Killian Jamieson Diverticulum, the Great Mimicker: A Case Series and Contemporary Review.

OBJECTIVE: To assess barium esophagram (BAS) as a diagnostic marker for patients with Killian Jamieson diverticula (KJD). METHODS: Prospective, multicenter cohort study of individuals enrolled in the Prospective OUtcomes of Cricopharyngeus Hypertonicity (POUCH) Collaborative. Patient demographics, comorbidities, radiographic imaging reports, laryngoscopy findings, patient-reported outcome measures (PROM), and operative reporting were abstracted from a REDCap database and summarized using means, medians, percentages, frequencies. Paired t-tests and Wilcoxon Signed Rank test were used to test pre- to post-operative differences in RSI, EAT-10, and VHI-10 scores. Diagnostic test evaluation including sensitivity, specificity, positive, and negative predictive value with 95% confidence intervals were calculated comparing BAS findings to operative report. RESULTS: A total of 287 persons were enrolled; 13 (4%) patients were identified with confirmed KJD on operative reports. 100% underwent open transcervical excision. BAS has a 46.2% (95% confidence interval [CI]: 23.2, 70.9) sensitivity and 97.8% (95% CI: 95.3, 99.0) specificity in detecting a KJD and 50% (95% CI: 25.4, 74.6) positive predictive value but 97.4% (95%CI: 94.8, 98.7) negative predictive value. Preoperatively, patients reported mean (SD) RSI and EAT-10 of 19.4 (9) and 8.3 (7.5) accordingly. Postoperatively, patients reported mean (SD) RSI and EAT-10 as 5.4 (6.2) and 2.3 (3.3). Both changes in RSI and EAT-10 were statistically significant (p = 0.008, p = 0.03). CONCLUSION: KJD are rare and represent <5% of hypopharyngeal diverticula undergoing surgical intervention. Open transcervical surgery significantly improves symptoms of dysphagia. BAS has high specificity but low sensitivity in detecting KJD. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:2110-2115, 2023.

Cricopharyngeus Muscle Dysfunction and Hypopharyngeal Diverticula (e.g., Zenker): A Multicenter Study.

OBJECTIVE: To describe demographics and imaging and compare findings and symptoms at presentation in a large cohort of persons with cricopharyngeus muscle dysfunction (CPMD) with and without hypopharyngeal diverticula. METHODOLOGY: Prospective, multicenter cohort study of all individuals enrolled in the Prospective OUtcomes of Cricopharyngeal Hypertonicity (POUCH) Collaborative. Patient survey, comorbidities, radiography, laryngoscopy findings, and patient-reported outcome measures (e.g., Eating Assessment Tool [EAT-10]) data were abstracted from a REDCap database and summarized using means, medians, percentages, and frequencies. Diagnostic categories were compared using analysis of variance. RESULTS: A total of 250 persons were included. The mean age (standard deviation [SD]) of the cohort was 69.0 (11.2). Forty-two percent identified as female. Zenker diverticula (ZD) was diagnosed in 85.2%, 9.2% with CPMD without diverticula, 4.4% with a Killian Jamieson diverticula (KJD), and 1.2% traction-type diverticula. There were no differences between diagnostic categories in regard to age, gender, and duration of symptoms (p = 0.25, 0.19, 0.45). The mean (SD) EAT-10 score for each group was 17.1 (10.1) for ZD, 20.2 (9.3) for CPMD, and 10.3 (9.4) for KJD. Patients with isolated CPMD had significantly greater EAT-10 scores compared to the other diagnostic groups (p = 0.03). CONCLUSION: ZD is the most common, followed by CPMD without diverticula, KJD, and traction-type. Patients with isolated obstructing CPMD may be more symptomatic than persons with ZD or KJD. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1349-1355, 2023.

Absent Posterior Belly of Digastric Muscle.

