A challenging diagnosis of narcolepsy type 2 in a patient with Duane syndrome.

Duane syndrome is a form of congenital strabismus with horizontal eye movement limitation. This may present a diagnostic challenge when assessing for rapid eye movements during stage REM utilizing PSG or MSLT. We present a case of a child with Duane syndrome who presented with excessive daytime sleepiness and underwent evaluation for hypersomnia. His eye movement limitation provided a challenge in identifying stage REM. In patients with eye movement limitations or prosthetic eyes, it is necessary to look for other features of stage REM such as low chin EMG tone and EEG pattern.

Delayed onset sleep-related stridor due to radiation for thyroid cancer.

Radiation therapy is the mainstay of treatment for head and neck cancers with both acute and delayed complications. While obstructive sleep apnea is common in the few series of patients undergoing radiation therapy to the neck, the development of sleep-related stridor is exceedingly rare and has typically been reported in the acute treatment setting. We describe a 65-year-old female with 1 year of nocturnal groaning beginning 2 years after radiation therapy for thyroid carcinoma. Polysomnography revealed mild obstructive sleep apnea and sleep-related stridor responsive to nasal continuous positive airway pressure. Our case highlights the importance of screening patients with a history of head and neck radiation for sleep-related breathing complaints at each follow-up visit and consideration of both obstructive sleep apnea and stridor in these patients. Identification of sleep-disordered breathing in these patients may lead to timely treatment and improvement in quality of life. CITATION: McCarter SJ, Mansukhani MP, Herold DL, Kolla BP. Delayed onset sleep-related stridor due to radiation for thyroid cancer. J Clin Sleep Med. 2022;18(9):2327-2329.

A certified child life specialist influences the emotional response during polysomnography setup.

OBJECTIVES: To examine if the presence of a certified child life specialist (CCLS) had a positive impact on patient emotion at the time of polysomnography setup and to determine the optimal timing of CCLS intervention prior to polysomnography (PSG) in our sleep center. METHODS: We implemented a study which measured the impact of a CCLS on the emotional manifestation score (EMS) of pediatric patients (4 months-17 years, median 7 years) during PSG setup. CCLS intervention was either at the time of sleep medicine consultation (daytime) or during PSG setup (evening). We used Emotional Manifestations Scores (EMS) as well as patient/caregiver satisfaction data to measure the impact of a CCLS and inform decision-making regarding ongoing employment of a CCLS in our sleep lab. RESULTS: High EMS scores were noted during PSG setup in all groups indicating the emotional distress of children undergoing PSG. The EMS improved more when CCLS was present at the time of PSG setup. Statistically significant improvements occurred in level of cooperation, pain/discomfort, and child coping. Based on the results of the study, we hired a CCLS to work in our sleep center at the time of PSG setup 2 evenings per week. CONCLUSIONS: PSG is emotionally stressful for pediatric patients as seen on EMS. A CCLS present in the evening at our sleep lab led to an improvement in EMS in children being prepared for PSG set-up. After implementation of a CCLS two nights per week in our sleep lab, parents/caregiver satisfaction scores were higher on nights a CCLS was present at setup.

Rapid response to medical device recalls: an organized patient-centered team effort.

As more patients depend upon mechanical or electronic technologies for treatment, medical device recalls-like the recent recall of common positive airway pressure treatment devices-impact millions of patients, often causing significant anxiety, extra costs, and interruption of care for patients. When recalls require health care and/durable medical equipment providers to be part of the solution, the burden on practices and businesses can be significant, creating strains on access for new patients and on limited medical supplies. We have observed that having an established and well-organized medical device recall plan in place allows for a rapid response, decreased practice burden, and reduced provider stress. Coupling the organized response with proactive, empathic, and clear communication with patients reduces their anxiety, provides clear directions for how to address the issue constructively, and reduces reactive communications. We share what we believe are key components of a medical device and produce recall procedure as we describe our institutions response in hopes that others can build on these basics as they design their own response plans. CITATION: Morgenthaler TI, Linginfelter EA, Gay PC, et al. Rapid response to medical device recalls: an organized patient-centered team effort. J Clin Sleep Med. 2022;18(2):663-667.

Efficiency in COVID-19 Vaccination Campaigns-A Comparison across Germany's Federal States.

