Annals of Otology,Rhinology & Laryngology

To discuss the presentation and management of infants with arhinia or congenital absence of the nose.

This case report describes an infant with arhinia that was diagnosed prenatally. In addition to a discussion of the case, a review of the literature was completed to define appropriate postnatal work-up and management.

The patient is a term male infant, diagnosed with arhinia on ultrasound and magnetic resonance imaging (MRI) performed at 21-weeks gestational age. Upon birth, the patient was subsequently intubated, followed by tracheostomy due to complete nasal obstruction. Through a genetics evaluation, the patient was found to be heterozygous for the SMCHD1 gene, with hypomethylation at the D4Z4 locus. Plans for reconstruction will be based on future imaging and the development of any nasal patency, however, the patient’s family plans to utilize a prosthetic nose until the patient is older.

Arhinia is a rare condition causing respiratory distress in the neonatal period. While stabilization of the airway is the first priority, further management is not clearly defined given the rarity of the malformation. This case discusses stabilization of the airway with a review of treatment and reconstructive options.

Double aortic arch is a rare congenital malformation of the aortic arch that most frequently presents in childhood. Early surgical intervention typically yields excellent outcomes.

To describe aortotracheal fistula as a rare, yet serious complication of vascular ring and subsequent aortic aneurysm in an adult patient.

Clinical history, as well as radiographic and endoscopic imaging were obtained to describe the development, diagnosis, and clinical course of this patient’s aortotracheal fistula. Additionally, follow up data was obtained to document the healing of this fistula after surgical repair.

We describe a case of a 46-year-old male with DiGeorge Syndrome and a double aortic arch, repaired in childhood, which developed into an aortotracheal fistula after tracheostomy placement as an adult.

This case demonstrates that dangerous complications of a double aortic arch can persist into adulthood, even after surgical repair in infancy. Each patient’s unique anatomy must be considered when thinking about airway management and prevention of complications of this rare congenital anomaly.

Congenital midline cervical cleft (CMCC) is a rare congenital anterior neck anatomical anomaly. We present the case of two related patients (grandchild and maternal grandmother) who were both born with a congenital midline cervical cleft along with genetic analysis.

Clinical examination of both patients and surgical excision of the grandchild was performed. Genetic analysis with exome sequencing (ES) was conducted for both patients.

Genetic analysis with exome sequencing (ES) revealed apparently novel single nucleotide variants in 66 genes present in both proband and grandmother. Five of these variants are predicted to cause frameshifting in the coding region of the respective genes and truncated proteins (OVGP1, TYW1B, ZAN, SSPO, FOLR3). Two of these genes (TYW1B and SSPO) have homozygous indel mutations in both patients.

To our knowledge, this is the first case of two related patients with a congenital midline cervical cleft. The results of our genetic analysis reveal potential relevance to CMCC development.

Medullary thyroid carcinoma (MTC) accounts for 1% to 2% of thyroid cancers in the United States. When identified early, total thyroidectomy is most often curative. However, in advanced disease, more aggressive treatment such as laryngectomy and esophagectomy may be indicated. Postsurgical fistula formation and leak is a potential complication in such cases. These fistulas are most likely to occur at the anastomotic site in cases of laryngectomy or esophagectomy. Concomitant chemotherapy and radiation increase this risk. Tyrosine kinase inhibitors (TKI) such as Cabozantinib are used as therapy for metastatic MTC. These drugs have previously been associated with dehiscence of anastomotic sites in the gastrointestinal tract. While previously identified in the bowel, this report represents the first documented case of gastropharyngeal anastomosis leak described in the context of TKI use for head and neck cancer.

We present the case of a 72-year-old male previously diagnosed with MTC. His gastropharyngeal anastomosis status-post laryngopharyngectomy and gastric pull up had been stable for 23 years. Over the past year, he developed back pain and was found to have spinal metastases of MTC. He was subsequently started on Cabozantinib to slow the progression of the disease. Within months of starting this TKI, a bleeding pharyngocutaneous fistula developed at the anastomosis site of the gastric pull up and pharynx. Upon discontinuation of Cabozantinib, the fistula healed with no further complications.

To our knowledge, this is the first documented case of gastropharyngeal anastomotic leak related to TKI use. A causal relationship is highly plausible given the previously stable anastomosis and the suspicious advent of complications within months of initiation of this new drug. While previously limited to cases of intraabdominal bowel dehiscence, this report now suggests that wound dehiscence must be considered a known side effect of TKIs throughout the gastrointestinal tract, including the gastropharynx. As such, the risk of anastomotic dehiscence should be discussed with the patient prior to starting a TKI.

Cricoid chondronecrosis is a rare entity that has significant consequences for patients. Reports of its occurrence are scattered in the literature and currently there is no comprehensive review to help guide providers.

