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Background: The CLEFT-Q is a validated instrument designed to elicit patient-reported outcomes among people affected by cleft lip and/or palate. However, it has not been reported how use of CLEFT-Q data alters routine cleft care. This study analyzed the impact of CLEFT-Q data integration on patient care and clinical decision-making., Methods: Patients were sequentially, prospectively evaluated during scheduled cleft team visits. The CLEFT-Q was completed before the clinic encounter, but results were initially masked from the surgeon and family. In the encounter, a study observer characterized patients' verbalized attitudes across 7 specific domains of appearance and function, and the provisional assessment and plan was noted. CLEFT-Q data were then introduced into the clinical encounter and discussed. Discordance between patients' initially verbalized attitudes and their self-reported scores on the CLEFT-Q was documented along with any resultant modifications to their care plan., Results: Seventy patient visits were observed; the mean patient age was 12.7 years (range, 8 to 19 years). Forty-one patients (59%) had cleft lip and palate/alveolus and 29 (41%) had isolated cleft palate. Discordance was observed in 36% of visits and in 9.2% of specific domains assessed. Highest discordance rates were observed in domains of psychosocial function (12.5%), speech function/distress (11.6%), and lips/lip scar appearance (11.6%). No age group or sex was associated with increased discordance. Integration of CLEFT-Q results altered the assessment and plan in 11 visits (16%)., Conclusion: The CLEFT-Q provides clinically relevant insight into patient perspectives that are not captured by routine interview and examination alone, and regularly leads to a change in the management plan., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Background: This study updates the authors' institutional experience with modified Furlow palatoplasty, evaluating speech outcomes and incidence of secondary speech surgery throughout development and at skeletal maturity., Methods: Nonsyndromic patients undergoing primary modified Furlow palatoplasty between 1985 and 2005 with postoperative speech evaluations were reviewed retrospectively. Secondary speech surgery and Pittsburgh Weighted Speech Scale (PWSS) scores before secondary speech or orthognathic operations were assessed in the groups aged 5 to 7 years, 8 to 11 years, 12 to 14 years, and older than 15 years and analyzed by Veau cleft type., Results: A total of 551 patients with 895 total speech assessments were analyzed. Of 364 patients followed up to age 15 years or older, 19.8% underwent secondary speech surgery. Speech assessment of patients aged 15 years or older without prior secondary speech surgery showed competent velopharyngeal mechanisms in 77% of patients. PWSS nasal emission scores were worse in the 5- to 7-year age range ( P = 0.02), whereas resonance scores remained stable throughout development ( P = 0.2). Patients with Veau type I or II clefts had worse overall PWSS classifications in the groups aged 5 to 7 years and 8 to 11 years ( P = 0.01 and P = 0.03), with greater odds of secondary speech surgery relative to those with Veau type III (OR, 2.9; P < 0.001) or IV clefts (OR, 3.6; P = 0.001)., Conclusions: Most patients undergoing primary modified Furlow palatoplasty do not require secondary speech surgery and achieve socially acceptable speech at skeletal maturity. However, Veau type I and II clefts are associated with increased risk for early velopharyngeal dysfunction and secondary speech surgery. The incidence of secondary speech surgery was 19.8%, an increase from our previously reported rate of 8%., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Objective: Surgical treatment of velopharyngeal insufficiency (VPI) includes a wide array of procedures. The purpose of this study was to develop a classification for VPI procedures and to describe variations in how they are performed. Design/participants/setting/outcomes: We completed an in-depth review of the literature to develop a preliminary schema that encompassed existing VPI procedures. Forty-one cleft surgeons from twelve hospitals across the USA and Canada reviewed the schema and either confirmed that it encompassed all VPI procedures they performed or requested additions. Two surgeons then observed the conduct of the procedures by surgeons at each hospital. Standardized reports were completed with each visit to further explore the literature, refine the schema, and delineate the common and unique aspects of each surgeon's technique., Results: Procedures were divided into three groups: palate-based surgery; pharynx-based surgery; and augmentation. Palate-based operations included straight line mucosal incision with intravelar veloplasty, double-opposing Z-plasty, and palate lengthening with buccal myomucosal flaps. Many surgeons blended maneuvers from these three techniques, so a more descriptive schema was developed classifying the maneuvers employed on the oral mucosa, nasal mucosa, and muscle. Pharynx-based surgery included pharyngeal flap and sphincter pharyngoplasty, with variations in design for each. Augmentation procedures included palate and posterior wall augmentation., Conclusions: A comprehensive schema for VPI procedures was developed incorporating intentional adaptations in technique. There was substantial variation amongst surgeons in how each procedure was performed. The schema may enable more specific evaluations of surgical outcomes and exploration of the mechanisms through which these procedures improve speech., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To examine the impact of primary rhinoplasty on subsequent rhinoplasties for patients with cleft lip nasal deformity., Design: Retrospective cohort study., Setting: Tertiary care pediatric hospital., Patients/participants: Individuals who underwent definitive cleft lip repair at our institution from 2000 to 2006 with a current age of 18 or older., Main Outcome Measures: Number and timing of subsequent rhinoplasties., Results: Among 199 individuals, 94 (47.2%) underwent primary rhinoplasty. Follow-up was 15.0 ± 4.8 years in the PR cohort and 15.0 ± 5.1 years in the NPR cohort ( p  = 0.993). In bilateral cleft lip, interdomal suture predicted fewer subsequent rhinoplasties (β=-0.310, p  = 0.033), while history of primary rhinoplasty predicted greater age at subsequent rhinoplasty (β=1.800, p  = 0.040). Among individuals with follow-up beyond age 18, intranasal stenting predicted fewer subsequent rhinoplasties (β=-0.609, p  = 0.015). Most underwent subsequent nasal correction aside from 7 (19.4%) and 9 (20.9%) in the PR and NPR cohorts, respectively ( p  = 0.536). There was no difference in mean subsequent rhinoplasties between cohorts (1.1 ± 0.8 versus 1.3 ± 1.1, p =  0.284). Individuals with complete cleft lip underwent more lifetime rhinoplasties (1.9 ± 1.0 versus 1.2 ± 1.2, p  = 0.007)., Conclusions: Primary rhinoplasty with interdomal tip sutures in bilateral cleft lip was associated with fewer subsequent rhinoplasties. Primary rhinoplasty may delay subsequent nasal correction, though most who underwent primary rhinoplasty ultimately required nasal correction later in childhood. Postoperative nasal stenting may provide longer-term nasal benefits and should be considered at time of definitive cleft lip repair., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Ocular Ischemic Syndrome (OIS) is a rare, vision threatening condition associated with severe carotid artery disease. There are few cases of OIS reported in the literature., Methods: We present the case of a 54-year-old male with history of multiple previous carotid interventions including a right carotid stent, who presented with right-sided OIS., Results: CTA and angiogram showed a severe calcific plaque causing restenosis of the right carotid stent, with a patent right internal carotid artery (ICA) in the very distal neck. The right common carotid artery (CCA) was patent but diseased with ulcerated plaque extending proximally to below the level of the clavicle. The left CCA was chronically occluded from its origin all the way to the bifurcation. Given our patient's surgical history, the imperative to revascularize the ipsilateral carotid, and a diffusely diseased ipsilateral CCA, he was successfully treated with an ipsilateral subclavian to internal carotid bypass., Conclusion: There is paucity of data regarding the best approach for carotid revascularization in OIS. This case report discusses our unique perioperative decision making as well as relevant literature., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Pancreatic resection not infrequently requires portal vein (PV) repair or replacement. PV reconstruction often requires bypass grafting or patch venoplasty, and these grafts and patches require time to thaw or harvest. Mesenteric ischemia and congestion with associated bowel edema may result from prolonged venous occlusion during thawing, harvesting, and reconstructing. Temporary shunting of the mesenteric venous circulation may mitigate these adverse effects. Twenty-one patients were shunted using Argyle shunts during PV reconstruction from 2010 to 2020. Reconstructions in this series consisted of aortic homograft interposition grafts (52%), bovine pericardial patches (38%), internal jugular vein interposition grafts (5%), and internal jugular patches (5%). No intraoperative complications resulted from shunt placement; technical success of PV reconstruction was 100%. Temporary venous shunting during PV reconstruction is safe, technically straightforward, and may serve to decrease the duration of venous mesenteric occlusion., Competing Interests: None., (© 2024 The Authors.)

