Faculty Bibliography

Introduction: Pediatric-type follicular lymphoma (PTFL) is a rare B-cell lymphoma that primarily affects the head and neck, accounting for approximately 1.5"“2% of childhood lymphomas. Distinguishing between follicular lymphoma and PTFL is critical due to their distinct clinical characteristics, which influence prognosis and treatment. Case presentation: Two patients, a 14-year-old male (Case 1) and a 17-year-old male (Case 2), presented to the pediatric otolaryngology clinic with complaints of an isolated neck and parotid masses, respectively. In Case 1, physical examination revealed a 5 cm upward-growing neck mass without accompanying B symptoms or discomfort. CT scan, needle biopsy, and MRI with contrast confirmed the presence of the mass, which was subsequently surgically excised. In Case 2, the patient had an enlarging left-sided parotid mass, along with night sweats, fatigue, and arthralgias. Ultrasound and contrast-enhanced MRI confirmed a well-defined solid vascular mass within the left parotid gland, which was subsequently surgically excised. Pathological examination confirmed PTFL in both cases, leading to a positron emission tomography (PET)/CT scan for further evaluation and staging. Conclusion: This case series underscores the importance of pediatric surgeons to consider PTFL in the differential diagnosis of head and neck masses, as it can be effectively cured through surgical excision.

BACKGROUND AND OBJECTIVES/OBJECTIVE:An international, multicenter, retrospective study was conducted to evaluate the long-term clinical outcomes and tumor control rates after stereotactic radiosurgery (SRS) for trigeminal schwannoma. METHODS:Patient data (N = 309) were collected from 14 international radiosurgery centers. The median patient age was 50 years (range 11-87 years). Sixty patients (19%) had prior resections. Abnormal facial sensation was the commonest complaint (49%). The anatomic locations were root (N = 40), ganglion (N = 141), or dumbbell type (N = 128). The median tumor volume was 4 cc (range, 0.2-30.1 cc), and median margin dose was 13 Gy (range, 10-20 Gy). Factors associated with tumor control, symptom improvement, and adverse radiation events were assessed. RESULTS:The median and mean time to last follow-up was 49 and 65 months (range 6-242 months). Greater than 5-year follow-up was available for 139 patients (45%), and 50 patients (16%) had longer than 10-year follow-up. The overall tumor control rate was 94.5%. Tumors regressed in 146 patients (47.2%), remained unchanged in 128 patients (41.4%), and stabilized after initial expansion in 20 patients (6.5%). Progression-free survival rates at 3 years, 5 years, and 10 years were 91%, 86%, and 80 %. Smaller tumor volume (less than 8 cc) was associated with significantly better progression-free survival ( P = .02). Seventeen patients with sustained growth underwent further intervention at a median of 27 months (3-144 months). Symptom improvement was noted in 140 patients (45%) at a median of 7 months. In multivariate analysis primary, SRS ( P = .003) and smaller tumor volume ( P = .01) were associated with better symptom improvement. Adverse radiation events were documented in 29 patients (9%). CONCLUSION/CONCLUSIONS:SRS was associated with long-term freedom (10 year) from additional management in 80% of patients. SRS proved to be a valuable salvage option after resection. When used as a primary management for smaller volume tumors, both clinical improvement and prevention of new deficits were optimized.

OBJECTIVE:This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA). METHODS:We conducted a retrospective analysis of 2 children who were referred for an otolaryngology consultation between 2019 and 2022 for surgical treatment of PFAPA syndrome. Both patients had symptoms strongly suggestive of PFAPA and were at risk for total tonsillectomy (TT) complications. ITA was performed using a microdebrider. Both patients were followed up postoperatively to assess for symptomatic resolution and complications. RESULTS:Two children exhibited recurrent febrile episodes prior to ITA. The procedure was efficacious in both patients, with neither experiencing postoperative complications or recurring PFAPA symptoms for over 1 year after surgery. CONCLUSION/CONCLUSIONS:Our study reported on the use of ITA as a surgical treatment option for PFAPA. We showed that ITA eliminated febrile attacks and was safely performed without postoperative complications in 2 pediatric patients after 1-year follow-up. Future studies involving larger cohorts of PFAPA patients and lengthier follow-ups will need to be conducted to further evaluate ITA as a surgical option. Laryngoscope, 2023.