A case report from our institution is presented. A review of available literature is then provided with assessment of risk factors, signs and symptoms at presentation, laryngeal exam findings, radiologic findings, and surgical techniques.

Twenty-four cases of cricoid chondronecrosis were reviewed from the literature. Patient age ranged from 8 months to 76 years. A history of endotracheal intubation for various reasons was present in all but two cases, and duration of intubation ranged from 6 hours to 28 days. Patients presented with airway compromise in all but two cases—one asymptomatic patient with prior tracheostomy and another with dysphagia after radiation. Dysphonia (n = 6) and dysphagia (n = 3) were less commonly present. Subglottic stenosis (n = 19) was the most common exam finding followed by vocal fold impairment (n = 11). When CT scan findings were reported, fragmentation of the cartilage and/or hypodensity of the central lamina were described in all but one case. Interventions ranged from observation on antibiotics and steroids to surgical therapies including tracheostomy, dilation, and posterior cricoid split, with or without stent placement.

Cricoid chondronecrosis is a serious, rare entity that can occur even after a short period of endotracheal intubation. Providers must have a high level of suspicion in patients that present with upper airway dyspnea with a history of prior intubation.

Level 4

Open partial laryngeal surgery (OPLS) represents a wide array of procedures that can be fitted to treat different types of laryngeal cancer (LC). We would like to present our 30-years’ institutional experience, to analyze survival outcomes and to critically discuss prognostic factors.

We reviewed all cases of OPLS performed at our Institution from 1982 to 2016 for LC. Survival analysis by Kaplan–Meier estimate was performed and prognostic variables by multivariate analysis were identified.

Mean follow-up time was 68.3 months, 30-day mortality 0.2%, subsequent functional total laryngectomy (TL) was 1.01%. Over 80% of cases were stage I to II. We had 25 local, 62 regional and eight distant recurrences. Local control was 94.9%, overall survival (OS) was 83.4% and disease-specific survival (DSS) was 87.7%. The two major risk factors significantly associated with the risk of death were cT and cN stage. CONCLUSIONS: We have confirmed that OPLS represents an oncologically sound option in the treatment of LC despite the emergence of non-surgical strategies and new transoral mininvasive techniques. Our results highlight that accurate staging, correct selection of the patient and a strong surgical expertise are of paramount importance in this type of surgery.

Management of pain following endoscopic sinus surgery (ESS) often involves intermittent use of opioid medications. Given the current opioid crisis, many surgeons aim to minimize opioid prescriptions. However, surgeons often avoid the use of NSAIDs following ESS out of concern for increased bleeding risk. We sought to evaluate the effectiveness and safety of over the counter dosing of ibuprofen on pain and bleeding rates following ESS.

Prospective cohort pilot study of patients undergoing ESS. Patients self-selected either control arm (acetaminophen and PRN oxycodone) or intervention arm (alternating ibuprofen with acetaminophen, and PRN oxycodone). Outcome measures included pain (10-point visual analog scale [VAS]), bleeding rate (10-point VAS), and number of opiate pills consumed.

Thirty-nine patients completed the study (15 control and 24 intervention). Overall, patients in the intervention arm reported decreased pain levels at days 1 (−0.9 [95%CI: −2.2, 0.5], 3 (−0.9 [95%CI: −2.3, 0.5]), and 7 (−0.6 [95%CI: −1.8, 0.6]), as well as decreased postoperative bleeding at those same days −0.9 [95%CI: −2.1, 0.4], −0.9 [95%CI: −2.1, 0.4], and −0.7 [95% CI: −1.2, −0.7], compared to controls. Opiate consumption was similar between groups with patients consuming on average four opiate pills (oxycodone 5 mg).

Over the counter dosing of ibuprofen along with acetaminophen may yield better pain control after sinus surgery compared to acetaminophen alone. Additionally, there was no significant difference in epistaxis in the ibuprofen cohort compared to the cohort who did not take ibuprofen. Furthermore, this study showed very few opioid pills were consumed after routine ESS regardless of pain regimen prescribed. A larger trial is needed to make definitive statements on safety and efficacy.

2b

The purpose of this study was to evaluate the effect of tanning bed use on the behavior of subsequent melanomas.

Cases of invasive cutaneous melanoma who completed a baseline questionnaire within 1 year of biopsy were ascertained using an institutional registry. Patients were categorized into one of two groups: (1) no history of UV tanning bed usage or (2) any history of tanning bed usage. Data analysis looked for group differences on the following variables: TNM staging, mitotic rate, family history of melanoma, and basic demographic variables.

Among 141 cases, a higher percentage of women (48.28%) reported tanning bed usage compared to men (26.51%, P < .01). Additionally, the average age at biopsy for people who reported tanning bed usage was significantly lower compared to those who had not reported using a tanning bed. There was no significant difference in stage at presentation (P = .56). Those with tanning bed usage presented significantly less often with melanoma of unknown primary (MUP). After controlling for the effects of gender and nodal status, tanning bed usage was not a significant independent predictor of 5 year overall survival.

