Anaesthesia management of a patient with Bethlem Myopathy for elective tonsillectomy: a case report.

BACKGROUND: Bethlem Myopathy is a collagen VI-related myopathy presenting as a rare hereditary muscular disorder with progressive muscular weakness and joint contractures. Despite its milder clinical course relative to other myopathies, anaesthetic management can be challenging. High arched palates and fixed flexion deformities may contribute to a difficult airway. A progressive decline in pulmonary function can present later into adulthood. This respiratory decline can carry secondary cardiovascular consequences due to the progressive nature of restrictive lung disease, including right sided heart disease and pulmonary hypertension. We describe a case of a male patient with Bethlem Myopathy undergoing anaesthesia, to contribute to the limited body of literature on this condition and enhance awareness and guidance amongst anaesthesiologists on approaching patients with this condition. This is the first case report within the literature of its kind. CASE PRESENTATION: This case details a 33-year-old male with Bethlem Myopathy undergoing tonsillectomy. Diagnosed in childhood following developmental delays, the patient had no prior anaesthetic exposure and no family history of anaesthetic complications. Anaesthetic induction was achieved without complications, avoiding depolarizing muscle relaxants and careful airway management. Extreme care was taken in patient positioning to prevent complications. The surgery proceeded without incident and muscle paralysis was reversed with Suggammadex, resulting in no adverse post-operative respiratory complications. The patient was discharged on the first post-operative day without any respiratory or cardiovascular compromise. CONCLUSIONS: Bethlem Myopathy, while often exhibiting a mild clinical course, can present anaesthetic challenges. Awareness of potential complications including a difficult airway, cardiovascular and respiratory implications as well as the need for specialised monitoring and positioning is crucial to ensure a safe peri-operative course.

Depression risk in chronic tonsillitis patients underwent tonsillectomy: a global federated health network analysis.

Background: Tonsillectomy is a common surgery in the US, with possible postoperative complications. While small studies indicate postoperative depressive symptoms may occur, large-scale evidence is lacking on the tonsillectomy-depression link. Methods: We conducted a retrospective cohort study using the TriNetX US collaborative network, offering de-identified electronic health data from 59 collaborative healthcare organizations (HCOs) in the United States. In this study, people being diagnosed of chronic tonsillitis between January 2005 and December 2017 were enrolled. Patients deceased, with previous record of cancers or psychiatric events before index date were excluded. 14,874 chronic tonsillitis patients undergoing tonsillectomy were propensity score matched 1:1 to controls for age, sex, and race. New-onset depression risks were evaluated over 5 years post-tonsillectomy and stratified by age and sex. Confounders were adjusted for including demographics, medications, comorbidities and socioeconomic statuses. Results: After matching, the difference of key baseline characteristics including age, sex, comedications status and obesity status was insignificant between tonsillectomy and non-tonsillectomy groups. Tonsillectomy had a 1.29 times higher 5-year depression risk versus matched controls (95% CI, 1.19-1.40), with elevated risks seen at 1 year (HR=1.51; 95% CI, 1.28-1.79) and 3 years (HR=1.30; 95% CI, 1.18-1.43). By stratifications, risks were increased for both males (HR=1.30; 95% CI, 1.08-1.57) and females (HR=1.30; 95% CI, 1.18-1.42), and significantly higher in ages 18-64 years (HR=1.37; 1.26-1.49), but no significance observed for those 65 years and older. After performing sensitivity analyses and applying washout periods of 6, 12, and 36 months, the outcome remained consistent with unadjusted results. Conclusion: This real-world analysis found tonsillectomy was associated with a 30% higher 5-year depression risk versus matched non-tonsillectomy patients with chronic tonsillitis. Further mechanistic research is needed to clarify the pathophysiologic association between depression and tonsillectomy. Depression is not commonly mentioned in the current post-tonsillectomy care realm; however, the outcome of our study emphasized the possibility of these suffering condition after operation. Attention to psychological impacts following tonsillectomy is warranted to support patient well-being, leading to better management of post-tonsillectomy individuals.

Adenotonsillectomy for Obstructive Sleep Apnea in Children.

