Aliza P. Cohen

Advances in the diagnosis and management of chronic pulmonary aspiration in children

Chronic pulmonary aspiration (CPA) in children is an important cause of recurrent pneumonia, progressive lung injury, respiratory disability and death. It is sporadic, intermittent and variable, and often occurs in children with complicated underlying medical conditions and syndromes that produce symptoms indistinguishable from CPA. For most types of aspiration there is no gold-standard diagnostic test. The diagnosis of CPA is currently made clinically with some supporting diagnostic evaluations, but often not until significant lung injury has been sustained. Despite multiple diagnostic techniques, the diagnosis or exclusion of CPA in children is challenging. This is of particular concern given the outcome of unrecognised progressive lung injury and the invasiveness of definitive therapies. Although new techniques have been introduced since the 1990s and significant advances in the understanding of dysphagia and gastro-oesophageal reflux have been made, characterisation of the aspirating child remains elusive.

A diagnostic paradigm for childhood idiopathic sensorineural hearing loss.

OBJECTIVE: Our objective was to determine the diagnostic yield of laboratory testing, radiological imaging, and GJB2 mutation screening in a large cohort of patients with differing severities of idiopathic sensorineural hearing loss (SNHL). DESIGN AND SETTING: We undertook a retrospective study of patients presenting with SNHL at our institution from 1993 to 2002. RESULTS: Laboratory testing had an extremely low yield. Patients with unilateral SNHL had a significantly higher imaging yield than those with bilateral. The diagnostic yield of GJB2 screening was significantly higher in patients with severe to profound SNHL than in those with less severe SNHL. However, a relatively large number of patients with mild to moderate SNHL had positive GJB2 screens. CONCLUSIONS: Based on diagnostic yields, we propose a cost-effective stepwise diagnostic paradigm to replace the more commonly used and costly simultaneous testing approach. EBM rating: C. (Otolaryngol Head Neck Surg 2004;131: 804–9.)

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.)

Baroreflex Gain in Children with Obstructive Sleep Apnea

RATIONALE We previously demonstrated that children with obstructive sleep apnea have increased blood pressure associated with changes in left ventricular mass index. Others have shown in adults that blood pressure variability is an important predictor of changes in left ventricular mass. The baroreflex system buffers blood pressure changes by varying heart rate. We have thus hypothesized that (1) baroreflex system gain is increased during sleep, improving blood pressure buffering; (2) children with obstructive sleep apnea lack this baroreflex gain increase; and (3) reduced blood pressure buffering results in exaggerated blood pressure variability that is associated with end-organ damage. OBJECTIVES Compare measures of left ventricular mass index and nighttime baroreflex gain of healthy children to those of children with obstructive sleep apnea. METHODS A total of 169 children (50 control subjects, 63 with mild obstructive sleep apnea, and 56 with severe obstructive sleep apnea) with a mean age of 9.9 years (+/-2.2) underwent echocardiography followed by polysomnography with continuous blood pressure measurement. Baroreflex gain was calculated in time and frequency domains. MEASUREMENTS AND MAIN RESULTS Healthy children demonstrated a nighttime pattern of increasing baroreflex gain. Children with obstructive sleep apnea had decreased nighttime baroreflex gain compared with control subjects. Nighttime blood pressure and blood pressure variability were significantly correlated with left ventricular mass index. CONCLUSIONS Obstructive sleep apnea is associated with a decrease in nighttime baroreflex gain and an increase in blood pressure variability. This increase is correlated with changes in left ventricular mass index.

Endoscopic airway management in children

Purpose of reviewTo discuss the current status of endoscopic airway surgery in children. Recent findingsMore refined endoscopic instruments have been introduced, including balloon dilators, powered debriders, lasers with more exact modes of delivery, and innovative suspension laryngoscopes. The use of balloon dilatation for primary management of acquired subglottic stenosis is promising. Microdebriders are now considered a viable option to the CO2 laser for the management of a number of airway diseases. Knowledge regarding supraglottoplasty continues to evolve. Endoscopic vocal cord lateralization is being successfully used at some centers for the management of bilateral vocal cord paralysis. The da Vinci Surgical Robot (Intuitive Surgical, Inc., Sunnyvale, California, USA) has been introduced to facilitate endoscopic laryngeal cleft repair. SummaryEndoscopic airway surgery is presently regaining its early (1960s) popularity. This trend can be attributed to the availability of new and more sophisticated endoscopic instrumentation, the adjunctive use of new pharmaceuticals, and the realization that open and endoscopic techniques can often be used in a complementary fashion.

Systematic review of site of obstruction identification and non-CPAP treatment options for children with persistent pediatric obstructive sleep apnea.

