Malformación de Arnold Chiari / Arnold Chiari malformation

La malformación de Arnold Chiari es una enfermedad rara que consiste en una alteración anatómica de la base del cráneo, en la que se produce herniación del cerebelo y del tronco del encéfalo a través del foramen magnum hasta el canal cervical. Muchos niños afectados nunca llegan a tener síntomas. En el caso de que cause síntomas, estos no suelen aparecer hasta la infancia tardía o adolescencia. Presentamos el caso clínico de un paciente de 2 años con malformación de Arnold Chiari tipo I. (AU)

Arnold Chiari malformation is a rare disease that consists of an anatomical alteration of the base of the skull, in which herniation of the cerebellum and brainstem occurs through the foramen magnum to the cervical canal. Many affected children never develop symptoms. If it does cause symptoms, they usually don’t appear until late childhood or adolescence. We present the clinical case of a 2-year-old patient with Arnold Chiari malformation type I. (AU)

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.

The role of overnight pulse oximetry in risk stratification of paediatric sleep disordered breathing in a district general hospital.

OBJECTIVES: Adenoidectomy and tonsillectomy are common surgical interventions in paediatric patients with sleep disordered breathing. Post operative respiratory complications are a recognised risk, and pre-operative risk stratification of patients is important to enable safe delivery of peri-operative care. Due to easy accessibility, overnight pulse oximetry is commonly used for this purpose. However, its limitations have been widely reported and recent national guidance recommends limiting use to patients with significant risk factors. We reviewed the use of overnight pulse oximetry in our local unit to determine if local guidelines should be adapted in line with these national recommendations. METHODS: Retrospective analysis of all paediatric patients referred for overnight pulse oximetry over an eighteen month period between August 2020 to February 2022. Data collection included patient age, weight, and relevant co-morbidities. The McGill score was used to risk stratify patients and determine the need for an overnight bed. This was then correlated with the occurrence of post operative respiratory complications. RESULTS: 200 patients were referred for overnight pulse oximetry, with a mean age of 7.5 years. 7.5 % (15/200) had significant comorbidities. 64 % (128/200) of patients were subsequently listed for surgery. 20.3 % (26/128) were deemed at risk of post-operative complications due to McGill scores of 3 and 4 and planned for overnight ward observation. Of these, 15 % (4/26) were subsequently discharged the same day. None of our patients developed major respiratory complications in the post operative period. CONCLUSION: Our findings support national recommendations that overnight pulse oximetry is not an accurate predictor of post-operative respiratory complications and resulted in unnecessary inpatient stays. Following discussion with the trust paediatric anaesthetist lead, our local guidance was tightened, limiting overnight pulse oximetry to children with relevant identifiable co-morbidities.

Postoperative Outcomes of Intracapsular Tonsillectomy With Coblation: A Systematic Review and Meta-Analysis.

OBJECTIVE: Following tonsillectomy, postoperative pain and hemorrhage from the tonsillar bed are causes of significant morbidity. Intracapsular tonsillectomy with Coblation is suggested to minimize such morbidity while remaining efficacious in long-term outcomes. This systematic review and meta-analysis assessed short-term morbidity and long-term outcomes from intracapsular tonsillectomy with Coblation, focusing primarily on posttonsillectomy hemorrhage. DATA SOURCES: Medline, Embase, and the Cochrane Library. REVIEW METHODS: Guided by PRISMA guidelines, studies on intracapsular tonsillectomy with Coblation published between December 2002 and July 2022 evaluating frequency of posttonsillectomy hemorrhage were screened. Studies without primary data were excluded. Meta-analysis was conducted using the random-effect model. The primary outcome was the proportion of patients who experienced posttonsillectomy hemorrhage. The secondary outcomes were posttonsillectomy pain, the proportion requiring revision tonsillectomy, and severity of sleep-disordered breathing measured by polysomnography outcomes. RESULTS: From 14 studies there were 9821 patients. The proportion of total posttonsillectomy hemorrhage was 1.0% (95% confidence interval [CI] 0.5%-1.6%, n = 9821). The proportion experiencing primary hemorrhage, secondary hemorrhage, and those requiring further tonsil surgery were 0.1% (95% CI 0.0%-0.1%; study n = 7), 0.8% (95% CI 0.2%-1.4%; study n = 7), and 1.4% (95% CI 0.6%-2.2%; study n = 6), respectively. Mean reduction in apnea-hypopnea index was -16.0 events per hour (95% CI -8.8 to -23.3, study n = 3) and mean increase in oxygen nadir was 5.9% (95% CI 2.6%-9.1%, study n = 3). CONCLUSION: Intracapsular tonsillectomy with Coblation has been demonstrated to have a low rate of posttonsillectomy hemorrhage. Data regarding long-term tonsil regrowth and need for reoperation were encouraging of the efficacy of this technique.