Obstructed breathing is the most common indication for tonsillectomy in children. Although tonsillectomy is performed frequently worldwide, the surgery is associated with a number of significant complications such as bleeding and respiratory failure. Complication risk depends on a number of complex factors, including indications for surgery, demographics, patient comorbidities, and variations in perioperative techniques. While polysomnography is currently accepted as the gold standard diagnostic tool for obstructive sleep apnea, studies evaluating outcomes following surgery suggest that more research is needed on the identification of more readily available and accurate tools for the diagnosis and follow-up of children with obstructed breathing.

Absorption and pharmacokinetics of bupivacaine after bilateral topical administration in tonsillar fossae for posttonsillectomy pain relief.

No previous studies have investigated the systemic absorption of bupivacaine when used topically for posttonsillectomy pain. The present study was undertaken to investigate the pharmacokinetics of bupivacaine after administration by a swab in the tonsillar fossae over 4 min after tonsillectomy. Eleven adult patients undergoing elective tonsillectomy were recruited. After removal of both tonsils, each of the two tonsillar fossae was covered with a swab moistened with 2 mL of bupivacaine 5 mg/mL, that is, a total of 20 mg bupivacaine. Blood samples were drawn after 0, 5, 10, 20, 30, 45, and 60 min. Bupivacaine was analyzed with an ultra-high-performance liquid chromatography-tandem mass spectrometry method. The highest single measured bupivacaine serum concentration was 23.2 ng/mL and took place 10 min after drug administration. Mean (±SD) Cmax was 11.4 ± 6.0 ng/mL and mean tmax was 11.3 ± 4.7 min. Mean t1/2 was 31.6 ± 9.3 min. As the toxic concentration threshold has been reported to be in the interval 1500-4500 ng/mL, the concentrations measured were well below 2% of the lowest cited toxic threshold. In conclusion, this study shows that applying 4 mL of bupivacaine 5 mg/mL by a swab in the tonsillar fossae posttonsillectomy yields very low plasma concentrations, suggesting its safe application without any risk of systemic toxic effects.

Should paediatric tonsillar asymmetry be an indication for tonsillectomy? A single centre experience.

BACKGROUND: Paediatric tonsillar lymphoma (TL) is a rare diagnosis. Historically, the presence of clinical features such as tonsillar asymmetry, grossly abnormal tonsil appearance and cervical lymphadenopathy raise concern for this diagnosis. Tonsillar asymmetry is considered to be the most concerning clinical feature; however, asymmetry is often apparent due to differences in depth or shape of tonsillar fossa and tonsillar pillars, rather than a true difference in volume. There is debate whether a tonsillectomy is required in all cases of tonsil asymmetry to exclude lymphoma, and what clinical features should raise concern. The aim of this study was to establish whether the presence of clinical asymmetry can be deemed a reliable marker for genuine tonsil size discrepancies. We also sought to evaluate the clinical and examination characteristics that are concerning for lymphoma. METHODS: Retrospective review of clinical records for paediatric tonsil specimens sent for histological evaluation between 1 January 2012 and 1 January 2023 driven by a clinical suspicion of lymphoma at Starship Children's Hospital, New Zealand. Patient demographics and clinical data were recorded. A comparison was made between tonsil size asymmetry on clinical examination (Brodsky criteria) and tonsil volume difference based on dimensions given in pathology reports. RESULTS: One hundred and forty-three patients had tonsillectomies between 2012 and 2022 at Starship Children's Hospital due to concern for lymphoma. Of these, three were positive for lymphoma. Presence of pain and abnormal tonsil appearance were predictors for lymphoma (p<0.02). Interrater reliability agreement between clinical size difference and tonsil volume was poor, Kappa= -0.13 p<0.05. CONCLUSION: Clinical size difference is a poor predictor for true tonsil volume difference. We advise that assessment of tonsil size should be performed in conjunction with the examination of gross visual abnormalities and lymphadenopathy to guide clinical decision making.

A quality assessment of YouTube as an information resource for tonsillectomy.

