The Forgotten Flap: The Pedicled Trapezius Flap's Utility in Pediatric Head and Neck Reconstruction-A Systematic Review.

BACKGROUND: When free tissue transfer is precluded or undesired, the pedicled trapezius flap is a viable alternative for adults requiring complex head and neck (H&N) defect reconstruction. However, the application of this flap in pediatric reconstruction is underexplored. This systematic review aimed to describe the use of the pedicled trapezius flap and investigate its efficacy in pediatric H&N reconstruction. METHODS: A systematic review was performed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Articles describing the trapezius flap for H&N reconstruction in pediatric patients were included. Patient demographics, surgical indications, wound characteristics, flap characteristics, complications, and functional outcomes were abstracted. RESULTS: A systematic review identified 22 articles for inclusion. Studies mainly consisted of case reports (n = 11) and case series (n = 8). In total, 67 pedicled trapezius flaps were successfully performed for H&N reconstruction in 63 patients. The most common surgical indications included burn scar contractures (n = 46, 73.0%) and chronic wounds secondary to H&N masses (n = 9, 14.3%). Defects were most commonly located in the neck (n = 28, 41.8%). The mean flap area and arc of rotation were 326.4 ± 241.7 cm2 and 157.6 ± 33.2 degrees, respectively. Most flaps were myocutaneous (n = 48, 71.6%) and based on the dorsal scapular artery (n = 32, 47.8%). Complications occurred in 10 (14.9%) flaps. The flap's survival rate was 100% (n = 67). No instances of functional donor site morbidity were reported. The mean follow-up was 2.2 ± 1.8 years. CONCLUSION: This systematic review demonstrated the reliability of the pedicled trapezius flap in pediatric H&N reconstruction, with a low complication rate, no reports of functional donor site morbidity, and a 100% flap survival rate. The flap's substantial surface area, bulk, and arc of rotation contribute to its efficacy in covering soft tissue defects ranging from the proximal neck to the vertex of the scalp. The pedicled trapezius flap is a viable option for pediatric H&N reconstruction.

Risk Factors for Delayed Diagnosis of Positional Plagiocephaly: A Review of 25,322 Patients.

OBJECTIVE: This study identifies risk factors for late positional plagiocephaly (PP) diagnosis and impact on helmet therapy. DESIGN: We conducted a retrospective review of all patients diagnosed with PP over 10 years at five Southern California hospitals. SETTING: Patients diagnosed with PP at an included hospital. PATIENTS: 25,332 patients were diagnosed with PP over 10 years. INTERVENTIONS: Patients diagnosed with PP early (< = 6 months) and late (>6 months) were compared. MAIN OUTCOME MEASURES: Cohorts were evaluated for demographics, gestational history, associated conditions, and hospitalizations through direct comparison, logistic regression, and correlation analyses. Rates of referrals and helmet orders were compared. RESULTS: Of patients reviewed, 4.8% (n = 1216) were diagnosed late. On multivariate analysis, late diagnoses were more likely Hispanic or Black/African-American. Early gestational age, hydrocephalus, and VP shunt were more frequent in late diagnoses. Patients diagnosed late had longer NICU and overall hospital stays. Earlier gestational age, longer NICU or overall hospital stay correlated with later age at PP diagnosis. 8.9% of patients were referred for helmet therapy evaluation. Patients diagnosed late were 2.63 and 1.64 times as likely to be referred and require helmet therapy, respectively. CONCLUSIONS: Patients who are Hispanic or Black/African-American, premature, have hydrocephalus, or VP shunt have higher rates of delayed PP diagnosis. Shorter gestational age or longer NICU or hospital stay correlates with later diagnosis, which increases helmet therapy requirements. Additional interventions are needed for at risk patients to routinely evaluate for and minimize the risk of developing PP.

"Healthcare Disparities in the Care of Children with Craniosynostosis in the United States: A Systematic Review".