Vaccination programs are considered a central pillar of the efforts to stop COVID-19. However, vaccine doses are scarce and several organizational and logistical obstacles, such as the timing of and reserves for second shots and delivery failures, apparently slow down vaccination roll-outs in several countries. Moreover, it is an open question as to where vaccines are administered as efficiently as possible (vaccination centers, hospitals, doctor's offices, pharmacists, etc.). The first aim of our study was to systematically evaluate the efficiency of a country's vaccination campaign. The second aim was to analyze how the integration of doctors' offices into a campaign that formerly relied only on vaccination centers affected the speed of that campaign. Using data on vaccine deliveries and vaccinations given in Germany, we find considerable differences across federal states in terms of efficiency, defined as the ability to administer the most vaccinations out of a given number of available doses. Back-of-the-envelope calculations for January to May 2021 show that vaccinations would have been 3.4-6.9% higher if all federal states had adopted a similar ratio between vaccinations given and vaccines stored, as the most efficient states did. This corresponds to 1.7-3.3% of Germany's total population. In terms of our second research goal, we find evidence that the integration of doctors' offices into the vaccination campaign significantly increased the ratio of vaccinations administered out of a given stock of vaccine doses. On average, there appears to be a structural break in this ratio after doctors' offices were integrated into the vaccination campaign on 5 April 2021. On average, an additional 11.6 out of 100 available doses were administered each week compared to the period prior to that date. We conclude that there are considerable regional differences in the efficiency of the vaccination roll-out. Systematic efficiency analyses are one step to detecting inefficiencies and to identify best practices that can be adopted to eventually speed up the vaccination roll-out in a country.

A 64-Year-Old Man With Germline BRCA2-Mutated Breast Cancer: Known and Unknown Aspects of Male Breast Cancer.

Male breast cancer is a rather uncommon and understudied disease. It accounts for less than 1% of all breast cancers, but in recent decades its frequency has been on the rise. Clinical trials of breast cancer have traditionally excluded men. Due to the lack of large-scale prospective studies, most published data come from single-institution, small-cohort studies, and treatment recommendations are based on the extrapolation of data from clinical trials enrolling only women. Although to some extent etiology, diagnosis, and treatment characteristics can be similar, male breast cancer exhibits some distinct features. Men tend to be diagnosed with breast cancer at an older age and at a more advanced stage. A better understanding of the biologic features, clinically relevant differences, effective treatments, and outcomes of male breast cancer is crucial to appropriately manage these patients. We present a male breast cancer case with a germline BRCA2 mutation and discuss the epidemiologic, pathologic, and clinical characteristics along with treatment and follow-up recommendations in view of our recent understanding of this disease.

Mucinous Adenocarcinoma of the Appendix With Histologic Response to Neoadjuvant Chemotherapy: Review of Histologic and Clinical Spectrum of Epithelial Neoplastic Mucinous Lesions of the Appendix.

Appendiceal mucinous neoplasms are a rare and heterogeneous group of diseases with challenging clinical management decisions. They account for less than 1% of all cancers but their incidence is on the rise. Treatment is based on their stage and histology. Appendiceal neoplasms frequently metastasize inside the abdomen; this leads to tumor cell growth in the abdominal cavity, known as peritoneal carcinomatosis, and buildup of mucinous material, known as pseudomyxoma peritonei. While low-grade, early-stage tumors can be effectively treated with limited surgical resection, patients with low-grade, advanced-stage disease require peritoneal debulking and hyperthermic intraperitoneal chemotherapy. Therapeutic options for high-grade, advanced-stage mucinous tumors of the appendix have not been well established. Debulking surgery with hyperthermic intraperitoneal chemotherapy preceded and/or followed by systemic chemotherapy has been utilized based on some prospective but not randomized data. We present a case of mucinous adenocarcinoma of the appendix treated with neoadjuvant chemotherapy followed by cytoreductive surgery/hyperthermic intraperitoneal chemotherapy and adjuvant chemotherapy. Preoperative chemotherapy provided a favorable histologic response by converting initial mucinous appendiceal adenocarcinoma histology to a high-grade mucinous appendiceal neoplasm.

Enhancing the patient and family experience during pediatric sleep studies.

