International Adoptees With Cleft Lip and/or Palate: Lessons Learned From a Growing Population.

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

Maxillary involvement in hemifacial microsomia: an objective three-dimensional analysis of the craniofacial skeleton.

The aim of our study is to better understand the maxillary involvement in relation to the mandibular deformation in hemifacial microsomia (HFM). To do so, consecutive patients with HFM treated at The Children's Hospital of Philadelphia from 2000 to 2012 were included in our study. Both two-dimensional and three-dimensional analyses of the bony and sinus structures of the midface and mandible were performed using three-dimensional segmentation software. Patients were stratified into groups based on the Kaban-Pruzansky classification--mild (0-1), moderate (2A), and severe (2B-3)--as well as rank ordering based on overall severity. Analyses involved paired t-tests within severity groups, 1-way analysis of variance when assessing across groups (ipsilateral/contralateral ratio), and regression to assess for trends. Thirty patients were included (4 mild, 12 moderate, and 14 severe). The mandibular volume ratio differed across all patient groups (P < 0.001) and trended with rank order (P < 0.001). No significant difference in maxillary bony volume ratio was found across all patient groups (P = 0.16). In patients with severe disease, the maxillary bone volume was found to be significantly decreased on the ipsilateral side as compared with the contralateral side (P = 0.0123). There was no difference in maxillary sinus volume between ipsilateral and contralateral sides within any patient groups or in comparing across groups (P = 0.10). No significant trend was found in the volume ratio of mandible and maxilla (P = 0.41). To conclude, the maxillary sinus seems to show no difference in volume when comparing between laterality and severity groupings. These findings suggest that there may be alternative influences other than the vascular insult acting as the driving force behind the mandibular deformity and the additional classic clinical findings of HFM.

Analysis of the long-term growth of the mandible in Apert syndrome.

Apert syndrome carries a characteristic phenotype of midface hypoplasia, syndactyly, craniosynostosis, and developmental delay. These patients frequently require a large number of surgical procedures to produce a functional and aesthetically pleasing correction of their facial deformities. Although most of the focus for surgical planning is allocated to the cranial vault and the midface, controversy exists as to whether the mandible is intrinsically abnormal in this population.A retrospective chart review was performed to identify patients with Apert syndrome cared for at The Children's Hospital of Philadelphia. Patients with available craniofacial computed tomographic scans after skeletal maturity were examined using cephalometric and three-dimensional volumetric techniques. A comparison was made to age- and demographically matched controls, and statistical significance was determined using the Student t test (P < 0.05).Thirty-eight patients, in total, were identified, 9 of which had available three-dimensional computed tomographic scans. Most patients underwent frontal-orbital advancement in their infancy and at least 1 midface procedure later in life. Three-dimensional volumetric analysis identified a decreased maxillary volume (P = 0.03) in the population with Apert syndrome but found no difference in the mandibular volume (P = 0.59). Cephalometric analysis demonstrated that the patients with Apert syndrome have normal ramal height but a statistically significant decreased mandibular length.The mandible with Apert syndrome seems to be intrinsically normal on the basis of our three-dimensional analysis, and differences in appreciated mandibular length are likely related to the interrelationship with the maxilla. Patients can therefore be instructed that improving the midface position may likely also reduce the compensatory mandibular deformity.

Surgical Classification of the Mandibular Deformity in Craniofacial Microsomia Using 3-Dimensional Computed Tomography.

BACKGROUND: Grading systems of the mandibular deformity in craniofacial microsomia (CFM) based on conventional radiographs have shown low interrater reproducibility among craniofacial surgeons. We sought to design and validate a classification based on 3-dimensional CT (3dCT) that correlates features of the deformity with surgical treatment. METHODS: CFM mandibular deformities were classified as normal (T0), mild (hypoplastic, likely treated with orthodontics or orthognathic surgery; T1), moderate (vertically deficient ramus, likely treated with distraction osteogenesis; T2), or severe (ramus rudimentary or absent, with either adequate or inadequate mandibular body bone stock; T3 and T4, likely treated with costochondral graft or free fibular flap, respectively). The 3dCT face scans of CFM patients were randomized and then classified by craniofacial surgeons. Pairwise agreement and Fleiss' κ were used to assess interrater reliability. RESULTS: The 3dCT images of 43 patients with CFM (aged 0.1-15.8 years) were reviewed by 15 craniofacial surgeons, representing an average 15.2 years of experience. Reviewers demonstrated fair interrater reliability with average pairwise agreement of 50.4 ± 9.9% (Fleiss' κ = 0.34). This represents significant improvement over the Pruzansky-Kaban classification (pairwise agreement, 39.2%; P = 0.0033.) Reviewers demonstrated substantial interrater reliability with average pairwise agreement of 83.0 ± 7.6% (κ = 0.64) distinguishing deformities requiring graft or flap reconstruction (T3 and T4) from others. CONCLUSION: The proposed classification, designed for the era of 3dCT, shows improved consensus with respect to stratifying the severity of mandibular deformity and type of operative management.

An analysis of early oncologic head and neck free flap reoperations from the 2005-2012 ACS-NSQIP dataset.

