David W. Molter

Multidisciplinary management of Hunter syndrome

Hunter syndrome is a rare, X-linked disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase. In the absence of sufficient enzyme activity, glycosaminoglycans accumulate in the lysosomes of many tissues and organs and contribute to the multisystem, progressive pathologies seen in Hunter syndrome. The nervous, cardiovascular, respiratory, and musculoskeletal systems can be involved in individuals with Hunter syndrome. Although the management of some clinical problems associated with the disease may seem routine, the management is typically complex and requires the physician to be aware of the special issues surrounding the patient with Hunter syndrome, and a multidisciplinary approach should be taken. Subspecialties such as otorhinolaryngology, neurosurgery, orthopedics, cardiology, anesthesiology, pulmonology, and neurodevelopment will all have a role in management, as will specialty areas such as physiotherapy, audiology, and others. The important management topics are discussed in this review, and the use of enzyme-replacement therapy with recombinant human iduronate-2-sulfatase as a specific treatment for Hunter syndrome is presented.

Cochlear Implantation in the Congenitally Malformed Ear

A child with a congenital hearing loss and auricular malformations was evaluated for cochlear implantation. Preoperative radiologic studies demonstrated a common cavity cochlear malformation and a contralateral narrow internal auditory canal. Intraoperative findings included an aberrantly coursing facial nerve, preventing routine placement of the electrode array. The surgical approach was consequently modified and the cochlea was accessed through a malformed semicircular canal. Cochlear implantation can be performed safely and effectively in children with congenitally malformed ears.

Pediatric laryngeal dimensions: an age-based analysis.

BACKGROUND: In children, the cricoid is considered the narrowest portion of the “funnel-shaped” airway. Growth and development lead to a transition to the more cylindrical adult airway. A number of airway decisions in pediatric airway practice are based on this transition from the pediatric to the adult airway. Our primary aim in this study was to measure airway dimensions in children of various ages. The measures of the glottis and cricoid regions were used to determine whether a transition from the funnel-shaped pediatric airway to the cylindrical adult airway could be identified based on images obtained from video bronchoscopy. METHODS: One hundred thirty-five children (ASA physical status 1 or 2) aged 6 mo to 13 yr were enrolled for measurement of laryngeal dimensions, including cross-sectional area (G-CSA), anteroposterior and transverse diameters at the level of the glottis and the cricoid (C-CSA), using the video bronchoscopic technique under general anesthesia. RESULTS: Of the 135 children enrolled in the study, seven patients were excluded from the analysis mainly because of poor image quality. Of the 128 children studied (79 boys and 49 girls), mean values (±standard deviation) for the demographic data were age 5.9 (±3.3) yr, height 113.5 (±22.2) cm and weight 23.5 (±13) kg. Overall, the mean C-CSA was larger than the G-CSA (48.9 ± 15.5 mm2 vs 30 ± 16.5 mm2, respectively). This relationship was maintained throughout the study population starting from 6 mo of age (P < 0.001, r = 0.45, power = 1). The mean ratio for C-CSA: G-CSA was 2.1 ± 1.2. There was a positive correlation between G- and the C-CSA versus age (r = 0.36, P < 0.001; r = 0.27, P = 0.001, respectively), height (r = 0.34, P < 0.001; r = 0.29, P < 0.001, respectively), and weight (r = 0.35, P < 0.001; r = 0.25, P = 0.003, respectively). No significant gender differences in the mean values of the studied variables were observed. CONCLUSION: In this study of infants and children, the glottis rather than cricoid was the narrowest portion of the pediatric airway. Similar to adults, the pediatric airway is more cylindrical than funnel shaped based on these video bronchoscopic images. Further studies are needed to determine whether these static airway measurements in anesthetized and paralyzed children reflect the dynamic characteristics of the glottis and cricoid in children.

Sinonasal posttransplant lymphoproliferative disorder in pediatric lung transplant patients

OBJECTIVE: To evaluate sinonasal manifestations of posttransplant lymphoproliferative disorder (PTLD) in the pediatric lung transplant population. STUDY DESIGN AND METHODS: Case series of children less than 18 years presenting with PTLD after pulmonary transplantation at St Louis Childrens Hospital between Jan 1, 1990 and Dec 31, 2003. RESULTS: Two hundred eighty-two lung transplants were performed in 246 children. Thirty-two cases of histopathologically confirmed PTLD were identified with 8 (25%) presenting in the head and neck. Sinonasal PTLD was the most common site of head and neck involvement (63%), with 40% of patients presenting with occult disease. All patients with sinonasal PTLD had longstanding nasal polyposis related to cystic fibrosis (P = 0.07). CONCLUSIONS: This is, to our knowledge, the first report identifying an increased frequency of sinonasal PTLD after pediatric pulmonary transplantation, particularly in children with cystic fibrosis and associated nasal polyposis. Because sinonasal PTLD may be asymptomatic, this region should receive close scrutiny on surveillance evaluations.

