Safety and efficacy of intracranial vascularized submental lymph node transfer for treating hydrocephalus.

Hydrocephalus is routinely treated with ventriculoperitoneal shunt drainage of cerebrospinal fluid (CSF), a procedure plagued by high morbidity and frequent revisions. Vascularized submental lymph node (VSLN) transplants act as lymphatic pumps to drain interstitial fluid (ISF) from lymphedematous extremities. As the field of neuro-lymphatics comes to fruition, we hypothesize the efficacy of VSLN in the drainage of intracranial CSF-ISF. We report novel placement of VSLN in the temporal subdural space in two patients diagnosed with symptomatic communicating hydrocephalus. At a minimum follow-up of 1 month postoperatively, both experienced radiological and clinical improvements.

Sequential free fibula transfers: Quality of life and systematic review.

BACKGROUND: Successive osteoseptocutaneous fibula transfers for jaws reconstruction are rare but important options. This study contributes patient-reported and clinical outcomes, as well as systematically reviews all existing reports. METHODS: All sequential fibula transfers performed by the senior author were reviewed from a prospectively managed database, including University of Washington quality of life (UWQoL). Systematic review was conducted in PubMed and Cochrane databases for similar publications. RESULTS: Eighteen patients (average age 51.5 years) received sequential fibulas (mean 4.7 years between reconstructions). Secondary fibulas more often had benign indications (72.2% vs. 33.3%, p = .04), most commonly osteoradionecrosis (38.9%). At a mean follow-up of 30.5 months, the average interincisal distance increased from 21.8 to 27.6 mm, and 92.3% tolerated an oral diet following the second fibula. Eight patients completed the UW-QoL before and after the second fibula, and three prior to the first fibula. Composite physical function was significantly decreased from 96.7 prefibula reconstruction to 63.3 following the first (p < .001) and 64.2 after the second fibula (p < .001). There were no differences in other domains. The systematic review yielded six articles reporting 56 patients (mean 39 months between fibulas). Secondary fibulas were performed for repeat malignancy (45%) and osteoreadionecrosis (39%), resulting in elevated tube feeding from 20% following the first to 39% following the second, but overall high quality of life in two studies. CONCLUSIONS: Sequential osteoseptocutaneous fibula reconstructions of jaws are often performed for benign indications such as osteoradionecrosis. Overall function and QoL are comparable with those following the first fibula transfer.

Risk Factors for Hardware Removal Following Bimaxillary Surgery: A National Database Analysis.

Orthognathic surgery typically relies on the rigid fixation of fracture fragments using metal hardware. Though hardware is usually intended to be implanted permanently, the removal of hardware (ROH) is sometimes indicated for a variety of reasons. The authors sought to identify risk factors for ROH following orthognathic surgery. The authors conducted a retrospective analysis of the Merative MarketScan Research Databases, 2007-2021 using Current Procedural Terminology (CPT) and International Classification of Disease (ICD-9 and ICD-10) codes to identify patients who underwent an index Le Fort 1 osteotomy and bilateral sagittal split osteotomy operation on the same day. Statistical analysis involved χ2, Shapiro-Wilk, Wilcoxon-Mann-Whitney, Poisson regression, and multivariable logistic regression tests. 4698 patients met the inclusion criteria. The mean age at surgery was 25 years, and 57% were female. ROH occurred in 5.9% of patients. The mean time to hardware removal was 190.5±172.4 days. In a multivariate logistic regression, increased odds of ROH were associated with older patient age [OR: 1.02 (1.01-1.03), P=0.046], sleep apnea [OR: 1.62 (1.13-2.32), P=0.018], and craniofacial syndrome and/or cleft diagnoses [OR: 1.88 (1.14-2.55), P<0.001]. In the same model, postoperative oral antibiotic prophylaxis was not associated with ROH (P=0.494). The incidence of all-cause complications [IRR: 1.03 (1.01-1.05), P<0.001] rose over the study period, while the incidence of ROH did not change significantly (P=0.281). Patients at elevated risk should be counseled on the increased possibility of a second operation for ROH before having orthognathic surgery to ensure expectations and health care utilization decisions align with the evidence.