If we remove the subject with MUP on the basis that more of these are mucosal in origin, lower TNM stage at presentation are found in patients with no tanning bed melanoma compared to those with history of tanning bed usage. In addition, tanning bed patients are younger and more likely female but with an equivalent mortality rate.

Case Series

Surgical procedure is considered in patients with severe dysphagia when conservative treatment fails. This study aimed to evaluate laryngeal suspension (LS) and upper esophageal sphincter (UES) myotomy for treating severe dysphagia due to brain disease.

Fourteen patients underwent LS and UES myotomy, with a median follow-up of 5 years and 6 months when conservative treatment failed. The penetration-aspiration scale (PAS), the Dysphagia Severity Scale (DSS), the Eating Status Scale (ESS), and diet contents were evaluated just before surgery, at discharge, and at the last follow-up.

Preoperative intake was tube feeding in all patients. The patients learned the extended head and flexed neck posture to open the esophageal inlet. PAS, DSS and ESS scores, and diet contents were significantly improved at discharge compared with before surgery, and were maintained until the last follow-up. Eight patients had pneumonia during their hospital stay, and five had pneumonia between discharge and at the last follow-up. Age was significantly, negatively correlated with DSS and ESS at the last follow-up.

Although LS and UES myotomy require a long inpatient rehabilitation and the risk of pneumonia after surgery is high, the outcome is favorable and the effects last for a long time.

Demonstrate the potential of the 4K-3D exoscope during upper airway stimulation surgery (UAS).

A proof-of-concept study was performed to evaluate the feasibility of the exoscope during three consecutive UAS.

The exoscope was employed during UAS including cuff electrode and sensing lead placement. Three cases were successfully completed without adverse perioperative event; mean operative time was 200 minutes (range 188-218 minutes) with a successive reduction in operative time.

This experience demonstrates the potential viability of the exoscope for UAS; it is a safe, innovative, and effective alternative or adjunct to existing visualization modalities. Notable advantages include improved ergonomics, unobstructed surgical field access, wide depth of field visualization, and short learning curve. Future technological enhancements could allow the exoscope to become a promising next-generation tool in the armamentarium of the contemporary sleep surgeon.

Chronic rhinosinusitis (CRS) is a chronic inflammatory disease of the sinonasal mucosa and with strong associations to other immune-mediated comorbidities. Patients often require referral to both an otolaryngologist and an allergist/immunologist. This study is the first in the literature to describe a multidisciplinary clinic that offers patient care by subspecialists in rhinology and in allergy/immunology.

One hundred twenty-nine patients were seen in the Comprehensive Sinus and Allergy Clinic (CSAC) between January 2016 and June 2017 and 43 selected patients were seen in both the standalone allergy and rhinology clinics over the same time period. Patient satisfaction was retrospectively assessed using a modified Press-Ganey satisfaction survey. Time to evaluation and time to follow up appointment were compared between the CSAC and both the standalone rhinology and allergy/immunology clinics.

Patients seen in the CSAC reported high satisfaction with the amount of time spent with the physicians (98.3%), quality of medical care (9.3 ± 1.0), and most importantly, the convenience of seeing two physicians in one day (9.5 ± 1.2). Time from referral placement to clinic evaluation (P ≤ .02) and time to follow up appointment (P ≤ .002) was significantly shorter for the CSAC than for the standalone Rhinology or Allergy clinics.

Patients reported high satisfaction with the medical care provided and were also seen much faster in our multidisciplinary clinic as compared to standalone rhinology or allergy/immunology clinics. Overall, a multidisciplinary approach may be beneficial to patients presenting to tertiary referral centers with CRS and atopic conditions.

Clinical follow-up after endoscopic sinus surgery (ESS) for chronic rhinosinusitis (CRS) allows for assessment of the sinonasal cavity, debridement, and tailoring of medical therapies. Frequency and timing of postoperative clinical visits is debated, but the impact of adherence on disease-specific outcomes is not well understood. In this longitudinal study, we assessed the association between follow-up adherence and quality of life (QOL) outcomes in the 12 months after ESS.

A retrospective review of patients undergoing ambulatory ESS for CRS between 11/2016 and 1/2018 was performed. We assessed sociodemographic characteristics, radiographic severity, and QOL utilizing the 22-item sinonasal outcome test (SNOT-22). Patients were categorized as “non-adherent,” “moderately-adherent,” and “fully-adherent” to a 1-, 3- and 5-week postoperative visit schedule.