PURPOSE: Online resources are increasingly being utilised by patients to guide their clinical decision making, as an alternative or supplement to the traditional clinical-patient relationship. YouTube is an online repository of user and community generated videos, which is one of the most popular websites globally. We undertook a study to examine the quality of information presented in YouTube videos related to tonsillectomy. METHODS: We completed a systematic search of YouTube in May 2023 and identified 88 videos for inclusion in our study. Videos were published in the English language, focussing on tonsillectomy and tonsillectomy recovery, and were greater than 2 min in length. We recorded video quality metrics and two authors independently analysed the quality of information using three validated quality assessment tools described in the literature including the modified DISCERN, Global Quality Score, and the JAMA Benchmark Criteria. RESULTS: The overall quality of the information was low with mean quality scores of Modified DISCERN (1.8 ± 1.3), GQS (2.6 ± 1.2), and JAMA Benchmark Criteria (1.6 ± 0.7). Information published by medical sources including medical professionals, healthcare organisations, and medical education channels scored significantly higher compared to non-medical sources across all quality measures and were of moderate overall quality and usefulness: Modified DISCERN (2.5 ± 1.1 vs 0.8 ± 0.9, z = -6.0, p < 0.001), GQS (3.2 ± 1.0 vs 1.7 ± 0.9, z = -5.7, p < 0.001), and JAMA (1.9 ± 0.8 vs 1.1 ± 0.3, z = -5.2, p < 0.001). Videos published during or after 2018 scored higher on Modified DISCERN (z = -3.2,p = 0.001) but not on GQS or JAMA. Video quality metrics such as total view count, likes, and comments, and channel subscriber count, did not correlate with higher video quality. However, amongst videos published by authoritative medical sources, total view count correlated positively with higher Modified DISCERN quality scores (p = 0.037). CONCLUSION: The overall quality and usefulness of YouTube videos on tonsillectomy is of low quality, but information published by authoritative medical sources score significantly higher. Clinicians should be mindful of increasing use of online information sources such as YouTube when counselling patients. Further research is needed in the medical community to create engaging, high-quality content to provide guidance for patients.

Effect of perioperative ketorolac on postoperative bleeding after pediatric tonsillectomy.

INTRODUCTION: Ketorolac is a frequently used anesthetic pain agent which is traditionally avoided during tonsillectomy due to concern for postoperative hemorrhage. Our goal was to assess the degree of risk associated with the use of Ketorolac following pediatric tonsillectomy. METHODS: The TriNetX electronic health records research database was queried in January 2024 for patients undergoing tonsillectomy with or without adenoidectomy under the age of 18 years and without a diagnosed bleeding disorder. Patients were separated into two cohorts either having received or not having received ketorolac the same day as surgery. Propensity score matching was performed for age at the time of surgery, sex, race, ethnicity, and preoperative diagnoses. The outcomes assessed were postoperative hemorrhage requiring operative control within the first day (primary hemorrhage) and within the first month after surgery (secondary hemorrhage). RESULTS: 17,434 patients were identified who had undergone pediatric tonsillectomy with or without adenoidectomy and had received ketorolac the same day as surgery. 290,373 patients were identified who had undergone pediatric tonsillectomy with or without adenoidectomy and had not received ketorolac the same day as surgery. 1:1 propensity score matching resulted in 17,434 patients within each cohort. Receipt of ketorolac the same day as surgery resulted in an increased risk of primary hemorrhage OR 2.158 (95 % CI 1.354, 3.437) and secondary hemorrhage OR 1.374 (95 % CI 1.057, 1.787) requiring operative control. CONCLUSION: Ketorolac use during pediatric tonsillectomy with or without adenoidectomy was associated with an increased risk of postoperative primary and secondary bleeding requiring surgery.

Neurostimulation for Pediatric Obstructive Sleep Apnea.

Up to 80% of children with Down syndrome (DS) are affected by obstructive sleep apnea (OSA), and only 16% to 30% will have resolution of their OSA with adenotonsillectomy. Hypoglossal nerve stimulation is a well-established therapy for adults with OSA and was recently approved by the Food and Drug Administration for use in children with DS and residual OSA. There is robust experience with this therapy in adults that has led to well-established care pathways. However, given the challenges inherent to caring for a complex pediatric population, these pathways are not directly transferrable to children with DS.

Risk of post-tonsillectomy hemorrhage among COVID-19 positive pediatric patients.