ChatGPT in head and neck scientific writing: A precautionary anecdote.

PURPOSE: To evaluate the accuracy of ChatGPT references in scientific writing relevant to head and neck surgery. MATERIALS AND METHODS: Five commonly researched keywords relevant to head and neck surgery were selected (osteoradionecrosis of the jaws, oral cancer, adjuvant therapy for oral cancer, TORS, and free flap reconstruction in oral cancer). The AI chatbot was then asked to provide ten complete citations for each of the keywords. Two independent authors reviewed the results for accuracy and assigned each article a numerical score based on pre-selected criteria. RESULTS: Among 50 total references provided by ChatGPT, only five (10 %) were found to have the correct title, journal, authors, year of publication, and DOI. Merely 14 % of the presented references had correct DOI. References regarding free flap reconstruction for oral cancer were the least accurate from all the five categories, with no correct DOI. Complete inter-rater agreement was noted while evaluating the citations. CONCLUSION: Only 10 % of the articles provided by ChatGPT, relevant to head and neck surgery, were correct. A high degree of academic hallucination was noted.

Persistent Cranial Defects After Endoscopic Sagittal Synostosis Surgery.

INTRODUCTION: Incomplete cranial ossification is a rare complication of calvarial-vault remodeling for sagittal synostosis often requiring reoperation. Studies show an incidence ranging from 0.5% to 18%. METHODS: Infants with sagittal synostosis who underwent endoscopic sagittal synostectomy and barrel stave osteotomies with postoperative orthotic helmeting between 2003 and 2021 were included with minimum follow-up until the completion of helmeting. RESULTS: Of 90 patients, 86 met inclusion; 3 had defects (3.5%). Patients with and without cranial defects had no difference in age of surgery (113 versus 131 d), duration helmeting (6.6 versus 7.0 mo), or perioperative/postoperative complications. Two underwent reoperation for recurrence. Patients with cranial defects manifested the evidence of developmental concerns more than patients without (100% versus 16.9%).The average cranial defect size was 19.33 cm 2 and age at surgery 4.29 years. All were managed with cranial particulate bone grafting with addition of bone matrix and SonicWeld plate. The first had 6×6 cm posterior defect requiring cranioplasty at 4.86 years with excellent healing. The second had a 3×6 cm posterior and 1×1 cm anterior defect, underwent cranioplasty at 4.14 years with persistent 4×6 defect, requiring repeat cranioplasty at 5.3 years. The third had a 3×5 cm posterior defect and underwent cranioplasty at 3.88 years with continued defect, planning for repeat intervention. CONCLUSIONS: This is the largest documented series of reoperations for incomplete ossification after endoscopic sagittal synostectomy with postoperative helmet treatment. The authors report a 3.5% rate of cranial defects, managed with bone grafting, bone matrix, and absorbable plates. Patients with poor ossification may have a propensity toward developmental concerns.

Mutations in TFAP2B and previously unimplicated genes of the BMP, Wnt, and Hedgehog pathways in syndromic craniosynostosis.

Craniosynostosis (CS) is a frequent congenital anomaly featuring the premature fusion of 1 or more sutures of the cranial vault. Syndromic cases, featuring additional congenital anomalies, make up 15% of CS. While many genes underlying syndromic CS have been identified, the cause of many syndromic cases remains unknown. We performed exome sequencing of 12 syndromic CS cases and their parents, in whom previous genetic evaluations were unrevealing. Damaging de novo or transmitted loss of function (LOF) mutations were found in 8 genes that are highly intolerant to LOF mutation (P = 4.0 × 10-8); additionally, a rare damaging mutation in SOX11, which has a lower level of intolerance, was identified. Four probands had rare damaging mutations (2 de novo) in TFAP2B, a transcription factor that orchestrates neural crest cell migration and differentiation; this mutation burden is highly significant (P = 8.2 × 10-12). Three probands had rare damaging mutations in GLI2, SOX11, or GPC4, which function in the Hedgehog, BMP, and Wnt signaling pathways; other genes in these pathways have previously been implicated in syndromic CS. Similarly, damaging de novo mutations were identified in genes encoding the chromatin modifier KAT6A, and CTNNA1, encoding catenin α-1. These findings establish TFAP2B as a CS gene, have implications for assessing risk to subsequent children in these families, and provide evidence implicating other genes in syndromic CS. This high yield indicates the value of performing exome sequencing of syndromic CS patients when sequencing of known disease loci is unrevealing.