OBJECTIVE:To assess the safety of cochlear implants (CIs) in children under 9 months of age to better understand expected postoperative complication rates, and to provide a preliminary look at efficacy. STUDY DESIGN/METHODS:Single-center retrospective review. SETTING/METHODS:Tertiary referral center. PATIENTS/METHODS:Children 5 to 8 months of age who received a CI between 2011 and 2021. INTERVENTIONS/METHODS:Therapeutic-CI. MAIN OUTCOME MEASURES/METHODS:The primary outcome was 6-month postoperative complication rate. Secondary outcomes included length of stay, device explantation/reimplantation, rehabilitation supports, and hearing and language outcomes. Complications of children implanted under than 9 months of age was compared with a cohort of children implanted 9 to 18 months of age between the years 2011 and 2016 using a chi-squared test (p < 0.05). RESULTS:One hundred six children under 9 months of age were implanted (204 CIs) at a mean age of 6.6 months (range: 5-8). Postoperative complications occurred in 28 patients (26%) and were often minor. There were no mortalities. There was no statistically significant difference in complications, including reimplantation rates, between patients implanted under 9 months of age and those implanted 9 to 18 months of age. Speech discrimination outcomes were excellent, and mean spoken language outcomes were near normative for typically developing children. CONCLUSIONS:Cochlear implantation can be a safe and effective treatment option for children 5 to 8 months of age. Early hearing screening and referral for infants to receive appropriate intervention will continue to play a critical role in optimizing speech and language outcomes.

Astrocytes play crucial roles in regulating neural circuit function by forming a dense network of synapse-associated membrane specializations, but signaling pathways regulating astrocyte morphogenesis remain poorly defined. Here, we show the Drosophila lipid-binding G protein-coupled receptor (GPCR) Tre1 is required for astrocytes to establish their intricate morphology in vivo. The lipid phosphate phosphatases Wunen/Wunen2 also regulate astrocyte morphology and, via Tre1, mediate astrocyte-astrocyte competition for growth-promoting lipids. Loss of s1pr1, the functional analog of Tre1 in zebrafish, disrupts astrocyte process elaboration, and live imaging and pharmacology demonstrate that S1pr1 balances proper astrocyte process extension/retraction dynamics during growth. Loss of Tre1 in flies or S1pr1 in zebrafish results in defects in simple assays of motor behavior. Tre1 and S1pr1 are thus potent evolutionarily conserved regulators of the elaboration of astrocyte morphological complexity and, ultimately, astrocyte control of behavior.

OBJECTIVES/OBJECTIVE:A method for stimulating the cochlear apex using perimodiolar electrode arrays is described. This method involves implanting an electrode (ECE1) into the helioctrema in addition to standard cochlear implant placement. One objective is to verify a suitable approach for implanting ECE1 in the helicotrema. Another is to determine how placement of ECE1 reshapes electric fields. DESIGN/METHODS:Two cadaveric half-heads were implanted, and electric voltage tomography was measured with ECE1 placed in many positions. RESULTS:An approach for placing ECE1 was identified. Changes in electric fields were only observed when ECE1 was placed into the fluid in the helicotrema. When inside the helicotrema, electric voltage tomography modeling suggests an increased current flow toward the apex. CONCLUSIONS:Placement of ECE1 into the cochlear apex is clinically feasible and has the potential to reshape electric fields to stimulate regions of the cochlea more apical than those represented by the electrode array.