Objective: To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs)., Design: Cross-sectional retrospective chart review., Setting: Tertiary care pediatric hospital., Patients/participants: Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period., Main Outcome Measures: CLEFT-Q., Results: Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales., Conclusions: Patient perception of cleft outcomes is linked to psychosocial factors., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Objective: To compare patient-reported outcomes (PROs) in internationally adopted patients with cleft lip and palate to those in non-adopted peers., Design: Cross-sectional study., Setting: Multidisciplinary cleft team at tertiary care hospital., Patients: Patients aged ≥ 8 with cleft lip and palate attending routine cleft team evaluations September 2021 - September 2022., Main Outcome Measure: CLEFT-Q PRO scores., Results: Sixty-four internationally adopted patients and 113 non-adopted patients with a mean age of 13 years were included. Compared to non-adopted peers, adopted patients demonstrated worse satisfaction with face appearance (mean 59 vs. 66, p  = .044), speech function (mean 69 vs. 78, p  = .005), and speech distress (mean 80 vs. 84, p  = .032). No significant differences were observed on the nose, nostrils, teeth, lips, lip scar, jaws, psychological function, or social function scales ( p  > .05). Objective clinical evaluation corroborated these findings, with adopted patients demonstrating worse Pittsburgh Weighted Speech scores (mean 3.0 vs 1.9, p  = .027) and greater incidence of articulation errors (64% vs 46%, p = .021). No significant differences were observed in rates of mood, anxiety, or behavior concerns identified on psychosocial assessment ( p = .764). Among adopted patients, undergoing palatoplasty prior to adoption was associated with worse satisfaction with speech, appearance, school, and social function ( p  < .05)., Conclusions: Patient-reported outcomes among internationally adopted adolescents and young adults with cleft lip and palate show slightly lower satisfaction with facial appearance and speech but otherwise demonstrate similar results to non-adopted peers on most appearance and psychosocial measures. PRO data correlated well with objective speech assessment and did not portend worse psychosocial function.

Objective: To determine the impact of illustrated postoperative instructions on patient-caregiver knowledge and retention., Design: Prospective study with all participants receiving an educational intervention., Setting: Pediatric plastic surgical missions in Guatemala City, Guatemala, between 2019 and 2020., Participants: A total of 63 majority-indigenous Guatemalan caregivers of patients receiving cleft lip and/or palate surgery., Intervention: Illustrated culturally appropriate postoperative care instructions were iteratively developed and given to caregivers who were surveyed on illustration-based and text-based information at preoperative, postoperative, and four-week follow-up time points., Main Outcome Measure: Postoperative care knowledge of illustration-based versus text-based information as determined by the ability to answer 11 illustration- and 8 text-based all-or-nothing questions, as well as retention of knowledge as determined by the same survey given at four weeks follow-up., Results: Scores for illustration-based and text-based information both significantly increased after caregivers received the postoperative instructions (+13.30 ± 3.78 % SE, + 11.26 ± 4.81 % SE; P  < .05). At follow-up, scores were unchanged for illustration-based (-3.42 ± 4.49 % SE, P  > .05), but significantly lower for text-based information (-28.46 ± 6.09 % SE, P  < .01). Retention of text-based information at follow-up correlated positively with education level and Spanish literacy, but not for illustration-based., Conclusions: In the setting of language and cultural barriers on a surgical mission, understanding of illustration-based and text-based information both increased after verbal explanation of illustrated postoperative instructions. Illustration-based information was more likely to be retained by patient caregivers after four weeks than text-based information, the latter of which correlated with increased education and literacy.

This review aimed to update the clinical practice guidelines for managing children and adolescents with 22q11.2 deletion syndrome (22q11.2DS). The 22q11.2 Society, the international scientific organization studying chromosome 22q11.2 differences and related conditions, recruited expert clinicians worldwide to revise the original 2011 pediatric clinical practice guidelines in a stepwise process: (1) a systematic literature search (1992-2021), (2) study selection and data extraction by clinical experts from 9 different countries, covering 24 subspecialties, and (3) creation of a draft consensus document based on the literature and expert opinion, which was further shaped by survey results from family support organizations regarding perceived needs. Of 2441 22q11.2DS-relevant publications initially identified, 2344 received full-text reviews, including 1545 meeting criteria for potential relevance to clinical care of children and adolescents. Informed by the available literature, recommendations were formulated. Given evidence base limitations, multidisciplinary recommendations represent consensus statements of good practice for this evolving field. These recommendations provide contemporary guidance for evaluation, surveillance, and management of the many 22q11.2DS-associated physical, cognitive, behavioral, and psychiatric morbidities while addressing important genetic counseling and psychosocial issues., Competing Interests: Conflict of Interest The authors declare no conflicts of interest., (Copyright © 2022 American College of Medical Genetics and Genomics. Published by Elsevier Inc. All rights reserved.)