Labiaplasty: Analysis of the National Surgical Quality Improvement Program Database.

BACKGROUND: Labiaplasty is an increasingly popular procedure performed for both cosmetic and pathologic etiologies. Questions have been raised regarding the efficacy of the procedure, especially for cosmetic etiologies. OBJECTIVES: The aim of this study was to examine the complication profiles of labiaplasties for both cosmetic and pathologic etiologies. METHODS: The 2005 to 2017 National Surgical Quality Improvement Program database was analyzed for patients who, according to the relevant Current Procedural Terminology code, had undergone labiaplasties. Our cohort was further separated into cosmetic and pathologic groups based on International Classification of Diseases codes. Information was collected on patient demographic characteristics, patient comorbidities, and operative variables. Outcomes of interest included surgical complications and delayed length of stay (DLOS). A univariate analysis and multivariate logistic regression were applied to determine statistically significant predictors of our outcomes of interest for both etiologies. RESULTS: There were 640 patients in the cosmetic cohort and 1919 patients in the pathologic cohort. There were no significant differences in rates of surgical complications between the 2 groups, but there was a statistically significant increase in length of stay for the pathologic group. Univariate analysis revealed operative time and plastic surgeon specialty to be predictive of DLOS in the cosmetic cohort. No covariates were implicated with multivariate analysis for either surgical complications or for DLOS in the cosmetic cohort. CONCLUSIONS: Our findings suggest that cosmetic labiaplasty is a safe and efficacious procedure with low complication rates and no predictors of adverse outcomes.

Primary Cleft Palate Repair Among Older-Age Children and Adolescents in the United States.

PURPOSE: Older age cleft palate (CP) repair in international settings has been associated with increased surgical morbidity. This study assesses the prevalence and risks associated with late-age CP repair (age > 5 years) in the United States. METHODS: Primary CP repair patients less than the age of 18 years were identified in the National Surgical Quality Improvement pediatric database from 2012 to 2018. Total postoperative complications, readmissions, reoperations, duration of surgery, and length of stay were recorded. T-tests and χ2 analyses were used to compare variables between age groups 0-5, 6-10, and 11-17. RESULTS: A total of 10,022 primary CP procedures were identified from 2012 to 2018, of which 868 (8.6%) received repair at age > 5 years. Hispanic patients constituted a larger proportion of CP repair from ages 11 to 17 years than repair at other ages (P < .001). In comparison with children treated from ages 0 to 5 years, children operated on between ages 6 and 10 or 11 and 17 years experienced no increases in unplanned readmissions, reoperations, or complication rates after surgery. Patients of ages 6-10 years and 11-17 years had decreased operating room time (P < .001) compared with younger patients. Patients of ages 11-17 years also had decreased hospital length of stay (P = .04). CONCLUSIONS: Many children in the United States received primary CP repair after the age of 5 years likely due to late treatment of submucosal clefts or delayed care among international immigrants/adoptees. Old age procedures were not associated with increased short-term surgical morbidity in comparison with surgery at earlier time points. The causes and implications of older age primary surgery warrant further study.

The Impact of Premastectomy Versus Postmastectomy Radiation Therapy on Outcomes in Prepectoral Implant-Based Breast Reconstruction.

