics and quality of cost utility analyses (CUA) related to otolaryngology within the CEA registry and to summarize their collective results. METHODS: All cost-utility analyses published between 1976 and 2011 contained in the Cost-Effectiveness Analysis Registry (CEA Registry) were evaluated. Topics that fall within the care of an otolaryngologist were included in the review regardless of the presence of an otolaryngologist author. Potential associations between various study characteristics and CEA registry quality scores were evaluated using the Pearson product moment correlation coefficient. RESULTS: Sixty-one of 2913 (2.1%) total CUA publications screened were related to otolaryngology. Eighteen of 61 (29.5%) publications included an otolaryngologist as an author. Fourteen studies agreed on the cost effectiveness of at least unilateral cochlear implantation and six of seven (85.7%) studies demonstrated the cost effectiveness of continuous positive airway pressure (CPAP) for obstructive sleep apnea (OSA). Forty-six percent (28 of 61) of all manuscripts were published between 2008 and 2011. A more recent publication year was associated with a higher CEA registry quality score while the presence of an otolaryngologist author and journal impact factor had no significant correlation with the quality of the CUA. CONCLUSION: Based on current evidence in the CEA registry, unilateral cochlear implantation for hearing loss and CPAP for OSA are both cost-effective therapeutic interventions. Although CUAs in otolaryngology have increased in quantity and improved in quality in more recent years, there is a relative lack of CUAs in otolaryngology in comparison to other subspecialties.

Natural history and management of Fanconi anemia patients with head and neck cancer: A 10-year follow-up.

OBJECTIVES/HYPOTHESIS: To describe the management and outcomes of Fanconi anemia (FA) patients with head and neck squamous cell carcinoma. STUDY DESIGN: Cohort study. METHODS: Demographic information, prognostic factors, therapeutic management, and survival outcomes for FA patients enrolled in the International Fanconi Anemia Registry who developed head and neck squamous cell carcinoma (HNSCC) were analyzed. RESULTS: Thirty-five FA patients were diagnosed with HNSCC at a mean age of 32 years. The most common site of primary cancer was the oral cavity (26 of 35, 74%). Thirty patients underwent surgical resection of the cancer. Sixteen patients received radiation therapy with an average radiation dose of 5,050 cGy. The most common toxicities were high-grade mucositis (9 of 16, 56%), hematologic abnormalities (8 of 16, 50%), and dysphagia (8 of 16, 50%). Three patients received conventional chemotherapy and had significant complications, whereas three patients who received targeted chemotherapy with cetuximab had fewer toxicities. The 5-year overall survival rate was 39%, with a cause-specific survival rate of 47%. CONCLUSIONS: Fanconi anemia patients have a high risk of developing aggressive HNSCC at an early age. Fanconi anemia patients can tolerate complex ablative and reconstructive surgeries, but careful postoperative care is required to reduce morbidity. The treatment of FA-associated HNSCC is difficult secondary to the poor tolerance of radiation and chemotherapy. However, radiation should be used for high-risk cancers due to the poor survival in these patients. LEVEL OF EVIDENCE: 4.

Retrospective Assessment of the Utility of Imaging, Fine-Needle Aspiration Biopsy, and Intraoperative Frozen Section in the Management of Parotid Neoplasms: The Weill Cornell Medical College Experience.

BACKGROUND/AIMS: Preoperative identification of malignant parotid lesions remains challenging, and thus, some surgeons use frozen section (FS) to assist them in their decision making. We evaluated the pathologic and cost benefit of FS after fine-needle aspiration (FNA) at our institution. METHODS: We assessed medical data for 260 patients undergoing parotidectomy with FS. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value were calculated for radiology, FNA, and FS. RESULTS: The sensitivities, specificities, and accuracies of FNA and FS were 75.0, 96.4, and 93.2%, and 75.0, 100, and 96.8%, respectively. FS detected 0% of FNA false negatives and 80% of false positives. The additional pathology charge for FS alone per correctly identified benign lesion after a positive FNA was USD 1,443. CONCLUSION: FNA and FS are more reliable in the prognostication of the final pathology than radiology. At our center, FS appears to be of limited clinical use after benign FNAs, but may be more useful after positive, indeterminate, and nondiagnostic FNAs.

Differential subcellular Ca2+ signaling in a highly specialized subpopulation of astrocytes.