BACKGROUND: Healthcare inequity is a pressing concern in pediatric populations with craniofacial conditions. Little is known about the barriers to care affecting children with craniosynostosis. This systematic review investigates disparities impacting care for children with craniosynostosis in the U.S. METHODS: A comprehensive literature search was performed in the following databases from inception to December 2022: Ovid MEDLINE, Ovid EMBASE, and The Cochrane Library. Studies were screened for eligibility by two authors. All original articles that focused on disparities in access, treatment, or outcomes of craniosynostosis surgery were included. Studies describing disparities in other countries, those not written English, and review articles were excluded (Figure 1). RESULTS: An initial database search revealed 607 citations of which 21 met inclusion criteria (Figure 1). All included studies were retrospective reviews of databases or cohorts of patients. The results of our study demonstrate that barriers to access in treatment for craniosynostosis disproportionally affect minority children, children of non-English speaking parents and those of lower socioeconomic status or with Medicaid. Black and Hispanic children, non-English speaking patients, and children without insurance or with Medicaid were more likely to present later for evaluation, ultimately undergoing surgery at an older age. These patients were also more likely to experience complications and require blood transfusions compared to their more privileged, white peers. CONCLUSIONS: There is a discrepancy in treatment received by minority patients, patients with Medicaid, and those who are non-English speaking. Further research is needed to describe the specific barriers that prevent equitable care for these patients.

Rethinking the Rule of 10s: Early Cleft Lip Repair Improves Weight Gain.

OBJECTIVE: This study compares patients undergoing early cleft lip repair (ECLR) (<3-months) and traditional lip repair (TLR) (3-6 months) with/without nasoalveolar molding (NAM) to evaluate the effects of surgical timing on weight gain in hopes of guiding future treatment paradigms. DESIGN: Retrospective review. SETTING: Children's Hospital of Los Angeles, California. PATIENT, PARTICIPANTS: A retrospective chart review evaluated patients who underwent ECLR or TLR ± NAM from November 2009 through January 2020. INTERVENTIONS: No intervention was performed. MAIN OUTCOME MEASURE(S): Patient demographics, birth and medical history, perioperative variables, and complications were collected. Infant weights and age-based percentiles were recorded at birth, surgery, 8-weeks, 6-months, 12-months, and 24-months postoperatively. The main outcomes were weight change and weight percentile amongst ECLR and TLR ± NAM groups. RESULTS: 107 patients met inclusion criteria: ECLR, n = 51 (47.6%); TLR + NAM, n = 35 (32.7%); and TLR-NAM, n = 21 (19.6%). ECLR patients had significantly greater changes in weight from surgery to 8-weeks and from surgery to 24-months postoperatively compared with both TLR ± NAM (P < .05). Age-matched weights in the ECLR group were significantly greater than TLR ± NAM at multiple time points postoperatively (P < .05). CONCLUSIONS: ECLR significantly increased patient weights 24-months postoperatively when compared to TLR ± NAM. Specifically compared to TLR-NAM, ECLR weights were significantly greater at all time points past 6-months postoperatively. The results of this study demonstrate that ECLR can mitigate feeding difficulties and malnutrition traditionally seen in patients with cleft lip.

Tympanostomy Tubes: Are They Necessary? A Systematic Review on Implementation in Cleft Care.

OBJECTIVE: To understand the indication for and the effects of early ventilation tube insertion (VTI) on hearing and speech for patients with cleft lip and/or palate (CLP). DESIGN: We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA)-guided systematic review of relevant literature. SETTING: Setting varied by geographical location and level of clinical care across studies. PATIENTS, PARTICIPANTS: Patients with CLP who underwent VTI were included. INTERVENTIONS: No interventions were performed. MAIN OUTCOME MEASURE(S): Primary outcome measures were hearing and speech following VTI. Secondary outcome measures were tube-related and middle ear complications. Early VTI occurred before or at time of palatoplasty while late VTI occurred after palatoplasty. RESULTS: Twenty-three articles met inclusion criteria. Articles varied among study design, outcome measures, sample size, follow-up, and quality. Few studies demonstrated support for early VTI. Many studies reported no difference in hearing or speech between early and late VTI. Others reported worse outcomes, greater likelihood of complications, or needing repeat VTI following early tympanostomy placement. Several studies had significant limitations, including confounding variables, small sample size, or not reporting on our primary outcome. CONCLUSIONS: No consistency was found regarding which patients would benefit most from early VTI. Given the aforementioned variability and sub-optimal methodologies, additional studies are warranted to provide stronger evidence regarding VTI timing in cleft care.