PURPOSE: Prepectoral implant-based breast reconstruction is being increasingly performed over subpectoral reconstruction because of the reduced invasiveness of the procedure, postoperative pain, and risk of animation deformity. Radiation therapy is a well-known risk factor for complications in implant-based breast reconstruction. The effect of premastectomy versus postmastectomy radiation therapy on outcomes after prepectoral breast reconstruction has not been well-defined. The purpose of this study was to compare the impact of premastectomy versus postmastectomy radiation therapy on outcomes after prepectoral breast reconstruction. METHODS: A retrospective chart review was performed on all patients who underwent prepectoral implant-based breast reconstruction with inferior dermal flap and acellular dermal matrix performed by a single surgeon from 2010 to 2019. Demographic, clinical and operative data were reviewed and recorded. Outcomes were assessed by comparing rates of capsular contracture, infection, seroma, hematoma, dehiscence, mastectomy skin flap necrosis, rippling, implant loss, local recurrence and metastatic disease, between patients receiving premastectomy and postmastectomy radiation therapy and nonradiated patients. RESULTS: Three hundred and sixty-nine patients (592 breasts) underwent prepectoral implant-based breast reconstruction. Twenty-six patients (28 breasts) received premastectomy radiation, 45 patients (71 breasts) received postmastectomy radiation, and 305 patients (493 breasts) did not receive radiation therapy. Patients with premastectomy radiation had higher rates of seroma (14.3% vs 0.2%), minor infection (10.7% vs 1.2%), implant loss (21.4% vs 3.4%) and local recurrence (7.1% vs 1.0%), compared with nonradiated patients (P < 0.05). Patients with postmastectomy radiation had higher rates of major infection (8.4% vs 2.4%), capsular contracture (19.7% vs 3.2%), implant loss (9.9% vs 3.4%), and local recurrence (5.6% vs 1.0%) when compared with nonradiated patients (P < 0.03). Outcomes after prepectoral breast reconstruction were comparable between premastectomy and postmastectomy radiation patients, respectively, with regard to major infection (7.1% vs 8.4%), dehiscence (3.6% vs 1.4%), major mastectomy skin flap necrosis (7.1% vs 2.8%), capsular contracture (10.7% vs 19.7%), implant loss (21.4% vs 9.9%), and local recurrence (7.1% vs 5.6%) (P ≥ 0.184). However, premastectomy radiation patients had a higher rate of seroma compared with postmastectomy radiation patients (14.3% vs 0%; P = 0.005). CONCLUSIONS: In prepectoral implant breast reconstruction, premastectomy and postmastectomy radiation therapy were associated with higher rates of infection and implant loss compared with nonradiated patients. Postmastectomy radiation was associated with a higher rate of capsular contracture compared with nonradiated patients, and a comparable rate of capsular contracture compared with premastectomy radiation therapy patients. Premastectomy radiation was associated with a higher rate of seroma compared with postmastectomy radiation and nonradiated patients.

Neurologic Characterization of Craniosynostosis: Can Direct Brain Recordings Predict Language Development?

PURPOSE: Nonsyndromic craniosynostosis (NSC) is associated with language deficits. Conventional tests, such as the Bayley Scales of Infant Development (BSID), may not reflect accurate long-term cognition. Alternatively, mismatch negativity (MMN) waves recorded via electroencephalogram (EEG) measure neural responses to speech and may objectively predict language development. This study aimed to (1) correlate infant MMN to future language achievement and (2) compare MMN among subtypes of NSC. METHODS: Pre and postoperatively (mean operative age 9.5 months), NSC participants received the BSID and EEG phoneme-discrimination paradigm(80 dB,250 Hz). The MMN was the largest negative amplitude in the difference wave 80 to 300 ms after stimuli. To measure cognitive outcome, patients completed a neurodevelopmental battery (Wechsler-Abbreviated Scale of Intelligence and Wechsler-Fundamentals) at >6 years of age. RESULTS: Eleven NSC patients with EEG testing in infancy were neurocognitively tested (average age 8.0 years; 27% female; 55% sagittal, 27% metopic, 9% unicoronal, 9% sagittal/metopic). The left frontal cluster MMN strongly correlated with word-reading (r = 0.713, P = 0.031), reading-comprehension (r = 0.745, P = 0.021), and language-composites (r = 0.0771, P = 0.015). Conversely, BSID scores did not yield significant predictive value (r < 0.5, P > 0.05). Follow-up event related potentials (ERP) comparison included 39 normal control, 18 sagittal, 17 metopic, 6 unilateral-coronal infants. Preoperatively, sagittal (P = 0.003) and metopic (P = 0.003) patients had attenuated left frontal MMN compared to controls. Postoperatively, the sagittal cohort was normalized to controls while metopic patients retained attenuations (P = 0.041). CONCLUSION: ERP assessment in NSC had significantly better predictive value for future neurocognition than the BSID. Preoperatively, sagittal and metopic patients had attenuated neural response to language; postoperatively, sagittal patients had improved responses in comparison to metopic patients. Use of ERP assessment may help tailor treatment for language deficits earlier in development.

