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.

How Does Reduction Mammaplasty Surgical Technique Impact Clinical, Aesthetic, and Patient-Reported Outcomes?: A Comparison of the Superomedial and Inferior Pedicle Techniques.

BACKGROUND: A comprehensive comparison of surgical, aesthetic, and quality of life outcomes by reduction mammaplasty technique does not exist. We sought to ascertain the effect of technique on clinical, aesthetic, and patient-reported outcomes. METHODS: Patients with symptomatic macromastia undergoing a superomedial or inferior pedicle reduction mammoplasty by a single surgeon were identified. BREAST-Q surveys were administered. Postoperative breast aesthetics were assessed in 50 matched-patients. Patient characteristics, complications, quality of life, and aesthetic scores were analyzed. RESULTS: Overall, 101 patients underwent reductions; 60.3% had a superomedial pedicle. Superomedial pedicle patients were more likely to have grade 3 ptosis (P < 0.01) and had significantly shorter procedure time (P < 0.01). Only the inferior pedicle technique resulted in wound dehiscence (P = 0.03) and reoperations from complications (P < 0.01). Those who underwent an inferior pedicle reduction were 4.3 times more likely to experience a postoperative complication (P = 0.03). No differences in quality of life existed between cohorts (P > 0.05). Superomedial pedicle patients received significantly better scarring scores (P = 0.03). CONCLUSIONS: The superomedial pedicle reduction mammoplasty technique provides clinical and aesthetic benefits compared with the inferior pedicle technique.

Speech Outcomes Following Operative Management of Velopharyngeal Dysfunction (VPD) in Non-Syndromic Post-Palatoplasty Cleft Palate Patients.

OBJECTIVE: Approximately 30% of patients with a history of repaired cleft palate (CP) go on to suffer from velopharyngeal dysfunction (VPD). This study discusses the operative management of VPD and postoperative speech outcomes in a cohort of CP patients. SETTING: An academic tertiary pediatric care center. METHODS: Retrospective cohort study. PATIENTS: Patients with history of repaired CP (Veau I-IV) who underwent operative management of VPD between January 1st, 2010 and December 31st, 2020. Operative modalities were posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), Furlow palate re-repair, and buccal myomucosal flap palate lengthening (PL). OUTCOME MEASURES: The primary outcome measure is postoperative speech improvement evaluated by the Pittsburgh Weighted Speech Scale (PWSS). RESULTS: 97 patients met inclusion criteria. 38 patients with previous straight-line primary palatoplasty underwent Furlow re-repair; these patients were significantly younger (7.62 vs 11.14, P < .001) and were more likely to have severe VPD per PWSS (OR 4.28, P < .01, 95% CI 1.46-12.56) when compared to VPD patients with previous Furlow repair. 21.1% of these patients required an additional non-revisional VPD procedure. The remaining patients underwent a non-revision procedure (26 PPF, 22 SPP, 11 PL); all experienced significant (P < .001 on paired t-test) reductions in PWSS total and subgroup VPD severity scores without difference in improvement between operation types. SPP was statistically associated with all-cause complication (OR 2.79, 95% CI 1.03-7.59, P < .05) and hyponasality (OR 3.27, 95% CI 1.112-9.630, P < .05). CONCLUSION: Furlow re-repair reduced need for additional VPD operations. Speech outcomes between non-revisional operations are comparable, but increased complications were seen in SPP.

Trends in insurance coverage for adolescent reduction mammaplasty.

