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.

Speech Outcomes and Rates of Secondary Speech Surgery from Childhood to Skeletal Maturity Following Modified-Furlow Palatoplasty.

BACKGROUND: This study updates our 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 post-operative speech evaluations were retrospectively reviewed. Secondary speech surgery and Pittsburgh Weighted Speech Scale (PWSS) scores prior to secondary speech or orthognathic surgeries were assessed in the 5-7, 8-11, 12-14, and 15+ age groups and analyzed by Veau cleft type. RESULTS: Five hundred fifty-one patients with 895 total speech assessments were analyzed. Of 364 patients followed to age 15 or older, 19.8% underwent secondary speech surgery. Speech assessment of patients aged 15 or older without prior secondary speech surgery showed competent velopharyngeal mechanisms in 77% of patients. PWSS nasal emission scores were worse in the 5-7 age range (p=0.02), while resonance scores remained stable throughout development (p=0.2). Patients with Veau type I or II clefts had worse overall PWSS classifications in the age 5-7 and 8-11 age groups (p=0.01, 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. Incidence of secondary speech surgery was 19.8%, an increase from our previously reported rate of 8%.

How does CLEFT-Q change the way we practice? A prospective study integrating patient-reported outcomes.

OBJECTIVE: 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 prior to the clinic encounter but results were initially masked from the surgeon and family. In the encounter, a study observer characterized patients' verbalized attitudes across seven specific domains of appearance and function and the provisional assessment and plan was noted. CLEFT-Q data was then introduced in 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; mean patient age was 12.7 years (range 8-19). 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 gender was associated with increased discordance. Integration of CLEFT-Q results altered the assessment and plan in 11 (16%) visits. CONCLUSIONS: 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.

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.

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.

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.

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.

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.

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.

A Longitudinal Study of Improvement in Nasal Airway Obstruction after Secondary Cleft Rhinoplasty.

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.

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.

Trends in insurance coverage for adolescent reduction mammaplasty.

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

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

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

Poverty and Risk of Cleft Lip and Palate: An Analysis of United States Birth Data.

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.

Postnatal outcomes and risk factor analysis for patients with prenatally diagnosed oropharyngeal masses.

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 cm3 (range 1.73-742.5 cm3). 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.

Risk factor analysis and outcomes of airway management in antenatally diagnosed cervical masses.

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.

A Novel Approach in the Surgical Management of Nasal Tip Hemangiomas: A 26-Year Experience.

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.