Long-Term Neurocognitive Outcomes in Sagittal Synostosis: The Impact of Reoperation.

INTRODUCTION: Optimal age at surgery in nonsyndromic sagittal craniosynostosis continues to be debated. Previous reports suggest that earlier age at whole vault cranioplasty more frequently requires reoperation. It is unknown, however, whether reoperation affects neurocognitive outcome. This study examined the impact of reoperation on neurocognitive outcome in children with nonsyndromic sagittal craniosynostosis using comprehensive neurocognitive testing. METHODS: Forty-seven school-age children (age 5-16 years) with nonsyndromic sagittal craniosynostosis who underwent whole-vault cranioplasty were included in this analysis. Participants were administered a battery of standardized neuropsychological testing to measure neurocognitive outcomes. RESULTS: Thirteen of the 47 participants underwent reoperation (27.7%); 11 out of the 13 reoperations were minor revisions while 2 reoperations were cranioplasties. Reoperation rate was not statistically different between patients who had earlier surgery (at age ≤6 months) versus later surgery (at age >6 months) (P > 0.05). Nonreoperated patients who had only one later-in-life surgery did not perform statistically better than reoperated patients on any outcome measure of neurocognitive function, including IQ, academic achievement, visuomotor integration, executive function, and behavior. Comparing reoperated earlier surgery patients with nonreoperated later surgery patients, reoperated earlier surgery patients had higher full-scale and verbal IQ (P < 0.05), scored higher on word reading, reading comprehension, spelling, numerical operations, and visuomotor integration (P < 0.05), and had fewer indicators of suspected learning disabilities (P < 0.01) compared to nonreoperated later surgery patients. CONCLUSION: Reoperation rate after whole vault cranioplasty was 27.7%, with few cases of repeat cranioplasty (4.2% of all patients). Reoperation was not associated with worse neurocognitive outcome. Reoperated earlier surgery patients in fact performed better in IQ, academic achievement and visuomotor integration when compared to nonreoperated later surgery patients.

Assessing Facial Asymmetry in Postoperative Patients With Unilateral Coronal Craniosynostosis.

BACKGROUND: Despite surgical correction of unilateral craniosynostosis (ULC), complex cranial base angulation can result in partial reversion to preoperative deformity with growth and time. Using 3-dimensional imaging, dysmorphic facial features of ULC in school-age patients were quantified and related to how they contribute to overall facial asymmetry and patient-reported outcomes. METHODS: Children who underwent surgical correction of ULC were recruited from Yale University and Children's Hospital of Philadelphia. The 3D photographs were analyzed utilizing a Procrustes analysis of shape. Pearson's correlation was used to determine dysmorphic features' impact on overall asymmetry. Patients were stratified into "moderate" and "severe" asymmetry. Finally, asymmetry was correlated to patient-reported outcome scores. Statistical analysis was performed with SPSS-25 with P < 0.05 as statistically significant. RESULTS: Twenty-one patients were included with average age at analysis of 12.3 years. Fifty-seven percent of patients had right-sided fusion. The overall Procrustes analysis indicated a root mean square difference of 2.21 mm. Pearson's correlation indicated that the facial middle 3rd (P ≤ 0.001), orbital dystopia (P < 0.001), chin point deviation (P = 0.011), and nasal root angulation (P = 0.019) contributed most to overall asymmetry. Patients in the severe asymmetry cohort had greater facial middle-third asymmetry (P < 0.001) and orbital dystopia (P < 0.001). Asymmetry did not correlate with patient-reported outcomes. CONCLUSION: Patients with ULC have persistent facial asymmetry at school-age with the greatest levels of asymmetry in the facial middle-third, orbit, and nasal root. Beyond the cranial dysmorphology, initial skull base angulation in unilateral coronal craniosynostosis manifests in long-term mid and lower-third facial asymmetry.