Background: Nasal airway obstruction is an increasingly recognized phenomenon in patients with cleft lip and/or palate and has the potential to significantly affect quality of life in this patient population. To date, the effect of secondary cleft rhinoplasty on cleft-related nasal airway obstruction has not been studied., Methods: Patients undergoing secondary cleft rhinoplasty at the Children's Hospital of Philadelphia from 2015 to 2021 were identified. Preoperative and postoperative Nasal Obstruction Symptom Evaluation scores were recorded. Alterations in scores were evaluated for variation depending on patient characteristics, operative maneuvers, and postoperative nasal stenting., Results: Nasal airway obstruction was present in mild to moderate severity in patients before secondary cleft rhinoplasty. Postoperatively, obstruction improved or resolved in the domains of nasal blockage/obstruction, trouble breathing through the nose, and ability to get enough air through the nose during exertion (P < 0.05). Overall composite Nasal Obstruction Symptom Evaluation scores improved (P < 0.05). Lateral crural strut grafting was associated with improvement in nasal blockage, whereas alar revision and tip sutures were associated with worsening in specific nasal symptoms. Patients who underwent nasal stenting were found to report less trouble breathing after surgery than patients who did not (P < 0.05)., Conclusions: Nasal airway obstruction is present in mild to moderate severity in patients with cleft lip and/or palate, and the subjective severity of obstruction is decreased by secondary cleft rhinoplasty. Specific operative maneuvers are associated with alterations in nasal airway obstructive symptoms, and nasal stenting is associated with an improvement in trouble breathing after secondary cleft rhinoplasty., Clinical Question/level of Evidence: Therapeutic, IV., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Background: Facial palsy may have deleterious effects for pediatric patients. The most common reconstruction is 2-stage free gracilis muscle transfer (FGMT) after cross-face nerve graft (CFNG). This requires a prolonged period from time of surgery to smile. New techniques using both a CFNG and motor nerve to masseter (MNM) as dual power sources in a single-stage surgery have been described in adults. Here, we examine our experience with this technique in children., Methods: A retrospective study was performed examining patients who underwent dual-innervated single-stage FGMT at 2 pediatric hospitals from 2016 to 2019. Demographics, etiology, perioperative characteristics, time to mandibular and emotional smile, and Sunnybrook scores were recorded., Results: Five patients met inclusion criteria with a mean age of 11.8 (range, 8-20). Two patients had congenital facial palsy while 3 had acquired facial palsy. Four patients (80%) received dual end-to-end neural coaptations of the CFNG and MNM to the obturator nerve. One (20%) had end-to-side coaptation of the CFNG to the obturator nerve and end-to-end of the MNM to the obturator nerve. The average time to mandibular smile was 103 ± 15.4 days. The average time to emotional smile was 245 ± 48.1 days. The preoperative Sunnybrook scale was 32 ± 7.5 and improved to 55.3 ± 20.6 at 8 months postoperatively., Conclusions: Dual-innervated FGMT is effective for facial reanimation in children with unilateral facial palsy. Patients can harness a stronger motor source (MNM) and the component of an emotional stimulus (CFNG). This approach may be the new criterion standard pediatric facial reanimation., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: Declaration of competing interest The authors report no funding sources or conflict of interest concerning the materials or methods used in this study or the findings specified in this study. This study and subsequent manuscript preparation are a work product of Dr. Butler serving as the inaugural Society of Black Academic Surgeons (SBAS)/CIGNA Academic Fellow from July 2021–2022., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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., Competing Interests: Disclosure:The authors have no financial disclosures or conflicts of interest to report., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Objectives: To describe our experience treating prenatally diagnosed oropharyngeal masses in a novel, multidisciplinary collaboration. To identifying outcomes and risk factors associated with adverse postnatal outcomes., Methods: This is a sixty-two patient case series at an academic referral center. Patients with prenatally diagnosed oropharyngeal masses were identified through a programmatic database and confirmed in the electronic health record., Results: Sixty-two patient with prenatally diagnosed oropharyngeal mass were identified, with prenatal imaging at our institution confirming this diagnosis in fifty-seven patients, short term outcomes analysis conducted on forty-four patients, and long-term outcomes analysis conducted on seventeen patients. The most common pathology was lymphatic malformations (n = 27, 47.4%), followed by teratomas (n = 22, 38.6%). The median mass volume from all available patient imaging (n = 57) was 60.54 cm 3 (range 1.73-742.5 cm 3 ). Thirteen pregnancies were interrupted, six infants expired, and thirteen cases had an unknown fetal outcome. Confirmed mortality was 6/57 patients with imaging-confirmed oropharyngeal masses (10.5%). Fourteen (56%) of the surviving patients (n = 25) were delivered by Ex Utero Intrapartum Treatment (EXIT) procedure and the median NICU stay was thirty-six days (range: 3-215 days). There was no association between airway compression/deviation/displacement, stomach size, polyhydramnios, or mass size and mortality. Seventeen patients had more than one year of follow-up (mean 5.3 ± 2.4 years). These seventeen patients underwent general anesthesia a total of ninety-two times (mean 5.4 ± 4.3) and had a total of twenty-three mass-related surgeries. The great majority of patients required an artificial airway at birth, feeding support, and speech/swallow therapy., Conclusions: Oropharyngeal mass involvement of key anatomic structures-the neck, upper thorax, orbit, and ear, has a greater association with mortality than mass size. Regardless of the size and involved structures, oropharyngeal masses are associated with a high burden of intensive medical care and surgical care beginning at or before birth., (Copyright © 2021. Published by Elsevier B.V.)

Background: The relationship between poverty and incidence of cleft lip and cleft palate remains unclear. The authors investigated the association between socioeconomic status and cleft lip with or without cleft palate and cleft palate only in the United States after controlling for demographic and environmental risk factors., Methods: The U.S. 2016 and 2017 natality data were utilized. Proxies for socioeconomic status included maternal education, use of the Special Supplemental Nutrition Program for Women, Infants, and Children, and payment source for delivery. Multiple logistic regression controlled for household demographics, prenatal care, maternal health, and infant characteristics., Results: Of 6,251,308 live births included, 2984 (0.05 percent) had cleft lip with or without cleft palate and 1180 (0.02 percent) had cleft palate only. Maternal education of bachelor's degree or higher was protective against, and delayed prenatal care associated with, cleft lip with or without cleft palate (adjusted ORs = 0.73 and 1.14 to 1.23, respectively; p < 0.02). Receiving assistance under the Special Supplemental Nutrition Program for Women, Infants, and Children was associated with cleft palate only (adjusted OR = 1.25; p = 0.003). Male sex, first-trimester tobacco smoking, and maternal gestational diabetes were also associated with cleft lip with or without cleft palate (adjusted ORs = 1.60, 1.01, and 1.19, respectively; p < 0.05). Female sex, prepregnancy tobacco smoking, and maternal infections during pregnancy were associated with cleft palate only (adjusted ORs = 0.74, 1.02, and 1.60, respectively; p < 0.05)., Conclusions: Increased incidence of orofacial clefts was associated with indicators of lower socioeconomic status, with different indicators associated with different cleft phenotypes. Notably, early prenatal care was protective against the development of cleft lip with or without cleft palate., Cliincal Question/level of Evidence: Risk, III., (Copyright © 2021 by the American Society of Plastic Surgeons.)