OBJECTIVE: COVID-19 infection has been demonstrated to increase risk for post-operative bleeding. This study investigated the impact of COVID-19 infection on post-tonsillectomy hemorrhage in pediatric patients, a potentially devastating complication. STUDY DESIGN: Retrospective cohort study. METHODS: The TriNetX database was queried for pediatric patients who underwent tonsillectomy and evaluated for outcomes of primary and secondary post-tonsillectomy hemorrhage. RESULTS: Among subjects 18 years and younger, 1226 were COVID-19 positive and 38,241 were COVID-19 negative in the perioperative period. There was statistically significant increased risk of bleeding with perioperative COVID-19 infection at postoperative days 1, 5, and 10. Additionally, when assessing the role of COVID-19 infection before or after surgery, the risk of bleeding remained statistically significant at all three time points, however these results did not suggest that infection before surgery confers more/less risk compared to infection after. CONCLUSION: The results of this investigation suggest that the presence of COVID-19 in the perioperative period may pose an increased risk for acute or delayed post tonsillectomy hemorrhage. This study employed a large, diverse population and is the first to address this clinical question.

The utility and accuracy of ChatGPT in providing post-operative instructions following tonsillectomy: A pilot study.

OBJECTIVE: To investigate the utility of answers generated by ChatGPT, a large language model, to common questions parents have for their children following tonsillectomy. METHODS: Twenty Otolaryngology residents anonymously submitted common questions asked by parents of pediatric patients following tonsillectomy. After identifying the 16 most common questions via consensus-based approach, we asked ChatGPT to generate responses to these queries. Satisfaction with the AI-generated answers was rated from 1 (Worst) to 5 (Best) by an expert panel of 3 pediatric Otolaryngologists. RESULTS: The distribution of questions across the five most common domains, their mean satisfaction scores, and their Krippendorf's interrater reliability coefficient were: Pain management [6, (3.67), (0.434)], Complications [4, (3.58), (-0.267)], Diet [3, (4.33), (-0.357)], Physical Activity [2, (4.33), (-0.318)], and Follow-up [1, (2.67), (-0.250)]. The panel noted that answers for diet, bleeding complications, and return to school were thorough. Pain management and follow-up recommendations were inaccurate, including a recommendation to prescribe codeine to children despite a black-box warning, and a suggested post-operative follow-up at 1 week, rather than the customary 2-4 weeks for our panel. CONCLUSION: Although ChatGPT can provide accurate answers for common patient questions following tonsillectomy, it sometimes provides eloquently written inaccurate information. This may lead to patients using AI-generated medical advice contrary to physician advice. The inaccuracy in pain management answers likely reflects regional practice variability. If trained appropriately, ChatGPT could be an excellent resource for Otolaryngologists and patients to answer questions in the postoperative period. Future research should investigate if Otolaryngologist-trained models can increase the accuracy of responses.

Pain management after tonsil surgery in children and adults-A national survey related to pain outcome measures from the Swedish Quality Register for tonsil surgery.

OBJECTIVE: The primary aim of this study was to describe the current practice regarding pain management in relation to tonsil surgery among Ear Nose and Throat (ENT) clinics in Sweden. The secondary aim was to determine the impact of the provider's regime of rescue analgesics on the pain related Patient Reported Outcome Measures (pain-PROMs) from the Swedish Quality Register for Tonsil Surgery (SQTS). MATERIALS & METHODS: A descriptive cross-sectional study originating from a validated web-based questionnaire. The survey enrolled one respondent from each ENT clinic (47/48 participated) nationally. Pain-PROMs from the SQTS, recorded from October 2019 to October 2022, were included (8163 tonsil surgeries). RESULTS: Paracetamol was used by all enrolled ENT clinics as preemptive analgesia. The addition of COX inhibitors was used in 40% of the clinics. Betamethasone was usually administered, to prevent pain and nausea (92%). All clinics gave postdischarge instructions on multimodal analgesia with COX inhibitors and paracetamol. Rescue analgesics were prescribed after tonsillectomy for 77% of adults, 62% of older children, 43% of young children and less often after tonsillotomy. The most frequently prescribed rescue analgesic was clonidine in children (55%) and oxycodone in adults (72%). A high proportion of patients reported contact with health care services due to postoperative pain (pain-PROMs/ SQTS). Tonsillectomy procedures were associated with the highest rates of contacts (children/adolescents 13-15%; adults 26%), while tonsillotomy were associated with lower rates, (5-7% of children/adolescents). There was no significant difference in the frequency of health care contacts due to pain regarding whether clinics routinely prescribed rescue analgesics or not after tonsillectomy. CONCLUSION: The Swedish analgesic regimen after tonsil surgery is good overall. Nevertheless, there is a need for increased awareness and knowledge to achieve optimal patient recovery. Pain-PROM data demonstrate the call for improvement in pain management after tonsil surgery.