BACKGROUND: Reduction mammaplasty is an effective intervention for symptomatic macromastia. Studies of insurance policy criteria for adult patients have demonstrated nonuniformity. This study assesses trends in insurance preauthorization for reduction mammaplasty in the adolescent population. METHODS: A retrospective cohort study of patients aged ≤18 years at a pediatric breast clinic between January 1, 2010 and December 31, 2020. Demographic information and clinical variables submitted to insurers were analyzed, as well as third-party payer company, provider network type, and preauthorization criteria. RESULTS: 249 preauthorization requests were studied, with an approval rate of 79.5%. Submissions increased from 6 in 2010 to 59 in 2020. Variables deemed significantly associated with preauthorization denial included submission before 2015 (OR 2.04, 95% CI 1.04-3.95, p = .038), billing zip code median income <$60,000 (OR 2.11, 95% CI 1.12-3.98 p = .02), predicted resection mass below Schnur Sliding Scale threshold (OR 1.97, 95% CI 1.01-3.83 p = .047), and insurance company. CONCLUSIONS: Insurance criteria for preauthorization of reduction mammaplasty in adolescents differ from adults, are not supported by clinical studies, and may exclude low-income patients from receiving care.

Anterior Cranial Base Reconstruction in Complex Craniomaxillofacial Trauma: An Algorithmic Approach and Single-Surgeon's Experience.

Management of traumatic skull base fractures and associated complications pose a unique reconstructive challenge. The goals of skull base reconstruction include structural support for the brain and orbit, separation of the central nervous system from the aerodigestive tract, volume to decrease dead space, and restoration of the three-dimensional appearance of the face and cranium with bone and soft tissues. An open bicoronal approach is the most commonly used technique for craniofacial disassembly of the bifrontal region, with evacuation of intracranial hemorrhage and dural repair performed prior to reconstruction. Depending on the defect size and underlying patient and operative factors, reconstruction may involve bony reconstruction using autografts, allografts, or prosthetics in addition to soft tissue reconstruction using vascularized local or distant tissues. The vast majority of traumatic anterior cranial fossa (ACF) injuries resulting in smaller defects of the cranial base itself can be successfully reconstructed using local pedicled pericranial or galeal flaps. Compared with historical nonvascularized ACF reconstructive options, vascularized reconstruction using pericranial and/or galeal flaps has decreased the rate of cerebrospinal fluid (CSF) leak from 25 to 6.5%. We review the existing literature on this uncommon entity and present our case series of n = 6 patients undergoing traumatic reconstruction of the ACF at an urban Level 1 trauma center from 2016 to 2018. There were no postoperative CSF leaks, mucoceles, episodes of meningitis, or deaths during the study follow-up period. In conclusion, use of pericranial, galeal, and free flaps, as indicated, can provide reliable and durable reconstruction of a wide variety of injuries.

Direct-to-Implant Versus 2-Stage Breast Reconstruction: Which Technique Is Better? An Analysis of 104 Patients at a Single Institution.

BACKGROUND: Two-stage (TS) implant-based reconstruction is the most commonly performed method of reconstruction after mastectomy. A growing number of surgeons are offering patients direct-to-implant (DTI) reconstruction, which has the potential to minimize the number of surgeries needed and time to complete reconstruction, as well as improve health care utilization. However, there are conflicting data regarding the outcomes and complications of DTI, and studies comparing the 2 methods exclusively are lacking. METHODS: Patients undergoing implant-based reconstruction after mastectomy within a large interstate health system between 2015 and 2019 were retrospectively identified and grouped by reconstruction technique (TS and DTI). The primary outcomes were a composite of complications (surgical site occurrences), health care utilization (reoperations, unplanned emergency department visits, and readmissions), and time to reconstruction completion. Risk-adjusted logistic and generalized linear models were used to compare outcomes between TS and DTI. RESULTS: Of 104 patients, 42 underwent DTI (40.4%) and 62 underwent TS (59.6%) reconstruction. Most demographic characteristics, and oncologic and surgical details were comparable between groups ( P > 0.05). However, patients undergoing TS reconstruction were more likely to be publicly insured, have a smoking history, and undergo skin-sparing instead of nipple-sparing mastectomy. The composite outcome of complications, reoperations, and health care utilization was higher for DTI reconstruction within univariate (81.0% vs 59.7%, P = 0.03) and risk-adjusted analyses (odds ratio, 3.78 [95% confidence interval [CI], 1.09-13.9]; P < 0.04). Individual outcome assessment showed increased mastectomy flap necrosis (16.7% vs 1.6%, P < 0.01) and reoperations due to a complication (33.3% vs 16.1%; P = 0.04) in the DTI cohort. Although DTI patients completed their aesthetic revisions sooner than TS patients (median, 256 days vs 479 [ P < 0.01]; predicted mean difference for TS [reference DTI], 298 days [95% CI, 71-525 days]; P < 0.01), the time to complete reconstruction (first to last surgery) did not differ between groups (median days, DTI vs TS, 173 vs 146 [ P = 0.25]; predicted mean difference [reference, DTI], -98 days [95% CI, -222 to 25.14 days]; P = 0.11). CONCLUSIONS: In this cohort of patients, DTI reconstruction was associated with higher complications, reoperations, and health care utilization with no difference in time to complete reconstruction compared with TS reconstruction. Further studies are warranted to investigate patient-reported outcomes and cost analysis between TS and DTI reconstruction.