Purpose: To investigate antenatally-determined imaging characteristics associated with invasive airway management at birth in patients with cervical masses, as well as to describe postnatal management and outcomes., Study Design: A retrospective analysis of 52 patients with antenatally diagnosed neck masses was performed using single-center data from January 2008 to January 2019. Antenatal imaging, method of delivery, management, and outcomes data were abstracted from the medical record and analyzed., Results: Antenatal diagnosis of neck masses in this cohort consisted of 41 lymphatic malformations (78.8%), 6 teratomas (11.5%), 3 hemangiomas (5.8%), 1 hemangioendothelioma (1.9%), and 1 giant foregut duplication cyst (1.9%). Mean gestational age at time of diagnostic imaging was 29 weeks 3 days (range: 19w4d - 37w). Overall, 22 patients (42.3%) required invasive airway management at birth, specifically 18 patients (34.6%) required endotracheal intubation and 4 (7.7%) required tracheostomy. 15 patients (28.8%) underwent ex-utero intrapartum treatment (EXIT) for the purposes of securing an airway. Polyhydramnios, tracheal deviation and compression, and anterior mass location on antenatal imaging were significantly associated with incidence of invasive airway intervention at birth, EXIT procedure, and tracheostomy during the neonatal hospitalization (p < 0.025; Fisher's exact test). Logistic regression analysis demonstrated statistically significant association between increasing antenatally-estimated mass volume and incidence of invasive airway management at birth (p = 0.02). Post-natal cervical mass management involved surgical excision (32.7%), sclerotherapy (50%), and adjuvant therapy with rapamycin (17.3%). Demise in the neonatal period occurred in 4 (7.7%) patients., Conclusion: This series documents the largest single-center experience of airway management in antenatally diagnosed cervical masses. Fetal imaging characteristics may help inform the appropriate method of delivery, airway management strategy at birth, and prenatal counseling., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Infantile hemangiomas are common benign tumors of infancy with a predilection for the face. Nasal-tip hemangiomas, termed the "Cyrano deformity," can have especially devastating psychological effects. The ideal surgical approach and timing for these patients is unclear, as numerous designs have been reported in the literature. The authors present a novel approach to nasal tip reconstruction involving an algorithmic approach to incision selection and leaving a thin rim of hemangioma tissue under the dermis after debulking to minimize skin envelope atrophy. Nineteen cases were treated with the senior author's preferred method, with a mean age of 3.3 years and follow-up of 2.5 years. At follow-up, 10 patients were deemed by the senior author to have a very good aesthetic result, and the remaining nine patients were deemed to have an excellent one. The results of this study suggest that surgical intervention as early as 3 to 4 years of age is safe and provides good aesthetic outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE:: Therapeutic, IV.

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., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Definitive treatment of Paget-Schroetter syndrome (PSS) involves first rib resection (FRR), division of the anterior scalene muscle, and resection of the subclavius muscle. This is a single-institution experience with PSS, according to a treatment algorithm of preoperative venogram (accompanied by lysis and percutaneous mechanical thrombectomy as needed) followed by transaxillary FRR. In the later period of this experience, patients have often been discharged on aspirin only, with no plan for anticoagulation postoperatively. We sought to evaluate outcomes in light of this experience and these practice patterns., Methods: Between 2007 and 2018, 125 transaxillary FRRs were performed in 123 patients. All patients presented with documented venous thrombosis, underwent diagnostic venography and-if indicated-lysis and percutaneous mechanical thrombectomy (VPT) before FRR. The patient was not offered FRR if the vein could not be crossed with a wire and patency was not re-established during percutaneous treatment. The experience was divided into early (before 2012, n = 50) and late (n = 75) periods., Results: Mean patient age was 28.4 (12-64 years) years. Of the cohort, 33 were high-level competitive athletes, 13 presented with documented pulmonary embolism in addition to local symptoms, and 3 had a cervical rib fused to the first rib. Patients underwent FRR a median of 50 (4 days to 18 years) days after their initial symptoms, and a median of 22 (1 day to 9 months) days after their percutaneous intervention. Postoperative VPT was required in 23 patients and performed a median of 5 (1-137 days) days postoperatively; in 19 of these patients, postoperative VPT was required for postoperative re-thrombosis, whereas in 4 patients, postoperative VPT was planned before FRR due to vein stenosis or residual thrombus. All these patients were prescribed postoperative anticoagulation. No operative venous reconstruction or bypass was performed. Median follow-up time after FRR was 242 days; at last follow-up, 98.4% (123/125) of axillosubclavian veins were patent by duplex ultrasound (and all those patients were asymptomatic). Postoperative anticoagulation was less frequently prescribed in the late experience, with no difference in the rate of early re-thrombosis or follow-up patency., Conclusions: This experience demonstrates 98.4% patency at last follow-up with standard preoperative percutaneous venography and intervention, transaxillary FRR, and postoperative endovascular re-intervention only in cases with persistent symptoms, stenosis, or re-thrombosis. Patients presenting with both acute and chronic PSS did not require surgical venous reconstruction. In the later experience, patients frequently have not been anticoagulated postoperatively. Advantages of this algorithm include the following: (1) the cosmetic benefits of the transaxillary approach, (2) the preoperative assessment of the ability to recanalize the vein to determine which patients will benefit from surgery, (3) the capacity to use thrombolysis preoperatively, and (4) potential elimination of the risk and inconvenience of postoperative anticoagulation., (Copyright © 2020 Elsevier Inc. All rights reserved.)

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, χ 2 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.

Objective: To determine pre- and postoperative prevalence of obstructive sleep apnea (OSA) in patients with 22q11.2 deletion syndrome (DS) undergoing wide posterior pharyngeal flap (PPF) surgery for velopharyngeal dysfunction (VPD)., Design: Retrospective study using pre- and postoperative polysomnography (PSG) to determine prevalence of OSA. Medical records were reviewed for patients' medical comorbidities. Parents were surveyed about snoring., Setting: Academic tertiary care pediatric hospital., Patients: Forty patients with laboratory confirmed 22q11.2DS followed over a 6-year period., Interventions: Pre- and postoperative PSG, speech evaluation, and parent surveys., Main Outcome Measure: Severity and prevalence of OSA, defined by obstructive apnea hypopnea index (OAHI), before and after PPF surgery to determine whether PPF is associated with increased risk of OSA., Results: Mean OAHI did not change significantly after PPF surgery (1.1/h vs 2.1/h, P = .330). Prevalence of clinically significant OSA (OAHI ≥ 5) was identical pre- and postoperatively (2 of 40), with both cases having severe-range OSA requiring positive airway pressure therapy. All other patients had mild-range OSA. Nasal resonance was graded as severe preoperatively in 85% of patients. None were graded as severe postoperatively. No single patient factor or parent-reported concern predicted risk of OSA (OAHI ≥ 1.5)., Conclusions: Patients with 22q11.2DS are medically complex and are at increased risk of OSA at baseline. Wide PPF surgery for severe VPD does not significantly increase risk of OSA. Careful perioperative planning is essential to optimize both speech and sleep outcomes.