OBJECTIVE: Report a unique case of absent posterior belly of digastric muscle, with a literature review and discussion of its clinical importance. METHODS: Present a case report and review the current literature including PUBMED search terms; "absent posterior digastric", "digastric muscle", "posterior belly". RESULTS: While there were multiple reports of accessory anterior and posterior bellies and absence of anterior belly, there is a paucity of literature on absence of posterior belly of digastric muscle. CONCLUSION: To our knowledge, this is the first report of an absent posterior belly of the digastric muscle. The posterior belly of the digastric muscle is an important landmark in neck dissection, and its absence makes knowledge of other anatomic landmarks critically important. Laryngoscope, 131:1501-1502, 2021.

Does Ibuprofen Increase Bleed Risk for Pediatric Tonsillectomy?

OBJECTIVE: To evaluate risk factors for pediatric posttonsillectomy hemorrhage (PTH) and the need for transfusion using a national database. STUDY DESIGN: Retrospective cohort study. SETTING: The study was conducted using the Pediatric Health Information System (PHIS) database. METHODS: Children ≤18 years who underwent tonsillectomy with or without adenoidectomy (T±A) between 2004 and 2015 were included. We evaluated the risk of PTH requiring cauterization according to patient demographics, comorbidities, indication for surgery, medications, year of surgery, and geographic region. RESULTS: Of the 551,137 PHIS patients who underwent T±A, 8735 patients (1.58%) experienced a PTH. The risk of PTH increased from 1.33% (95% confidence interval [CI]: 1.15%, 1.53%) in 2010 to 1.91% (95% CI: 1.64%, 2.24%) in 2015 (P < .001). Older age (≥12 vs <5 years old: adjusted odds ratio [aOR] 3.17; 95% CI: 2.86, 3.52), male sex (aOR 1.11; 95% CI: 1.05, 1.17), medical comorbidities (aOR 1.18; 95% CI: 1.08, 1.29), recurrent tonsillitis (aOR 1.15; 95% CI: 1.07, 1.24), and intensive care unit admission (aOR 1.74; 95% CI: 1.55, 1.95) were significantly associated with an increased risk of PTH. Use of ibuprofen (aOR 1.36; 95% CI: 1.22, 1.52), ketorolac (aOR 1.39; 95% CI: 1.14, 1.69), anticonvulsant (aOR 1.23; 95% CI: 1.03, 1.76), and antidepressants (aOR 1.35; 95% CI: 1.03, 1.76) were also associated with an increased risk of PTH. The need for blood transfusion was 2.1% (181/8735). CONCLUSION: The incidence of PTH increased significantly between 2011 and 2015, and ibuprofen appears to be one contributing factor. Given the benefits of ibuprofen, it is unclear whether this increased risk warrants a change in practice.

Subcutaneous emphysema with pneumomediastinum after tonsillectomy: Case report and review of the literature.

Subcutaneous emphysema is a rare complication of tonsillectomy.We report a case of post-tonsillectomy crepitus with radiographic extravasation of contrast from the tonsil fossa into the neck, subcutaneous emphysema, pneumomediastinum and small pneumothorax in a patient with Down Syndrome. Subsequent direct laryngoscopy showed no visible defect in the mucosal or muscle layers of the tonsil fossa. We explore common presenting symptoms, clinical course, and treatment of subcutaneous emphysema secondary to tonsillectomy.

Does Surgical Volume and Complexity Affect Cost and Mortality in Otolaryngology-Head and Neck Surgery?