Secondary Alveolar Bone Grafting in Patients with Unilateral and Bilateral Complete Cleft Lip and Palate: A Single-Institution Outcomes Evaluation Using Three-Dimensional Cone Beam Computed Tomography.

BACKGROUND: Secondary alveolar bone grafting of autologous bone from the iliac crest is a widely accepted modality for repair of residual alveolar cleft in patients with mixed dentition with previously repaired complete cleft lip and palate. There remains debate regarding surgical timing and preoperative, perioperative, and postoperative management of these cases. METHODS: This retrospective study reviewed patient demographic information in addition to preoperative, perioperative, and postoperative course to evaluate how patient and practice factors impact graft outcomes as assessed by three-dimensional cone beam computed tomographic evaluation at the 6-month postoperative visit. RESULTS: On univariate analysis, age at operation older than 9 years, history of oronasal fistula, history of cleft lip or palate revision, and history of international adoption were all found to significantly increase likelihood of graft failure (p < 0.05). On multivariate analysis, age older than 9 years was found to be the single most significant predictor of graft failure (p < 0.05). There was no significant difference in graft outcomes between patients with unilateral or bilateral cleft lip and palate, and no single variable was found to significantly correlate to increased complication rates. The graft success rate overall of the authors' practice was 86.2 percent, with a complication rate of 7.7 percent. CONCLUSIONS: Secondary alveolar bone grafting is an integral part of the cleft lip and palate surgical treatment series; this study identified several outcome predictors for both graft failure and adverse events, the most significant of which was age at operation. Although the mixed dentition phase often extends to 12 years of age, it is recommended that bone grafting be performed before 9 years of age to optimize outcomes. CLINICAL QUESTIONS/LEVEL OF EVIDENCE: Risk, III.

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.

Is lymphangitic streaking associated with different pathogens?

OBJECTIVES: Little is known regarding the differences in microbiology associated with cellulitis or abscess with or without lymphangitic streaking. The objective of our study is to assess whether there are differences in the pathogens identified from wound cultures of patients with paronychia with and without associated lymphangitis. METHODS: Retrospective cross-sectional study at a tertiary pediatric emergency department over 25 years. We opted to assess patients with paronychia of the finger, assuming that these cases will have a greater variety of causative pathogens compared to other cases of cellulitis and soft tissue abscess that are associated with nail biting. Case identification was conducted using a computerized text-screening search that was refined by manual chart review. We included patients from 1 month to 20 years of age who underwent an incision and drainage (I&D) of a paronychia and had a culture obtained. The presence or absence of lymphangitis was determined from the clinical narrative in the medical record. We excluded patients treated with antibiotics prior to I&D as well as immune-compromised patients. We used descriptive statistics for prevalence and χ2 tests for categorical variables. RESULTS: Two hundred sixty-six patients met inclusion criteria. The median age was 9.7 years [IQR 4.7, 15.4] and 45.1% were female. Twenty-two patients (8.3%) had lymphangitic streaking associated with their paronychia. Patients with lymphangitis streaking were similar to those without lymphangitis in terms of age and sex (p = 0.52 and p = 0.82, respectively). Overall, the predominant bacteria was MSSA (40%) followed by MRSA (26%). No significant differences were found between the pathogens in the 22 patients with associated lymphangitis compared to the 244 patients without. CONCLUSION: Staphylococcus aureus represent the majority of pathogens in paronychia, although streptococcal species and gram-negative bacteria were also common. Among patients with paronychia of the finger, there seems to be no association between pathogen type and presence of lymphangitic streaking.