A total of 166 patients met the inclusion criteria. Of these, 55 (33.1%) were fully-adherent, 105 (63.2%) were moderately-adherent, and 6 (3.6%) were non-adherent within the 6 weeks following ESS. In the immediate postoperative period, fully-adherent patients demonstrated worse QOL (SNOT-22: 31.2 ± 23.1 vs 27.5 ± 17.6, P = .047). This cohort also had greater psychological dysfunction at baseline and 12-months (P < .05) after ESS. Extra-nasal symptom scores increased at a lower rate in the fully-adherent cohort (0.12 vs 1.29 points per 6 months, P = .038), as did ear/facial symptoms (1.17 vs 3.05 points per 6 months, P = .044).

Despite worse symptom severity in the immediate postoperative period, patients who are more adherent to the follow-up schedule demonstrated slower return of symptoms in the extra-rhinological and ear-facial domains. These findings suggest that clinical adherence and management may impact the long-term evolution of ESS outcomes.

Handheld otoscopy requires both technical and diagnostic skills, and is often reported to be insufficient after medical training. We aimed to develop and gather validity evidence for an assessment tool for handheld otoscopy using contemporary medical educational standards.

Educational study.

University/teaching hospital.

A structured Delphi methodology was used to develop the assessment tool: nine key opinion leaders (otologists) in undergraduate training of otoscopy iteratively achieved consensus on the content. Next, validity evidence was gathered by the videotaped assessment of two handheld otoscopy performances of 15 medical students (novices) and 11 specialists in otorhinolaryngology using two raters. Standard setting (pass/fail criteria) was explored using the contrasting groups and Angoff methods.

The developed Copenhagen Assessment Tool of Handheld Otoscopy Skills (CATHOS) consists 10 items rated using a 5-point Likert scale with descriptive anchors. Validity evidence was collected and structured according to Messick’s framework: for example the CATHOS had excellent discriminative validity (mean difference in performance between novices and experts 20.4 out of 50 points, P < .001); and high internal consistency (Cronbach’s alpha = 0.94). Finally, a pass/fail score was established at 30 points for medical students and 42 points for specialists in ORL.

We have developed and gathered validity evidence for an assessment tool of technical skills of handheld otoscopy and set standards of performance. Standardized assessment allows for individualized learning to the level of proficiency and could be implemented in under- and postgraduate handheld otoscopy training curricula, and is also useful in evaluating training interventions.

NA

This study aimed to evaluate the effect of preoperative sphenopalatine ganglion block (SPBG) on the postoperative pain (POP) in patients undergoing septorhinoplasty (SRP).

A retrospective cohort study was performed. A total of 42 patients that had received septorhinoplasty included in the study. The patients that had received SPBG before the surgery included in the Block group (n:20) and the patients that had not received SPBG before the surgery included in the Control group (n:22). POP was questioned with a numeric rating scale (NRS) at the 30th minute (t1), 1st hour (t2), 4th hour (t3), 12th hour (t4), and 24th hour (t5) and noted. The intraoperative details and the dose of the postoperative rescue analgesics were also noted.

The average dose of Paracetamol that was used in the postoperative first 24 hours was 500 mg in the Block group and 1363 mg in the Control group, and the difference was statistically significant (P = .001). The average dose of Tramadol was 0 mg in the Block group and 45 mg in the Control group, and the difference was statistically significant (P = .001). There was a statistically significant difference among the groups with respect to NRS in the first 24 hours postoperatively (P < .05). The number of the patients requiring rescue analgesics was lower in the Block group than the Control group. The difference was statistically significant at the t1, t2, and t5 time intervals (P > .05).

Preoperative SPGB is an effective option to reduce POP and the need for rescue analgesics for patients undergoing SRP.

NCT04020393

Acupuncture is a form of Traditional Chinese Medicine that has minimal side effects, is cost-effective, can be easily administered, and may serve as a useful non-pharmaceutical therapy for certain conditions. However, studies and clinical practice guidelines are inconsistent in conclusions and recommendations about acupuncture. This qualitative review addresses the evidence available for acupuncture use in otolaryngology.

A literature review was completed using EMBASE, PubMed, and The Cochrane Collection for conditions within otolaryngology that have used and studied the comparative effects of acupuncture. Thirty studies ultimately fit the inclusion criteria and were used for this qualitative review.

Studies included in this review contained a variety of conditions within otolaryngology, including allergic rhinitis, chronic rhinitis, tinnitus, sudden sensorineural hearing loss, post-viral olfactory dysfunction, dysphonia, and tonsillectomies.

Given its safety profile, cost, and perceived benefit, recommendations and guidelines supporting acupuncture as an alternative or adjunctive therapy are surfacing for certain conditions such as allergic rhinitis. However, stronger conclusions with specific recommendations are limited by varied methodology between majority of the studies. Additional high-quality randomized control studies with low risk of bias are required to continue to assess the effects of acupuncture in the field of otolaryngology before stronger recommendations can be made on other conditions.