STUDY OBJECTIVES: Pediatric polysomnography can result in suboptimal patient and provider (physician and advanced practice provider) experiences. We embarked on a project aimed at increasing the proportion of maximal satisfaction survey scores by a minimum of 10% in 1 year without adding personnel or major expenses. METHODS: We used a Six Sigma framework, define, measure, analyze, improve, and control (DMAIC), to conduct our analysis. For measurement, we designed a project-specific survey that was given to caregivers of children who underwent PSG in February 2018 and repeated the survey after interventions in February 2019. Lean and Six Sigma quality improvement tools were used to define important processes that influence patient satisfaction, including: supplier, input, process, output, customer, and requirements (SIPOC-R); journey mapping; 1-2-4-All brainstorming; and views solicited from our center's Patient and Family Advisory Council. We analyzed the relationships between identified processes and outcomes using usual descriptive statistics. We prioritized interventions using a Kano model and a quality function deployment (QFD) technique to rank priorities for interventions. Multiple opportunities to improve patient and family satisfaction before, during, and after a pediatric polysomnography were identified. Many were simple, one-step interventions and were implemented simultaneously. For those that required substantial training and/or scheduling changes, pilots were performed and plan, do, study, act (PDSA) cycles were used to check effectiveness. RESULTS: After implementation, top box scores rose 20%, from 51% (n = 47) in 2018 to 71% (n = 50) in 2019. CONCLUSIONS: Various quality improvement techniques employed in business, engineering, and manufacturing were used to identify and address areas of improvement in the pediatric polysomnography experience.

Practical Implementation of a Single-Night Split-Titration Protocol With BPAP-ST and AVAPS in Patients With Neuromuscular Disease.

STUDY OBJECTIVES: At the sleep laboratory, noninvasive positive pressure ventilation titration protocols in patients with neuromuscular disease (NMD) are based on standard pressure cycle devices in a spontaneous/timed mode (BPAP-ST). Experience integrating protocols on average volume-assured pressure support (AVAPS) mode is limited, prompting us to develop a practical single-night titration protocol that provides information to assist clinicians and patients as they decide between BPAP-ST and AVAPS modes. METHODS: We implemented a sequential titration protocol of BPAP-ST followed by AVAPS during a single-night polysomnography study in patients with NMD and reported polysomnographic and clinical metrics. RESULTS: There were 27 patients who completed the protocol: 14 (52%) were male with median and interquartile range (IQR) 64 (59 to 70) years of age and body mass index of 29.6 (25.6-32) kg/m2. They had median (IQR) maximal percent predicted inspiratory and expiratory pressures, and percent vital capacity of 33 (24 to 54), 34 (22 to 47) and 60 (47 to 74), respectively. At final titration of each device, average tidal volume and nadir non-rapid eye movement sleep oxyhemoglobin saturation (SpO2) were higher and respiratory rate/tidal volume, transcutaneous CO2, and arousal index were lower on AVAPS (P < .05) in comparison with BPAP-ST. Full face mask was used in 23 patients (85%). None of the other ventilatory or sleep parameters differed significantly between BPAP-ST and AVAPS (P > .05) sessions. CONCLUSIONS: A practical single-night split-titration protocol with BPAP-ST and AVAPS can successfully be implemented in patients with NMD, assisting clinicians and patients with the decision on initial treatment modalities and settings.

Analyses of the Complexity of Patients Undergoing Attended Polysomnography in the Era of Home Sleep Apnea Tests.

STUDY OBJECTIVES: Health care complexity includes dimensions of patient comorbidity and the level of services needed to meet patient demands. Home sleep apnea tests (HSAT) are increasingly used to test medically uncomplicated patients suspected of having moderate to severe obstructive sleep apnea (OSA). Patients with significant comorbidities or other sleep disorders are not candidates for HSAT and require attended in-center polysomnography. We hypothesized that this trend would result in increasingly complex patients being studied in sleep centers. METHODS: Our study had two parts. To ascertain trends in sleep patient comorbidity, we used administrative diagnostic codes from patients undergoing polysomnography at the Mayo Clinic Center for Sleep Medicine from 2005 to June 2015 to calculate the Charlson and the Elixhauser comorbidity indices. We measured the level of services provided in two ways: (1) in a subset of patients from the past 2 months of 2015, we evaluated correlation of these morbidity indices with an internally developed Polysomnogram Clinical Index (PSGCI) rating anticipated patient care needs from 0 to 3 and (2) we measured the sleep study complexity based on polysomnography protocol design. RESULTS: In 43,780 patients studied from 2005 to June 2015, the Charlson index increased from a mean of 1.38 to 1.88 (3.1% per year, P < .001) and the mean Elixhauser index increased from 2.61 to 3.35 (2.5% per year, P < .001). Both comorbidity indices were significantly higher at the highest (Level 3) level of the PSGCI (P < .001), and sleep study complexity increased over time. CONCLUSIONS: The complexity of patients undergoing attended polysomnography has increased by 28% to 36% over the past decade as measured by validated comorbidity indices, and these indices correlate with the complexity of rendered care during polysomnography. These findings have implications for increasing requirements for staffing, monitoring capabilities, and facility design of future sleep centers. COMMENTARY: A commentary on this article appears in this issue on page 499.

