A Retrospective and Prospective Cohort Study Comparing Pediatric Patients With Cleft Lip and Palate From the United States and Guatemala.

Orofacial clefts (OFC) remain among the most prevalent congenital abnormalities worldwide. In the United States in 2010 to 2014, 16.2 of 10,000 live births are born with OFC compared with 23.6 of 10,000 in Alta Verapaz, Guatemala in 2012. Demographics and cleft severity scores were retrospectively gathered from 514 patients with isolated OFC at the Children's Hospital of Philadelphia scheduled for surgery from 2012 to 2019 and from 115 patients seen during surgical mission trips to Guatemala City from 2017 to 2020. Risk factors were also gathered prospectively from Guatemalan families. The Guatemalan cohort had a significantly lower prevalence of cleft palate only compared with the US cohort, which may be a result of greater cleft severity in the population or poor screening and subsequent increased mortality of untreated cleft palate. Of those with lip involvement, Guatemalan patients were significantly more likely to have complete cleft lip, associated cleft palate, and right-sided and bilateral clefts, demonstrating an increased severity of Guatemalan cleft phenotype. Primary palate and lip repair for the Guatemalan cohort occurred at a significantly older age than that of the US cohort, placing Guatemalan patients at increased risk for long-term complications such as communication difficulties. Potential OFC risk factors identified in the Guatemalan cohort included maternal cooking-fire and agricultural chemical exposure, poor prenatal vitamin intake, poverty, and risk factors related to primarily corn-based diets. OFC patients who primarily rely on surgical missions for cleft care would likely benefit from more comprehensive screening and investigation into risk factors for more severe OFC phenotypes.

Epidemiology of Craniofacial Pathology in Northern Vietnam Using Digital Platforms: Utilization of Electronic Medical Records and an SMS-Based Reporting System in a Low-Middle Income Country.

INTRODUCTION: Current disease surveillance in Vietnam relies on underdeveloped electronic medical record (EMR) systems, which are insufficient for timely standardized data collection about craniofacial and cleft pathologies. Concurrently, the World Bank reports Vietnamese mobile cellular subscription rates (per 100 people) of 128, some of the highest in the world. Herein, we discuss the development of a short messaging service (SMS)-based surveillance system based in Hanoi, Vietnam, and its utility in contrast to an EMR-based approach to craniofacial epidemiology study. METHODS: Vietnam National Children's Hospital (VNCH) is a pediatric tertiary care center serving approximately 12 million children. Four plastic surgeons were trained in SMS data entry. The SMS database was designed using FrontlineSMS, a 2-way mobile gateway software application with automated messaging capabilities. The VNCH EMR database was queried for individual demographic data for patients seen by the department from 2010 to 2019. RESULTS: A corrected prevalence for cleft lip and palate of 0.098% (1 in 1020 live births) was calculated based on EMR data. Six rural provinces greater than 150 km from Hanoi were found to be significantly (P < 0.01) underserved surgically by the VNCH. A significantly (P < 0.01) higher rate of Pierre Robin in Lang Son province and facial clefts in Ha Tinh province were identified. The prospective data collection system received 7 patient data sets over 4 months, resulting in a capture rate of 2.9%. CONCLUSIONS: This feasibility study offers valuable insight into the true cleft and craniofacial prevalence in Vietnam and alternative methods to study in low- and middle-income countries.

Evaluating Quality of Life After Facial Reanimation Using the Facial Clinimetric Evaluation Scale in a Series of Pediatric Patients.

