Identifying Posterior Fossa Abnormality in Pediatric Aerodigestive Patients, a Case Series.

OBJECTIVES: Multidisciplinary clinics like Aerodigestive programs focus on issues associated with airway, pulmonary, and gastrointestinal issues. Rarely, significant neurological issues like posterior fossa abnormality are identified as the primary etiology. We describe 3 such patients and compare their clinical presentation to the other patients seen in Aerodigestive clinic. METHODS: A retrospective chart review was conducted to review the 3 posterior fossa patients and the remainder of children that were referred to the Aerodigestive Clinic at Children's Hospital Los Angeles from June 2016 to August 2018. Clinical characteristics including triple endoscopies and sleep studies were recorded. RESULTS: Of the 110 patients included for review, 3 patients (3%) had an underlying posterior fossa abnormality; all of whom had symptoms of sleep disordered breathing along with dysphagia compared with 30% incidence of this symptom profile in the remaining Aerodigestive population. CONCLUSION: Presence of sleep disordered breathing and dysphagia, with underlying vomiting history, warrants considering evaluation for posterior fossa abnormalities in addition to traditional workup for aerodigestive disorders. Due to the rarity of this presentation and small sample size, future studies with multicenter collaboration may help better describe identifiers to delineate this population with similar aerodigestive symptoms and clarify diagnostic algorithms.

A System Analysis of Delay in Outpatient Respiratory Equipment Delivery.

OBJECTIVES: To systematically assess barriers delaying home respiratory equipment requisition and to evaluate for temporal correlation between delays and emergency room or hospitalization episodes. BACKGROUND: Initiation of home respiratory treatments is delayed because of delays in delivery of durable medical equipment (DME). This study assesses root causes of such delays from a system perspective. We also describe clinical consequences by measuring emergency room visits and hospitalization days for temporal correlations. METHODS: We conducted a retrospective review of DME ordering records from April 2011 to March of 2012. SETTINGS: Outpatient DME records in Pediatric Pulmonary Division. RESULTS: Of 164 available orders studied, deliveries were made as followed: 31 (19%) within 24 hr: 18 (59%) oxygen orders and 10 (32%) nebulizer orders 50 (30%) within 1 week: 25 (50%) nebulizer orders and 10 (20%) oxygen orders Delays: 45 (27%) delivered > 1 month: Bilevel positive airway pressure (BPAP) = 16 (36%) Oxygen = 12 (26%) Cough assist device = 7 (16%) Nebulizer = 5 (11%) Miscellaneous devices = 5 (11%) Analysis of barriers includes (a) type of insurance, (b) human error, (c) communication barrier, (d) deficit in training or knowledge, (e) no clear policy, (f) differences in clinical policy/ standard, (g) no DME benefit, (h) no clinical justification, and (i) error in communication/record keeping. Six patients with 7 emergency department (ED) visits and 4 inpatient admissions, totaling 24 hospital days, were temporally associated with delays in delivery of equipment over 30 days. CONCLUSION: One half of commonly used DMEs were delivered within the first week. One quarter of more expensive required more steps for approval. Twenty-nine ED/hospital days with respiratory morbidities were temporally associated with delays.

Freeman-Sheldon syndrome and respiratory obstruction: a novel use of distraction osteogenesis.

Freeman-Sheldon syndrome, or distal arthrogryposis type IIA (DA 2A), is a rare and severe multiple congenital contracture syndrome that is associated with upper airway obstruction. This obstruction has been clinically significant enough to warrant tracheostomy and has been associated with mortality. We describe a patient who presented to us as a neonate and the novel management of her respiratory obstruction in the setting of DA 2A. Bilateral mandibular osteotomies were performed and bilateral internal mandibular distracters were placed. She was distracted a total of 3 cm over 15 days without event and successfully extubated on the postoperative day 16. Preoperative polysomnogram demonstrated an obstructive apnea hypopnea index of 43.7, but a repeat polysomnogram demonstrated an apnea hypopnea index of 8.1. In this study, we report the first use of distraction osteogenesis in the setting of severe obstructive sleep apnea syndrome secondary to DA 2A.

Diagnosis and management of childhood obstructive sleep apnea syndrome.