Adenotonsillectomy for Snoring and Mild Sleep Apnea in Children: A Randomized Clinical Trial.

Importance: The utility of adenotonsillectomy in children who have habitual snoring without frequent obstructive breathing events (mild sleep-disordered breathing [SDB]) is unknown. Objectives: To evaluate early adenotonsillectomy compared with watchful waiting and supportive care (watchful waiting) on neurodevelopmental, behavioral, health, and polysomnographic outcomes in children with mild SDB. Design, Setting, and Participants: Randomized clinical trial enrolling 459 children aged 3 to 12.9 years with snoring and an obstructive apnea-hypopnea index (AHI) less than 3 enrolled at 7 US academic sleep centers from June 29, 2016, to February 1, 2021, and followed up for 12 months. Intervention: Participants were randomized 1:1 to either early adenotonsillectomy (n = 231) or watchful waiting (n = 228). Main Outcomes and Measures: The 2 primary outcomes were changes from baseline to 12 months for caregiver-reported Behavior Rating Inventory of Executive Function (BRIEF) Global Executive Composite (GEC) T score, a measure of executive function; and a computerized test of attention, the Go/No-go (GNG) test d-prime signal detection score, reflecting the probability of response to target vs nontarget stimuli. Twenty-two secondary outcomes included 12-month changes in neurodevelopmental, behavioral, quality of life, sleep, and health outcomes. Results: Of the 458 participants in the analyzed sample (231 adenotonsillectomy and 237 watchful waiting; mean age, 6.1 years; 230 female [50%]; 123 Black/African American [26.9%]; 75 Hispanic [16.3%]; median AHI, 0.5 [IQR, 0.2-1.1]), 394 children (86%) completed 12-month follow-up visits. There were no statistically significant differences in change from baseline between the 2 groups in executive function (BRIEF GEC T-scores: -3.1 for adenotonsillectomy vs -1.9 for watchful waiting; difference, -0.96 [95% CI, -2.66 to 0.74]) or attention (GNG d-prime scores: 0.2 for adenotonsillectomy vs 0.1 for watchful waiting; difference, 0.05 [95% CI, -0.18 to 0.27]) at 12 months. Behavioral problems, sleepiness, symptoms, and quality of life each improved more with adenotonsillectomy than with watchful waiting. Adenotonsillectomy was associated with a greater 12-month decline in systolic and diastolic blood pressure percentile levels (difference in changes, -9.02 [97% CI, -15.49 to -2.54] and -6.52 [97% CI, -11.59 to -1.45], respectively) and less progression of the AHI to greater than 3 events/h (1.3% of children in the adenotonsillectomy group compared with 13.2% in the watchful waiting group; difference, -11.2% [97% CI, -17.5% to -4.9%]). Six children (2.7%) experienced a serious adverse event associated with adenotonsillectomy. Conclusions: In children with mild SDB, adenotonsillectomy, compared with watchful waiting, did not significantly improve executive function or attention at 12 months. However, children with adenotonsillectomy had improved secondary outcomes, including behavior, symptoms, and quality of life and decreased blood pressure, at 12-month follow-up. Trial Registration: ClinicalTrials.gov Identifier: NCT02562040.