Purpose: The purpose of this study was to characterize airway problems, speech outcomes, and facial growth in patients with Stickler syndrome undergoing cleft palate repair., Methods: A retrospective, longitudinal study was performed at the Children's Hospital of Philadelphia on 25 patients with Stickler syndrome and 53 nonsyndromic patients with clefts of the secondary palate repaired between 1977 and 2000. Airway problems were characterized by the incidence of Pierre Robin Sequence (PRS) and the necessity for surgical airway management. Speech was analyzed using the Pittsburgh weighted values for speech symptoms associated with velopharyngeal incompetence (VPI). Longitudinal anthropometric measurements represented up to 12 years of longitudinal cephalofacial growth., Results: Seventy-two percent of patients with Stickler syndrome were diagnosed with PRS, 55.6% of whom required surgical airway management. Conversely, 20.8% of nonsyndromic patients were diagnosed with PRS (P < 0.0001), 18% of whom required surgical intervention (P < 0.05). Speech outcomes were poorer in patients with Stickler syndrome with 40% demonstrating borderline VPI and 13.3% demonstrating VPI, compared with 21.8% and 9.1%, respectively, in the nonsyndromic group. Both groups exhibited significantly shallower upper and mid facial depths and wider upper facial breadths when compared with normal standards of facial growth. Although there was a tendency toward decreased facial depths in patients with Stickler syndrome relative to nonsyndromic patients, the differences were nonsignificant., Conclusions: Patients with Stickler syndrome show significant potential for early airway compromise and a poorer prognosis for speech outcome after cleft palate repair. Their cephalofacial growth does not differ significantly from that of nonsyndromic cleft palate patients.

Background: Cleft repair requires multiple operations from infancy through adolescence, with repeated exposure to opioids and their associated risks. The authors implemented a quality improvement project to reduce perioperative opioid exposure in their cleft lip/palate population., Methods: After identifying key drivers of perioperative opioid administration, quality improvement interventions were developed to address these key drivers and reduce postoperative opioid administration from 0.30 mg/kg of morphine equivalents to 0.20 mg/kg of morphine equivalents. Data were retrospectively collected from January 1, 2015, until initiation of the quality improvement project (May 1, 2017), tracked over the 6-month quality improvement study period, and the subsequent 14 months. Metrics included morphine equivalents of opioids received during admission, administration of intraoperative nerve blocks, adherence to revised electronic medical record order sets, length of stay, and pain scores., Results: The final sample included 624 patients. Before implementation (n =354), children received an average of 0.30 mg/kg of morphine equivalents postoperatively. After implementation (n = 270), children received an average of 0.14 mg/kg of morphine equivalents postoperatively (p < 0.001) without increased length of stay (28.3 versus 28.7 hours; p = 0.719) or pain at less than 6 hours (1.78 versus 1.74; p = 0.626) or more than 6 hours postoperatively (1.50 versus 1.49; p = 0.924)., Conclusions: Perioperative opioid administration after cleft repair can be reduced in a relatively short period by identifying key drivers and addressing perioperative education, standardization of intraoperative pain control, and postoperative prioritization of nonopioid medications and nonpharmacologic pain control. The authors' quality improvement framework has promise for adaptation in future efforts to reduce opioid use in other surgical patient populations., Clinical Question/level of Evidence: Therapeutic, III.

Purpose: Postoperative hip pain is commonly reported after anterior iliac crest harvest for alveolar bone grafting. The goal of this study is to describe our institution's experience and examine the efficacy of our pain management protocols., Methods: A retrospective review was performed by abstracting demographic, operative, and pain management data from January 2011 to April 2013. Paired t-tests and Fisher exact tests were used to examine differences when comparing 2 groups, while ANOVA was used to examine difference between the 3 protocols for harvest and pain management: trapdoor technique and local anesthetic injection (TD+LAI), TD and pain catheter (TD+PC), and split crest and LAI., Results: Eighty-four patients, 52 males (61.9%), averaging 8.8 years old (±2.9) were included. Postoperatively, 17 (71%) patients in the PC group received IV narcotics compared to 27 (45%) in those without a PC (P = .03). When comparing all 3 protocols, no significant difference was found in IV morphine usage or duration of IV morphine treatment. In subgroup analysis, when patients in the groups TD+PC versus TD+LAI were examined, those in the TD+PC group had significantly shorter hospital stays and were more likely to go home postoperative day 1 (P = .03; P = .04)., Conclusions: Overall, patients tolerated alveolar bone grafting well regardless of harvest technique or pain management approach. While indwelling PCs did not significantly decrease IV morphine usage, these patients had significantly shorter lengths of stays.

Background: Lengthening temporalis myoplasty (LTM) and cross-face nerve graft with free gracilis muscle transfer (CFNG-FGMT) are the 2 most common procedures used to restore dynamic facial animation and improve facial symmetry. There has not been direct comparison or consensus. Here, the authors compare our experience with respect to muscle activity, symmetry, and excursion., Methods: A retrospective review was performed of patients with facial palsy who had CFNG-FGMT or LTM from 2008 to 2016 at a single institution. Postoperative surface EMG was recorded at maximum open smile. Normal and paralyzed sides of the face were analyzed with Facial Assessment by Computer Evaluation software. Commissure excursion and symmetry was assessed., Results: Six patients with LTM and 10 with CFNG-FGMT met inclusion criteria. Muscle activity was 1st identified in LTM patients after 3 months (47.42 mV, P < 0.001) and CFNG-FGMT patients after 3 months (28.30 mV, P < 0.001) compared to immediate postoperative period. Relative to preoperative excursion, there was significant increase of 3.33 mm in commissure excursion seen at the 0 to 3 month period for LTM patients (P = 0.04). Commisure excursion for CFNG-FGMT was seen later, in the 3- to 6-month postoperative period (4.01, P = 0.024). During smile, CFNG-FGMT patients had better symmetry than unilateral LTM patients. In bilateral LTM patients, there was no significant change in symmetry., Conclusion: Dynamic facial animation improved in both surgical groups. The LTM demonstrates a faster rate of muscle recruitment compared to CFNG-FGMT. After 3 months, both LTM and CFNG-FGMT groups had comparable excursions. A decision-making algorithm is presented.

Objective: Deficiencies of the upper lip vermilion occur with some frequency following repair of unilateral and bilateral clefts of the lip and can compromise the aesthetic outcome. The presence of dense scar tissue within the lip at the cleft site as well as intrinsic vermillion deficiencies can make long-lasting correction of this deformity challenging. We describe a technique to address vermillion deficiencies after cleft lip repair., Design: A novel lip augmentation technique for correction of residual vermilion deficiencies after unilateral and bilateral cleft lip repair is presented. This technique combines precise placement of a contoured dermal fat graft with local tissue (V-Y) rearrangement., Conclusion: In our experience, this method of lip augmentation following either unilateral or bilateral cleft lip repair can restore upper lip vermillion symmetry and provide predictable and durable results in patients with mild to severe vermillion deficiency.

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.