BACKGROUND: Facial palsy can have significant functional and psychological impact. Dynamic facial reanimation methods have provided means of restoration of smile. There remains a dearth of quality of life data in children. The authors aim to assess patient reported outcomes in this underreported population. METHODS: A retrospective review was performed of pediatric patients with facial palsy who had dynamic facial reanimation from 2009 to 2017 at a single institution. Patients were sent a digital Facial Clinimetric Evaluation (FaCE) questionnaire, a validated tool to assess patient quality of life. FaCE statements are grouped into 6 categories: facial movement, facial comfort, oral function, eye comfort, lacrimal control, and social control. RESULTS: Twenty patients met inclusion criteria. Fourteen successfully completed the survey. With normal function being 100, our cohort scored: facial movement 38.7 ±â€Š14.3; facial comfort 67.3 ±â€Š23, oral function 75 ±â€Š22.7, eye comfort 68.75 ±â€Š18.2, lacrimal control 53.6 ±â€Š32.5, social function 64.3 ±â€Š25.6. The overall FaCE score was 61.07 ±â€Š10.85. The facial movement subscores were lower for brow raise 16.1 ±â€Š33.4 relative to smiling 53.6 ±â€Š29.2 and lip pucker, 46.4 ±â€Š23.7. No correlation was found between facial movement scores and total scores (R2 < 0.01). There was a strong negative correlation between total scores and time since procedure (Spearman's rho R =  -0.45). CONCLUSION: Our pediatric facial reanimation cohort demonstrated excellent social function, in contrast to a relatively lower facial movement score. The low facial movement score was mediated by low score for brow raise and was not correlated to overall FaCE score. Interestingly, overall FaCE score tended to decrease with time from procedure.

Buccal Myomucosal Flap Reconstruction After Resection of Palatal Mucoepidermoid Carcinoma.

Salivary gland tumors are rare, constituting approximately 0.5% of pediatric malignancies, yet account for over 50% of malignant salivary gland neoplasms, of which a majority are mucoepidermoid carcinomas (MECs). We present a case of MEC involving the palatal minor salivary gland of an adolescent patient successfully reconstructed using buccal myomucosal flap. The subject presented as a 17-year-old male with a painless left hard palatal mass found to have imaging suspicious for minor salivary gland tumor and punch biopsy consistent with a low-grade MEC. The subject underwent wide local excision of a 1.0 × 1.2 cm mass with 1.0 cm margins down to hard palate nasal mucosa excluding the abutting first and second molars of the ipsilateral maxilla. Pathology confirmed low-grade MEC confined to hard palate. Two weeks, thereafter, the subject underwent buccal myomucosal flap reconstruction inset into the palatal defect and divided 2 weeks thereafter. Postoperative course was complicated by a pinpoint oronasal fistula at the posterior aspect of the flap-palate junction requiring reelevation and advancement. The subject subsequently recovered without complication. Mucoepidermoid carcinomas represent rare, malignant minor salivary gland tumors with nonspecific presentations that require multidisciplinary workup and management. The authors recommend reconstruction of resultant palatal defects to prevent progression to oronasal fistulae or speech and swallow impairment.

Detection of Pulmonary Embolism in High-Risk Children.

OBJECTIVE: To evaluate 2 commonly used adult-based pulmonary embolism (PE) algorithms in pediatric patients and to derive a pediatric-specific clinical decision rule to evaluate children at risk for PE, given the paucity of data to guide diagnostic imaging in children for whom PE is suspected. STUDY DESIGN: We performed a single-center retrospective study among 561 children <22 years of age undergoing either D-dimer testing or radiologic evaluation (computed tomography or ventilation-perfusion scan) in the emergency department setting for concern of PE. A diagnosis of PE required radiologic confirmation and anticoagulant treatment. We evaluated the test characteristics of the Wells criteria and Pulmonary Embolism Rule-out Criteria (PERC) low-risk rule and used recursive partition analysis to derive a clinical decision rule. RESULTS: Among the 561 patients included in the study, 36 (6.4%) were diagnosed with PE. The Wells criteria demonstrated a sensitivity and specificity of 86% and 60%, respectively. The sensitivity and specificity of the PERC were 100% and 24%, respectively. A clinical decision rule including the presence of oral contraceptive use, tachycardia, and oxygen saturation <95% demonstrated a sensitivity and specificity of 90% and 56%, respectively, a positive and negative likelihood ratio of 2.0 and 0.2, and a positive and negative predictive value of 0.12 and 0.99, respectively. CONCLUSIONS: The risk of PE is low among children not receiving estrogen therapy and without tachycardia and hypoxia in those with an initial suspicion of PE. Application of the PERC rule and Wells criteria should be used cautiously in the pediatric population.