Gary B. Skolnick

An E23K single nucleotide polymorphism in the islet ATP-sensitive potassium channel gene (Kir6.2) contributes as much to the risk of Type II diabetes in Caucasians as the PPARγ Pro12Ala variant

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Assessing long-term outcomes of open and endoscopic sagittal synostosis reconstruction using three-dimensional photography.

AbstractSagittal synostosis has been successfully managed with numerous surgical techniques. Nevertheless, few data on long-term outcomes exist to justify use of one surgical technique over another. In this study, we compared children with surgically corrected sagittal synostosis to their age-matched control subjects to assess the longevity of their corrections. Furthermore, the outcomes of open repairs were compared with endoscopic repairs.Following institutional review board approval, three-dimensional photographs of patients who underwent surgical reconstruction for nonsyndromic sagittal synostosis were analyzed to determine biparietal and anterior-posterior diameter, circumference, cephalic index, cranial vault volume, cranial height, and forehead inclination. Thirteen patients who had undergone open repair, including 6 total cranial vault and 7 modified-pi reconstructions, and 6 patients who had undergone endoscopic strip craniectomy with barrel-stave osteotomies and postoperative helmeting were compared with nonsynostotic age-matched control subjects. Mean follow-up was 97.5 months after open and 48.9 months after endoscopic repair. Student t tests were used for analysis. In the second arm of this study, 33 patients who had undergone endoscopic repair were compared with the 13 patients who had undergone open repair; mean follow-up was 24.8 months after endoscopic repair. Linear regression models were used to adjust for age and sex.After comparing three-dimensional photographs of children who were more than 3 years postoperative from surgical correction for sagittal synostosis with their age-matched control subjects, no statistically significant differences were found in any of the measured parameters. In addition, no differences were detected between open reconstruction versus endoscopic repair, suggesting equivalence in final results for both procedures.

Are endoscopic and open treatments of metopic synostosis equivalent in treating trigonocephaly and hypotelorism

BackgroundPatients with metopic craniosynostosis are traditionally treated with fronto-orbital advancement to correct hypotelorism and trigonocephaly. Alternatively, endoscopic-assisted treatment comprises narrow ostectomy of the fused suture followed by postoperative helmet therapy. Here we compare the preoperative and 1-year postoperative results in open versus endoscopic repairs. MethodsWe reviewed preoperative and 1-year postoperative three-dimensional reconstructed computed tomography scans of patients treated for nonsyndromic metopic craniosynostosis by either open (n = 15) or endoscopic (n = 13) technique. Hypotelorism was assessed by interzygomaticofrontal distance and intercanthal distance. Trigonocephaly was assessed by 2 independent angles: first, an axial-plane two-dimensional angle between zygomaticofrontal suture bilaterally and the glabella (ZFR-G-ZFL); second, an interfrontal angle (IFA) between the most anterior point from a reconstructed midsagittal plane and supraorbital notch bilaterally. Age-matched scans of unaffected patients (n = 28) served as controls for each postoperative scan. ResultsPatients with open repair (9.5 ± 1.8 months) were older at time of surgery than patients with endoscopic repairs (3.3 ± 0.4 months) (P = 0.004). Male-to-female ratios were equivalent at roughly 7:3 in both groups. Preoperatively, the endoscopic group had worse hypotelorism and ZFR-G-ZFL than the open group (P ⩽ 0.04). After accounting for preoperative differences, all of the postoperative measurements (ie, interzygomaticofrontal distance, intercanthal distance, ZFR-G-ZFL angle, IFA) of the 2 groups were statistically equivalent (P ≥ 0.135). Trigonocephaly was significantly improved after repair in both the open (8 degrees [ZFR-G-ZFL] and 18 degrees [IFA]) and endoscopic (13 degrees [ZFR-G-ZFL] and 16 degrees [IFA]) groups (P < 0.001). Postoperative measures in both groups were equivalent to controls (0.12 < P < 0.89). Intrarater reliability ranged from 0.93 to 0.99 for all measurements. ConclusionOur retrospective series shows that endoscopic and open repairs of metopic craniosynostosis are equivalent in improving hypotelorism and trigonocephaly at 1-year follow-up. Additional studies are necessary to better define minor differences in morphology, which may result from the different techniques.

Comparison of Traditional versus Normative Cephalic Index in Patients with Sagittal Synostosis: Measure of Scaphocephaly and Postoperative Outcome.