High prevalence of deleterious mutations in concomitant nonsyndromic cleft and outflow tract heart defects.

Our previous work demonstrating enrichment of outflow tract (OFT) congenital heart disease (CHD) in children with cleft lip and/or palate (CL/P) suggests derangements in common underlying developmental pathways. The current pilot study examines the underlying genetics of concomitant nonsyndromic CL/P and OFT CHD phenotype. Of 575 patients who underwent CL/P surgery at Children's Hospital Los Angeles, seven with OFT CHD, negative chromosomal microarray analysis, and no recognizable syndromic association were recruited with their parents (as available). Whole genome sequencing of blood samples paired with whole-blood-based RNA sequencing for probands was performed. A pathogenic or potentially pathogenic variant was identified in 6/7 (85.7%) probands. A total of seven candidate genes were mutated (CHD7, SMARCA4, MED12, APOB, RNF213, SETX, and JAG1). Gene ontology analysis of variants predicted involvement in binding (100%), regulation of transcription (42.9%), and helicase activity (42.9%). Four patients (57.1%) expressed gene variants (CHD7, SMARCA4, MED12, and RNF213) previously involved in the Wnt signaling pathway. Our pilot analysis of a small cohort of patients with combined CL/P and OFT CHD phenotype suggests a potentially significant prevalence of deleterious mutations. In our cohort, an overrepresentation of mutations in molecules associated with Wnt-signaling was found. These variants may represent an expanded phenotypic heterogeneity within known monogenic disease genes or provide novel evidence of shared developmental pathways. The mechanistic implications of these mutations and subsequent developmental derangements resulting in the CL/P and OFT CHD phenotype require further analysis in a larger cohort of patients.

Increased Distraction Magnitude Leads to Greater Reduction in the Apnea-Hypopnea Index in Infants With Robin Sequence.

PURPOSE: Upper airway obstruction seen in Robin Sequence (RS) is commonly treated with mandibular distraction osteogenesis (MDO). The purpose of this study is to evaluate the impact of distraction distance on sleep study outcomes in patients with obstructive sleep apnea (OSA) secondary to RS. METHODS: A retrospective cohort study was conducted for patients with isolated RS who underwent MDO at Children's Hospital Los Angeles between January 2006-September 2021. The predictor variable was distraction distance (maximal distraction using a 30 mm device vs sub-maximal distraction), and the primary outcome variable was OSA scores. Relationships between covariates, including demographic characteristics, preoperative sleep variables, and postoperative OSA outcomes using polysomnography, were also analyzed. Descriptive statistics and tests of statistical significance were performed using the Statistical Package for Social Sciences (SPSS) (version 28.0), including Student's t-test, proportions testing, multiple linear regression, and correlation analysis. RESULTS: Seventy-one patients met inclusion criteria (39.4% female, 60.6% male). Average age at MDO was 3.0 ± 10.2 months. Fifty-six patients were distracted maximally with a 30 mm distractor, while the remaining 15 patients experienced shorter distraction due to distractor limitations (25 mm distractor), persistent infection or family request. Looking at absolute values of postoperative sleep study variables, there were no significant differences between patients who were maximally and sub-maximally distracted across apnea-hypopnea index (AHI), highest carbon dioxide, lowest oxygen saturation, and oxygen requirement. However, both cohorts demonstrated significant improvements in lowest oxygen saturation, AHI, highest carbon dioxide level, and highest oxygen requirement compared to their pre-distraction levels. Compared with patients distracted <30 mm, maximal distraction had a significantly greater improvement in AHI when controlling for preoperative sleep study variables (P = .047). CONCLUSION: Patients with isolated RS who have more severe OSA experienced greater improvements in AHI, oxygen requirement, and oxygen saturation after MDO. Two-thirds of patients no longer had oxygen requirements after MDO. Our results suggest that MDO is helpful in treating patients with RS regardless of distraction distance. However, our study provides evidence that increasing the distraction distance may further improve AHI, which is particularly beneficial to patients with a significant preoperative AHI.