Familial premature coronary artery disease mortality and obstructive sleep apnea.

BACKGROUND: Obstructive sleep apnea (OSA) is linked to both coronary artery disease (CAD) and sudden death, but any causal role remains unclear. A family history of premature CAD and related mortality is an independent risk factor for the development of CAD. We hypothesized that OSA is associated with a family history of premature mortality from ischemic heart disease. METHODS: We prospectively studied 588 subjects who underwent polysomnography from May 2000 to June 2004. Demographics, comorbidities, family history of cardiovascular disease, and the ages and causes of death for 10 strata of family members were recorded for all subjects. We excluded those subjects with known causes of premature cardiac death, such as hypertrophic cardiomyopathy and long-QT syndrome. OSA was defined by American Academy of Sleep Medicine criteria (ie, apnea-hypopnea index >or= 5). Premature CAD mortality was defined as death due to ischemic heart disease or sudden cardiac death before 55 years of age (men) or 65 years of age (women). RESULTS: Polysomnography confirmed OSA in 316 subjects and excluded it in 202 subjects. The unadjusted odds ratio (OR) for OSA and a family history of premature CAD mortality was 2.11 (95% confidence interval [CI], 1.10 to 4.31; p = 0.031). After adjusting for each subject's sex, body mass index, and history of CAD, there was a significant and independent association between OSA and family history of premature CAD mortality (OR, 2.13; 95% CI, 1.04 to 4.66; p = 0.046). CONCLUSIONS: Regardless of their own CAD status, people with OSA are more likely than those without OSA to have a family history of premature CAD mortality.

A randomized, double-blind clinical trial comparing continuous positive airway pressure with a novel bilevel pressure system for treatment of obstructive sleep apnea syndrome.

STUDY OBJECTIVES: To obtain efficacy, objective compliance, and self-assessment data from obstructive sleep apnea syndrome (OSAS) patients treated with continuous positive airway pressure (CPAP) or a novel bilevel (NBL) therapy. DESIGN: Randomized, controlled, double-blind trial. SETTING: Home treatment after diagnosis and titration by split-night polysomnography (PSG) in a sleep laboratory. PATIENTS: Twenty-seven adults (22 men) newly referred for suspected OSAS but without concomitant medical or sleep disorders. INTERVENTIONS: If the subject's apnea-hypopnea index was greater than 10 and less than 100, the CPAP was titrated during PSG and then followed by NBL titration. Treatment was randomly and blindly set to either CPAP or NBL mode for 1 month. MEASUREMENTS & RESULTS: There were no significant baseline group differences in age, body mass index, apnea-hypopnea index (mean +/- SD, CPAP group vs NBL group of 46.1 +/- 23.1/hour vs 41.8 +/- 25.8), CPAP requirement, or scores on the Epworth Sleepiness Scale and Functional Outcomes of Sleep Questionnaire. Treatment with CPAP and NBL equivalently reduced the apnea-hypopnea index during the laboratory titration (7.6 +/- 11.9/hour vs. 3.7 +/- 4.4, respectively). At 1 month, there were no significant group compliance differences as determined by percentage of nights with at least 4 hours of use (CPAP, 80.5 +/- 24 vs NBL, 77.6 +/- 24.8) and hours of use per night (CPAP, 5.6 +/- 1.4 hours/night vs NBL, 5.6 +/- 1.7). Similar improvements were seen in scores on the Epworth Sleepiness Scale and Functional Outcomes of Sleep Questionnaire. CONCLUSIONS: The NBL appeared to be as effective as CPAP for the treatment of OSAS but offered no advantages in patients receiving first-time therapy for OSAS.