[New Swedish National care process for pediatric obstructive sleep disordered breathing]. / Nytt vårdförlopp för barn med OSDB är godkänt att tas i bruk.

Obstructive sleep disordered breathing (OSDB) is a spectrum from habitual snoring and labored breathing to obstructive sleep apnea (OSA), which is common and potentially serious in children. The process contains a new question at child care centers, directed at caretakers with children at age 18 months and 3 years, concerning habitual snoring (3 times a week or more). A primary care doctor verifies the suspicion of OSDB in case of a positive answer to one of 7 additional questions or 4 status findings (e.g. tonsil hypertrophy). The process starts with the suspicion of OSDB, from the age of 18 months to 18 years, and ends when symptoms are improved after watchful waiting or upper airway surgery. National equality is a goal, with increased access to nocturnal respiratory recordings of children with comorbidities or doubtful cases. Also, with short waiting time to first visit at ORL department, and to surgery. Children with comorbidities or severe symptoms get postoperative follow-ups with a nurse after 6 months. The new ICD code for OSDB is R06.8A.

Intracapsular tonsillectomy: setting a new standard.

PURPOSE OF REVIEW: Tonsillectomy is one of the most common surgical procedures performed on children in the United States. Since 2002, the intracapsular technique has been studied as a safer and less painful alternative to total tonsillectomy. Concerns have been raised, however, as to the potential for regrowth and long-term outcomes regarding this technique. RECENT FINDINGS: Studies support the use of intracapsular tonsillectomy in the management of sleep disordered breathing, including in syndromic populations, as well as for tonsillitis. In addition, safety profiles continue to be improved over that of extracapsular dissection. While the incidence of regrowth ranges depending on the study and duration of follow up, it remains acceptably low. The most consistent independent risk factor for revision surgery includes young age. SUMMARY: While total tonsillectomy is more thoroughly studied historically, an important absence in the literature is a definitive superiority over the intracapsular technique. With continued high-level studies, as well as additional examination of long-term outcomes, we should continue to see greater acceptance of intracapsular tonsillectomy as a standard of practice in a vulnerable population.

Reconsidering routine admission for children under age 3 undergoing partial tonsillectomy: a prospective study.

BACKGROUND: Partial Tonsillectomy (PT) is an alternative method to treat sleep disordered breathing (SDB) and/or obstructive sleep apnea (OSA). The current guidelines do not differentiate it from traditional tonsillectomy. Thus, children younger than 3 years old undergoing PT are admitted for surveillance similar to traditional tonsillectomy due to possible postoperative complications. The aim of this study is to assess the risks of PT in children 3 years old and younger, compared to older children. METHODS: Children underwent inpatient partial tonsillectomy and/or adenoidectomy, due to SDB/OSA, from 2018 to 2020. A special protocol was designed, including follow-up at 2-, 4-, 6-, 8- and 24-h after surgery. Variables analyzed included visual analogue pain score, oral intake, oxygen saturation, pulse rate, postoperative hemorrhage, urine output, temperature, analgesics and fluid administration. Furthermore, major interventions were recorded. Comparison of all variables between children younger than 3 years old with older children was performed. RESULTS: Ninety-two children were included; mean age of the whole cohort was 44.5 ± 21.9 months. Thirty-five (38%) children were 3-years old or younger and n = 57 (62%) were older than 3 years old, with no significant statistical difference in sex (p = 0.22). Mean age in the younger group was 25.7 ± 6.9 months, and 56.1 ± 20.1 months in the older group. In total we had 7 children with post-operative complications; 4 with fever, 3 with low intake. There were no major interventions recorded in either group. The complications were more common in the older group (n = 5) than the younger group (n = 2) without a statistical significance (p = 0.59). There were no differences in VAS, use of painkillers, oral intake, urine output, oxygen saturation and tachycardia among the two groups. CONCLUSION: This study supports that children undergoing ambulatory PT may be at low risk of complications, regardless of age.