OBJECTIVES: This revised clinical practice guideline, intended for use by primary care clinicians, provides recommendations for the diagnosis and management of the obstructive sleep apnea syndrome (OSAS) in children and adolescents. This practice guideline focuses on uncomplicated childhood OSAS, that is, OSAS associated with adenotonsillar hypertrophy and/or obesity in an otherwise healthy child who is being treated in the primary care setting. METHODS: Of 3166 articles from 1999-2010, 350 provided relevant data. Most articles were level II-IV. The resulting evidence report was used to formulate recommendations. RESULTS AND CONCLUSIONS: The following recommendations are made. (1) All children/adolescents should be screened for snoring. (2) Polysomnography should be performed in children/adolescents with snoring and symptoms/signs of OSAS; if polysomnography is not available, then alternative diagnostic tests or referral to a specialist for more extensive evaluation may be considered. (3) Adenotonsillectomy is recommended as the first-line treatment of patients with adenotonsillar hypertrophy. (4) High-risk patients should be monitored as inpatients postoperatively. (5) Patients should be reevaluated postoperatively to determine whether further treatment is required. Objective testing should be performed in patients who are high risk or have persistent symptoms/signs of OSAS after therapy. (6) Continuous positive airway pressure is recommended as treatment if adenotonsillectomy is not performed or if OSAS persists postoperatively. (7) Weight loss is recommended in addition to other therapy in patients who are overweight or obese. (8) Intranasal corticosteroids are an option for children with mild OSAS in whom adenotonsillectomy is contraindicated or for mild postoperative OSAS.

Diagnosis and management of childhood obstructive sleep apnea syndrome.

OBJECTIVE: This technical report describes the procedures involved in developing recommendations on the management of childhood obstructive sleep apnea syndrome (OSAS). METHODS: The literature from 1999 through 2011 was evaluated. RESULTS AND CONCLUSIONS: A total of 3166 titles were reviewed, of which 350 provided relevant data. Most articles were level II through IV. The prevalence of OSAS ranged from 0% to 5.7%, with obesity being an independent risk factor. OSAS was associated with cardiovascular, growth, and neurobehavioral abnormalities and possibly inflammation. Most diagnostic screening tests had low sensitivity and specificity. Treatment of OSAS resulted in improvements in behavior and attention and likely improvement in cognitive abilities. Primary treatment is adenotonsillectomy (AT). Data were insufficient to recommend specific surgical techniques; however, children undergoing partial tonsillectomy should be monitored for possible recurrence of OSAS. Although OSAS improved postoperatively, the proportion of patients who had residual OSAS ranged from 13% to 29% in low-risk populations to 73% when obese children were included and stricter polysomnographic criteria were used. Nevertheless, OSAS may improve after AT even in obese children, thus supporting surgery as a reasonable initial treatment. A significant number of obese patients required intubation or continuous positive airway pressure (CPAP) postoperatively, which reinforces the need for inpatient observation. CPAP was effective in the treatment of OSAS, but adherence is a major barrier. For this reason, CPAP is not recommended as first-line therapy for OSAS when AT is an option. Intranasal steroids may ameliorate mild OSAS, but follow-up is needed. Data were insufficient to recommend rapid maxillary expansion.

Age specific differences in pediatric obstructive sleep apnea.

BACKGROUND: Some have suggested that younger children have a more severe form of obstructive sleep apnea than older children and therefore are at a higher risk for respiratory compromise after tonsillectomy and adenoidectomy. However, at present there are few studies that have identified any significant correlation between age and severity of obstructive sleep apnea. OBJECTIVE: To determine if age specific differences in obstructive sleep apnea are present in children. DESIGN: Retrospective chart review. SETTING: Tertiary care children's hospital. PATIENTS: The records of children (1-18 years of age) with obstructive sleep apnea diagnosed by overnight polysomnography between January 1998 and January 2001 were reviewed. Children included in the study also had evidence of adenotonsillar hypertrophy and had no other co-existing medical problems. MAIN OUTCOME MEASURES: Overnight polysomnography was performed in all children. Apnea-hypopnea index (AHI), baseline and lowest O(2) saturation, baseline and peak end tidal CO(2), and total number of obstructive apneas, hypopneas, central apneas and mixed apneas were measured during each polysomnogram. Children were subdivided into the following age groups: 1-2, 3-5, 6-11 and 12-18 years. Polysomnograms were classified into normal, mild, moderate and severe categories. RESULTS: Three hundred and sixty-three children were studied; 45 children were ages 1-2 years, 159 children were ages 3-5 years, 137 children were 6-11 years and 22 children were 12-18 years. Although there appears to be a trend towards a greater mean number of obstructive apneas, hypopneas, central apneas, mixed apneas, a higher mean AHI, lower mean SaO(2) nadir, and a higher mean PETCO(2) in the younger age groups when compared to the older groups, a Student's t-test demonstrates that there is no statistical significance for most OSA parameters. An analysis of variance using the F-test reveals statistical significance (p<0.01) when children ages 1-2 were compared to those 3-5, 6-11 or 12-18 years of age for the variables AHI, mean number of central apneas, hypopneas and mixed apneas. When comparing patients in the various severity categories, children ages 1-2 years show a distinct distribution with a larger percentage in the moderate to severe categories. Chi square analysis reveals a significant difference between the frequency distribution of children in age group 1-2 years and that of the other age groups (p<0.01). CONCLUSION: There is a predilection for children less than 3 years of age to have more severe obstructive sleep apnea as documented by polysomnography. Central apnea also appears to be more common in this age group. These findings may be explained by anatomic and physiologic differences related to age and support a period of observation following adenotonsillectomy in younger children.

