PHACES syndrome: otolaryngic considerations in recognition and management.

OBJECTIVES: To describe the otolaryngic manifestations of PHACES and evaluate current diagnostic and management principles for these patients. METHODS: A retrospective review was performed within a tertiary children's hospital. Children with segmental facial hemangiomas of infancy and one extracutaneous manifestation comprising PHACES (posterior fossa malformation, arteriovenous malformations, cardiac/aortic defects, eye anomalies, and sternal defect) were identified. Otolaryngic problems were evaluated with physical examination, audiogram, swallow evaluation, polysomnography, and laryngoscopy. Extracutaneous manifestations were diagnosed using radiology, echocardiogram, and EEG. Treatment for cutaneous and airway hemangiomas included oral and intralesional steroids, CO(2) or pulse-dye laser, tracheotomy, and surgical excision. Management of extracutaneous problems was system-dependent. RESULTS: Of 246 children with segmental facial hemangiomas of infancy evaluated since January 2000, 5 girls (2.0%) met diagnostic criteria for PHACES. Mean age at last follow-up was 2.6 years (range 0.4-5.8). Each child had one extracutaneous manifestation of aortic anomaly (2/5), sternal clefting (2/5), and brain malformation (1/5). Otolaryngic abnormalities included middle ear atelectasis (1/5), tympanic membrane hemangiomas with conductive hearing loss (3/5), skin and cartilage ulceration (2/5), dysphagia (4/5), and airway hemangiomas with stridor (3/5). Three children received oral steroids and required pulse-dye laser for cutaneous hemangiomas of infancy. One child underwent tracheotomy. Additional interventions included tympanostomy tubes and resection of nasal hemangioma. CONCLUSIONS: Diagnosis of PHACES requires awareness of the association of facial hemangiomas of infancy with systemic and airway problems. Otolaryngology-related manifestations of PHACES are not commonly described, and management should be tailored to the individual patient.

Velopharyngeal insufficiency: current concepts in diagnosis and management.

PURPOSE OF REVIEW: To discuss principles of evaluation and treatment of children with velopharyngeal insufficiency, and to review recent reports of surgical outcomes. RECENT FINDINGS: Velopharyngeal insufficiency significantly impacts both quality-of-life and speech intelligibility in children. Diagnosis is made through history and physical, perceptual speech assessment, and instrumental measures including nasendoscopy and radiographic multiplanar videofluoroscopy. Treatment options for velopharyngeal insufficiency consist of prosthetic management or surgery, supplemented with speech therapy when appropriate. Surgical interventions are palatal, palatopharyngeal or pharyngeal in nature. Despite some controversy, most recent reports identify no significant difference in outcomes following pharyngeal flap or sphincter pharyngoplasty. Complications of surgical therapy relate to postoperative obstructive breathing or persistent velopharyngeal insufficiency. CONCLUSION: Although there is no universally accepted measure to assess velopharyngeal insufficiency severity, nasendoscopy and multiplanar videofluoroscopy are most commonly used for clinical diagnosis. A speech pathologist is an integral member of the velopharyngeal insufficiency team, and momentum toward a standardized reporting system of perceptual speech measurements is increasing. Treatment of velopharyngeal insufficiency should be tailored to the specific needs of the child and family. Surgical therapy may improve velopharyngeal function but may negatively impact upper airway patency and respiration during sleep. The otolaryngologist should be familiar with strengths and limitations of different surgical options for velopharyngeal insufficiency.

Pediatric obstructive sleep apnea and quality of life: a meta-analysis.