BACKGROUND: There are limited population-based studies that examine perioperative factors that influence postoperative surgical take-backs to the OR following free flap (FF) reconstruction for head/neck cancer extirpation. The purpose of this study was to critically analyse head/neck free flaps (HNFF) captured in the ACS-NSQIP dataset with a specific focus on postoperative complications and the incidence of factors associated with re-operation. METHODS: The 2005-2012 ACS-NSQIP datasets were accessed to identify patients undergoing FF reconstruction after a diagnosis of head/neck cancer. Patient demographics, comorbidities, and perioperative risk factors were examined as covariates, and the primary outcome was return to OR within 30 days of surgery. A multivariate regression was performed to determine independent preoperative factors associated with this complication. RESULTS: In total, 855 patients underwent FF for head/neck reconstruction most commonly for the Tongue (24.7%) and Mouth/Floor/cavity (25.0%). Of these, 153 patients (17.9%) returned to the OR within 30 days of surgery. Patients in this cohort had higher rates of wound infections and dehiscence (p < 0.01). Medical complications were significantly higher and included pneumonia (12.4% vs 5.0%, p < 0.01), prolonged ventilation (16.3% vs 4.8%, p < 0.01), myocardial infarction (2.6% vs 0.6%, p = 0.017), and sepsis (7.2% vs 3.4%, p = 0.033). Regression analysis demonstrated that visceral flaps (OR = 9.7, p = 0.012) and hypoalbuminemia (OR = 2.4, p = 0.009) were significant predictors of a return to the OR. CONCLUSION: Based on data from the nationwide NSQIP dataset, up to 17% of HNFF return to the OR within 30 days. Although this data-set has some significant limitations, these results can cautiously help to improve preoperative patient optimisation and surgical decision-making.

A Comparative Cost Analysis of Cleft Lip Adhesion and Nasoalveolar Molding before Formal Cleft Lip Repair.

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

The mandibular deformity in hemifacial microsomia: a reassessment of the Pruzansky and Kaban classification.

BACKGROUND: The authors examined hemifacial microsomia using three-dimensional computed tomography and the Kaban modification of the Pruzansky classification to determine its relationship with traditional evaluation and its reproducibility among evaluators. METHODS: A retrospective review of all patients diagnosed with hemifacial microsomia was performed. Three-dimensional computed tomographic scans were reviewed by expert evaluators and rated according to evaluators' understanding of the Kaban modification of the Pruzansky classification. The clinical Kaban-Pruzansky score was recorded at the time of initial clinical presentation and an in-house score stratified the population into mild (0-I), moderate (IIA), and severe (IIB-III). These two standards and the evaluators' scores were compared. Fleiss's kappa was used to assess interrater variability (p < 0.05). RESULTS: Forty-one patients met inclusion criteria, and 38 had documented clinical Kaban-Pruzansky scores. Sixteen craniofacial surgeons with an average of 15.5 years (range, 6 to 38 years) of experience were surveyed. Fair interrater reproducibility was found among all expert evaluators (Fleiss ĸ = 0.238). When comparing raters' three-dimensional computed tomography-based classification to the clinical Kaban-Pruzansky scores, the average agreement was 39.17 ± 8.83 percent (average ĸ = 0.257 ± 0.147) (p = 0.90). When comparing raters' classification to the in-house score, the average agreement was 69.71 ± 9.42 percent (p = 0.97) (average ĸ = 0.576 ± 0.140). CONCLUSIONS: The introduction of three-dimensional computed tomography into the diagnostic paradigm highlights the inaccuracy and variability of traditional classification systems. The results question the accuracy and reproducibility of the current clinical paradigm, suggesting the need to reexamine the classification of hemifacial microsomia. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, III.

Sustained delivery of rhBMP-2 by means of poly(lactic-co-glycolic acid) microspheres: cranial bone regeneration without heterotopic ossification or craniosynostosis.

BACKGROUND: Commercially available recombinant human bone morphogenetic protein 2 (rhBMP2) has demonstrated efficacy in bone regeneration, but not without significant side effects. The authors used rhBMP2 encapsulated in poly(lactic-co-glycolic acid) (PLGA) microspheres placed in a rabbit cranial defect model to test whether low-dose, sustained delivery can effectively induce bone regeneration. METHODS: The rhBMP2 was encapsulated in 15% PLGA using a double-emulsion, solvent extraction/evaporation technique, and its release kinetics and bioactivity were tested. Two critical-size defects (10 mm) were created in the calvaria of New Zealand white rabbits (5 to 7 months of age, male and female) and filled with a collagen scaffold containing either (1) no implant, (2) collagen scaffold only, (3) PLGA-rhBMP2 (0.1 µg per implant), or (4) free rhBMP2 (0.1 µg per implant). After 6 weeks, the rabbits were killed and defects were analyzed by micro-computed tomography, histology, and finite element analysis. RESULTS: The rhBMP2 delivered by means of bioactive PLGA microspheres resulted in higher volumes and surface area coverage of new bone than an equal dose of free rhBMP2 by micro-computed tomography (p=0.025 and p=0.025). Finite element analysis indicated that the mechanical competence using the regional elastic modulus did not differ with rhBMP2 exposure (p=0.70). PLGA-rhBMP2 did not demonstrate heterotopic ossification, craniosynostosis, or seroma formation. CONCLUSIONS: Sustained delivery by means of PLGA microspheres can significantly reduce the rhBMP2 dose required for de novo bone formation. Optimization of the delivery system may be a key to reducing the risk for recently reported rhBMP2-related adverse effects.