Recent evidence suggests that astrocytes do not serve a mere buffering function, but exhibit complex signaling pathways, disturbance of which contributes significantly to the pathophysiology of CNS diseases. Little is known regarding the intracellular signaling pathways in the specialized optic nerve head astrocytes (ONHAs), the major glia cell type in non-myelinated optic nerve head. Here we show the differential subcellular expression of intracellular Ca(2+) channels in ONHAs. Expression of type 1 and type 3 inositol-1-4-5,-trisphosphate receptors (IP3Rs) in the endoplasmic reticulum and type 2 IP3Rs in the nuclear envelope causes differential Ca(2+) release from intracellular stores in nuclear vs. cytosolic compartments. Our study identifies differential distribution and activity of Ca(2+) channels as molecular substrate and mechanism by which astrocytes independently regulate Ca(2+) transients in both cytoplasm and nucleoplasm, thereby controlling genomic and non-genomic cellular signaling, respectively. This provides excellent targets for therapeutics restoring pathological disturbances of intracellular Ca(2+) signaling present in glaucoma and other neurodegenerative disorders with astrocyte involvement.

Retrospective evaluation of frozen section use for thyroid nodules with a prior fine needle aspiration diagnosis of Bethesda II-VI: The Weill Cornell Medical College experience.

OBJECTIVE: To evaluate the Weill Cornell Medical College (WCMC)/New York Presbyterian Hospital (NYPH) experience with intraoperative frozen (IOF) section in the management of thyroid nodules with a fine needle aspiration (FNA) diagnosis of Bethesda II-VI and to analyze the cost and pathology benefit it provides. METHODS: The surgical and cytopathology files at WCMC/NYPH were searched within the time period of January 2008 to May 2013. A total of 435 thyroid specimens were identified for which both an FNA and subsequent IOF section was performed. The FNA was correlated with the locations of the resected nodule and the nodule frozen for intraoperative diagnosis. The results of the FNA were compared to the IOF section diagnosis and final diagnosis (FD). RESULTS: Among 435 cases, the FNA diagnosis was Bethesda II: 149 cases, Bethesda III: 170 cases, Bethesda IV: 91 cases, Bethesda V: 19 cases, and Bethesda VI: 6 cases. There were a total of 83 carcinomas identified on FD, which included 69 papillary thyroid carcinomas (PTCs), 12 follicular carcinomas, and 2 poorly differentiated carcinomas. The preoperative FNA diagnosis for these carcinomas was as follows: Bethesda II, 11/149 (7.4%), Bethesda III, 24/170 (14%), Bethesda IV, 26/91 (29%), Bethesda V, 16/19 (84%), and Bethesda VI, 6/6 (100%). IOF section contributed to the diagnosis of malignancy in 16/429 (4%) cases: 1/149 (0.7%) Bethesda II, 5/170 (3%) Bethesda III, 2/91 (1.1%) Bethesda IV, and 8/19 (42%) Bethesda V. The diagnosis of malignancy was confirmed in the 6 Bethesda VI cases by IOF section. There were no false positives on IOF section. IOF had a sensitivity and specificity of 26% and 100%, respectively. CONCLUSION: The role of IOF section is limited in the evaluation of thyroid nodules. IOF section is most useful for nodules with an FNA diagnosis of Bethesda V lesions. The diagnosis of follicular variant of PTC remains difficult on frozen section.

Intravenous pantoprazole utilization in a level 1 trauma center.

BACKGROUND: In recent years there has been a rapid increase in the use of proton pump inhibitors. Our institution has recently had several shortages of IV pantoprazole, each lasting 7-10 days. The purpose of our study was to evaluate in-patient usage of IV pantoprazole. We hypothesized that hospitalized patients with upper gastrointestinal bleeding (GIB) or risk for stress ulcers inappropriately received IV pantoprazole based on current literature. METHODS: This was a retrospective study of 165 consecutive in-patients identified as receiving pantoprazole from December 2004 to March 2005. Only patients receiving IV pantoprazole were included (n = 78). Data collected included demographics, indication and dosing of pantoprazole, admitting team (surgery vs. medicine), and risk factors for stress ulcers. RESULTS: Our study population had a mean age of 54 +/- 17 years and 62% were male. Overall, 45% (35/78) of patients receiving IV pantoprazole had an appropriate indication, and 19% (15/78) received the correct dose. Of the 78 patients, 43 (55%) were treated with pantoprazole for stress ulcer prophylaxis (SUP), and 35 (45%) patients were treated for GIB. We found that none of the 43 patients treated for SUP had an appropriate indication for pantoprazole, but all of the patients with GIB (35) had an appropriate indication. Of the 35 patients treated for GIB with pantoprazole, only 40% (14/35) received the correct dose. In all cases of incorrect dosing, the patients were underdosed. CONCLUSIONS: Pantoprazole is not being prescribed appropriately for stress ulcer prophylaxis in our patient population. Even in patients appropriately receiving pantoprazole the majority were prescribed an incorrect dose. Appropriate indications and dosing of pantoprazole could eliminate the shortages seen at our institution.