Sivakumar Chinnadurai

Beyond adenotonsillectomy: Outcomes of sleep endoscopy-directed treatments in pediatric obstructive sleep apnea

OBJECTIVES In this study we determine the subjective and objective outcomes of pediatric patients with refractory OSA undergoing drug-induced sleep endoscopy (DISE)-directed surgical treatment. METHODS 31 consecutive children with OSA following TA underwent DISE. 26 completed DISE-directed operative management of the level(s) of ongoing upper airway obstruction. Pre- and postoperative OSA were assessed through a detailed history (of nighttime symptoms (NS) and daytime symptoms (DS)), physical examination, and polysomnography. RESULTS Age ranged 5-18 years (mean 9.7 ± 3.4). Fourteen of 26 had trisomy 21 (51%). Operations were performed in the following frequencies: lingual tonsillectomy (LT) (22), midline posterior glossectomy (MPG) (16), revision adenoidectomy (11), inferior turbinate submucosal resection (7), uvulopalatoplasty (2), and supraglottoplasty (2). Overall, 92% reported subjective improvement. NS improved from 5.8 ± 2.9 preoperatively to 2.1 ± 2.5 postoperatively (p<0.05), while DS improved from 2.1 ± 1.3 preoperatively to 0.6 ± 1.1 postoperatively (p<0.05). Seventeen patients completed preoperative polysomnography, while only 11 of them also completed postoperative polysomnography. Mean OAHI fell from 7.0 (±5.8) events/hr to 3.6 (±1.8) events/hr (t-test, p=0.09). CONCLUSIONS Individualized, multilevel, DISE-directed operative therapy was associated with substantial improvement in subjective measures of sleep.

Pharmacologic Interventions for Infantile Hemangioma: A Meta-analysis.

CONTEXT: Infantile hemangiomas (IH) may be associated with significant functional impact. OBJECTIVE: The objective of this study was to meta-analyze studies of pharmacologic interventions for children with IH. DATA SOURCES: Data sources were Medline and other databases from 1982 to June 2015. STUDY SELECTION: Two reviewers assessed studies using predetermined inclusion criteria. DATA EXTRACTION: One reviewer extracted data with review by a second. RESULTS: We included 18 studies in a network meta-analysis assessing relative expected rates of IH clearance associated with β-blockers and steroids. Oral propranolol had the largest mean estimate of expected clearance (95%; 95% Bayesian credible interval [BCI]: 88%–99%) relative to oral corticosteroids (43%, 95% BCI: 21%–66%) and control (6%, 95% BCI: 1%–11%). Strength of evidence (SOE) was high for propranolol’s effects on reducing lesion size compared with observation/placebo. Corticosteroids demonstrated moderate effectiveness at reducing size/volume (moderate SOE for improvement in IH). SOE was low for effects of topical timolol versus placebo. LIMITATIONS: Methodologic limitations of available evidence may compromise SOE. Validity of meta-analytic estimates relies on the assumption of exchangeability among studies, conditional on effects of the intervention. Results rely on assumed lack of reporting bias. CONCLUSIONS: Propranolol is effective at reducing IH size compared with placebo, observation, and other treatments including steroids in most studies. Corticosteroids demonstrate moderate effectiveness at reducing IH size/volume. The meta-analysis estimates provide a relative ranking of anticipated rates of lesion clearance among treatments. Families and clinicians making treatment decisions should also factor in elements such as lesion size, location, number, and type, and patient and family preferences.

Laser treatment of infantile hemangioma: A systematic review.

To systematically review studies of laser treatment of infantile hemangioma (IH).

Prevalence of hearing loss in children with 22q11.2 deletion syndrome

OBJECTIVE The purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS). METHODS Fifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicines database with a focus on hearing loss and 22q11DS was performed. RESULTS 22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears. CONCLUSION Hearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population.

Treatment of Ankyloglossia for Reasons Other Than Breastfeeding: A Systematic Review

BACKGROUND AND OBJECTIVE: Children with ankyloglossia, an abnormally short, thickened, or tight lingual frenulum, may have restricted tongue mobility and sequelae, such as speech and feeding difficulties and social concerns. We systematically reviewed literature on feeding, speech, and social outcomes of treatments for infants and children with ankyloglossia. METHODS: Medline, PsycINFO, Cumulative Index of Nursing and Allied Health Literature, and Embase were searched. Two reviewers independently assessed studies against predetermined inclusion/exclusion criteria. Two investigators independently extracted data on study populations, interventions, and outcomes and assessed study quality. RESULTS: Two randomized controlled trials, 2 cohort studies, and 11 case series assessed the effects of frenotomy on feeding, speech, and social outcomes. Bottle feeding and social concerns, such as ability to use the tongue to eat ice cream and clean the mouth, improved more in treatment groups in comparative studies. Supplementary bottle feedings decreased over time in case series. Two cohort studies reported improvement in articulation and intelligibility with treatment. Other benefits were unclear. One randomized controlled trial reported improved articulation after Z-frenuloplasty compared with horizontal-to-vertical frenuloplasty. Numerous noncomparative studies reported speech benefits posttreatment; however, studies primarily discussed modalities, with outcomes including safety or feasibility, rather than speech. We included English-language studies, and few studies addressed longer-term speech, social, or feeding outcomes; nonsurgical approaches, such as complementary and alternative medicine; and outcomes beyond infancy, when speech or social concerns may arise. CONCLUSIONS: Data are currently insufficient for assessing the effects of frenotomy on nonbreastfeeding outcomes that may be associated with ankyloglossia.