Utility of Blood Cultures and Empiric Antibiotics in Febrile Pediatric Hemophilia Patients With Central Venous Access Devices.

BACKGROUND: Children with hemophilia frequently require long-term central venous access devices (CVADs) for regular infusion of factor products. Hemophilia patients are not immunocompromised, but the presence and use of CVADs are associated with infections including bacteremia. Currently, the utility of blood cultures in evaluation of the febrile hemophilia patient with an indwelling CVAD is unknown, nor is optimal empiric antibiotic use. METHODS: We performed a retrospective cross-sectional study of febrile immunocompetent hemophilia patients with CVADs presenting to a large academic urban pediatric emergency department from 1995 to 2017. We used a natural language processing electronic search, followed by manual chart review to construct the cohort. We analyzed rate of pathogen recovery from cultures of blood in subgroups of hemophilia patients, the pathogen profile, and the reported pathogen susceptibilities to ceftriaxone. RESULTS: Natural language processing electronic search identified 181 visits for fever among hemophilia patients with indwelling CVADs of which 147 cases from 44 unique patients met study criteria. Cultures of blood were positive in 56 (38%) of 147 patients (95% confidence interval, 30%-47%). Seventeen different organisms were isolated (10 pathogens and 7 possible pathogens) with Staphylococcus aureus and coagulase-negative Staphylococcus species as the most common. Thirty-four percent of isolates were reported as susceptible to ceftriaxone. Positive blood cultures were more common in cases involving patients with inhibitors (n = 71) versus those without (n = 76), odds ratio, 7.4 (95% confidence interval, 3.5-15.9). This was observed irrespective of hemophilia type. CONCLUSIONS: Febrile immunocompetent hemophilia patients with indwelling CVADs have high rates of bacteremia. Empiric antimicrobial therapy should be targeted to anticipated pathogens and take into consideration local susceptibility patterns for Staphylococcus aureus.

Bacteriology of pediatric breast abscesses beyond the neonatal period.

BACKGROUND: Limited data exist regarding the presentation and bacteriology of nonneonatal pediatric breast abscess. OBJECTIVE: To determine the bacteriology and characteristic presentation of pediatric breast abscesses in a tertiary care center. METHODS: Cross-sectional study of patients age 1 month to 21 years admitted to a pediatric Emergency Department (ED) between 1996 and 2018 with a breast abscess. Patients with pre-existing conditions were excluded. Records were reviewed to determine demographics, history, physical exam findings, wound culture results, imaging and ED disposition. We used descriptive statistics to describe prevalence of different bacteria. RESULTS: We identified 210 patients who met study criteria. Median age was 13.6 years [IQR 6.6, 17.4], and 91% (191/210) were females. Ninety-two patients (43.8%) were 'pre-treated' with antibiotics prior to ED visit, and 33/210 (16%) were febrile. Ultrasound was obtained in 85 patients (40.5%), 69 patients had a single abscess and 16 had multiple abscesses. Most patients were treated with antibiotics and 100 had a surgical intervention, of these 89 had I&D and 11 a needle aspiration. Admission rate was 45%. Culture results were available for 75 (75%). Thirty-three (44%) had a negative culture, or grew non-aureus staphylococci or other skin flora. Culture were positive for MSSA 21 (28%), MRSA 13 (17%), Proteus mirabilis 2 (2.6%), Serratia 1 (1.3%). Other organisms include Gram-negative bacilli, group A Streptococcus and enterococcus. CONCLUSIONS: Non-neonatal pediatric breast abscess bacteriology is no different than data published on other skin abscesses. MRSA coverage should be considered based on local prevalence in skin infections.