Antimicrobial therapy for children with adenotonsillar hypertrophy and obstructive sleep apnea: a prospective randomized trial comparing azithromycin vs placebo.

OBJECTIVE: Multiple clinical trials demonstrate the value of administering antibiotics for recurrent tonsillitis. However, there is no consensus as to the role of antibiotics in the management of adenotonsillar hypertrophy and obstructive sleep apnea (OSA). It has been suggested that antibiotics may reduce adenotonsillar size, improve obstructive symptoms, and obviate the need for surgery. The goal of this pilot study was to evaluate the efficacy of a broad-spectrum antibiotic in the management of adenotonsillar hypertrophy and OSA. METHODS: Twenty-two children ages 2 through 12, with evidence of OSA, were enrolled in this double-blinded, prospective clinical trial. Children were randomly assigned into 1 of 2 groups. Group I received azithromycin for a 30-day period (12 mg/kg on days 1-5; the regimen was repeated on days 11-15 and days 21-25). Group II received a placebo administered in the same regimen for a 30-day period. Overnight polysomnography was performed before and within 2 weeks after drug administration. RESULTS: Polysomnographic indices of OSA severity appear to indicate an improvement in the antibiotic as compared to the placebo group, but a comparison of mean +/- standard error for each parameter failed to demonstrate statistical significance using the paired t test. Only 1 of 11 (9%) children had normalization of their polysomnographic parameters. CONCLUSIONS: These results suggest that a course of a broad-spectrum antibiotic may be effective in temporarily improving OSA due to adenotonsillar hypertrophy, but does not appear to obviate the need for surgery.

Infant polysomnography: reliability and validity of infant arousal assessment.

Infant arousal scoring based on the Atlas Task Force definition of transient EEG arousal was evaluated to determine (1). whether transient arousals can be identified and assessed reliably in infants and (2). whether arousal and no-arousal epochs scored previously by trained raters can be validated reliably by independent sleep experts. Phase I for inter- and intrarater reliability scoring was based on two datasets of sleep epochs selected randomly from nocturnal polysomnograms of healthy full-term, preterm, idiopathic apparent life-threatening event cases, and siblings of Sudden Infant Death Syndrome infants of 35 to 64 weeks postconceptional age. After training, test set 1 reliability was assessed and discrepancies identified. After retraining, test set 2 was scored by the same raters to determine interrater reliability. Later, three raters from the trained group rescored test set 2 to assess inter- and intrarater reliabilities. Interrater and intrarater reliability kappa's, with 95% confidence intervals, ranged from substantial to almost perfect levels of agreement. Interrater reliabilities for spontaneous arousals were initially moderate and then substantial. During the validation phase, 315 previously scored epochs were presented to four sleep experts to rate as containing arousal or no-arousal events. Interrater expert agreements were diverse and considered as noninterpretable. Concordance in sleep experts' agreements, based on identification of the previously sampled arousal and no-arousal epochs, was used as a secondary evaluative technique. Results showed agreement by two or more experts on 86% of the Collaborative Home Infant Monitoring Evaluation Study arousal scored events. Conversely, only 1% of the Collaborative Home Infant Monitoring Evaluation Study-scored no-arousal epochs were rated as an arousal. In summary, this study presents an empirically tested model with procedures and criteria for attaining improved reliability in transient EEG arousal assessments in infants using the modified Atlas Task Force standards. With training based on specific criteria, substantial inter- and intrarater agreement in identifying infant arousals was demonstrated. Corroborative validation results were too disparate for meaningful interpretation. Alternate evaluation based on concordance agreements supports reliance on infant EEG criteria for assessment. Results mandate additional confirmatory validation studies with specific training on infant EEG arousal assessment criteria.