Obstructive Sleep Apnea Syndrome in Children with 22q11.2 Deletion Syndrome after Operative Intervention for Velopharyngeal Insufficiency

Introduction: Surgical treatment of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome is often warranted. In this patient population, VPI is characterized by poor palatal elevation and muscular hypotonia with an intact palate. We hypothesize that 22q11.2 deletion patients are at greater risk of obstructive sleep apnea (OSA) after surgical correction of VPI, due, in part, to their functional hypotonia, large velopharyngeal gap size, and the need to surgically obstruct the velopharynx. Methods: We performed a retrospective analysis of patients with 22q11.2 deletion syndrome treated at a tertiary pediatric hospital between the years of 2002 and 2012. The incidence of VPI, need for surgery, post-operative polysomnogram, post-operative VPI assessment, and OSA treatments were evaluated. Results: Forty-three patients (18 males, 25 females, ages 1–14 years) fitting the inclusion criteria were identified. Twenty-eight patients were evaluated by speech pathology due to hypernasality. Twenty-one patients had insufficient velopharyngeal function and required surgery. Fifteen underwent pharyngeal flap surgery, three underwent sphincter pharyngoplasty, two underwent Furlow palatoplasty, and one underwent combined sphincter pharyngoplasty with Furlow palatoplasty. Of these, eight had post-operative snoring. Six of these underwent polysomnography (five underwent pharyngeal flap surgeries and one underwent sphincter pharyngoplasty). Four patients were found to have OSA based on the results of the polysomnography (average apnea/hypopnea index of 4.9 events/h, median = 5.1, SD = 2.1). Two required continuous positive airway pressure (CPAP) due to moderate OSA. Conclusion: Surgery is often necessary to correct VPI in patients with 22q11.2 deletion syndrome. Monitoring for OSA should be considered after surgical correction of VPI due to a high occurrence in this population. Furthermore, families should be counseled of the risk of OSA after surgery and the potential need for treatment with CPAP.

Tonsillectomy for Obstructive Sleep-Disordered Breathing: A Meta-analysis.

CONTEXT: The effectiveness of tonsillectomy or adenotonsillectomy (hereafter, “tonsillectomy”) for obstructive sleep-disordered breathing (OSDB) compared with watchful waiting with supportive care is poorly understood. OBJECTIVE: To compare sleep, cognitive or behavioral, and health outcomes of tonsillectomy versus watchful waiting with supportive care in children with OSDB. DATA SOURCES: Medline, Embase, and the Cochrane Library. STUDY SELECTION: Two investigators independently screened studies against predetermined criteria. DATA EXTRACTION: Two investigators independently extracted key data. Investigators independently assessed study risk of bias and the strength of the evidence of the body of literature. Investigators synthesized data qualitatively and meta-analyzed apnea–hypopnea index (AHI) scores. RESULTS: We included 11 studies. Relative to watchful waiting, most studies reported better sleep-related outcomes in children who had a tonsillectomy. In 5 studies including children with polysomnography-confirmed OSDB, AHI scores improved more in children receiving tonsillectomy versus surgery. A meta-analysis of 3 studies showed a 4.8-point improvement in the AHI in children who underwent tonsillectomy compared with no surgery. Sleep-related quality of life and negative behaviors (eg, anxiety and emotional lability) also improved more among children who had a tonsillectomy. Changes in executive function were not significantly different. The length of follow-up in studies was generally <12 months. LIMITATIONS: Few studies fully categorized populations in terms of severity of OSDB; outcome measures were heterogeneous; and the durability of outcomes beyond 12 months is not known. CONCLUSIONS: Tonsillectomy can produce short-term improvement in sleep outcomes compared with no surgery in children with OSDB. Understanding of longer-term outcomes or effects in subpopulations is lacking.