OBJECTIVE: 1) To assess the quality of life (QOL) in children with obstructive sleep apnea (OSA) compared with QOL of children with chronic medical conditions, and 2) To determine QOL in children with OSA after adenotonsillectomy in short- and long-term follow-up. DATA SOURCES/REVIEW METHODS: A literature review on QOL in pediatric OSA using the PubMed database. RESULTS: The literature search yielded 10 articles that satisfied inclusion and exclusion criteria. In three studies, the Child Health Questionnaire (CHQ) survey was used to compare 193 patients who had OSA with 93 children who had juvenile rheumatoid arthritis (JRA) and with 815 healthy children. Of 12 CHQ subscale scores for children with OSA, 8 scores were significantly lower (indicating a poorer QOL) than controls. Children with OSA scored 19.23 points lower than healthy children in the subscale of parental impact-emotional. Children with OSA had QOL scores that were similar to those of children with JRA. In seven publications, 369 children with OSA undergoing adenotonsillectomy were studied by using the OSA-18 QOL instrument. The total OSA-18 score and each of the domain scores showed significant improvement (P < 0.0001) after adenotonsillectomy. At long-term follow-up, QOL scores remained significantly improved. CONCLUSIONS: Pediatric OSA has a significant impact on QOL. QOL in pediatric OSA is similar to that of children with JRA. Large improvements in QOL occur after adenotonsillectomy, and these findings are maintained in the long-term. The literature lacks control studies on QOL in pediatric OSA.

Long-term improvements in quality-of-life after surgical therapy for pediatric sinonasal disease.

OBJECTIVE: To study long-term changes in quality-of-life (QOL) in children after surgery for chronic sinonasal disease. DESIGN: Prospective, nonrandomized. METHODS: Caregivers completed the SN-5 QOL instrument before surgery. Children underwent either adenoidectomy or endoscopic sinus surgery (ESS). Caregivers then completed a survey <6 months (short-term) and between six and 24 months after surgery (long-term). Scores were compared using a paired t test. RESULTS: Twenty-two children were enrolled (mean age, 5.7 years). The majority (59%) of children underwent ESS. The mean preoperative total score was 25.6. Postoperatively, the mean total score was 11.7 at short-term (P < 0.0001) and 11.5 at long-term follow-up (P < 0.0001). Short- and long-term postoperative scores for all domains were significantly different from preoperative scores. In the long-term scores, the domain with the greatest change was "emotional distress" (SRM 1.9) and with the smallest change was "allergy symptoms" (SRM 1.0). There were no significant differences between children who underwent adenoidectomy compared with ESS. CONCLUSIONS: After adenoidectomy or ESS, caregivers observe a long-term improvement in QOL of children with sinonasal disease.

Prevalence and ethnicity of sleep-disordered breathing and obesity in children.

OBJECTIVES: To compare the prevalence of obesity in children with sleep-disordered breathing (SDB) who undergo adenotonsillectomy to that in the general pediatric population. DESIGN: Cross-sectional controlled study. METHODS: A body-mass-index (BMI-for-age) percentile was determined for children with SDB and for matched controls from a general pediatric clinic. Children who were obese (>85th %) were analyzed. Groups were compared using a t test or chi(2) analysis. Risk factors were estimated with logistic regression. RESULTS: The study population included 299 children, of whom 170 (56.9%) had SDB. Compared with controls, more children with SDB were obese (46% vs 33%, P = 0.029) or underweight (8% vs 3%, P = 0.110), and fewer children with SDB were healthy weight (46% vs 64%, P = 0.002). Among African American children, those who were obese were more likely to have SDB (OR, 2.22, P < 0.01). CONCLUSIONS: Children with SDB who undergo adenotonsillectomy are more likely to be obese than children seen in a general pediatric clinic. African American children who are obese are more likely to have SDB.

Behavior and obstructive sleep apnea in children: is obesity a factor?