Factors affecting polysomnography compliance and delays to surgical treatment of obstructive sleep apnea.

OBJECTIVE: To evaluate adherence to polysomnography in pediatric patients and determine if demographics, socioeconomic status, polysomnography indication, or prior otolaryngology intervention are associated with polysomnography adherence and time to definitive surgery. STUDY DESIGN: Retrospective review study. SETTING: Tertiary-care children's hospital. METHODS: Electronic medical record was queried to identify patients ordered for a sleep study between January and May 2019. Demographic information, time to sleep study, and time to surgery were collected and calculated. RESULTS: 304 patients were recommended to obtain polysomnography, with adherence rate of 65.4%. There was no significant difference in adherence or loss to follow-up rates based on patient sex, age, language, socioeconomic status, state of residence, single-parent status, or polysomnography indication. There was no difference between time to surgery for patients who did or did not obtain polysomnography (181 vs. 161 days, P = .51). Patients with prior otolaryngology intervention were more likely to obtain polysomnography and less likely to be lost to follow-up (P < .05). Median household income demonstrated a significant inverse relationship with time to polysomnography (P < .05) as well as time to surgery (P < .05). Medically complex patients tended to experience longer time to surgery compared with non-complex patients. CONCLUSION: Families with lower socioeconomic status or medically complex children may require assistance to obtain polysomnography and pursue surgery for sleep-disordered breathing. Patients without prior otolaryngology intervention may be less likely to follow up and may need assistance with navigating the polysomnography process.

Parental Experiences With Access to Care for Obstructive Sleep-Disordered Breathing: A Qualitative Study.

OBJECTIVE: Despite evidence-based guidelines for obstructive sleep-disordered breathing (SDB), recent studies continue to highlight treatment inequities. We used qualitative research methods to examine parental facilitators and barriers to SDB treatment. STUDY DESIGN: Qualitative interviews. SETTING: Tertiary care center. METHODS: Semistructured interviews were conducted (January-April 2022) with parents of children with SDB who underwent tonsillectomies to understand the processes of SDB detection and accessing specialty care. Interviews were conducted until thematic saturation was reached and coded using NVivo software. RESULTS: Of the 17 parents who completed the key informant interviews, 6 (35%) were of non-Hispanic black race, and 3 (17.6%) interviews were conducted in Spanish. Parents noted that the more knowledge their primary care provider (PCP) had about SDB, the easier it was to obtain a diagnostic workup (41%). The most common barrier included difficulty obtaining a specialist (otolaryngology or sleep medicine) referral from their PCP and encountering providers who were dismissive of parent-reported symptoms related to SDB, leading them to seek a second opinion or self-refer (53%). Medicaid coverage was a strong facilitator to receipt of care (59%). Three (17.6%) parents noted alienation in the process due to racial bias or language barriers. CONCLUSION: Parental interviews revealed that facilitators of SDB treatment included high clinician knowledge and perceived importance of SDB as well as Medicaid insurance which decreased financial strain. Parents also cited the attainment of referrals as a significant barrier to obtaining specialty evaluation. These findings identify potential modifiable areas to tailor future interventions for timely and equitable SDB care.

Pediatric obesity education and counseling in otolaryngology clinics: A survey of ASPO members.

