Pindborg tumor in early childhood: a rare tumor in the youngest patient reported to date.

Pindborg tumor is a benign expansile and slow growing odontogenic tumor that occurs mainly in adulthood. Limited management data exist for its treatment in young patients. We report the case of a 5-year-old patient and provide recommendations for the care of pediatric patients diagnosed with this rare odontogenic tumor.

Decannulation and Airway Outcomes With Maxillomandibular Distraction in Treacher Collins and Nager Syndrome.

BACKGROUND: Treacher Collins syndrome is a rare disorder (1/50,000 live births) with features that include hypoplastic orbitozygomatic complex with downward slanting eyes, and maxillary/mandibular retrusion. Obstructive sleep apnea and tracheostomy-dependence are common. This study presents the outcomes of skeletal distraction on avoidance of tracheostomy and decannulation in this patient population. METHODS: The authors reviewed charts of all patients with Treacher Collins syndrome who underwent craniofacial reconstruction from 2003 to 2016. Primary outcome measures included decannulation of tracheostomy dependent patients and avoidance of tracheostomy. Secondary outcome measures included cephalometric parameters, polysomnography scores, and airway exposure scores on direct laryngoscopy. RESULTS: Twenty-five patients underwent mandibular and maxillary advancement to resolve upper airway obstruction. Mandibular distraction was performed in 24 of 25 patients, and maxillary distraction in 14 of 25 patients. Maxillary distraction was combined with mandibular distraction in 13 of 17 to accomplish greater advancement and counter-clockwise rotation of the entire maxillary-mandibular complex. Six of 7 patients, 85.7%, avoided a tracheostomy and 39% (7 of 18) were decannulated. Cephalometric changes in sella-nasion-A point, sella-nasion-B , occlusal plane angle, and posterior airway space were equivalent between the groups who were able to clear their obstruction and those who were not. CONCLUSIONS: Treacher Collins is a very challenging disease in which to resolve airway obstruction. Thus, thorough evaluation of the entire airway for all levels of obstruction is critical to successful outcomes. Future collaborative efforts between multiple institutions can help to increase our understanding and effective management of this rare disease.

What is the Cost of Free Cleft Surgery in the Middle East?

BACKGROUND: This project explores the costs of cleft lip and/or palate surgeries in Palestine and Sudan, two low- and middle-income countries (LMIC), in the Middle East. Our purpose is to examine the veracity of advertisements from international cleft organizations claiming that "250 US dollars (USD) covers the cost of a single cleft surgery." We hypothesize that the actual cost of surgery is greater than 250 USD. METHODS: Costs for each cleft surgery were organized broadly into 5 categories: hospital charges, personnel (time and money spent for health professionals to travel to LMIC, including lost wages), tests, consumables, and reusables. Each item was priced at market value during the time of data collection. Following itemization of actual costs, we compared the costs per cleft surgery among four surgical practice models: (1) visiting international surgical teams, (2) visiting international surgeon working with local teams, (3) local teams working at government hospitals, and (4) local teams working at private hospitals. RESULTS: Our results suggest that 250 USD is an underestimate of actual costs per cleft surgery in all models. The most expensive model in both Palestine and Sudan was the first model, visiting international teams performing all team functions; the cheapest surgical model in both countries was a local team working at government hospitals. The largest cost for any of these models is travel and lost wages for international team members. Eliminating this single cost (travel) decreases overall cost tremendously, but still does not approach the advertised cost of 250 USD. CONCLUSIONS: We conclude that 250 USD underestimates the actual costs to perform a single cleft surgery in Palestine and Sudan. If international cleft organizations are genuinely committed to creating sustainable international cleft programs, they should focus exclusively on training local professionals to perform surgery in hospitals of their own choosing.

Decision Making for Children with Obstructive Sleep Apnea without Tonsillar Hypertrophy.

OBJECTIVE: Evidence-based medicine is the gold standard practice model for patient management. Our aim was to determine whether decisions made by pediatric subspecialists regarding management of obstructive sleep apnea in children without tonsillar hypertrophy adhered to this model or were based on clinical experiences. STUDY DESIGN: Single-institution prospective study. SETTING: Multidisciplinary upper airway center in an academic pediatric hospital. SUBJECTS AND METHODS: Twelve pediatric subspecialists representing 8 specialties participating in upper airway clinics and management conferences. Real-time decisions made in treatment conferences and upper airway clinics were collected. Physicians were queried regarding the basis of their decisions, and these decisions were then classified into 10 categories. RESULTS: Over 13 days (10 case conferences, 3 half-day clinics), 324 decisions were made for 58 patients (mean age = 8.9 ± 7.4 years, mean body mass index percentile = 75 ± 29); 34% (n = 108) of decisions were evidence based; 59% (n = 193) were nonevidence based; and 7% (n = 23) were based on parental preference. Providers were able to cite specific studies for <20% of these decisions. There was no significant increase in the proportion of evidence-based decisions made over time. CONCLUSIONS: We deemed 34% of decisions regarding the management of obstructive sleep apnea in children without tonsillar hypertrophy to be evidence based and found that sharing the basis for decisions did not improve the percentage of evidence-based decisions over time. These findings reflect significant evidence gaps and highlight the need for a systematic literature evaluation to identify best practice in managing this population. We recommend that these evidence gaps be further characterized and incorporated into an agenda for future research.