OBJECTIVES: (1) To evaluate whether admission volume and case complexity are associated with mortality rates and (2) evaluate whether admission volume and case complexity are associated with cost per admission. STUDY DESIGN: Retrospective case series. SETTING: Tertiary academic hospital. SUBJECTS AND METHODS: The Vizient database was queried for inpatient admissions between July 2015 and March 2017 to an otolaryngology-head and neck surgery service. Data collected included admission volume, length of stay, intensive care unit (ICU) status, complication rates, case mix index (CMI), and cost data. Regression analysis was performed to evaluate the relationship between cost, CMI, admission volume, and mortality rate. RESULTS: In total, 338 hospitals provided data for analysis. Mean hospital admission volume was 182 (range, 1-1284), and mean CMI was 1.69 (range, 0.66-6.0). A 1-point increase in hospital average CMI was associated with a 40% increase in odds for high mortality. Admission volume was associated with lower mortality, with 1% lower odds for each additional case. A 1-point increase in CMI produces a $4624 higher total cost per case (95% confidence interval, $4550-$4700), and for each additional case, total cost per case increased by $6. CONCLUSION: For otolaryngology inpatient services at US academic medical centers, increasing admission volume is associated with decreased mortality rates, even after controlling for CMI and complication rates. Increasing CMI levels have an anticipated correlation with higher total costs per case, but admission volume is unexpectedly associated with a significant increase in average cost per case.

Mandibular Lytic Lesion in Familial Paraganglioma Syndrome Type I: A Clinical Conundrum.

OBJECTIVE: The entity of primary mandibular paraganglioma (PGL) is not well accepted within the head and neck. Mandibular PGLs hitherto reported in literature are malignant metastatic lesions, mostly from a pheochromocytoma. METHODS: We report a case of mandibular lytic lesion in a young female with multifocal PGLs but no family history of PGLs. We also performed a literature search to identify published cases of mandibular PGL. RESULTS: Lack of established criteria for malignancy in a PGL made diagnosis and treatment challenging. Testing was negative for a pheochromocytoma and positive for mutation of succinate dehydrogenase gene encoding subunit D (SDHD), thus rendering a diagnosis of familial PGL syndrome type I. Due to the absence of prior published reports of nonmalignant, primary mandibular PGL, patient was treated with surgery and postoperative radiotherapy. Our literature search revealed 4 published cases of mandibular PGL, all of which had an osteoblastic appearance and were malignant. CONCLUSIONS: Isolated mandibular PGL does not always indicate a malignant metastatic lesion. Genetic testing is recommended in patients with early onset of PGL and/or multifocality even without a positive family history. Surgical resection alone with surveillance can be offered for such isolated lesions in the presence of familial PGL syndrome type I.

Do post-tonsillectomy patients who report bleeding require observation if no bleeding is present on exam?

OBJECTIVE: Three to ten percent of tonsillectomy patients experience post-tonsillectomy hemorrhage. Examination of those patients who return to the Emergency Department (ED) with a history of hemorrhage may be found to have active bleeding, a coagulum within the fossa, or a normal post-operative exam. It is not known if those with a normal postoperative exam require inpatient observation. METHODS: This is a retrospective series from 1/1/2010 to 12/31/2014 at a tertiary pediatric hospital. We evaluated outcomes in patients who presented to our ED with a history of post tonsillectomy hemorrhage, but after a thorough inspection failed to demonstrate active bleeding or clot, and were thus deemed to have a normal postoperative exam. This cohort was then evaluated for subsequent active bleeding requiring cauterization. Demographics and clinical data were extracted from the medical record. RESULTS: In 337 visits with a history of bleeding, and a normal postoperative exam, 38 (11%) subsequently bled requiring cauterization. 32/38 (84%) bled within 24 h of admission to the ED. No demographic or clinical variables predicted an increased risk of bleeding during observation. CONCLUSIONS: Eleven percent of patients who presented to the ED with a history of bleeding at home but a normal postoperative exam subsequently bled and required cautery, usually within 24 h. Aside from the history of bleeding at home, we found no additional predictors of subsequent bleeding and recommend this group of patients should be considered for 24 hour in-hospital observation prior to discharge.

Characterizing mortality in pediatric tracheostomy patients.