Midface Growth Potential in Unoperated Clefts: A Systematic Review and Meta-Analysis.

ABSTRACT: Competing hypotheses for the development of midface hypoplasia in patients with cleft lip and palate include both theories of an intrinsic restricted growth potential of the midface and extrinsic surgical disruption of maxillary growth centers and scar growth restriction secondary to palatoplasty. The following meta-analysis aims to better understand the intrinsic growth potential of the midface in a patient with cleft lip and palate unaffected by surgical correction. A systematic review of studies reporting cephalometric measurements in patients with unoperated and operated unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), and isolated cleft palate (iCP) abstracted SNA and ANB angles, age at cephalometric analysis, syndromic diagnosis, and patient demographics. Age and Region-matched controls without cleft palate were used for comparison. SNA angle for unoperated UCLP (84.5 ±â€Š4.0°), BCLP (85.3 ±â€Š2.8°), and ICP (79.2 ±â€Š4.2°) were statistically different than controls (82.4 ±â€Š3.5°), (all P ≤ 0.001). SNA angles for operated UCLP (76.2 ±â€Š4.2°), BCLP (79.8 ±â€Š3.6°), and ICP (79.0 ±â€Š4.3°) groups were statistically smaller than controls (all P ≤ 0.001). SNA angle in unoperated ICP (n = 143) was equivalent to operated ICP patients (79.2 ±â€Š4.2° versus 79.0 ±â€Š4.3° P = 0.78). No unoperated group mean SNA met criteria for midface hypoplasia (SNA < 80). Unoperated UCLP/BLCP exhibit a more robust growth potential of the maxilla, whereas operated patients demonstrate stunted growth compared to normal phenotype. Unoperated ICP demonstrates restricted growth in both operated and unoperated patients. As such, patients with UCLP/BCLP differ from patients with ICP and the factors affecting midface growth may differ.Level of Evidence: IV.

The Local Mission: Improving Access to Surgical Care in Middle-Income Countries.

BACKGROUND: Billions of people lack access to quality surgical care. Short-term missions are used to supplement the delivery of surgical care in regions with poor access to care. Traditionally known for using international teams, Operation Smile has transitioned to using a local mission model, where surgical service is delivered to areas of need by teams originating within that country. This study investigates the proportion and location of Operation Smile missions that use the local mission model. METHODS: A retrospective review was performed of the Operation Smile mission database for fiscal years 2014 to 2019. Missions were classified into local or international missions. Countries were also classified by their income levels as well as their specialist surgical workforce (SAO) density. As no individual patient or provider data was recorded, ethics board approval was not warranted. RESULTS: Between 2014 and 2019, Operation Smile held an average of 144.8 (range 135-154) surgical missions per year. Local missions accounted for 97 ± 5.6 (67%) of the missions. Of the 34 program countries, 26 (76%) used local missions. Of the countries that had only international missions, six (75%) were low-income countries and the average SAO density was 1.54 (range 0.19-5.88) providers per 100,000 people. Of the countries with local missions, 24 (92%) were middle-income, and the average SAO density was 30.9 (range 3.4-142.4). CONCLUSION: International investments may assist in the creation of local surgical teams. Once teams are established, local missions are a valuable way to provide specialized surgical care within a country's own borders.

Impact of an All-Female Surgical Team on Moroccan Patient Perspectives of Female Healthcare Providers.