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.

Four-Year Follow-Up of the World's First Pediatric Bilateral Hand-Forearm Transplants: Do They Grow as Expected?

BACKGROUND: In 2015, the first bilateral pediatric hand-forearm transplant was performed in an 8-year-old boy. The growth rate of the transplanted upper extremities was unknown at the time. METHODS: Forearm and hand radiographs were obtained annually. Radius and ulna measurements were performed by multiple coauthors and mathematically normalized using a standardized methodology. The Greulich and Pyle atlas was used to estimate hand bone age. RESULTS: From July of 2015 to July of 2019, unadjusted bone length (metaphysis to metaphysis) increased 38.8 and 39.6 mm for the left radius and ulna, and 39.5 and 35.8 mm for the right radius and ulna, respectively. Distal physes of the donor limbs increasingly contributed to overall bone length relative to proximal physes. Normalized growth between the two limbs was statistically similar. At each annual follow-up, the bone age increased by 1 year. CONCLUSIONS: Successful pediatric hand-forearm transplantation offers the advantage of growth similar to that of nontransplanted pediatric patients. The transplanted distal physes contributes more to the overall growth, paralleling normal pediatric growth patterns. Chronologic age parallels the increase in bone age. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Magnetic resonance angiography (MRA) in preoperative planning for patients with 22q11.2 deletion syndrome undergoing craniofacial and otorhinolaryngologic procedures.

INTRODUCTION: Patients with 22q11.2 deletion syndrome (22q11.2DS) have a variety of anatomic anomalies. For surgeons operating in proximity to the retropharynx, the most pertinent is medial displacement of the internal carotid arteries. The purpose of this study is to describe the preoperative use of magnetic resonance angiography (MRA) in surgical planning and update the incidence rate of medial carotid displacement in patients with 22q11.2DS. METHODS: This is a retrospective cohort study of patients with a confirmed diagnosis of 22q11.2 deletion and preoperative MRA <18 years old who underwent tonsillectomy, adenoidectomy, Furlow palatoplasty (FPP), posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), or submucosal cleft palate (SMCP) repair between January 1st, 2008 and December 31st, 2019. RESULTS: Ninety patients who met the inclusion criteria underwent 133 procedures. The majority identified as Caucasian (84.4%); 52.2% were female. Cervical MRA was more likely to be ordered before a PPF (80.9%) and tonsillectomy (72.7%) over a FPP (47.6%) or adenoidectomy (11.1%). Carotid medialization was visualized in 23 patients (25.6%) and was mild in 11 cases, moderate in 7 cases, and significant in 5 cases. There was no association between sex, race/ethnicity, or genetic diagnosis with carotid medialization. Flap shortening was necessary in 20% of PPF cases to avoid injuring the medialized vessel. CONCLUSION: Patients with 22q11.2DS may have higher rates of medialization of the carotid arteries than previously thought. Given the risk for complications in these patients during pharyngeal operations, there may be a critical place for MRA in surgical planning for patients with 22q11.2DS.

Sociodemographic Factors That Influence the Choice to Pursue Nasoalveolar Molding: One Pediatric Hospital's Experience.