OBJECTIVES/HYPOTHESIS: To assess the longitudinal risk of death following tracheostomy in the pediatric age group. STUDY DESIGN: Retrospective cohort study. METHODS: Hospital records of 513 children (≤18 years) at a tertiary care children's hospital who underwent tracheostomy between 1984 and 2015 were reviewed. The primary outcome measure was time from tracheostomy to death. Secondary patient demographic and clinical characteristics were assessed, with likelihood of death using χ2 tests and the Cox proportional hazards model. RESULTS: Median age at time of tracheostomy was 0.8 years (interquartile range, 0.3-5.2 years).The highest mortality rate (27.8%) was observed in patients in the 13- to 18-year-old age category; their mortality rate was significantly higher when compared to the lowest mortality risk group patients (age 1-4 years, P = .031). Timing of death was evenly distributed: <90 days (37.6%), 90 days to 1 year (27.1%), and >1 year after tracheostomy (35.3%). Patients who underwent tracheostomy for cardiopulmonary disease had an increased risk of mortality compared with airway obstruction (adjusted hazard ratio: 3.53, 95% confidence interval: 1.72-7.24, P < .001) and other indications. Adjusted hazard ratios for bronchopulmonary dysplasia (BPD) and congenital heart disease (CHD) were 2.63 and a 2.61, respectively (P < .001). CONCLUSIONS: Pediatric patients with tracheostomy have a high mortality rate, with an increased risk of death associated with a cardiopulmonary indication for undergoing tracheostomy. The majority of deaths occur after the index hospitalization during which the tracheostomy was performed. BPD and CHD are independent predictors of mortality in pediatric tracheostomy patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:1701-1706, 2017.

Complications of turbinate reduction surgery in combination with tonsillectomy in pediatric patients.

OBJECTIVES/HYPOTHESIS: To examine whether the addition of turbinoplasty to tonsillectomy and adenoidectomy (T&A) increases the risk of postoperative complications. STUDY DESIGN: Retrospective cohort study of children (18 years old and younger) who underwent tonsillectomy and/or turbinoplasty between July 1, 2013 and June 30, 2015 using the 2016 Pediatric Health Information System administrative database METHODS: Patients were divided into three groups: 1) T&A and turbinoplasty, 2) T&A alone, and 3) turbinoplasty alone. Postoperative revisit, hemorrhage requiring cautery, and blood transfusion rates were compared between groups. RESULTS: A total of 75,761 patients met inclusion criteria: 3,079 underwent both T&A and turbinoplasty, 72,043 underwent T&A alone, and 639 underwent turbinoplasty alone. The rate of 14-day relevant revisits after T&A in combination with turbinate reduction surgery was not significantly higher than that of T&A alone (9.4% vs. 8.6%; P = .11). The revisit rate after turbinoplasty alone was 1.4%. Indications for revisits did not differ between the T&A and turbinoplasty group versus T&A alone group (P = .23). Furthermore, the rates of hemorrhage requiring cauterization was similar between the two groups (1.4% vs. 1.5%; P = .64). Twenty-one patients who underwent T&A alone required blood transfusion after they were readmitted; no cases in the other two groups required blood transfusion. CONCLUSIONS: Turbinoplasty and T&A performed together do not increase the risk of postoperative revisit or hemorrhage requiring cauterization, and can therefore be considered as a combined procedure. Pediatric patients will benefit from avoiding the additional risk of multiple anesthetics and repeated intubation. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1920-1923, 2017.

Novel Alleles of gon-2, a C. elegans Ortholog of Mammalian TRPM6 and TRPM7, Obtained by Genetic Reversion Screens.