INTRODUCTION: Increasing numbers of women in medicine could address Morocco's 5.5-fold deficit in surgical providers. Cultural perceptions towards women limit female advancement in healthcare. This study evaluates the impact of an all-female surgical team on Moroccan attitudes. OBJECTIVE: This study aimed to evaluate how attitudes towards female healthcare professionals changed for Moroccan patients after exposure to a unique, all-female medical environment. METHODS: Cleft patients were surveyed after a surgery mission with all-female volunteers in Oujda, Morocco. Analysis included quantitative, qualitative, and mixed-methods approaches. RESULTS: Of 121 respondents (94%), 85% and 77% had prior exposure to a female nurse or doctor, respectively. 94% of respondents strongly agreed to receiving high-quality care. 75% developed increased confidence in female providers. 68% and 69% of respondents, regardless of gender (p = 0.950), felt that having a female nurse or doctor did not impact care. Female patients were more likely than male patients to strongly encourage female relatives to pursue medical careers (p = 0.027). Respondents without prior exposure to female nurses were more likely to: pursue medical careers (p = 0.034), believe female relatives could pursue medical careers (p = 0.006), and encourage them to do so (p = 0.011). CONCLUSIONS: Increased visibility of women improved patient attitudes towards female providers, especially in patients without prior exposure. Initiatives that increase female representation in healthcare may have greater effects in cultures with more gender inequity.

A Meta-Analysis of Palatal Repair Timing.

ABSTRACT: Developing midface hypoplasia is common after palatoplasty and has been hypothesized to be influenced by the timing of hard palate repair. This meta-analysis assesses the risk of developing midface hypoplasia based on age at hard palate repair. A Pubmed PRISMA systematic review and meta-analysis was completed for literature focused on palatoplasty and midface hypoplasia published between 1970 and 2019. Cephalometric data were extracted and categorized by age at hard palate repair: <6, 7 to 12, 13 to 18, 19 to 24, and 25 to 83 months. Analysis of these groups and a control were compared using independent T-tests and Spearman correlation coefficients. SNA angles for each group were 77.9 ±â€Š3.1° (<6 months), 77.7 ±â€Š4.2° (7-12 months), 78.7 ±â€Š4.2° (13-18 months), 75.1 ±â€Š4.2° (19-24 months), 75.5 ±â€Š4.8° (25-83 months), and were statistically different than the control group 82.4 ±â€Š3.5° (P < 0.0001). Hard palate repair at 13 to 18 months had a statistically significant greater SNA angle than all other groups except for the repair at <6 months group (P = 0.074). As age at hard palate closure increased beyond 18 months, the SNA decreased, corresponding to a more hypoplastic maxilla (Spearman's correlation coefficient -0.381, P = 0.015). Analysis suggests that younger age at the time of repair is less likely to create in midface hypoplasia in adulthood. Minimizing midface hypoplasia in cleft palate patients by optimizing algorithms of care is a practical way to decrease the burden of disease on patients, families and medical systems. Further studies are needed to evaluate the role of technique on outcomes.Level of Evidence: IV.

The Likelihood of Orthognathic Surgery After Orofacial Cleft Repair.

ABSTRACT: Midface hypoplasia is one of the most significant sequelae of cleft lip and/or palate surgery. A complete understanding of the rate of orthognathic surgery across varying cleft phenotypes is a powerful tool for educating patients and families as to the treatment course that the patient will incur during their lifetime. Understanding the average rates of orthognathic intervention also can act to develop metrics for outcome evaluation with different treatment protocols. Attempting to identify the average rates of orthognathic intervention, the authors conducted a systematic review and meta-analysis by combining studies from 1987 to 2016 describing the frequency of orthognathic intervention on the different cleft phenotypes as the primary outcome. Secondary outcomes included identification of surgical protocol, age of patient at orthognathic intervention, and the method by which patients were evaluated for orthognathic intervention. The rate of orthognathic surgery was 38.1% for bilateral cleft lip and palate (BCLP), 30.2% for unilateral cleft lip and palate (UCLP), 4.4% for isolated cleft palate (ICP), and 1.8% for patients with isolated cleft lip (ICL). 71% (n = 10) reported using lateral cephalograms for orthognathic surgery evaluation and only one of those studies reported specific objective cephalometric measurements for orthognathic intervention. Our findings demonstrated that BCLP possessed the highest rate of orthognathic intervention followed by UCLP, ICP, and ICL. ICP and ICL both possessed low rates of orthognathic intervention. By sharing our findings, the authors hope to provide a useful tool for informing patients' families as to their risk of needing orthognathic intervention.