Surgical Management of Pediatric Obstructive Sleep Apnea Beyond Adenotonsillectomy: The Nose, Nasopharynx, and Palate.

While adenotonsillectomy is the primary treatment of pediatric obstructive sleep apnea (OSA), persistent OSA after surgery is common and may be due to residual obstruction at the nose, nasopharynx, and/or palate. Comprehensive evaluation for persistent pediatric OSA ideally includes clinical examination (with or without awake nasal endosocpy) as well as drug-induced sleep endoscopy in order to accurately identify sources of residual obstruction. Depending on the site of obstruction, some of the surgical management options include submucous inferior turbinate resection, septoplasty, adenoidectomy, and expansion sphincter pharyngoplasty.

Surgical Management of Pediatric Obstructive Sleep Apnea Beyond Tonsillectomy & Adenoidectomy: Tongue Base and Larynx.

Pediatric patients with persistent obstructive sleep apnea (OSA) after adenotonsillectomy often have additional sites of upper airway obstruction such as the tongue base or larynx. Sleep endoscopy and cross-sectional, dynamic imaging can be used to direct surgical management of persistent OSA. The tongue base is one of the most common sites of obstruction in children with persistent OSA, especially for patients with Trisomy 21. Lingual tonsillectomy, tongue suspension, and/or posterior midline glossectomy may be used to address lingual tonsil hypertrophy and tongue base obstruction. Epiglottopexy and/or supraglottoplasty may be used to address laryngomalacia and epiglottic prolapse resulting in OSA.

Adenotonsillectomy for the treatment of OSA in children with mucopolysaccharidosis: A systematic review.

OBJECTIVE: To study the role of adenotonsillectomy (ADT) for obstructive sleep apnea (OSA) in children with mucopolysaccharidosis (MPS). METHODS: A systematic review were performed following the PRISMA guideline. PubMed and Embase were searched for studies regarding adenotonsillectomy for OSA in children with MPS. The MINOR Score were applied for quality assessment of the included studies. RESULTS: Nineteen studies were eligible for inclusion: fifteen were retrospective and four prospective. A total of 1406 subjects were included. The samples size varied from 2 to 336, the male to female ratio is 1.2 and mean age varied from 2.4 to 11 years. Overall, 56.2 % (IC 95%: 53.6-58.8) of the included subjects underwent ADT. MPS I and II are the two most operated types. Three studies, including 50 children, reported improvement in polysomnographic parameters after surgery. Two authors described the duration of follow-up: 8.4 and 9.8 years, respectively. CONCLUSIONS: More than half of children with MPS underwent ADT for the treatment of OSA, although few evidence demonstrated improvement in term of polysomnographic parameters. The two types of MPS most involved are type I and II. Considering the disease progression and anesthetic risks, multidisciplinary management may help identify the subgroup of children with MPS who benefit from ADT for the treatment of OSA.

The impact of intranasal corticosteroids in a prospective cohort of children with sleep disordered breathing.

INTRODUCTION: Sleep disordered breathing (SDB) is common in children and the most common reason for adenotonsillectomy. This large observational cohort study from a specialist outpatient clinic describes the impact of intranasal steroids (INS) on symptom improvement and the need for surgery. METHOD: Observational cohort study of 568 children assessing the impact of INS using the OSA-5 questionnaire with clinical and surgical outcome measures. RESULTS: The mean OSA-5 score at first visit was 7.78. Symptoms were persistent for a median 9 months (range 2-72). 51% underwent a trial of INS with 56% reporting symptomatic improvement. The mean score decreased from 8.2 to 5.5 (p < 0.0001) in those prescribed INS. They had a significantly higher symptom load (p < 0.01), turbinate size (p < 0.005) and history of atopy (p < 0.01) than the non-trial group. The rate of surgery in the non-trial group was 56% compared with 38% in those who had INS (p < 0.001). With increasing symptom burden, the reported improvement with INS and comparative reduction in surgery increased. Baseline OSA-5 scores were predictive of rates of surgery. Atopic status or age did not influence response to INS. CONCLUSION: The mean score at first visit and the median duration of symptoms indicated significant persistent symptoms in this cohort. The use of INS improved symptoms of SDB in 56%. The need for surgery in the group that received INS was 38% compared with 56% in those not trialling INS, despite the non-trial group having significantly less symptoms and signs. Symptomatic improvement was not influenced by age or atopic status.