Classification of Subtypes of Crouzon Syndrome Based on the Type of Vault Suture Synostosis.

BACKGROUND: Patients with Crouzon syndrome develop various types of anatomic deformities due to different forms of craniosynostosis, yet they have similar craniofacial characteristics. However, exact homology is not evident. Different pathology then may be best treated by different forms of surgical technique. Therefore, precise classification of Crouzon syndrome, based on individual patterns of cranial suture involvement is needed. METHODS: Ninety-five computed tomography (CT) scans (Crouzon, n = 33; control, n = 62) were included in this study. All the CT scans are divided into 4 types based on premature closure of sutures: class I = coronal and lambdoidal synostosis; class II = sagittal synostosis; class III = pansynostosis; and class IV = "Others." The CT scan anatomy was measured by Materialise software. RESULTS: The class III, pansynostosis, is the most prevalent (63.6%). The classes I, III, and IV of Crouzon have significantly shortened entire anteroposterior cranial base length, with the shortest base length in class III. The external cranial measurements in class I show primarily a decreased posterior facial skeleton, while the class III presented with holistic facial skeleton reduction. Class II has the least severe craniofacial malformations, while class III had the most severe. CONCLUSION: The morphology of patients with Crouzon syndrome is not identical in both cranial base and facial characteristics, especially when they associated with different subtypes of cranial suture synostosis. The classification of Crouzon syndrome proposed in this study, summarizes the differences among each subgroup of craniosynostosis suture involvement, which, theoretically, may ultimately influence both the timing and type of surgical intervention.

Spring-Assisted Strip Craniectomy Versus Cranial Vault Remodeling: Long-Term Psychological, Behavioral, and Executive Function Outcomes.

BACKGROUND: Controversy exists regarding the optimal surgical approach for non-syndromic sagittal synostosis. This study provides the first comparative analysis of the long-term behavioral, psychological, and executive function outcomes for patients who underwent either cranial vault remodeling (CVR) or spring-assisted strip craniectomy (SAS). METHODS: Thirty-six CVR patients and 39 SAS patients were evaluated. Parents and caregivers completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Behavior Assessment System for Children, Second Edition (BASC-2) to evaluate behavioral, emotional, social, adaptive, and executive functioning skills. RESULTS: There were no statistically significant differences between the CVR and the SAS groups (P > 0.05) in any of the BRIEF areas of function. Furthermore, the BASC-2 battery illustrated no significant differences in all areas analyzed except one. Among the 2 groups, the CVR group was rated as having fewer social withdrawal symptoms on the BASC-2 (47.00 ±â€Š10.27) compared to the SAS cohort (54.64 ±â€Š10.96), F = 6.79, P = 0.012, Cohen d = 0.688. However, both means were still within the normal range. CONCLUSIONS: Children undergoing SAS and CVR procedures for isolated sagittal synostosis were not rated as having clinically significant behavioral, emotional, social, adaptive, or executive functioning problems on parental forms.

Segmental Maxillary Osteotomy to Close Wide Alveolar Clefts.

Wide alveolar clefts are challenging to treat and are often associated with a higher chance of failure requiring repeated bone grafts. Even if successful, an excessively large cleft leads to a wide edentulous space, and patients are faced with prolonged orthodontic treatment. We present a series of large alveolar clefts closed with the aid of segmental maxillary osteotomies. Cases of alveolar clefts treated by segmental maxillary osteotomy from 2014 to 2016 were reviewed. Preoperative and postoperative computed tomography scans were analyzed to assess the alveolar gap and bone filling. Demographic characteristics, diagnoses, and operative techniques were reviewed and described. A total of 10 alveolar fistulae were addressed, using 6 segmental osteotomies in 6 patients. Of the cases, 66% were bilateral. There were 4 female and 2 male patients with an average age of 12.6 years. The average preoperative cleft width was 14.2 mm on computed tomography scans. Postoperatively, all clefts were successfully closed and no adverse effects were observed. Segmental maxillary osteotomy with concurrent bone grafting and fistula closure is an effective approach to treat excessively wide alveolar clefts. This enables a prudent dental substitution or prosthodontic plan while minimizing prolonged orthodontia, in addition to achieving the traditional goals of alveolar bone grafting (stabilizing the maxillary arch, closing fistulae, and permitting canine eruption).