The Evolution of Unilateral Cleft Lip Repair.

ABSTRACT: The understanding of cleft lip etiology and approaches for surgical repair have evolved over time, allowing for improved ability to restore form and function. The variability of cleft lip presentations has necessitated a nuanced surgical approach with multidisciplinary cleft care. The earliest documentation of unilateral cleft lip repair predates the 19th century, with crude outcomes observed before the advent of curved incisions and advancement flaps. In the 20th century, straight line, quadrilateral flap, and triangular flap repairs were introduced to mitigate post-repair surgical scarring, increase lip length, and restore the symmetry of the Cupid's bow. Towards the latter part of the century, the development of rotation-advancement principles allowed for improved functional and aesthetic outcomes. Future technical improvements will continue to address the goals of lip and nasal symmetry, muscular continuity, precise scar concealment, and improved patient satisfaction in an increasing range of cleft phenotypes and during subsequent years of growth.

Factors Associated With Delay in Cleft Surgery at a Tertiary Children's Hospital in a Major US Metropolitan City.

OBJECTIVE: To identify factors associated with late cleft repair at a US tertiary children's hospital. DESIGN: Retrospective study of children with CL/P using Children's Hospital Los Angeles (CHLA) records. SETTING: US tertiary children's hospital. PATIENTS/PARTICIPANTS: Patients undergoing primary CL or CP repair at CHLA from 2009 to 2018. MAIN OUTCOME MEASURES: Proportion of children who had delayed primary CL repair or CP repair using CHLA and American Cleft Palate-Craniofacial Association (ACPA) guidelines and factors associated with late surgery. RESULTS: In total, 805 patients-503 (62.5%) who had CL repair, 302 (37.5%) CP repair-were included. Using CHLA protocol, 14.3% of patients seeking CL repair had delayed surgery. Delay was significantly associated with female gender, non-Hispanic ethnicity, Spanish primary language, government insurance, bilateral cleft, cleft lip and palate (CLP), and syndromic diagnosis. Using ACPA guidelines, 5.4% had delayed surgery. Female gender and syndromic diagnosis were significantly associated with delay and remained significant after adjustment for confounders in multivariate models. For CP repair, 60.3% of patients had delayed surgery using CHLA protocol. Cleft lip and palate diagnosis, complete cleft, syndromic diagnosis, and longer travel distance were significantly associated with delay. Using ACPA guidelines, 28.5% had delayed surgery; however, significant association with patient variables was not consistently observed. CONCLUSIONS: Delay in cleft surgery occurs most often for patients seeking CP repair and is associated with female gender, non-Hispanic ethnicity, Spanish language, government insurance, and bilateral CL, CLP, or syndromic diagnoses. Initiatives should aim to optimize cleft surgery delivery for these subpopulations.

The experiences of female surgeons around the world: a scoping review.

INTRODUCTION: The Lancet Commission for Global Surgery identified an adequate surgical workforce as one indicator of surgical care accessibility. Many countries where women in surgery are underrepresented struggle to meet the recommended 20 surgeons per 100,000 population. We evaluated female surgeons' experiences globally to identify strategies to increase surgical capacity through women. METHODS: Three database searches identified original studies examining female surgeon experiences. Countries were grouped using the World Bank income level and Global Gender Gap Index (GGGI). RESULTS: Of 12,914 studies meeting search criteria, 139 studies were included and examined populations from 26 countries. Of the accepted studies, 132 (95%) included populations from high-income countries (HICs) and 125 (90%) exclusively examined populations from the upper 50% of GGGI ranked countries. Country income and GGGI ranking did not independently predict gender equity in surgery. Female surgeons in low GGGI HIC (Japan) were limited by familial support, while those in low income, but high GGGI countries (Rwanda) were constrained by cultural attitudes about female education. Across all populations, lack of mentorship was seen as a career barrier. HIC studies demonstrate that establishing a critical mass of women in surgery encourages female students to enter surgery. In HICs, trainee abilities are reported as equal between genders. Yet, HIC women experience discrimination from male co-workers, strain from pregnancy and childcare commitments, and may suffer more negative health consequences. Female surgeon abilities were seen as inferior in lower income countries, but more child rearing support led to fewer women delaying childbearing during training compared to North Americans and Europeans. CONCLUSION: The relationship between country income and GGGI is complex and neither independently predict gender equity. Cultural norms between geographic regions influence the variability of female surgeons' experiences. More research is needed in lower income and low GGGI ranked countries to understand female surgeons' experiences and promote gender equity in increasing the number of surgical providers.