TRP (Transient Receptor Potential) cation channels of the TRPM subfamily have been found to be critically important for the regulation of Mg2+ homeostasis in both protostomes (e.g., the nematode, C. elegans, and the insect, D. melanogaster) and deuterostomes (e.g., humans). Although significant progress has been made toward understanding how the activities of these channels are regulated, there are still major gaps in our understanding of the potential regulatory roles of extensive, evolutionarily conserved, regions of these proteins. The C. elegans genes, gon-2, gtl-1 and gtl-2, encode paralogous TRP cation channel proteins that are similar in sequence and function to human TRPM6 and TRPM7. We isolated fourteen revertants of the missense mutant, gon-2(q338), and these mutations affect nine different residues within GON-2. Since eight of the nine affected residues are situated within regions that have high similarity to human TRPM1,3,6 and 7, these mutations identify sections of these channels that are potentially critical for channel regulation. We also isolated a single mutant allele of gon-2 during a screen for revertants of the Mg2+-hypersensitive phenotype of gtl-2(-) mutants. This allele of gon-2 converts a serine to phenylalanine within the highly conserved TRP domain, and is antimorphic against both gon-2(+) and gtl-1(+). Interestingly, others have reported that mutation of the corresponding residue in TRPM7 to glutamate results in deregulated channel activity.

Treatment of retinopathy of prematurity in extremely premature infants over an 18-year period.

BACKGROUND: To report the efficacy of laser diode therapy in the treatment of retinopathy of prematurity (ROP) in extremely premature (EP) infants over an 18-year period. DESIGN: Retrospective study. PARTICIPANTS: One hundred twenty-eight eyes in 66 infants treated between 23 and 25.6 weeks. METHODS: Five hundred fifty-four infants between 23 and 25.6 weeks gestational age (GA) were admitted to The Royal Brisbane and Women's Hospital Neonatal Intensive Care Unit (NICU) between 1992 and 2009. Three hundred seventy-three patients survived to undergo screening, 304 were diagnosed with ROP, and 66 infants required diode laser therapy. MAIN OUTCOME MEASURES: Success of treatment, visual and refractive outcomes. RESULTS: One hundred twenty-eight eyes from 66 infants (18.8% of those screened) underwent laser treatment with a mean GA of 24.3 weeks and mean birth weight of 711.4g. Fifty-six eyes were treated at pre-threshold disease, and 72 eyes at threshold disease. Over the study period, the number of laser spots and regression rate of ROP increased, while the frequency of re-treatment decreased. At 40 weeks, 119 eyes had regressed ROP (93%), two advanced to stage 4a, three to stage 4b and four to stage 5. Aggressive posterior ROP (AP-ROP) occurred in 15 eyes (11.7% of those treated). Forty-three patients (65%) were followed up for a mean of 56.5 months. The number of laser spots correlated well with subsequent refractive error but poorly with corrected visual acuity. CONCLUSIONS: In EP infants, laser diode therapy is an effective technique to halt the progression of ROP in most cases. AP-ROP is uncommon, even in this subgroup of extremely premature infants.

Lacrimal outflow dysgenesis.

PURPOSE: To evaluate patients with lacrimal dysgenesis. DESIGN: Retrospective, nonrandomized, comparative case series. PARTICIPANTS: Fifty patients with lacrimal dysgenesis managed between 1992 and 2003. TESTING/INTERVENTION: The diagnosis of lacrimal outflow dysgenesis was made based on the following criteria: absent or hypoplastic punctum, canaliculus, lacrimal sac, and nasolacrimal duct, appearing in isolation or combination. Our management algorithm included observation, punctoplasty with intubation, or open lacrimal surgery, with or without intubation. MAIN OUTCOME MEASURE: Success, partial success, or failure of treatment. RESULTS: Lacrimal dysgenesis in our 50 patients (23 male and 27 female) involved 83 eyes, distributed as follows: proximal in 74 eyes (89%), distal in 27 (33%), and both in 18 (22%). Thirty (60%) patients presented with isolated lacrimal dysgenesis, and 20 (40%) presented with a systemic syndrome or dysmorphism. Thirty-three (66%) patients had bilateral involvement. Epiphora was the most common presenting symptom. Eighteen (36%) patients had a positive family history. Open lacrimal surgery was performed in a total of 29 (35%) of the 83 eyes: 25 had success, 3 had partial success, and 1 had failure. CONCLUSION: Both sporadic and hereditary forms of lacrimal outflow dysgenesis may present as an isolated finding or a part of a systemic syndrome or dysmorphism, occurring usually with bilateral involvement and presenting at a younger age in the setting of systemic anomalies. Proximal and distal lacrimal outflow systems may be involved with epiphora as the most common presenting symptom. The algorithm we have presented provides a systematic approach to the management of lacrimal outflow dysgenesis.

Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome.

OBJECTIVE: To evaluate the distribution and clinical as well as treatment outcome characteristics of idiopathic orbital inflammation with the aim of delineating a more systematic approach to diagnosis and treatment. METHODS: A 10-year retrospective review of patients with idiopathic orbital inflammation treated at one institution. RESULTS: Ninety eyes in 65 patients (22 men and 43 women) were studied. Diagnoses were isolated dacryoadenitis (n = 21), isolated myositis (n = 19), concurrent dacryoadenitis and myositis (n = 5), orbital apex syndrome (n = 6), and idiopathic inflammation involving the preseptal region, supraorbital region, sclera, Tenon capsule, orbital fat, or optic nerve (n = 14). The mean age at presentation was 45 years. Pain and periorbital swelling were the most common clinical features and were observed in 45 (69%) and 49 (75%) patients, respectively. Seventeen patients (26%) had bilateral involvement. Biopsy was performed in 19 patients (29%) with atypical presentations or who failed to respond to the initial therapy. Patients were treated with steroids alone (n = 45), steroids and subsequent radiation therapy (n = 8), steroids and nonsteroidal anti-inflammatory agents (n = 6), nonsteroidal anti-inflammatory agents alone in mild cases (n = 2), and, rarely, radiation therapy without steroids (n = 1) or surgical debulking alone (n = 1). Of 65 patients, 41 (63%) represented treatment successes, with complete symptom relief at the time of the last follow-up, and 24 (37%) represented treatment failures, with partial or no relief of symptoms. Treatment failures were often characterized by recurrence of inflammation after a period of quiescence (58%) and unremitting, recalcitrant inflammation (38%); 1 patient ultimately required an exenteration. CONCLUSION: Systemic steroid with a slow taper has been the established first-line treatment for idiopathic orbital inflammation, but refractory cases accounted for a significant portion of treatment failures in our study, reflecting the need for a more systematic approach to the study of this multifaceted disease and for therapeutic alternatives to systemic steroids.

Retrobulbar chlorpromazine injections for the management of blind and seeing painful eyes.

PURPOSE: To report the efficacy of primary retrobulbar chlorpromazine injections for the management of blind and seeing painful eyes. PATIENTS AND METHODS: Retrospective case series from three university hospitals was done. Twenty eyes of 20 patients with at least 3 months of follow-up are reported. Ten of these 20 eyes did not receive any other ophthalmic medications either 2 weeks before chlorpromazine injection or at any time point after injection. Treatment was considered successful if pain control lasted for at least 3 months, without the need for reinjection or enucleation. RESULTS: Sixteen of 20 (80.0%) eyes achieved successful pain control. There were no permanent complications in any patient. CONCLUSIONS: Retrobulbar chlorpromazine appears to be safe and effective for the management of pain in blind eyes.

Idiopathic orbital inflammation: ocular mechanisms and clinicopathology.

Pathogenesis of idiopathic orbital inflammation remains elusive but several lines of evidence point to immune-mediated processes as the likely underlying ocular mechanism. The exact nature of the immunological process and possible role of infection need to be elucidated in greater depth. There is an obvious need for a more satisfactory animal model for orbital pseudotumor. Given the prevalence of recurrent and persistent orbital inflammation, therapeutic alternatives to corticosteroids need to be further explored and systematically investigated.