OBJECTIVES: Children with obstructive sleep apnea (OSA) frequently exhibit behavioral and neurocognitive problems. There is a high prevalence of OSA among obese children. This study aims to evaluate the relationship between OSA and behavioral problems in obese children as compared with normal-weight children (controls). STUDY DESIGN: Prospective, nonrandomized, controlled study of obese and normal-weight children with OSA presenting to a tertiary medical center for adenotonsillectomy. METHODS: All study participants underwent preoperative polysomnography to document OSA. Obesity was defined as age- and sex-adjusted body mass index at the 95th percentile or higher. Behavior was evaluated using the Behavior Assessment System for Children (BASC). Preoperatively, the Behavioral Symptoms Index (BSI), a global measure of behavior, and BASC scores for obese and normal-weight children were compared using an unpaired t test. RESULTS: The study population included 52 children, 18 (35%) of whom were obese. The mean age of obese children was 8.6 (range, 2.0-14.9) years. The mean age of normal-weight children was 6.4 (range, 2.1-12.9) years. Demographics were otherwise similar. The mean apnea-hypopnea index for obese children was 17.2 (5.0-38.0) and for normal-weight children was 15.7 (5.3-88.0). The BSI score was 55.3 (SD, 15.9) for obese and 55.9 (SD, 15.0) for normal-weight children. Seven (38.9%) obese and 12 (35.3%) normal-weight children had clinically significant or abnormal behavior. Similar results were seen for the BASC scales of atypicality, depression, hyperactivity, and somatization in both groups. CONCLUSIONS: Behavioral problems are highly prevalent in children with OSA. However, these problems exist independently of whether children are obese or normal weight.

Orbital sequelae of rhinosinusitis after cochlear implantation in children.

OBJECTIVES: The objectives of this retrospective case review were to describe orbital complications in children after cochlear implantation, to define rhinosinusitis as a possible preoperative risk factor, and to suggest a possible pathophysiological mechanism for this previously unreported occurrence. METHODS: Records of children undergoing cochlear implantation over a 7-year period at a tertiary academic medical center were reviewed. Four children who experienced postoperative orbital sequelae were identified. We describe the demographics, clinical course, and radiologic findings in these children. RESULTS: The records of 91 children who underwent cochlear implantation were reviewed. The mean age was 6.0 years (range, 0.9-16.9 years). Forty-nine children (54%) were female and 51 (56%) were white. Four children developed postoperative orbital complications on the ipsilateral side to implantation. Orbital complications were characterized by periorbital edema and preseptal cellulitis necessitating prolonged hospitalization in all four children (mean length of stay, 3.3 days). Each child's orbital complication resolved with medical therapy that included intravenous antibiotics and nasal saline. Temporal bone images before implantation showed evidence of rhinosinusitis in all four cases. Of 76 available preoperative scans from the unaffected children, only 11 (14%) studies showed evidence of rhinosinusitis. CONCLUSIONS: Children with preoperative radiologic evidence of rhinosinusitis may be at risk of orbital sequelae after cochlear implantation. Positioning of the patient during surgery, length of surgery, and minor trauma to the lamina papyracea during drilling of the mastoid may be important etiologic factors. A careful review of medical history and computed tomography imaging before implantation may identify at-risk children.

Improvements in quality of life in children after surgical therapy for sinonasal disease.

OBJECTIVES: To study changes in quality of life in children with chronic sinonasal disease after surgical therapy. STUDY DESIGN AND SETTING: Prospective, nonrandomized quality of life study of children with sinonasal disease who undergo adenoidectomy or functional endoscopic sinus surgery (FESS) at an academic institution. Caregivers completed a preoperative SN-5 quality of life survey and a second survey within 6 months after surgery. RESULTS: Twenty-two children were enrolled in the study (mean, 6.5 years; range, 1.4 to 15.9). The majority (59%) of children underwent adenoidectomy. The mean total score was 25.8 at initial presentation and 12.0 at follow-up (P>0.001). Postoperative scores for all domains were significantly different from preoperative scores. The domains with the greatest change in mean score were nasal obstruction and emotional distress. There were no significant differences in outcome between children who underwent adenoidectomy and those who underwent FESS. CONCLUSIONS: After either adenoidectomy or FESS, caregivers report a dramatic improvement in the quality of life of children with sinonasal disease. EBM RATING: B-3b.