Outcomes of Intracranial Versus Subcranial Approaches to the Frontofacial Skeleton.

PURPOSE: An intracranial approach to the craniomaxillofacial skeleton can be effective for correcting complex craniofacial dystoses such as frontofacial hypoplasia, hypertelorism, and orbital dystopia. However, the significant morbidity resulting from the high complication rates has limited intracranial use. Given the need for intracranial approaches for certain clinical indications, the present study reevaluated intracranial frontofacial procedures to determine their safety and outcomes. PATIENTS AND METHODS: A retrospective review was performed of all frontofacial procedures completed between 2007 and 2017 at a single institution. Patients who had undergone monobloc distraction alone or with facial bipartition, facial bipartition alone, or box osteotomy were included in the intracranial cohort. Those who had undergone Le Fort III distraction, advancement, or a combination with Le Fort I were included in the subcranial cohort. The recorded data included demographics, previous craniofacial surgery, and operative events. The complications rates were compared between the 2 cohorts. RESULTS: The present study included 65 patients-35 subcranial and 30 intracranial. The rates of previous craniomaxillofacial (P = .193) and intracranial (P = .340) surgery were equivalent between the 2 cohorts. Of the 30 intracranial and 35 subcranial patients, 26.7% and 34.3% experienced complications (P = .218). The intracranial patients experienced more dural tears (53.3 vs 5.7%; P < .0001); however, no significant differences were observed in cerebrospinal fluid leakage. Reintubations (n = 3; 4.5%) occurred exclusively in the subcranial group. No significant differences in the major and minor complication rates were observed between the 2 cohorts. CONCLUSIONS: Intracranial and subcranial frontofacial procedures are associated with an equal risk of major and minor complications. Given the lack of an increase in risk, intracranial frontofacial procedures should be considered for the management of complex craniofacial dystoses.

The Monobloc Distraction With Facial Bipartition: Outcomes of Simultaneous Multidimensional Facial Movement Compared With Monobloc Distraction or Facial Bipartition Alone.

BACKGROUND: Although combined monobloc facial bipartition with distraction (MFBD) may simultaneously correct multiple facial dimensions in patients with syndromic craniosynostoses, complication risks limit its use. This study reassesses MFBD complications and outcomes to compare safety and efficacy to monobloc distraction (MD) and facial bipartition (FB) alone. METHODS: A retrospective review of MFBD, MD, and FB cases for 10 years at a tertiary children's hospital was performed. Patient demographics and surgical variables were compared between cohorts. Distraction distance was compared between MFBD and MD. Correction of interdacryon distance in MFBD compared with FB was measured on preoperative and postoperative computed tomographic scans. SPSS 17 was used for data analysis. RESULTS: Twenty-two total patients, 11 MFBD, 4 MD, and 7 FB, met the inclusion criteria. Three MFBD (27.3%) patients experienced complications, including 1 osteomyelitis and 2 hardware displacements. One MD patient (25%) experienced a postoperative complication consisting of a wound infection. Three FB patients (42.9%) experienced either cerebrospinal fluid leak, seroma, mucocele, hardware exposure, and/or orbital dystopia (n = 1 each). Patients with MFBD had significantly longer intensive care unit stay (P ≤ 0.05), but no difference in hospital stay (P = 0.421). Mean distraction length was similar between MFBD and MD (P = 0.612). There was no significant difference in final (P = 0.243) or change (P = 0.189) in interdacryon distance between MFBD and FB patients. CONCLUSIONS: In our experience, MFBD has similar complication rates compared with MD and FB alone. Given equivalent safety and postoperative correction of facial dimensions, MFBD could be more widely considered for select patients.