BACKGROUND: Adenotonsillectomy can cure pediatric obstructive sleep apnea (OSA) in 80% of children without obesity but only 20-30% of children with obesity. Despite this, there is a current lack of consistent guidelines and practices around pediatric obesity management in otolaryngology. This study evaluated the extent of counseling, referrals, confidence, and barriers in addressing childhood obesity in pediatric otolaryngology. METHODS: A 20-question electronic survey assessing pediatric obesity practice patterns regarding sleep-disordered breathing (SDB) and OSA was distributed to all American Society of Pediatric Otolaryngology (ASPO) members. Descriptive statistics were performed. Pediatric otolaryngologists were compared using logistic regression models based on their practice type and frequency of counseling and referral. RESULTS: Of all ASPO members, 19.6% (114/583) completed the survey. Half (50.0%) of physicians counsel and 14.0% refer to obesity management >75% of the time. Only 8.8% of physicians are confident their counseling helps reduce their patient's obesity, and 35.9% of physicians are satisfied with their services available for referral. Most physicians cite time/clinical workload (81.6%) and parent/rearing habits (50.0%) as barriers to obesity counseling. Non-academic physicians had lower odds of counseling and referring their patients >50% of the time (aOR 0.23 [95%CI 0.06-0.81], aOR 0.23 [95%CI 0.06-0.83]). Almost all (92.1%) physicians counsel on residual SDB or OSA after adenotonsillectomy >75% of the time if the child is obese. CONCLUSIONS: Most pediatric otolaryngologists do not regularly counsel or refer patients to available obesity management programs, with differences seen between academic and non-academic surgeons. Confidence is low in currently available measures to reduce pediatric obesity. These results highlight the challenges of pediatric obesity and provide context for guideline implementation and additional resource development for pediatric otolaryngologists.

Sleep disordered breathing improvement prevents worsening of autonomic dysfunction in children with Down syndrome.

BACKGROUND: Resolution of sleep disordered breathing (SDB) in typically developing children normalises heart rate variability (HRV), a measure of autonomic control, to that of non-snoring controls. Children with Down Syndrome (DS) have dampened heart rate variability (HRV) but the effect of treatment is not known. To assess the effect of improvement of SDB on autonomic control we compared HRV in children with DS whose SDB improved over 2 y, to those whose SDB did not improve. METHODS: 24 children (3-19 y) had a baseline and follow-up polysomnographic study 2 y later. Improved SDB was defined as a reduction in obstructive apnea hypopnea index (OAHI) to ≤ 50% of baseline. Children were grouped into Improved (n = 12) and Unimproved (n = 12). Power spectral analysis of the ECG determined low frequency (LF), high frequency (HF) power and the LF/HF ratio. Seven children in the Improved and 2 in the Unimproved group were treated following the baseline study. RESULTS: In the Unimproved group at follow-up, LF power was lower compared to baseline during N3 and Total Sleep (p < 0.05 for both). HF power was lower during REM (p < 0.05). HRV remained unchanged between studies in the Improved group. CONCLUSION: Autonomic control worsened as indicated by lower LF and HF power in children whose SDB was not improved. In contrast, in those children with improved SDB, autonomic control remained the same, suggesting improvement in SDB severity prevents further worsening of autonomic control in children with DS.

Racial Disparity in Residual Sleep Apnea After Adenotonsillectomy.

OBJECTIVE: Black children have a higher risk of residual obstructive sleep apnea after adenotonsillectomy than non-Black children. We analyzed Childhood Adenotonsillectomy Trial data to better understand this disparity. We hypothesized that (1) child-level factors, such as asthma, smoke exposure, obesity, sleep duration, and (2) socioeconomic factors, such as maternal education, maternal health, and neighborhood disadvantage, may confound, modify, or mediate the association between Black race and residual obstructive sleep apnea after adenotonsillectomy. STUDY DESIGN: Secondary analysis of a randomized controlled trial. SETTING: Seven tertiary care centers. METHODS: We included two hundred and twenty-four 5-to-9-year-olds with mild-to-moderate obstructive sleep apnea who underwent adenotonsillectomy. The outcome was residual obstructive sleep apnea 6 months after surgery. Data were analyzed with logistic regression and mediation analysis. RESULTS: Of 224 included children, 54% were Black. Compared with non-Black children, Black children had 2.7 times greater odds of residual sleep apnea (95% confidence interval [CI]: 1.2, 6.1; p = .01), adjusted for age, sex, and baseline Apnea Hypopnea Index. There was significant effect modification by obesity. Among obese children, there was no association between Black race and outcome. However, nonobese Black children were 4.9 times as likely to have residual sleep apnea than non-Black children (95% CI: 1.2, 20.0; p < 0.01). There was no significant mediation by any of the child-level or socioeconomic factors tested. CONCLUSION: There was substantial effect modification by obesity on the association between Black race and residual sleep apnea after adenotonsillectomy for mild-to-moderate sleep apnea. Black race was associated with poorer outcome among nonobese but not obese children.