OBJECTIVE: To identify demographic factors that influence choosing nasoalveolar molding (NAM) in the treatment of cleft lip with or without cleft palate (CL±P), and NAM treatment compliance. DESIGN: This work is a retrospective cohort study. SETTING: Tertiary pediatric hospital. PATIENTS, PARTICIPANTS: One hundred forty-nine patients with a diagnosis of unilateral complete CL±P receiving treatment when NAM was offered (January 1, 2008-July 26, 2016). MAIN OUTCOME MEASURE(S): Demographic variables collected included race, ethnicity, ZIP code, number of caregivers, caregiver employment status, and health insurance status. Medical variables collected included diagnosis, treatment pursued, compliance with NAM, completion of NAM, and the treating cleft surgeon and orthodontist. Data were analyzed via Fisher exact tests, χ2 tests, and multivariate logistic regression to identify factors that influence the decision to pursue NAM and treatment compliance. RESULTS: Univariate analyses identified the following significant factors predicting the pursuit of NAM: race and insurance type (both P < .001), surgeon (P = .005), income level (P = .009), comorbidities (P = .015), and syndromic diagnosis (P = .033). Driving distance trended toward significance (P = .078). Multivariate regression analyses indicated that Asian race (P = .047), insurance type (P = .046), driving distance (P = .019), and surgeon (P = .017) were significant predictors of pursuit of NAM. CONCLUSIONS: There are disparities in patient choice of NAM at our center for children with complete cleft lip. African American patient families were less likely to pursue this intervention. A stronger understanding of the barriers that lower income and minority patients face is needed in order to better characterize disparities in cleft care.

Cultured Epidermal Autograft for Total Scalp Reconstruction in a Neonate Following Necrotizing Fasciitis.

Necrotizing fasciitis in the pediatric population is a particularly difficult diagnostic and management challenge. Options for soft tissue reconstruction of wounds following surgical debridement have been historically limited, yet recent advancements in bioengineered tissue and matrices have introduced alternative methods of treatment for these patients. We present a case of neonatal necrotizing fasciitis of the scalp requiring full-thickness surgical debridement, which was successfully reconstructed using Epicel cultured epidermal autograft (CEA). A 4-day-old female neonate (gestational age 40 weeks) presented with scalp erythema, blistering, and sepsis following peripartum fetal scalp electrode monitoring. She underwent surgical excision of the scalp to healthy bleeding tissue resulting in a defect of approximately 97% of the scalp, measuring 18 × 19 cm including 4 × 3.5 cm of exposed bone at the occiput. Initial provisional coverage of the defect was obtained with Integra collagen matrix bilayer dressing to stimulate granulation over exposed bone. Concurrently, a 2 × 4-cm excisional biopsy of the left groin skin was obtained for CEA in vitro expansion over 21 days. Then, autograft sheets were applied to achieve total scalp coverage. Clinical assessments at 1 week, 1 month, and 2 months postoperatively exhibited, approximately, 60% take, 80% take, and 90% take, respectively. Scalp involvement in neonatal necrotizing infections is a notably rare presentation as surmised by our review of the literature, and to our knowledge, this is the first report on the use of Integra and CEA for near-total neonatal scalp coverage.

Retinal Function in X-Linked Juvenile Retinoschisis.

Purpose: To assess retinal function in young patients with X-linked juvenile retinoschisis (XLRS), a disorder that is known to alter ERG postreceptor retinal components and also possibly photoreceptor components. Methods: ERG responses to full-field stimuli were recorded under scotopic and photopic conditions in 12 XLRS patients aged 1 to 15 (median 8) years. A- and b-wave amplitudes and implicit times were examined over a range of stimulus intensities. Rod and cone photoreceptor (SROD, RROD, SCONE, RCONE) and rod-driven postreceptor (log σ, VMAX) response parameters were calculated from the a- and b-waves. Data from XLRS patients were evaluated for significant change with age. Results: A- and b-wave amplitudes were smaller in XLRS patients compared with controls under both scotopic and photopic conditions. Saturated photoresponse amplitude (RROD), postreceptor b-wave (log σ), and saturated b-wave amplitude (VMAX) were significantly lower in XLRS patients than in controls; SROD did not differ between the two groups. SCONE and RCONE values were normal. In XLRS patients, neither a- and b-wave amplitudes nor calculated parameters (SROD, RROD, log σ, VMAX,SCONE, and RCONE) changed with age. Conclusions: In these young XLRS patients, RROD and a-wave amplitudes were significantly smaller than in controls. Thus, in addition to XLRS causing postreceptor dysfunction, an effect of XLRS on rod photoreceptors cannot be ignored.

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.