Videofluoroscopic and Nasendoscopic Correlates of Speech in Velopharyngeal Dysfunction

Objective To compare videonasendoscopy, lateral videofluoroscopy, and perceptual speech examination in the assessment of velopharyngeal dysfunction. Design Retrospective observational. Setting Multidisciplinary cleft palate team at a tertiary academic institution. Patients, Participants Patients who had undergone videonasendoscopy and lateral videofluoroscopy for suspected velopharyngeal dysfunction at our center were evaluated. Inclusion required that videonasendoscopy, lateral videofluoroscopy, and the perceptual speech exam were performed on the same day. A total of 88 patients were analyzed. Main outcome Measure(s) Primary outcome measures included percent closure on videonasendoscopy, percent closure on lateral videofluoroscopy, and quantitative scores for hypernasal resonance, nasal emission, and facial grimace. Additional outcome measures included linear and angular anatomic measurements obtained from lateral videofluoroscopy. Results Moderately strong correlation was found between closure estimates of videonasendoscopy and lateral videofluoroscopy (ρ = .583; p < .001). Lateral videofluoroscopy estimates of closure averaged 11.7% higher than videonasendoscopy. Closure correlated moderately with overall speech severity (ρ = .304; p = .005); whereas, a stronger correlation was seen with hypernasal resonance (ρ = –.479; p < .001). Patients exhibiting grimace had worse closure than those without (79.1% versus 70.7%; ρ = .035). Movement angle of the velum and change in genu angle correlated significantly with closure function (ρ = –.304; p = .034 and ρ = –.395; p < .001, respectively). Conclusions Videonasendoscopy and lateral videofluoroscopy closure estimates correlated moderately. Lateral videofluoroscopy tended to give smaller gap estimates. Hypernasal resonance and facial grimace are useful clinical indicators of large gap size. Velar movement angle and change in genu angle were identified as anatomical correlates of closure function.

Official american thoracic society technical standards: Flexible airway endoscopy in children

BACKGROUND Flexible airway endoscopy (FAE) is an accepted and frequently performed procedure in the evaluation of children with known or suspected airway and lung parenchymal disorders. However, published technical standards on how to perform FAE in children are lacking. METHODS The American Thoracic Society (ATS) approved the formation of a multidisciplinary committee to delineate technical standards for performing FAE in children. The committee completed a pragmatic synthesis of the evidence and used the evidence synthesis to answer clinically relevant questions. RESULTS There is a paucity of randomized controlled trials in pediatric FAE. The committee developed recommendations based predominantly on the collective clinical experience of our committee members highlighting the importance of FAE-specific airway management techniques and anesthesia, establishing suggested competencies for the bronchoscopist in training, and defining areas deserving further investigation. CONCLUSIONS These ATS-sponsored technical standards describe the equipment, personnel, competencies, and special procedures associated with FAE in children.

The Furlow palatoplasty for velopharyngeal dysfunction: velopharyngeal changes, speech improvements, and where they intersect.

Objective We investigated how Furlow palatoplasty changes velopharyngeal morphology and speech characteristics, as well as how the anatomical and clinical results might be related. We hypothesized that Furlow palatoplasty would result in measurable velar elongation, tightening of the genu angle, and retropositioning of the levator sling and that the achievement of these modifications might be associated with clinical speech improvement. Design Retrospective analysis of preoperative and postoperative videofluoroscopic and speech data. Setting Tertiary care center. Patients/Participants A total of 29 patients with velopharyngeal insufficiency in the setting of previous cleft palate repair or submucous cleft palate. Interventions Furlow palatoplasty for treatment of velopharyngeal insufficiency. Outcome Measures Lateral videofluoroscopy and perceptual speech examination were conducted preoperatively and postoperatively in order to measure velopharyngeal dimensions and speech quality. We describe anatomical and speech changes associated with the Furlow palatoplasty and undertake an exploratory analysis of the relationship between surgical changes to the velopharynx and clinical outcomes. Results Furlow palatoplasty results in significant velar elongation, increased acuity of the genu angle, and retropositioning of the levator sling. Postoperative speech improvement was identified on the three subscales of resonance, nasal emission, and stops/plosives. Speech improvement and the absence of need for reoperation were most consistently associated with tightening of the genu angle. Conclusions Furlow palatoplasty lengthens the palate, while both tightening and retropositioning the levator sling. These changes reflect transverse recruitment of lateral velar tissues, along with transverse tightening and anterior release of the muscle fibers, respectively. Levator tightening is most consistently associated with improved speech outcomes.

Neonatal Outcomes of Pierre Robin Sequence: An Institutional Experience

Objective: The aim of this study was to characterize the outcomes of one institute’s experience regarding upper airway and feeding management in Pierre Robin sequence (PRS) neonates. Study Design: A retrospective review of 33 neonates was performed. Average daily weight gain for all patients and average length of stay for each group was calculated and the results were compared using Student’s t test. Result: Average daily weight gain was 16.5 g in the mandibular distraction osteogenesis (MDO) group (MDO; N = 12) and 5.6 g in the nonsurgical intervention (NSI) group (N = 18; P = .043). Average length of stay was longer in the MDO group (P = .01). In all, 67% of MDO patients were discharged with total PO (per os) feeds compared with 22% of NSI patients. Conclusion: Neonates with PRS who do not require surgical airway intervention are more likely to require assisted feeding, have slower weight gain, and a shorter average hospital stay compared to neonates undergoing mandibular MDO.

Upper airway dimensions in children using rigid video-bronchoscopy and a computer software: description of a measurement technique.

Background:  Pediatric airway management decisions are based primarily on results derived from indirect measures of laryngeal and tracheal dimensions. More recent methods could provide more direct information about absolute and relative changes in airway dimensions associated with growth and development.

Difficult airway consultation service for children: steps to implement and preliminary results

Failed airway management remains one of the most common causes of cardiopulmonary arrest in the pediatric population. Practice guidelines addressing the difficult airway (DAW) in adults provide anesthesiologists a framework for managing the airway during the perioperative period; however, similar consensus guidelines are lacking in the pediatric population. Many of the adverse events associated with difficult pediatric airway management occur outside the perioperative setting and often result in worse outcomes. The lower frequency of DAW management required in children, lesser awareness of pediatric health care professionals about DAW management, and the need for guiding principles led us to develop a DAW consultative service. This report outlines the steps to establish the Difficult Airway Service (DAS) and the initial experiences with this new consultation service.