Paliga Jt

An evaluation of complications, revisions, and long-term aesthetic outcomes in nonsyndromic metopic craniosynostosis.

Background: The authors evaluated the complications, revisions, and long-term aesthetic outcomes of patients with isolated metopic synostosis. Methods: A retrospective chart review was performed on consecutive metopic craniosynostosis patients treated from June of 1987 to June of 2012 at The Children’s Hospital of Philadelphia. Patient demographics, operative details, and postoperative data were collected. Outcomes were reported as Whitaker classification and postoperative clinical characteristics assessed before additional interventions. Reoperation in patients with greater than 5 years of follow-up was noted. Appropriate statistical analyses were applied. Results: From 1987 to 2012, 178 patients underwent surgical correction of isolated metopic craniosynostosis, and 147 met inclusion criteria. Average age at surgery was 0.83 year (range, 0.3 to 4.7 years); average follow-up was 5.8 years (range, 1.0 to 17.8 years). There were 13 surgical complications (8.8 percent), three major (2.0 percent), and 10 minor (6.8 percent). At follow-up, 67 patients (56.8 percent) were classified as Whitaker class I, six (5.1 percent) as class II, 43 (36.4 percent) as class III, and two (1.7 percent) as class IV. Patients with greater than 5 years’ follow-up (n = 57) were more likely to have temporal hollowing (OR, 2.9; 95 percent CI, 1.2 to 7.3; p = 0.021), lateral orbital retrusion (OR, 4.9; 95 percent CI, 1.9 to 12.7; p = 0.001), and Whitaker class III or IV classification (OR, 4.0; 95 percent CI, 1.5 to 10.6; p = 0.006) compared with those with less than 5 years’ follow-up. Conclusion: This study reports low complication and reoperation rates in the treatment of isolated metopic craniosynostosis, but demonstrates a clear trend toward worsening aesthetic outcomes over time. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Cranioplasty: indications and advances.

Purpose of reviewCranioplasty has been long practiced, and the reconstructive techniques continue to evolve. With a variety of options available for filling cranial defects, a review of the current practices in cranioplasty allows for reporting the most advanced techniques and specific indications. Recent findingsOverwhelming support remains for the use of autologous bone grafts in filling the cranial defects. Alloplastic alternatives have relative advantages and disadvantages depending on the patient population and specific indications. Application of imaging technology has allowed for the utilization of custom-made alloplastic implants when autologous bone grafts are not feasible. SummaryAutologous bone grafts remain the best option for adult and pediatric patients with viable donor sites and small-to-medium defects. Large defects in the adult population can be reconstructed with titanium mesh and polymethylmethacrylate overlay with or without the use of computer-assisted design and manufacturing customization. In pediatric patients, exchange cranioplasty offers a viable technique for using an autologous bone graft, while simultaneously filling the donor site with particulate bone graft. Advances in alloplastic materials and custom manufacturing of implants will have an important influence on cranioplasty techniques in the years to come.

A critical evaluation of long-term aesthetic outcomes of fronto-orbital advancement and cranial vault remodeling in nonsyndromic unicoronal craniosynostosis.

Background: This study reports long-term aesthetic outcomes with fronto-orbital advancement and cranial vault remodeling in treating unicoronal synostosis over a 35-year period. Methods: Retrospective review was performed on patients with isolated unicoronal synostosis from 1977 to 2012. Demographic, preoperative phenotypic, and long-term aesthetic outcomes data were analyzed with chi-squared and Fisher’s exact test for categorical data and Wilcoxon rank-sum and Kruskal-Wallis rank for continuous data. Results: A total of 238 patients were treated; 207 met inclusion criteria. None underwent secondary intervention for intracranial pressure. At definitive intervention, there 96 (55 percent) Whitaker class I patients, 11 (6 percent) class II, 62 (35 percent) class III, and six (3 percent) class IV. Nasal root deviation and occipital bossing each conferred an increased risk of Whitaker class III/IV [OR, 4.4 (1.4 to 13.9), p = 0.011; OR, 2.6 (1.0 to 6.8), p = 0.049]. Patients who underwent bilateral cranial vault remodeling with extended unilateral bandeau were less likely Whitaker class III/IV at latest follow-up compared with those undergoing strictly unilateral procedures [OR, 0.2 (0.1 to 0.7), p = 0.011]. Overcorrection resulted in decreased risk of temporal hollowing [OR, 0.3 (0.1 to 1.0), p = 0.05]. Patients with 5 years or more of follow-up were more likely to develop supraorbital retrusion [OR, 7.2 (2.2 to 23.4), p = 0.001] and temporal hollowing [OR, 3.7 (1.5 to 9.6), p = 0.006] and have Whitaker class III/IV outcomes [OR, 4.9 (1.8 to 12.8), p = 0.001]. Conclusion: Traditional fronto-orbital advancement and cranial vault remodeling appears to mitigate risk of intracranial pressure but may lead to aesthetic shortcomings as patients mature, namely fronto-orbital retrusion and temporal hollowing. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Perioperative complications associated with intracranial procedures in patients with nonsyndromic single-suture craniosynostosis.

