Sally R. Shott

Down syndrome: Common otolaryngologic manifestations†

Otolaryngologic or ear, nose, and throat (ENT) problems are common in children with Down Syndrome (DS). This includes problems with chronic ear infections and chronic middle ear effusions with associated hearing loss, airway obstruction, and sleep apnea, as well as problems with chronic rhinitis and sinusitis. In addition, many of these ENT problems require surgical interventions, and there are special anesthesia considerations that need to be addressed in children with DS. These include subglottic stenosis, post‐operative airway obstruction, and cervical spine concerns. As the care of children with DS has become more consistent and proactive, outcomes from the treatment of these ENT manifestations have improved. Aggressive interventions, both medical and surgical, have led to a decreased incidence of hearing loss, good control of the chronic rhinitis, and a better awareness of the incidence of sleep apnea and sleep‐disordered breathing in individuals with DS. These common otolaryngologic manifestations of DS are reviewed with recommendations for ongoing care and monitoring.

Cine Magnetic Resonance Imaging: Evaluation of Persistent Airway Obstruction after Tonsil and Adenoidectomy in Children with Down Syndrome†

Objective/Hypothesis: Although usually successful, not all obstructive sleep apnea is cured by removal of the tonsils and adenoids (T&A). This is particularly true in children with Down syndrome and craniofacial anomalies. This is because of the multiple levels of obstruction in their airways, with obstruction present not only at the level of the tonsils and adenoids but also from base of tongue obstruction, soft palate collapse, and hypopharyngeal collapse. The cine magnetic resonance image (MRI) is useful in evaluating the upper airway in those patients who have not achieved a normal polysomnogram after T&A surgery.

Impalement injuries of the palate in children: review of 131 cases.

Impalement injuries of the soft palate and oropharynx are common injuries especially in children. Devastating neurologic sequelae secondary to thrombus propagation in the internal carotid artery are rare but well-documented complications of these innocuous appearing injuries. Neurologic sequelae have been reported anywhere from 3 to 60 hours after the injury. Protocols that include hospitalization up to 72 hours have been advocated. In order to evaluate the effectiveness and practicality of such protocols, all hospital admissions for palate injuries in the past 17 years were reviewed. Of the 131 cases reviewed, no complications were discovered during hospitalization, nor were there any complications in these patients seen in follow up. Based on this review, the majority of soft plate and oropharyngeal impalement injuries can be managed on an outpatient basis. Parental counseling, similar to instructions given in instances of mild head trauma, as well as close follow up in the office are felt to be a more reasonable and as well as a safe treatment plan. A suggested instruction form is presented.

Down Syndrome: Analysis of Airway Size and a Guide for Appropriate Intubation

Objectives/Hypothesis The purpose of this study is to prospectively evaluate the airway size of children with Down syndrome (DS). Previous studies have observed an increase in postintubation stridor in children with DS. Anesthetic literature suggests using a smaller endotracheal tube in children with DS, but more specific recommendations are not offered. With this study, recommendations are presented for the appropriate endotracheal tube size to use in children with DS undergoing intubation.

Airway Management in Pierre Robin Sequence

There is a great deal of controversy regarding the long-term management of airway obstruction in children with Pierre Robin sequence. All 23 pediatric otolaryngology fellowship programs were surveyed to determine their current practice patterns. Issues that were addressed included methods of evaluation of airway obstruction, including polysomnography, choices for home care in children in whom observation and positioning treatment failed, and complications of the various treatment modalities. The lack of unanimity among the respondents mirrors the controversy found in the literature. Though tracheotomy appears to be favored as the method of long-term management, responses from those surveyed often were based on emotions as much as on scientific data. This report details those findings and offers suggestions to clinicians involved with the management of patients with Pierre Robin sequence with airway compromise. (Otolaryngol Head Neck Surg 1998;118:630–5.)

Association of Anterior Glottic Webs with Velocardiofacial Syndrome (Chromosome 22q11.2 Deletion)

OBJECTIVE: An association between anterior glottic webs and velocardiofacial syndrome (chromosome 22q11.2 deletion) has previously been noted in a number of case reports. Our objective was to determine if the presence of such webs warrants a high index of suspicion for this chromosome deletion. STUDY DESIGN AND SETTING: This study was carried out in the Division of Pediatric Otolaryngology-Head and Neck Surgery at Cincinnati Childrens Hospital Medical Center. Chromosome 22q11.2 deletion status was determined for all patients endoscopically diagnosed with anterior glottic webs between July 1998 and December 2000. Families of patients who tested positive for the deletion were referred to the Cincinnati Childrens Division of Human Genetics for additional evaluation and counseling. RESULTS: Eleven of 17 patients (65%) with anterior glottic webs were positive for chromosome 22q11.2 deletion. Of these 11 patients, 5 showed subtle clinical manifestations of velocardiofacial syndrome and underwent genetic testing due only to the presence of a web. All 11 patients were diagnosed with velocardiofacial syndrome. CONCLUSION: We strongly recommend that all patients diagnosed with anterior glottic webs undergo fluorescence in situ hybridization evaluation for this chromosome deletion. (Otolaryngol Head Neck Surg 2004;130:415-7.)