Harvesting of iliac crest bone graft for alveolar cleft repair in children is associated with significant postoperative pain. Transversalis fascia plane block has emerged as an alternative to local wound infiltration for iliac crest bone graft harvesting and has been used with good effect in adult patients requiring bone graft for orthopedic surgery. Little is known about the use of the transversalis fascia plane block in children or the feasibility of placing a continuous infusion catheter. In this report, we describe our experience using a continuous transversalis fascia plane infusion catheter in the management of a 6-year-old girl undergoing alveolar cleft repair with iliac crest bone graft.

Objective: To characterize the epidemiology and risk factors for nasal obstruction among subjects with cleft lip and/or cleft palate (CL/P) utilizing the well-validated Nasal Obstruction Symptom Evaluation (NOSE) survey., Design: Retrospective cross-sectional study., Setting: Cleft Lip and Palate Program, Children's Hospital of Philadelphia., Patients, Subjects: One thousand twenty-eight surveys obtained from 456 subjects (mean age: 10.10 (4.48) years) with CL/P evaluated between January 2015 and August 2017 with at least 1 completed NOSE survey., Interventions: Nasal Obstruction Symptom Evaluation surveys completed at each annual visit., Main Outcome Measures: Composite NOSE and individual symptom scores., Results: Sixty-seven percent of subjects had nasal obstruction at some point during the study period, with 49% reporting nasal obstruction at latest follow-up. subjects aged 14 years and older reported the most severe symptoms ( P = .002). Subjects with cleft lip and alveolus (CL+A) and unilateral cleft lip and palate (CLP) reported more severe nasal blockage than other phenotypes ( P = .021). subjects with a history of either posterior pharyngeal flap (PPF) or sphincter pharyngoplasty (SP) had significantly higher NOSE scores than subjects with no history of speech surgery ( P = .006). There was no significant difference ( P > .050) in NOSE scores with regard to history of primary tip rhinoplasty, nasal stent use, or nasoalveolar molding., Conclusions: There are more severe nasal obstructive symptoms among subjects older than 14 years of age, with CL+A or unilateral CLP, and with a history of PPF or SP. Future studies utilizing the NOSE are needed to evaluate and address this prevalent morbidity in the CLP population.

Objective: To determine demographics and practice patterns of surgeons treating velopharyngeal dysfunction (VPD) in patients with 22q11.2 deletion syndrome (22q11.2DS)., Methods: An anonymous electronic survey study was administered to the surgical membership of the American Cleft Palate-Craniofacial Association and the Society for Ear Nose and Throat Advances in Children. The survey queried surgeon demographics and differences in management practices for submucous cleft palate (SMCP), pharyngoplasty algorithms, and self-reported complications for nonsyndromic versus 22q11.2DS patients., Results: 126 surveys were returned from 9 international regions with the majority from the United States (73%), followed by Western Europe (9.5%) and Canada (7.9%). Plastic surgery was the most common specialty (61.9%), followed by otolaryngology (27.8%). 88.1% reported fellowship training, and 33% completed multiple fellowships. Prior to proceeding with pharyngoplasty in 22q11.2DS patients, surgeons required the following assessments: speech evaluation (79.4%), velopharyngeal imaging (51.6%), cardiac evaluation (50.0%), carotid artery MRI (29.4%), and cervical spine x-rays (11.1%). Nasoendoscopy was the most common modality used for imaging the velopharynx. Overall, providers managed patients with 22q11.2DS similarly to nonsyndromic patients, with several significant exceptions including that they were more likely to perform SMCP repair alone as a first approach in nonsyndromic patients (p = 0.031) and posterior pharyngeal flap without SMCP repair in those with 22q11.2DS (p = 0.017)., Conclusions: Practice patterns for the management of VPD in patients with 22q11.2DS vary across providers. Further collaborative studies are needed to develop optimal treatment paradigms for VPD in patients with 22q11.2 DS., (Copyright © 2018. Published by Elsevier B.V.)

22q11.2 deletion syndrome (22q11.2DS) is a disorder caused by recurrent, chromosome-specific, low copy repeat (LCR)-mediated copy-number losses of chromosome 22q11. The Children's Hospital of Philadelphia has been involved in the clinical care of individuals with what is now known as 22q11.2DS since our initial report of the association with DiGeorge syndrome in 1982. We reviewed the medical records on our continuously growing longitudinal cohort of 1,421 patients with molecularly confirmed 22q11.2DS from 1992 to 2018. Most individuals are Caucasian and older than 8 years. The mean age at diagnosis was 3.9 years. The majority of patients (85%) had typical LCR22A-LCR22D deletions, and only 7% of these typical deletions were inherited from a parent harboring the deletion constitutionally. However, 6% of individuals harbored other nested deletions that would not be identified by traditional 22q11.2 FISH, thus requiring an orthogonal technology to diagnose. Major medical problems included immune dysfunction or allergies (77%), palatal abnormalities (67%), congenital heart disease (64%), gastrointestinal difficulties (65%), endocrine dysfunction (>50%), scoliosis (50%), renal anomalies (16%), and airway abnormalities. Median full-scale intelligence quotient was 76, with no significant difference between individuals with and without congenital heart disease or hypocalcemia. Characteristic dysmorphic facial features were present in most individuals, but dermatoglyphic patterns of our cohort are similar to normal controls. This is the largest longitudinal study of patients with 22q11.2DS, helping to further describe the condition and aid in diagnosis and management. Further surveillance will likely elucidate additional clinically relevant findings as they age., (© 2018 Wiley Periodicals, Inc.)

Objective: This study aimed to identify risk factors for adverse perioperative events (APEs) after cleft palatoplasty to develop an individualized risk assessment tool., Design: Retrospective cohort., Setting: Tertiary institutional., Patients: Patients younger than 2 years with cleft palate., Interventions: Primary Furlow palatoplasty between 2008 and 2011., Main Outcome Measure(s): Adverse perioperative event, defined as laryngo- or bronchospasm, accidental extubation, reintubation, obstruction, hypoxia, or unplanned intensive care unit admission., Results: Three hundred patients averaging 12.3 months old were included. Cleft distribution included submucous, 1%; Veau 1, 17.3%; Veau 2, 38.3%; Veau 3, 30.3%; and Veau 4, 13.0%. Pierre Robin (n = 43) was the most prevalent syndrome/anomaly. Eighty-three percent of patients received reversal of neuromuscular blockade, and total morphine equivalent narcotic dose averaged 0.19 mg/kg. Sixty-nine patients (23.0%) had an APE, most commonly hypoventilation (10%) and airway obstruction (8%). Other APEs included reintubation (4.7%) and laryngobronchospasm (3.3%). APE was associated with multiple intubation attempts (odds ratio [OR] = 6.6, P = .001), structural or functional airway anomaly (OR = 4.5, P < .001), operation >160 minutes (OR = 2.2, P = .04), narcotic dose >0.3 mg/kg (OR = 2.3, P = .03), inexperienced provider (OR = 2.1, P = .02), and no paralytic reversal administration (OR = 2.0, P = .049); weight between 9 and 13 kg was protective (OR = 0.5, P = .04). Patients were risk-stratified according to individual profiles as low, average, high, or extreme risk (APE 2.5%-91.7%) with excellent risk discrimination (C-statistic = 0.79)., Conclusions: APE incidence was 23.0% after palatoplasty, with a 37-fold higher incidence in extreme-risk patients. Individualized risk assessment tools may enhance perioperative clinical decision making to mitigate complications.