Although adenotonsillectomy is accepted as a first‐line therapy for pediatric obstructive sleep apnea (OSA), there is currently no consensus regarding optimal methods for identifying the sites of obstruction or treatment of children with persistent disease after surgery. With this in mind, our aim was to systematically review the English‐language literature pertaining to these issues.

Survival probabilities of patients with childhood spinal muscle atrophy.

Objective: Medical and technological advances over the past 2 decades have resulted in improved patient care for children with spinal muscular atrophy (SMA). The objective of the present study was to describe changes in the life expectancy of pediatric patients with SMA over time and to compare these findings with previously reported survival patterns. Methods: Medical records of all patients diagnosed with SMA over a 16-year period (1989-2005) at Cincinnati Childrens Hospital Medical Center were reviewed. Data pertaining to date of birth, type of SMA, medical and surgical interventions, pulmonary complications, and date of death were obtained. Results: Kaplan-Meier survival analyses showed a significant improvement in survival probabilities in the severest form of SMA. Conclusions: We found a positive trend in the survival of patients with severe SMA. Although we cannot attribute this trend to any single factor, it is likely that advances in pulmonary care and aggressive nutritional support have played a significant role.

Complications of Metallic Stents in the Pediatric Airway

OBJECTIVE: Our aim was to present our experience with complications caused by placement of metallic stents in the pediatric airway. DESIGN AND SETTING: We conducted a retrospective study of the medical records of patients with complications resulting from metallic stent placement, managed by the senior authors between 1993 and 2002. RESULTS: Nine children had complications associated with the placement of metallic airway stents. Of these, 8 children required stent removal. Granulation tissue and tracheal stenosis were seen in all 7 children with long standing stent placement. There was 1 stent death in this series. CONCLUSIONS: Metallic airway stents can cause significant complications in the pediatric airway. These complications may supersede the airway compromise that necessitated their initial placement. As such, metallic stent placement should be approached with caution. The likelihood and severity of complications increase with time, as do the difficulties encountered upon removal. The proportion of patients in whom metallic stents may be placed “permanently” without complications is not known. Therefore we recommend that metallic airway stents be considered a temporizing measure of limited duration.

Diagnostic and Treatment Implications of Nasal Obstruction in Snoring and Obstructive Sleep Apnea

LEARNING OBJECTIVES The purpose of this review is to highlight fundamental aspects of obstructive sleep apnea (OSA), and to present an overview of the medical literature that pertains to the clinical interplay between various allergy-related disorders, nasal patency, and OSA. This should enable the reader to play a more proactive role in the diagnosis, management, and prevention of OSA. DATA SOURCES Relevant reviews, texts, and articles. The MEDLINE database was used to find related literature. CONCLUSIONS In predisposed individuals, OSA, sleep fragmentation, and the sequelae of disturbed sleep often result from nasal obstruction. Since breathing through the nose appears to be the preferred route during sleep, nasal obstruction frequently leads to nocturnal mouth breathing, snoring, and ultimately to OSA. Allergists can thus play a vital role in assessing sleep problems in their patients with allergic rhinitis and other upper respiratory disorders, in treating these problems more aggressively, and in some instances, in preventing them.

Determinants of Regional Cerebral Oxygenation in Children with Sleep-disordered Breathing

RATIONALE An association between neurocognitive deficits and pediatric sleep-disordered breathing has been suggested; however, weak correlations between disease severity and functional outcomes underscore the lack of knowledge regarding factors modulating cognitive morbidity of sleep-disordered breathing. OBJECTIVES To identify the parameters affected by sleep-disordered breathing that modulate cerebral oxygenation, an important determinant of cognition. A further objective was to use these parameters with demographic data to develop a predictive statistical model of pediatric cerebral oxygenation. METHODS Ninety-two children (14 control subjects, 32 with primary snoring, and 46 with obstructive sleep apnea) underwent polysomnography with continuous monitoring of cerebral oxygenation and blood pressure. Analysis of covariance was used to relate the blood pressure, sleep diagnostic parameters, and demographic characteristics to regional cerebral oxygenation. MEASUREMENTS AND MAIN RESULTS To account for anatomic variability, an index of cerebral oxygenation during sleep was derived by referencing the measurement obtained during sleep to that obtained during wakefulness. In a repeated measures model predicting the index of cerebral oxygenation, mean arterial pressure, rapid eye movement (REM) sleep, female sex, age, and oxygen saturation had a positive effect on cerebral oxygenation levels, whereas arousal index and non-REM (NREM) sleep had a negative effect. CONCLUSIONS Increasing mean arterial pressure, age, oxygen saturation, and REM sleep augment cerebral oxygenation, while sleep-disordered breathing, male sex, arousal index, and NREM sleep diminish it. The proposed model may explain the sources of variability in cognitive function of children with sleep-disordered breathing.