Mandibular Spatial Reorientation and Morphological Alteration of Crouzon and Apert Syndrome.

BACKGROUND: From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. METHODS: One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analysis. RESULTS: In Crouzon syndrome, the angle between the cranial base and gnathion was increased prior to 6 months of age by 10.29 degrees (P < 0.001) and by adulthood to 11.95 degrees (P = 0.003) compared with normal. After 6 months of age, the distance between bilateral mandibular condylions (COR-COL) was narrower by 15% (P < 0.001) in Crouzon syndrome compared with control subjects. Before 6 months of age, Apert COR-COL decreased 16% (P < 0.001) compared with control subjects and 13% (P = 0.006) narrower than Crouzon. During 2 to 6 years of age, Apert mandibular ramus height caught up to, and became longer than, Crouzon by 12% (P = 0.011). The nasion-sella-articulare angle of the Apert skull was 5.04 degrees (P < 0.001) less than Crouzon overall. CONCLUSIONS: In Crouzon syndrome, the changes of the spatial relationship of the mandible to the cranial base develop earlier than the mandibular shape deformity, whereas in Apert syndrome, the spatial and morphological changes are synchronous. The morphological changes of the mandible are disproportional in 3 directions, initially significant shortening of the mandibular width and length, and, subsequently, reduced height. Crouzon has more shortening in mandibular height compared with Apert, reflecting the more shortened posterior cranial base length. The narrowed angle between the mandible and the posterior cranial base in Apert skulls is consistent with the more limited nasopharyngeal and oropharyngeal airway space.

Hospital Volume Improves Primary, Revision, and Delayed Cleft Palate Repair.

BACKGROUND: High volume centers (HVC) is commonly associated with increased resources and improved patient outcomes. This study assesses efficacy and outcomes of high volume centers in cleft palate repair. METHODS: Cleft palate procedures were identified in the Kids' Inpatient Database from 2003-2009. Demographics, perioperative factors, co-morbidities, and complications in HVC (90th percentile, >48 cases/year) and non-high volume centers (NHVC) were compared across various cohorts of cleft repair. RESULTS: Four thousand five hundred sixty-three (61.7%) total cleft palate surgeries were performed in HVC and 3388 (38.3%) were performed in NHVC. The NHVC treated a higher percentage of Medicaid patients (P = 0.005) and patients from low-income quartiles (P = 0.018). HVC had larger bedsizes (P <0.001), were more often government/private owned (P <0.001), and were more often teaching hospitals (P <0.001) located predominantly in urban settings (P <0.001). The HVC treated patients at younger ages (P = 0.008) and performed more concurrent procedures (P = 0.047). The most common diagnosis at HVC was complete cleft palate with incomplete cleft lip, while the most common diagnosis at NHVC was incomplete cleft palate without lip. Overall, length of stay and specific complication rates were lower in HVC (P = 0.048, P = 0.042). Primaries at HVCs showed lower pneumonia (P = 0.009) and specific complication rates (P = 0.023). Revisions at HVC were associated with older patients, fewer cardiac complications (P = 0.040), less wound disruption (P = 0.050), but more hemorrhage (P = 0.040).

Temporal Evaluation of Craniofacial Relationships in Apert Syndrome.