The single-segment great saphenous vein continues to be a conduit of choice for lower extremity arterial bypass. In patients without an adequate continuous segment of great saphenous vein, a spliced vein graft may be used as an alternative. Creating a spliced vein conduit can be technically challenging and time consuming. We present a technique of creating a spliced vein conduit by using a microvascular anastomotic coupler., (Copyright © 2017 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: This study aims to better understand patient-reported outcomes for iliac bone grafting surgery for alveolar cleft repair and to determine how standardizing perioperative patient instruction affects patient-reported outcomes., Design: Retrospective survey-based assessment of patients undergoing iliac bone grafting with and without hospital-based systems standardization., Setting: Academic tertiary care hospital., Patients: Of the 195 identified patients, 127 participated., Interventions: Survey on pain and satisfaction regarding iliac bone grafting surgery., Main Outcome Measures: Survey answers measured patient opinions about the surgery. Answers of the pre- and poststandardization patients were compared to determine the effect of standardizing patient instructions., Results: Patients rated their satisfaction with the surgery and recovery a 4.5 and 4.4 out of 5, respectively. They rated their overall pain in the hospital a 5.5 out of 10 (4.9 in the mouth, 5.7 in the hip). Patients were discharged an average of 1.2 days after surgery and could return to normal daily activity in 6.1 days. Poststandardization patients were more likely to adhere to instructions regarding use of an antibacterial mouthrinse and a protective oral splint., Conclusions: Patients were highly satisfied with the iliac bone grafting procedure and the recovery and reported only moderate levels of postoperative pain. Implementing standardized patient instructions may not affect patient satisfaction or pain severity, but it significantly increased patient adherence to physician instructions.

Background: International adoptees with cleft lip and palate (CLP) are a growing population in the United States. They represent a clinical challenge, presenting at various ages and stages of cleft repair., Methods: A retrospective review of patients seen at the CLP Program at the Children's Hospital of Philadelphia (CHOP) between 1998 and 2012 with a history of international adoption was performed. Demographics, surgical histories, and long-term speech outcomes were reviewed., Results: Seventy-four female and 77 male patients were evaluated. Patients were adopted at an average age of 2.3 years (range, 0.4-8.6 years); 80.8% (n = 122) of patients were adopted from China. The rate of international cleft adoption increased by approximately 1.5 patients per year (r = 0.7739, P < 0.001); 13.2% (n = 19) of all subjects with cleft palates had oronasal fistulas (ONFs) that required repair. The ONF rates for primary palatoplasties at CHOP were significantly lower compared to both preadoption repairs (P = 0.002) and postadoption repairs at outside hospitals (P = 0.01); 14.8% (n = 21) of all patients had secondary surgeries for velopharyngeal incompetence (VPI). Rates of secondary surgery for VPI were also significantly lower for primary palatoplasties at CHOP compared to both preadoption repairs (P = 0.0018) and postadoption repairs at outside hospitals (P = 0.0033)., Conclusions: International adoptees with CLP are a growing population and are clinically challenging with high ONF rates and high secondary surgery rates for VPI. We recommend expedited repair of unoperated cleft palates in adoptees older than 18 months. Adopted patients with CLP should be rigorously evaluated for the need for speech therapy and secondary surgeries to correct for VPI.

Background: Patients with complete cleft lip and palate may benefit from cleft lip adhesion or nasoalveolar molding before formal cleft lip repair. The authors compared the relative costs to insurers of these two treatment modalities and the burden of care to families., Methods: A retrospective analysis was performed of cleft lip and palate patients treated with nasoalveolar molding or cleft lip adhesion at The Children's Hospital of Philadelphia between January of 2007 and June of 2012. Demographic, appointment, and surgical data were reviewed; surgical, inpatient hospital, and orthodontic charges and costs were obtained. Multivariate linear regression and two-sample, two-tailed independent t tests were performed to compare cost and appointment data between groups., Results: Forty-two cleft adhesion and 35 nasoalveolar molding patients met inclusion criteria. Mean costs for nasoalveolar molding were $3550.24 ± $667.27. Cleft adhesion costs, consisting of both hospital and surgical costs, were $9370.55 ± $1691.79. Analysis of log costs demonstrated a significant difference between the groups, with the mean total cost for nasoalveolar molding significantly lower than that for adhesion (p < 0.0001). Nasoalveolar molding patients had significantly more made, cancelled, no-show, and missed visits and a higher missed percentage than adhesion patients (p < 0.0001) for all except no-show appointments, (p = 0.0199), indicating a higher burden of care to families., Conclusions: Nasoalveolar molding may cost less before formal cleft lip repair treatment than cleft lip adhesion. Third-party payers who cover adhesion and not nasoalveolar molding may not be acting in their own best interest. Nasoalveolar molding places a higher burden of care on families, and this fact should be considered in planning treatment.

Background: Associated comorbidities can put syndromic patients with cleft palate at risk for poor speech outcomes. Reported rates of velopharyngeal insufficiency (VPI) vary from 8% to 64%, and need for secondary VPI surgery from 23% to 64%, with few studies providing long-term follow-up. The purpose of this study was to describe our institutional long-term experience with syndromic patients undergoing cleft palatoplasty., Methods: A retrospective review was conducted of all patients with syndromic diagnoses undergoing primary Furlow palatoplasty from 1975 to 2011. Outcomes included postoperative oronasal fistula (ONF) and need for secondary VPI surgery. Speech scores for verbal patients 5 years or older were collected via the Pittsburgh scale for speech assessment. Aggregate scores categorized the velopharyngeal mechanism as competent, borderline, or incompetent. Outcomes were analyzed by patient and operative factors., Results: One hundred thirty-two patients were included with average age at repair of 20.7 months. Cleft type was 9% submucosal, 16% Veau class I, 50% class II, 12% class III, and 13% class IV. Forty-five syndromes were recorded, most commonly Stickler syndrome (n = 32) and 22q11.2 deletion syndrome [22q11.2DS (n = 19)]. Forty-four patients also had associated Pierre Robin sequence (PRS). The overall ONF rate was 4.5% and was highest in Veau class IV clefts (P = 0.048). Seventy-six patients were included in speech analysis, with an average age at last assessment of 10.4 years. Overall, 60.5% of patients had a competent velopharyngeal mechanism, 23.7% borderline, and 15.8% incompetent mechanism. Fifty percent of 22q11.2DS patients had borderline speech and none had competent speech, compared to 73.3% with Stickler syndrome (P = 0.01) and 71.4% of patients with associated PRS (P = 0.02). Secondary VPI surgery was performed in 11.4% of patients overall. Patients with PRS (13.6%) and with Stickler syndrome (15.6%) had secondary VPI surgery, compared to 31.6% of patients with 22q11.2DS (P = 0.01)., Conclusions: This study demonstrates low rates of postoperative ONF after modified Furlow palatoplasty in syndromic patients. Speech outcomes were comparable to nonsyndromic patients at our institution, but patients with 22q11.2DS consistently had borderline-incompetent speech and a 3-fold higher incidence of secondary VPI surgery.