AbstractWithin the diagnosis “craniosynostosis,” there is a subset of patients who present with isolated, nonsyndromic, single-suture involvement. This study evaluates perioperative complications in this specific subset of patients over 4 decades at a single institution. To do so, we performed a retrospective review on consecutive patients undergoing correction of single-suture synostosis from May 1977 to January 2013 at a tertiary pediatric craniofacial center. Demographic information, operative details, and perioperative course were collected. Complications were categorized as either major or minor. A &khgr;2 test and Fisher exact test were used to compare all categorical variables. Continuous variables were analyzed using Wilcoxon rank-sum and Kruskal-Wallis tests.Seven hundred forty-six patients underwent surgical correction of nonsyndromic craniosynostosis. Of these, there were 307 (41.2%) sagittal, 201 (26.9%) metopic, and 238 (31.9%) unicoronal. Thirty-four patients had complications (4.6%). Eight were considered major (1.1%), including one postoperative mortality in a patient with hypoplastic left-sided heart syndrome. Minor complications occurred in 26 patients (3.5%) and included subgaleal hematoma (n = 3), seroma (n = 4), and superficial wound infection (n = 5). Metopic and sagittal suture involvement was significantly associated with a higher complication rate (P = 0.04). A child with isolated single suture synostosis and any comorbidity had a significantly greater risk of any complication (P < 0.001; odds ratio, 3.8) and specifically an increased risk of major complication (P = 0.031; odds ratio, 6.0). Subclassification of patients by time period yielded no statistically significant changes in perioperative morbidity. To conclude, these data allow us to counsel families more accurately with regard to morbidity and mortality and may potentially serve as a benchmark for future quality improvement work.

Earlier evidence of spheno-occipital synchondrosis fusion correlates with severity of midface hypoplasia in patients with syndromic craniosynostosis.

Background: The spheno-occipital synchondrosis is an important driver of facial and cranial base growth. The current study characterizes its fusion in patients with Apert, Crouzon, and Pfeiffer syndromes and correlates early fusion with the presence, and degree, of midface hypoplasia. Methods: A retrospective case-control study was performed of all syndromic patients treated between 1996 and 2012. Case computed tomographic scans and age- and sex-matched control scans were analyzed as demonstrating either open, partially fused, or completely fused synchondroses, and patient age at each scan was recorded. Midface hypoplasia as determined by sella-nasion–A point angle measurement at the time of midface surgery was correlated to fusion status. Results: Fifty-four patients with 206 computed tomographic scans met inclusion criteria. Two hundred six age- and sex-matched control scans were also identified. Average age at computed tomographic scanning was 6.1 years. The earliest ages of partial and complete fusion were 1.1 and 7.0 years, respectively, among cases; and 6.2 and 12.7 years, respectively, among controls. The odds of synchondrosis fusion in case computed tomographic scans was 66.0 times that of controls (95 percent CI, 9.2 to 475.5 times that of controls; p < 0.000001). Average age of synchondrosis fusion was 3.5 years (range, 0.5 to 6.0 years). Average sella-nasion–A point angle at the time of midface surgery was 67.5 degrees (range, 58 to 76 degrees), with a positive correlation between earlier age of fusion and more severe midface hypoplasia (p = 0.028). Conclusions: The spheno-occipital synchondrosis fuses earlier in syndromic patients compared with age-matched controls. Moreover, there is a positive correlation between earlier fusion and degree of midface hypoplasia, although definitive causality cannot be concluded. This is the first study to demonstrate such a correlation in human subjects. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Premature closure of the spheno-occipital synchondrosis in Pfeiffer syndrome: a link to midface hypoplasia.