Comparative Effectiveness of Partial versus Total Tonsillectomy in Children: A Systematic Review:

Objective To assess the effectiveness of partial versus total tonsillectomy in children. Data Sources MEDLINE, EMBASE, and Cochrane Library from January 1980 to June 2016. Review Methods Two investigators independently screened studies and extracted data. Investigators independently assessed risk of bias and strength of evidence of the literature. Heterogeneity precluded quantitative analysis. Results In 16 eligible randomized controlled trials (RCTs), definitions of “partial” tonsillectomy varied. In addition to comparing partial with total tonsil removal, 11 studies compared surgical techniques (eg, coblation). In studies comparing the same technique, return to normal diet or activity was faster with partial removal (more favorable outcomes in 4 of 4 RCTs). In studies with differing surgical techniques, return to normal diet and activity was faster with partial versus total tonsillectomy (more favorable outcomes in 5 of 6 studies). In 3 of 4 RCTs, partial tonsillectomy was associated with more throat infections than total tonsillectomy. Differences between groups were generally not statistically significant for obstructive symptom persistence, quality of life, or behavioral outcomes. Across all studies, 10 (6%) of roughly 166 children had tonsillar regrowth after partial tonsillectomy. Conclusions Data do not allow firm conclusions regarding the comparative benefit of partial versus total removal; however, neither surgical technique nor extent of surgery appears to affect outcomes markedly. Partial tonsillectomy conferred moderate advantages in return to normal diet/activity but was also associated with tonsillar regrowth and symptom recurrence. Effects may be due to confounding given differences in populations and surgical approaches/techniques. Heterogeneity and differences in the operationalization of “partial” tonsillectomy limited comparative analyses.

Postoperative Bleeding and Associated Utilization following Tonsillectomy in Children: A Systematic Review and Meta-analysis

Objective To assess posttonsillectomy hemorrhage (PTH), associated nonoperative readmissions/revisits, and reoperations in children. Data Sources MEDLINE, EMBASE, and the Cochrane Library. Review Methods Two investigators independently screened studies against predetermined criteria and extracted key data. Investigators independently assessed study risk of bias and the strength of the evidence of the body of literature. We calculated unadjusted pooled estimates of PTH frequency and conducted a Bayesian meta-analysis to estimate frequency of primary and secondary PTH and PTH-associated reoperation and revisits/readmissions by partial and total tonsillectomy and surgical approach. Results In meta-analysis, the frequency of primary and secondary PTH associated with total and partial tonsillectomy was <4% for any technique and with overlapping confidence bounds. Pooled frequencies of PTH were also <5% overall (4.2% for total tonsillectomy, 1.5% for partial tonsillectomy) in comparative studies. Fewer PTH episodes occurred with tonsillectomy for obstructive sleep-disordered breathing than for throat infection. In meta-analysis, frequency of PTH-associated nonoperative revisits/readmission or reoperation ranged from 0.2% to 5.7% for total tonsillectomy and from 0.1% to 3.7% for partial tonsillectomy. At least 4 deaths were reported in case series including 1,778,342 children. Conclusions PTH occurred in roughly 4% of tonsillectomies in studies included in this review. Although studies typically did not report bleeding severity or amount, relatively few episodes of PTH necessitated reoperation for hemostasis. Nonetheless, tonsillectomy is not without risk of harm. Frequency of PTH across techniques was similar; thus, we cannot conclude that a given technique is superior.

Velopharyngeal dysfunction in children with Prader-Willi syndrome after adenotonsillectomy

INTRODUCTION Prader-Willi syndrome (PWS) is a rare genetic disorder with an incidence rate of 1 in 10,000-30,000. Patients with PWS typically have symptoms related to hypotonia, obesity, and hypothalamic dysfunction. A high rate of obstructive sleep apnea (OSA) is found among this population of patients. Adenotonsillectomy has been advocated as a first line approach for treatment of OSA in patients with PWS. Velopharyngeal dysfunction (VPD) is a known complication of adenotonsillectomy. VPD can also be present in patients with global hypotonia, such as those with PWS. The objective of this study is to review the occurrence of VPD in patients with PWS after adenotonsillectomy for OSA. METHODS A retrospective review was performed of all patients with PWS and OSA from a tertiary pediatric hospital between the years of 2002 and 2012. Pre- and post-operative sleep studies and sleep disordered breathing symptoms, post-operative VPD assessment by the speech-language pathologist (SLP), and VPD treatments were evaluated. RESULTS Eleven patients (five males and six females), fitting the inclusion criteria, were identified. The age of the patient at the initial otolaryngologic evaluation ranged from 2 to 9 years. All patients underwent adenotonsillectomy for sleep disordered breathing. Four patients were diagnosed with post-operative hypernasality after assessment by a speech-language pathologist. The hypernasality ranged from mild to moderately severe. Of the four patients with hypernasality, two were found to have structural issues requiring surgery (pharyngeal flap). Both of the surgical patients experienced significant improvement in their VPD after surgery. The remaining two patients were found to have articulation error patterns that were considered more developmental in nature and both responded to speech therapy. All patients, except one, had improvement in their polysomnogram or sleep symptoms after adenotonsillectomy. However, three patients continue to require continuous positive airway pressure at night. CONCLUSION Velopharyngeal dysfunction may occur after adenotonsillectomy in patients with Prader-Willi Syndrome. Families should be counseled of this risk and the potential need for operative intervention to correct it.