Complicated craniofacial malformations interfacing with multiple intracellular regulatory mechanisms, lead to ambiguous growth patterns in Apert syndrome. This study aims to explore the chronology and pathogenesis of the development of craniofacial anatomic relationships and to verify the positional correlates between skull and facial structures in Apert syndrome. Fifty-four computed tomography scans (Apert, n = 18; control, n = 36) were included and divided into 3 age subgroups. Craniofacial 3-dimensional cephalometries were analyzed by Materialize software. The angle between sella-nasion plane and maxillary plane widens 7.74° (P = 0.003) prior to 6 months of age; thereafter, this widening increases by 10.36° (P < 0.001) in 6 months to 2 years of age, and remains increased by 8.9° (P = 0.046) throughout childhood. The angle between Frankfort horizontal plane and maxillary plane widens 5.17° (P = 0.022) before 6 months. Angles SNA, SNB, and ANB showed decreases, averaging 12.23° (P < 0.001), 5.19° (P = 0.004), and 6.72° (P = 0.001), respectively. The linear measurements showed synchronicity and continuing deformity into adulthood. Between 6 months to 2 years of age, the distance from sella to nasion (S-N), anterior nasal spine (S-ANS), and posterior nasal spine (S-PNS) decreased 8% (P = 0.006), 16% (P < 0.001), and 19% (P = 0.002), respectively, and remained shortened into adulthood. The angulation changes occur earlier in development than linear distance reduction in Apert syndrome patients compared with controls. Angular adjustments were not sufficient to maintain normal cranial base length. Facial deformity of Apert syndrome temporally begins with the midface, and affects orbit and mandible later in life.

A Quantitative Analysis of Weight Gain Following Mandibular Distraction Osteogenesis in Robin Sequence.

Mandibular distraction osteogenesis (MDO) is an effective treatment modality for children suffering from upper airway obstruction from Robin Sequence (RS). Mandibular distraction osteogenesis has been shown to have positive effects on oral feeding and for relieving respiratory obstruction, but its effects on postoperative weight gain are poorly understood. This study quantitatively analyzes weight gain following MDO. A retrospective chart review identified 22 RS children who underwent MDO. Patient weight data, feeding methods pre- and postoperatively, and polysomnography data pre- and postoperatively were collected. Each patient's weight plotted over time was then compared with his or her closest standardized growth curve, and linear regression analysis was utilized to quantify patient growth by calculating actual and expected average daily weight gain (g/d). Percentile changes were analyzed as well. Children gained significantly less weight than expected from birth to time of MDO and significantly more weight than expected from MDO to device removal, MDO to 6 months postoperatively, and MDO to 12 months postoperatively. The average growth percentile for the cohort was 37.3 at birth, declined to 22.7 by MDO, and increased to 28.5 and 33.5 at device removal and 6 months postoperatively, respectively. More than 70% of children were exclusively orally fed within 6 months of MDO. Children with isolated Robin Sequence had superior weight gain than children with syndromic Robin Sequence following surgery. In conclusion, MDO helps improve weight gain following surgery, particularly for infants with isolated Robin Sequence, and has positive effects on oral feeding and respiration.

National Longitudinal Comparison of Patients Undergoing Surgical Management of Craniosynostosis.

PURPOSE: Limited cross-institutional studies compare strip craniectomy versus cranial vault remodeling (CVR) for craniosynostosis management. Given competing surgical preferences, the authors conducted a large-scale analysis of socioeconomic differences, costs, and complications between treatment options. METHODS: Nonsyndromic craniosynostosis patients receiving strip craniectomies or CVR were identified in the Kids' Inpatient Database for years 2000 to 2009. Demographics, socioeconomic background, hospital characteristics, charge, and outcomes were tabulated. Univariate and multivariate analyses were performed for comparison. RESULTS: Two hundred fifty-one strip craniectomies and 1811 CVR patients were captured. Significantly more strip craniectomy patients were White while more CVR patients were Hispanic or Black (P < 0.0001). Strip craniectomy patients more often had private insurance and CVR patients had Medicaid (P < 0.0001). Over time, CVR trended toward treating a higher proportion of Hispanic and Medicaid patients (P = 0.036). Peri-operative charges associated with CVR were $27,962 more than strip craniectomies, and $11,001 after controlling for patient payer, income, bedsize, and length of stay (P < 0.0001). Strip craniectomies were performed more frequently in the West and Midwest, while CVR were more common in the South (P = 0.001). Length of stay was not significant. Postsurgical complications were largely equivocal; CVR was associated with increased accidental puncture (P = 0.025) and serum transfusion (P = 0.002). CONCLUSION: Our national longitudinal comparison demonstrates widening socioeconomic disparities between strip craniectomy and CVR patients. Cranial vault remodeling is more commonly performed in underrepresented minorities and patients with Medicaid, while strip craniectomy is common in the White population and patients with private insurance. While hospital charges and complications were higher among CVR, differences were smaller than expected.