AbstractThe spheno-occipital synchondrosis (SOS) is a critical component of midfacial and cranial base growth. Premature closure has been associated with midface hypoplasia in animal models and syndromic craniosynostosis subpopulations with Apert and Muenke syndromes. To link premature SOS closure and midface hypoplasia in patients with Pfeiffer syndrome, a retrospective case-control study was performed in patients treated at a large craniofacial center between 1982 and 2012 diagnosed with Pfeiffer syndrome. At least 1 computed tomography (CT) scan was required to assess SOS patency. Age-/sex-matched control CT scans were also assessed for SOS patency. Three independent reviewers with high interrater reliability (&kgr; = 0.88) graded SOS patency as open, partially closed, or completely closed. Wilcoxon rank sum test compared the Pfeiffer patients with control subjects.A total of 63 CT scans in 16 patients with Pfeiffer syndrome, all with midface hypoplasia, and 63 age-/sex-matched control scans, none of whom had midface hypoplasia, met inclusion criteria. Earliest partial SOS closure in patients with Pfeiffer syndrome was seen at 5 days compared with control subjects at 7.07 years. Earliest age at complete fusion was 2.76 years in the Pfeiffer cohort and 12.74 years in control subjects. Average age at partial closure was significantly younger (4.99 ± 3.33 years; n = 31 scans) in patients with Pfeiffer syndrome compared with control subjects (10.92 ± 3.53 years) (P = 0.0005), whereas average age at complete closure (11.90 ± 7.04 years) was not significantly different than that in control subjects (16.07 ± 3.39 years). Although definitive causality cannot be concluded, a strong correlation exists between midface hypoplasia and premature SOS closure in Pfeiffer syndrome.

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.

Clarifying the relationships among the different features of the OMENS+ classification in craniofacial microsomia.

Background: The OMENS+ classification is commonly used to describe the phenotypically diverse craniofacial features of craniofacial microsomia. The purpose of this study was to evaluate associations among the individual components of the OMENS+ criteria. Methods: An institutional review board–approved retrospective chart review was performed for patients who presented with a diagnosis of unilateral or bilateral craniofacial microsomia to the craniofacial clinic from January of 1990 to December of 2012. Demographic, diagnosis, classification, treatment, and radiographic data were abstracted for all patients who met inclusion criteria. Associations and correlations were evaluated using the Spearman rank test and a logistic regression model. Results: One hundred five patients (61 male and 44 female) with craniofacial microsomia met inclusion criteria. Eighty-one patients (77.1 percent) had unilateral microsomia and 24 (22.9 percent) had bilateral microsomia. Twenty-eight patients (26.7 percent) had macrostomia. Correlations were all significantly interrelated (p = 0.000 to p = 00.018) between the degree of orbital, mandibular, and soft-tissue deformities. Moreover, the severity of ear deformity and facial nerve involvement were also significantly correlated (p = 0.008). Between these two groupings, there was a significant correlation between soft-tissue deficiency and nerve involvement (p = 0.010). Macrostomia was associated with the individual components of the group orbit (p = 0.008), mandible (p = 0.000), and soft tissue (p = 0.005). Conclusions: The association between structures using the OMENS+ classification may be caused by their branchial arch origin. Structures mainly developed from the first branchial arch (orbit, mandible, and soft tissue) are associated in degree of severity, as are the structures mainly derived from the second branchial arch (facial nerve and ear). CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Utility of screening for obstructive sleep apnea syndrome in children with craniofacial disorders.

Background: Children with craniofacial disorders are at increased risk for obstructive sleep apnea syndrome. Methods for diagnosing obstructive sleep apnea syndrome in this population remain controversial. Sleep studies are the criterion standard but are impractical for all patients. The utility of obstructive sleep apnea syndrome questionnaires such as the Pediatric Sleep Questionnaire is unknown in children with craniofacial disorders. The authors hypothesized that the Pediatric Sleep Questionnaire would be a sensitive tool for detecting obstructive sleep apnea syndrome in children with craniofacial abnormalities. Methods: A retrospective review of consecutive children with diagnosed craniofacial disorders who both completed the Pediatric Sleep Questionnaire and underwent polysomnography was performed. Demographics, Pediatric Sleep Questionnaire score, and polysomnographic data were recorded. Statistical analysis included calculation of sensitivity, specificity, positive predictive value, and negative predictive value for the Pediatric Sleep Questionnaire. Results: Eighty-three children aged 2 to 18 years were included in the study. Of these, 44 (53.0 percent) screened positive on the Pediatric Sleep Questionnaire and 23 (27.7 percent) had polysomnographic evidence of obstructive sleep apnea syndrome, but the sensitivity of the Pediatric Sleep Questionnaire for detecting obstructive sleep apnea syndrome in this sample was only 0.57 and the specificity was 0.48. Positive predictive value and negative predictive value were 0.30 and 0.74, respectively. The correlation between the apnea hypopnea index and Pediatric Sleep Questionnaire score was 0.152 (p = 0.17). Conclusions: A substantial portion of craniofacial patients referred for polysomnography was found to have obstructive sleep apnea syndrome. However, the Pediatric Sleep Questionnaire is not a good screening tool for obstructive sleep apnea syndrome in children with craniofacial conditions. More research is needed to determine which patients with craniofacial disorders should be evaluated for obstructive sleep apnea syndrome by polysomnography or other means. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.

Monobloc minus Le Fort II for single-stage treatment of the Apert phenotype.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences.Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.AbstractTreatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences.Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.