BACKGROUND:There are no effective medical therapies for patients with meningioma who progress beyond surgical and radiotherapeutic interventions. Somatostatin receptor Type 2 (SSTR2) represents a promising treatment target in meningiomas. In this multicenter, single-arm phase II clinical study (NCT03971461), the SSTR2-targeting radiopharmaceutical 177Lu-DOTATATE is evaluated for its feasibility, safety, and therapeutic efficacy in these patients. PATIENTS AND METHODS/METHODS:Adult patients with progressive intracranial meningiomas received 177Lu-DOTATATE at a dose of 7.4 GBq (200 mCi) every eight weeks for four cycles. 68Ga-DOTATATE PET-MRI was performed before and six months after begin of treatment. The primary endpoint was progression-free survival (PFS) at 6 months (PFS-6). Secondary endpoints were safety and tolerability, overall survival (OS) at 12 months (OS-12), median PFS, and median OS. RESULTS:Fourteen patients (F=11, M=3) with progressive meningiomas (WHO 1=3, 2=10, 3=1) were enrolled. Median age was 63.1 (range 49.7-78) years. All patients previously underwent tumor resection and at least one course of radiation. Treatment with 177Lu-DOTATATE was well tolerated. Seven patients (50%) achieved PFS-6. Best radiographic response by modified Macdonald criteria was stable disease (SD) in all seven patients. A >25% reduction in 68Ga-DOTATATE (PET) was observed in five meningiomas and two patients. In one lesion, this corresponded to >50% reduction in bidirectional tumor measurements (MRI). CONCLUSIONS:Treatment with 177Lu-DOTATATE was well tolerated. The predefined PFS-6 threshold was met in this interim analysis, thereby allowing this multicenter clinical trial to continue enrollment. 68Ga-DOTATATE PET may be a useful imaging biomarker to assess therapeutic outcome in patients with meningioma.

PURPOSE/OBJECTIVE:Rhinoplasty is amongst the most challenging surgeries to perfect and can take decades. This process begins during residency; however, residents often have limited exposure to rhinoplasty during their training and lack a standardized method for systematically analyzing and formulating a surgical plan. The DESS (Deformity, Etiology, Solution, Sequence) is a novel educational format for residents that serves to increase their pre-operative comfort with the surgical evaluation and intraoperative planning for a rhinoplasty. MATERIALS AND METHODS/METHODS:A qualitative study performed at a tertiary academic institution with an otolaryngology residency program evaluating three consecutive residency classes comprised of four residents per class. A 9-item questionnaire was distributed to measure change in resident comfort after utilizing the DESS during their facial plastics rotation. Questionnaire responses highlighted resident comfort with facial nasal analysis, identifying deformities, suggesting surgical maneuvers, and synthesizing a comprehensive surgical plan. RESULTS:Ten of the twelve residents surveyed responded. Of those that responded, comfort in facial nasal analysis, identification of common nasal deformities, surgical planning, and development of an overall surgical plan were significantly improved after completion of the facial plastic rotation. These residents largely attributed their success to the systematic educational format, with an average score of 4.8/5.0 (SD 0.42). CONCLUSION/CONCLUSIONS:While rhinoplasty is a challenging artform to master, systematic approaches to analysis and operative planning are vital for teaching and guiding residents. Through this novel methodology, residents display significant improvement in their comfort with facial nasal analysis and overall surgical preparation.

OBJECTIVE:This systematic review aimed to describe the method followed during physical examination and the anatomical structures of the head and neck assessed in screening for oral cancer and oral potentially malignant disorders (OPMDs). STUDY DESIGN/METHODS:An extensive literature search was carried out using MEDLINE/PubMed, EMBASE, Scopus, LILACS, Web of Science, Cochrane databases, and gray literature. The risk of bias was available in all papers included. RESULTS:Of 9,688 records identified, 27 were included in this review, reporting data from 356,250 individuals screened and distributed across 11 countries. Most of these (n = 19) were based on 1 round of screening conducted by a dental professional or other health care workers. Most screening programs included visual inspection and palpation of the lips, oral cavity, and the most visible oropharyngeal sites, but the descriptions reported were imprecise. Additional inspection and palpation of the neck (submental, submandibular, cervical, and supraclavicular regions) to assess for the presence of swellings and any palpable neck nodes were also performed in 15 programs. CONCLUSION/CONCLUSIONS:In conclusion, there was considerable heterogeneity in the method of physical examination in screening programs for oral cancer and OPMDs among the included studies.