Objectives: Published reports and previous studies from our institution have reported worse overall speech results, including significantly higher rates of persistent articulation errors, in patients undergoing palatoplasty at age >18 months. This study further investigates the effects of late repair on long term speech outcomes., Methods: A retrospective review was performed of non-syndromic patients undergoing primary palatoplasty at age >18 months between 1980 and 2006 at our institution. Longitudinal speech results were compared based on reason for late repair and age at repair., Results: Forty-one patients were greater than 18 months of age at the time of palatoplasty, and 24 fit criteria for longitudinal data analysis. There was a statistically significant improvement in nasality scores at Time Point 1 for international adoptees compared to the non-adopted population (p=0.04). Patients with submucosal clefts were found to have significantly less severe nasal emission scores at Time Point 1 compared to those with overt clefts (p=0.04), but not at Time Point 2. There were no significant differences between scores if repair was performed between 18 and 36 months or >36 months, nor any difference in incidence of articulation errors between subgroups of patients with late repair at either Time Point., Conclusion: Our experience demonstrates that cleft palate repair after 18 months of age is associated with a significantly increased incidence of articulation errors associated with VPI, irrespective of reason for late repair, highlighting the persistence of learned compensatory behaviors in speech and the importance of proceeding with early repair., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Angiolymphoid hyperplasia with eosinophilia is a rare, benign vascular lesion characterized by discrete, painful papules. Although the exact etiology is unknown, trauma precedes many cases. We present a case of angiolymphoid hyperplasia with eosinophilia in the earlobes of a 15-year-old girl after ear piercing., (© 2014 Wiley Periodicals, Inc.)

Unlabelled: Otolaryngologic problems are common in the 22q11.2 deletion syndrome (DS) population. Structural anomalies and retrognathia may predispose these patients to obstructive sleep apnea (OSA). The current association of OSA in this population is not defined., Objective: (1) Define the frequency of OSA in 22q11.2 DS patients referred for polysomnography (PSG). (2) Determine if OSA is present before and/or after surgery to correct velopharyngeal insufficiency (VPI). (3) Determine effect of prior adenotonsillectomy on OSA following VPI surgery., Methods: Retrospective review of children treated from 2006 to 2013 in a tertiary care setting identified by ICD-9 758.32 (velocardiofacial syndrome) and 279.11 (DiGeorge syndrome). Surgical history and PSG data were abstracted from the identified records., Results: We identified 323 patients with 22q11.2 DS; 57 (18%) were screened at any point in care using PSG and 15 patients had PSG at multiple time points in care. In most cases, indication for PSG was sleep disordered breathing or pre-operative planning. Overall, 33 patients met criteria for OSA on PSG, accounting for 10.2% of our study population; however, the percentage of patients with OSA was significantly higher within the group of 57 patients (58%) who were screened with PSG. Twenty-one of the screened patients (54%) had PSG prior to any pharyngeal surgery and had mild to severe OSA (obstructive apnea/hypopnea index (AHI): median 5.1/h, range 1.9-25.6). Eighteen patients had PSG after adenotonsillectomy; 8 of these patients (44%) had mild to moderate OSA (median AHI 2.95/h, range 1.9-5.4). Seventeen patients had PSG after VPI surgery (palatopharyngeal flap (PPF) n=16, sphincteroplasty n=1). Nine of these patients (53%) had mild to severe OSA (median AHI 3/h, range 1.9-15). Patients who underwent adenotonsillectomy prior to VPI surgery had similar prevalence of OSA (50%, n=12) than those who did not (OSA: 60%, n=5, p=0.70). Most children had mild OSA., Conclusion: Prevalence of OSA in this population of 22q11.2 DS patients is higher than expected in the general population. OSA risk is highest after VPI surgery, and may be decreased by adenotonsillectomy. Providers should have awareness of increased prevalence of OSA in patients with 22q11.2 DS. Close monitoring for OSA is warranted given the likelihood of subsequent surgical intervention that can worsen OSA., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: The separation of conjoined twins presents a unique challenge to many pediatric surgical specialties. A multidisciplinary approach is essential for successful twin separation, and plastic surgeons are critical members of this surgical team when soft-tissue requirements preclude simple closure., Methods: In this article, the authors review multiple techniques available to plastic surgeons for providing adequate soft-tissue coverage in this unique clinical situation and present lessons learned from their experience at The Children's Hospital of Philadelphia with seven conjoined twin separations performed between the years 1980 and 2007., Results: Successful separation was accomplished in six of the seven conjoined twin pairs, expander complications requiring reoperation occurred in three twin pairs, and skin grafting was required because of insufficient expansion in two twin pairs. The techniques found to be critical to successful separation include maximal extracavitary tissue expansion with smooth-wall remote port tissue expanders, protection of expanded skin through pressure-reducing strategies, fluorescein vascular territory mapping, the use of frozen banked autogenous skin for salvage, and the use of supplemental nutritional support., Conclusions: The overall success of conjoined twin separation depends on the experience and preparedness of the treating team and the resources available at the pediatric specialty center. Multiple techniques are available to plastic surgeons to optimize success in this challenging clinical situation., Clinical Question/level of Evidence: Therapeutic, V.

Objectives: 1) To examine the orthodontic referral behaviour of dentists and 2) to examine dentists' familiarity with the Index of Orthodontic Treatment Need (IOTN)., Design: Postal questionnaire survey., Setting: West Sussex Primary Care Trust (PCT) from September to December 2006., Subjects and Methods: Questionnaires were sent to all dentists listed on the West Sussex PCT database, except those assumed not to make orthodontic referrals (n = 325)., Results: Two hundred and twenty-nine questionnaires were returned, representing a 70% response rate. Fifty-two percent of dentists in West Sussex correctly identified which type of orthodontic provider to refer three different malocclusions using picture tests. Twenty percent of dentists made correct decisions on the timing of referral for three different malocclusions using picture tests. IOTN is not routinely used by 76% of West Sussex dentists when making an orthodontic referral., Conclusions: This study provides evidence that there is a need for postgraduate training or the development of referral guidelines to assist West Sussex dentists in making referrals for orthodontic treatment to the most appropriate provider at the most appropriate time. If dentists are to act as gatekeepers of orthodontic provision on the NHS there is a need to provide more support and education for them about the use of IOTN.