Surgical outcome and treatment trends in 1289 infants with micrognathia: a multicenter cohort.

BACKGROUND: Studies of infants with micrognathia, especially Robin Sequence (RS), are limited by its rarity and both phenotypic and diagnostic variability. Most knowledge of this condition is sourced from small, single-institution samples. METHODS: This is a cross-sectional study including infants with micrognathia admitted to 38 Children's Hospital Neonatal Consortium centers from 2010-2020. Predictor variables included demographic data, birth characteristics, cleft and syndrome status. Outcome variables included length of stay (LOS), death, feeding or respiratory support, and secondary airway operations. RESULTS: 1289 infants with micrognathia had a surgery to correct upper airway obstruction. Mean age and weight at operation were 34.8±1.8 weeks and 3515.4±42 grams, respectively. A syndromic diagnosis was made in 150 (11.6%) patients, with Stickler (5.4%) and Treacher Collins Syndromes (2.2%) most common. Operations included: mandibular distraction osteogenesis (MDO), 66.3%; tracheostomy, 25.4%; and tongue-lip adhesion (TLA), 8.3%. Tracheostomy patients had a lower birth weight, head circumference, gestational age, and APGAR scores. MDO patients were less likely to need a second airway operation compared to TLA patients (3.5%vs17.8%,p<0.001). The proportion of infants feeding exclusively orally at hospital discharge differed significantly, from most to least: MDO, TLA, tracheostomy. Hospital LOS was not statistically different for patients that had MDO and TLA, but was longer for those with primary tracheostomy. Mortality was low for all operations (0.5%). CONCLUSION: In this 1289 surgical patient cohort, MDO was associated with shorter hospital stay, improved oral feeding, and lower rates of secondary airway operations. Prospective multi-center studies are necessary to support these conclusions.

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. STUDY DESIGN: Blinded modified Delphi consensus process. SETTING: Tertiary care center. METHODS: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. RESULTS: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. CONCLUSIONS: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:1168-1174, 2021.

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.

An Evidence-Based Practical Approach to Pediatric Otolaryngology in the Developing World.

Despite humanitarian otolaryngology groups traveling in record numbers to resource-limited areas treating pediatric otolaryngology disease processes and training local providers, there remains a large burden of unmet needs. There is a meager amount of published information that comes from the developing world from an otolaryngology standpoint. As would be expected, the little information that does comes involves some of the most common pediatric otolaryngology diseases and surgical burdens including childhood hearing loss, otitis media, adenotonsillectomies, airway obstructions requiring tracheostomies, foreign body aspirations, and craniomaxillofacial surgeries, including cleft lip and palate.

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

BACKGROUND: 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. METHODS: The mission of the DAS is to identify children with known or anticipated DAWs, communicate the diagnosis and collaborate with referring medical and surgical services, and to manage children in those settings that airway management might be required in the context of the patient's ongoing medical care. RESULTS: The initial 3-month experience confirmed that a majority of pediatric DAW events are associated with congenital or acquired abnormalities. Through appropriate consultation and leadership, the DAS was able to physically and electronically identify pediatric patients with a DAW and provide management. Hospital-wide participation was instrumental in the success and exponential growth of DAS: planned preoperative tracheostomy in complicated posterior spinal fusion candidates, participation in EXIT procedures, standardization of airway carts, and implementation of education forums. CONCLUSION: In developing the DAS, our goal was to provide a more comprehensive approach to caring for a child with a DAW that included their entire hospital stay and follow-up care. We believe this approach has improved health care professional awareness as well as the safe management of routine and difficult pediatric airway. Additional studies are needed to determine whether measurable changes in morbidity and mortality are observed over time.

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.

Hearing loss in pediatric patients with isolated nonsyndromic sagittal synostosis.

OBJECTIVE: Recent studies have shown increased rates of speech, language, cognitive and behavioral abnormalities in patients with isolated nonsyndromic sagittal synostosis. Little is known regarding the prevalence and type of hearing abnormalities in children with craniosynotosis. This study characterizes hearing loss in pediatric patients with isolated nonsyndromic sagittal synostosis. METHODS: Retrospective chart review of isolated nonsyndromic sagittal synostosis patients in a pediatric craniofacial clinic to determine the prevalence, severity, and type of hearing loss. RESULTS: Fifteen of 57 patients with isolated nonsyndromic sagittal synostosis had hearing loss (26% with a 95% confidence interval of 14.9 to 37.7%). Twelve patients demonstrated a conductive loss and three an unspecified type of loss (no bone conduction thresholds were recorded for these patients). The prevalence of conductive hearing loss in our population was 21% with a 95% confidence interval of 10.4 to 31.6%. All cases of hearing loss were mild or moderate in severity. CONCLUSIONS: The most common hearing impairment in patients with isolated nonsyndromic sagittal synostosis is conductive hearing loss likely secondary to middle ear effusion. These patients do not appear to have a higher frequency of middle ear effusion and conductive hearing loss than the normal population of comparable age. We conclude that there is no evidence of increased risk of hearing loss in our study population. We infer that hearing loss does not play a causative role in the elevation of risk for speech, language, behavioral and cognitive impairments which these patients experience. Health care professionals are obligated to ensure that conductive loss from middle ear effusion does not exacerbate these impairments. This obligation can safely be performed by managing hearing loss in the INSS population similar to hearing loss in comparably aged normal children.

Giant intracranial teratoma with epignathus in a neonate. Case report and review of the literature.

Epignathus, a congenital oropharyngeal teratoma, is a rare clinical entity with variable clinical outcomes described in the literature. Even fewer cases of epignathus with intracranial extension have been reported, all with poor outcomes. In this manuscript, the authors present a case of epignathus with intracranial extension, emphasizing clinical presentation, imaging findings, a staged surgical approach, multidisciplinary management, and outcome.

Otologic and audiologic features of Nager acrofacial dysostosis.

OBJECTIVE: To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis. DESIGN: Retrospective case series. SETTING: Multidisciplinary clinic in a tertiary care children's hospital. SUBJECTS: Patients less than 18 years of age with Nager acrofacial dysostosis. METHODS: Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline. RESULTS: External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population. CONCLUSIONS: Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended.

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 Children's 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.