Psychosocial Status and Self-Perception in Patients with Cleft Lip and/or Palate.

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.

Updated clinical practice recommendations for managing children with 22q11.2 deletion syndrome.

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.

Subclavian to carotid artery bypass for a case of ocular ischemic syndrome.

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.

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

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

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

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

Long-Term Patient-Reported Outcomes in Internationally Adopted Children with Cleft Lip and Palate.

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.

Surgery for Velopharyngeal Insufficiency Following Cleft Palate Repair: An Audit of Contemporary Practice and Proposed Schema of Techniques and Variations.

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.

Impact of Illustrated Postoperative Instructions on Knowledge and Retention During a Cleft Lip and Palate Surgical Mission.

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.

Dual-Innervated Free Gracilis Muscle Transfer for Facial Reanimation in Children.

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.

Is Routine Postoperative Anticoagulation Necessary in All Patients after First Rib Resection for Paget-Schroetter Syndrome?

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.

Orofacial Manifestations of Stickler Syndrome: An Analysis of Speech Outcome and Facial Growth After Cleft Palate Repair.

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.

Perioperative Outcome Differences Between Pain Management Protocols in Cleft Alveolar Bone Grafting.

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.

Comparison of Lengthening Temporalis Myoplasty and Free-Gracilis Muscle Transfer for Facial Reanimation in Children.

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.

Precision Dermal Fat Grafting for Vermillion Deficiencies in Patients With Unilateral and Bilateral Cleft Lip.

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.

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

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

Defining Risk of Postoperative Obstructive Sleep Apnea in Patients With 22q11.2DS Undergoing Pharyngeal Flap Surgery for Velopharyngeal Dysfunction Using Polysomnographic Evaluation.

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.

Buccal Myomucosal Flap Reconstruction After Resection of Palatal Mucoepidermoid Carcinoma.

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

Nasal Obstruction in Children With Cleft Lip and Palate: Results of a Cross-Sectional Study Utilizing the NOSE Scale.

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.

What is new with 22q? An update from the 22q and You Center at the Children's Hospital of Philadelphia.

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.

Predicting Adverse Perioperative Events in Patients Undergoing Primary Cleft Palate Repair.

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.