Patterns of Pharyngeal Obstruction and Collapse in Obese and Nonobese Children on Drug-Induced Sleep Endoscopy.

OBJECTIVE: (1) Compare proportions of collapse, obstruction, or mixed instances on drug-induced sleep endoscopy findings of obese and nonobese children with obstructive sleep disordered breathing. (2) Determine the frequency of collapse in general between both groups. STUDY DESIGN: Retrospective case-control study. SETTING: Tertiary pediatric center. METHODS: Obese (body mass index >95 percentile) children presenting with obstructive sleep disordered breathing (>33 on the pediatric sleep questionnaire) were identified from a prospectively kept surgical database. Only those who had undergone drug-induced sleep endoscopy were eligible. Age and sex pair-matched nonobese children were identified. Only nonsyndromic, neurologically normal, surgically naïve patients were included. The frequency of obstructive, collapse, and mixed pharyngeal patterns was documented in both groups. A comparison of proportions was then undertaken (χ2 test). RESULTS: Over a 5-year period, 73 consecutive children with obesity were identified (40 males; mean of 8.5 ± 3.0 years, 2.8-13.1). They were matched with 73 nonobese children (8.4 ± 3.0 years, 2.6-14.1). The obese group exhibited significantly more pharyngeal collapses (62:47) (p = .0021 odds ratio [OR] 3.358, 95% confidence interval [CI] 1.52-7.42). The proportion of pharyngeal findings on drug-induced sleep endoscopy was significantly different (p = .000129) between the 2 groups; obese (61 mixed: 3 obstruction: 9 collapse) and nonobese (48 mixed: 22 obstruction: 4 collapse). CONCLUSION: The predominance of hypopharyngeal collapse in children with obesity may explain the likelihood of failure of surgery directed at obstructive findings. This may also strengthen the case for drug-induced sleep endoscopy in this group at the initial surgery to guide it rather than after the failure of adenotonsillectomy.

Tonsil volume and outcome of radiofrequency uvulopalatoplasty with or without tonsillectomy in adults with sleep-disordered breathing.

PURPOSE: Predictors for the outcome of uvulopalatopharyngoplasty with and without tonsillectomy (UPPP ± TE) in sleep-disordered breathing have not been fully established. This study investigates tonsil grade, volume, and preoperative examination in predicting radiofrequency UPP ± TE outcomes. METHODS: All patients undergoing radiofrequency UPP with tonsillectomy if tonsils were present between 2015 and 2021 were retrospectively analyzed. Patients underwent a standardized clinical examination, including Brodsky palatine tonsil grade from 0 to 4. Preoperatively and 3 months after surgery, sleep apnea testing was performed using respiratory polygraphy. Questionnaires were administered assessing daytime sleepiness using the Epworth Sleepiness Scale (ESS) and snoring intensity on a visual analog scale. Tonsil volume was measured intraoperatively using water displacement. RESULTS: The baseline characteristics of 307 patients and the follow-up data of 228 patients were analyzed. Tonsil volume increased by 2.5 ml (95% CI 2.1-2.9 ml; P < 0.001) per tonsil grade. Higher tonsil volumes were measured in men, younger patients, and patients with higher body mass indices. The preoperative apnea-hypopnea index (AHI) and AHI reduction strongly correlated with tonsil volume and grade, whereas postoperative AHI did not. The responder rate increased from 14% to 83% from tonsil grade 0 to 4 (P < 0.01). ESS and snoring were significantly reduced after surgery (P < 0.01), but the reduction was not influenced by tonsil grade or volume. No other preoperative factor other than tonsil size could predict surgical outcomes. CONCLUSIONS: Tonsil grade and intraoperatively measured volume correlate well and predict the reduction of AHI, while they are not predictive of ESS and snoring response after radiofrequency UPP ± TE.