Efficacy of tonsillectomy and adenoidectomy as an outpatient procedure: a preliminary report☆

One of the recent trends in medical practice has been the use of ambulatory surgery for a substantial number of surgical procedures. A prospective evaluation of tonsillectomy and tonsillectomy with adenoidectomy as outpatient procedures at the Childrens Hospital Medical Center in Cincinnati, OH, is reported. Patients who were felt to be inappropriate candidates for adenotonsillectomies as outpatients included patients under 3 years of age, patients who lived more than one hour from the hospital and patients who demonstrated obstructive sleep apnea or who had other significant medical problems. A review of this 9-month experience indicates that outpatient surgery for adenotonsillectomy is both safe and cost-effective in most circumstances. In an analysis of complications requiring readmission to the hospital (bleeding, dehydration, poor social situation), patients who had their original surgery performed on an ambulatory basis had no more complications than patients who were hospitalized following their surgery. Excluding the professional fees for the surgeon and anesthesiologist, a savings of approximately 50% can be expected from the performance of a tonsillectomy and adenoidectomy on an outpatient basis.

The Frequency of Lingual Tonsil Enlargement in Obese Children

OBJECTIVE Enlargement of the lingual tonsils is being increasingly recognized as a not uncommon and treatable cause of obstructive sleep apnea, particularly in patients with Down syndrome who have undergone palatine tonsillectomy and adenoidectomy. We have recognized an increasing number of patients who are obese and have obstructive sleep apnea with enlarged lingual tonsils. The purpose of this study was to evaluate the frequency of enlarged lingual tonsils in obese children. SUBJECTS AND METHODS Seventy-one obese children (mean body mass index = 41.6 kg/m(2)) underwent sagittal fast spin-echo inversion recovery imaging. Lingual tonsils were identified and measured in the greatest anteroposterior diameter. Lingual tonsils > 10 mm were considered markedly enlarged. The subgroup with absent palatine tonsils (previous tonsillectomy) (n = 41) were compared with those with palatine tonsils present (n = 30). RESULTS Forty-four (62%) of the obese children had measurable lingual tonsils, which is greater than the frequency previously reported in normal subjects (0%), subjects with obstructive sleep apnea (33%), or subjects with Down syndrome and obstructive sleep apnea (50%). Ten (14%) had lingual tonsils > 10 mm. Obese subjects with absent palatine tonsils (previous tonsillectomy) had a higher prevalence of measurable lingual tonsils than those with palatine tonsils (78% vs 22%, respectively; p < 0.001) and a higher prevalence of lingual tonsils > 10 mm (90% vs 10%, p < 0.001). CONCLUSION Obese children have a high frequency of enlargement of the lingual tonsils with a significantly higher prevalence in those with previous tonsillectomy. Enlarged lingual tonsils may play a role in the pathogenesis of obstructive sleep apnea in obese children.

The effects of Anesthesia and opioids on the upper airway: A systematic review

Drug‐induced sleep endoscopy (DISE) is used to determine surgical therapy for obstructive sleep apnea (OSA); however, the effects of anesthesia on the upper airway are poorly understood. Our aim was to systematically review existing literature on the effects of anesthetic agents on the upper airway.

Evolving Therapies to Treat Retroglossal and Base-of-Tongue Obstruction in Pediatric Obstructive Sleep Apnea

OBJECTIVE To describe our experience treating retroglossal and base-of-tongue collapse in children and young adults with obstructive sleep apnea using combined genioglossus advancement (Repose THS; MedtronicENT, Jacksonville, Florida) and radiofrequency ablation of the tongue base. DESIGN Retrospective institutional review board-approved analysis of 31 operations. SETTING Tertiary pediatric medical center. PATIENTS Thirty-one patients with a mean age of 11.5 years (age range, 3.1-23.0 years). INTERVENTIONS Combined genioglossus advancement and radiofrequency ablation. MAIN OUTCOME MEASURES Preoperative and postoperative polysomnographic data were evaluated for each patient. Success of surgery was determined using the criteria of a postoperative apnea-hypopnea index of 5 or fewer events per hour, without evidence of hypoxemia (oxygen saturation as measured by pulse oximetry), and without prolonged hypercarbia (end-tidal carbon dioxide). RESULTS Thirty-one patients who underwent genioglossus advancement were analyzed. Nineteen (61%) had Down syndrome. The overall success rate was 61% (19 of 31) (58% [12 of 19] success among patients with Down syndrome and 66% [7 of 12] success among patients without Down syndrome). Overall, the mean apnea-hypopnea index improved from 14.1 to 6.4 events per hour (P < .001); the mean nadir oxygen saturation as measured by pulse oximetry during apnea improved from 87.4% to 90.9% (P = .07). CONCLUSIONS Pediatric obstructive sleep apnea refractory to adenotonsillectomy that is due to retroglossal and base-of-tongue collapse remains difficult to treat. However, most patients in this analysis benefited from combined genioglossus advancement and radiofrequency ablation.