Congenital Heart Disease in Patients With Cleft Lip/Palate and Its Impact on Cleft Management.

OBJECTIVE: To evaluate characteristics of congenital heart disease (CHD) in patients with cleft lip and/or palate (CL/P) and assess potential associations with cleft outcomes. DESIGN: Retrospective review of all patients with CL/P who underwent primary cleft treatment from 2009 to 2015. SETTING: Children's Hospital Los Angeles, a tertiary hospital. PATIENTS: Exclusion criteria included microform cleft lip diagnosis, international patients, and patients presenting for secondary repair or revision after primary repair at another institution. MAIN OUTCOMES MEASURED: Patient demographics, prenatal and birth characteristics, CL/P characteristics, syndromic status, postoperative complications, and other outcomes were analyzed relative to CHD diagnoses and management. Patients with CL/P with (+CHD) were compared to those without (-CHD) CHD using χ2 tests and analysis of variance. RESULTS: Among 575 patients with CL/P, 83 (14.4%) had CHD. Congenital heart disease rates were significantly higher in patients with cleft palate (CP) compared to other cleft types (χ2, P = .009). Eighty-one (97.6%) out of 83 +CHD patients were diagnosed prior to initial CL/P surgical assessment. Twenty-three (27.7%) +CHD patients required surgical repair of 10 cardiac anomalies prior to cleft care. Congenital heart disease was associated with delayed CP repair and increased rates of fistula in isolated patients with CP. CONCLUSIONS: Congenital heart disease is known to be more prevalent in patients with CL/P. These data suggest the condition is particularly increased in patients with CP. Severe forms of CHD are diagnosed and treated prior to cleft care however postoperative fistula may be more common in patients with CHD. Therefore, careful attention is required for patient optimization and palatal flap dissection in patients with coexisting CHD and CL/P.

The Prevalence of Congenital Heart Disease in Nonsyndromic Cleft Lip and/or Palate: A Systematic Review of the Literature.

BACKGROUND: Life-threatening anomalies, such as congenital heart disease (CHD) must be identified in patients with cleft lip and/or palate (CL/P) to minimize perioperative risk. Nevertheless, screening practices vary highly among cleft teams and programs, and little is known about the prevalence and clinical significance of CHD in nonsyndromic CL/P patients. Through a systematic literature review, this study examines the demographics and severity of CHD in the nonsyndromic CL/P population. The implications of concomitant CHD in providing safe and comprehensive cleft care both in the United States and abroad are discussed. METHODS: A systematic review of PubMed literature from 1980 to September 2015 was performed following PRISMA guidelines. Studies describing rates of CHD and severity of lesions specifically in nonsyndromic and all CL/P patients were included. Analysis of cumulative data was performed according to nonsyndromic status and cleft type. RESULTS: Twelve studies were found to meet inclusion criteria. Of the 4055 nonsyndromic CL/P patients who met inclusion criteria, 7.42% (n=301) had CHD, which was significantly greater than the general population (~1%; odds ratio [OR], 7.94; P<0.0001). Congenital heart disease was significantly more common in cleft palate (CP) (OR, 15.1), combined CL and palate (CL+P) (OR, 13.5), and CL (OR, 4.23) compared with the general population. Palatal clefts (CP and CL+P) had significantly increased odds of CHD compared with CL (OR, 3.58 and 3.19, respectively, both P<0.0001). The most common forms of CHD were atrial or ventricular septal defects (n=210, 74.2%), which typically do not require surgical intervention in the general population. Clinical significance of these CHD lesions in CL/P patients is not fully known. CONCLUSIONS: Cleft management programs aim to maximize the number of patients receiving care while maintaining patient safety. Appropriate evaluation of perioperative risk necessitates understanding the prevalence of CHD in CL/P patients and the severity of those lesions. Patients with CL/P, particularly patients with palatal clefts, have significantly higher odds of having CHD than the general population. Congenital heart disease is most likely to present as atrial or ventricular septal defects, which are lesions that are unlikely to impact safety during cleft repair surgery.