Background: Preoperative severity and postoperative success for patients with sagittal synostosis are measured by cephalic index, but this metric does not describe the appropriateness of euryon location. The authors hypothesize that cephalic index in patients with sagittal synostosis is an inaccurate measure of scaphocephaly. Methods: Preoperative and 1-year postoperative cranial computed tomographic scans of children with sagittal synostosis treated before 6 months of age by either total calvarial reconstruction or endoscope-assisted craniectomy and helmet therapy (n = 10 for each) were reviewed retrospectively. The location of euryons in age-matched controls was measured as a fraction of the glabella-opisthocranion distance (horizontal point of maximum width) and as the fraction of the nasion-vertex vertical distance (vertical point of maximum width). Cephalic index at this ideal location (normative cephalic index) and traditional cephalic index were determined in all patients. Results: Ideal euryon location from preoperative controls was 56 percent by the horizontal point of maximum width and 56 percent by the vertical point of maximum width. Normative cephalic index (0.60) was significantly less than traditional cephalic index (0.66) in patients preoperatively (p < 0.001) and remained smaller postoperatively (0.68 versus 0.73) for patients who underwent open reconstruction (p < 0.001). Patients treated endoscopically also had a smaller normative cephalic index (0.71) than traditional cephalic index (0.76) postoperatively (p < 0.001). Conclusions: Anterocaudal displacement of euryon in patients with sagittal synostosis influences cephalic index. Normative cephalic index, assessed at ideal euryon location, is a more accurate measure of preoperative severity and postoperative outcome. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, III.

Cranial Base and Posterior Cranial Vault Asymmetry After Open and Endoscopic Repair of Isolated Lambdoid Craniosynostosis.

Background:Previous studies have shown that open cranial vault remodeling does not fully address the endocranial deformity. This study aims to compare endoscopic-assisted suturectomy with postoperative molding helmet therapy to traditional open reconstruction by quantifying changes in cranial base morphology and posterior cranial vault asymmetry. Methods:Anthropometric measurements were made on pre- and 1-year postoperative three-dimensionally reconstructed computed tomography scans of 12 patients with unilateral lambdoid synostosis (8 open and 4 endoscopic-assisted). Cranial base asymmetry was analyzed using: posterior fossa deflection angle (PFA), petrous ridge angle (PRA), mastoid cant angle (MCA), and vertical and anterior–posterior (A–P) displacement of external acoustic meatus (EAM). Posterior cranial vault asymmetry was quantified by volumetric analysis. Results:Preoperatively, patients in the open and endoscopic groups were statistically equivalent in PFA, PRA, MCA, and A–P EAM displacement. At 1 year postoperatively, open and endoscopic patients were statistically equivalent in all measures. Mean postoperative PFA for the open and endoscopic groups was 6.6 and 6.4 degrees, PRA asymmetry was 6.4 and 7.6%, MCA was 4.0 and 3.2 degrees, vertical EAM displacement was −2.3 and −2.3 millimeters, and A–P EAM displacement was 6.8 and 7.8 millimeters, respectively. Mean volume asymmetry was significantly improved in both open and endoscopic groups, with no difference in postoperative asymmetry between the 2 groups (P = 0.934). Conclusions:Patients treated with both open and endoscopic repair of lambdoid synostosis show persistent cranial base and posterior cranial vault asymmetry. The results of endoscopic-assisted suturectomy with postoperative molding helmet therapy are similar to those of open calvarial vault reconstruction.

Comparative study of cranial anthropometric measurement by traditional calipers to computed tomography and three-dimensional photogrammetry.

AbstractCraniofacial anthropometry by direct caliper measurements is a common method of quantifying the morphology of the cranial vault. New digital imaging modalities including computed tomography and three-dimensional photogrammetry are similarly being used to obtain craniofacial surface measurements. This study sought to compare the accuracy of anthropometric measurements obtained by calipers versus 2 methods of digital imaging.Standard anterior-posterior, biparietal, and cranial index measurements were directly obtained on 19 participants with an age range of 1 to 20 months. Computed tomographic scans and three-dimensional photographs were both obtained on each child within 2 weeks of the clinical examination. Two analysts measured the anterior-posterior and biparietal distances on the digital images. Measures of reliability and bias between the modalities were calculated and compared.Caliper measurements were found to underestimate the anterior-posterior and biparietal distances as compared with those of the computed tomography and the three-dimensional photogrammetry (P < 0.001). Cranial index measurements between the computed tomography and the calipers differed by up to 6%. The difference between the 2 modalities was statistically significant (P = 0.021). The biparietal and cranial index results were similar between the digital modalities, but the anterior-posterior measurement was greater with the three-dimensional photogrammetry (P = 0.002). The coefficients of variation for repeated measures based on the computed tomography and the three-dimensional photogrammetry were 0.008 and 0.007, respectively.In conclusion, measurements based on digital modalities are generally reliable and interchangeable. Caliper measurements lead to underestimation of anterior-posterior and biparietal values compared with digital imaging.

Endoscope-assisted management of sagittal synostosis: wide vertex suturectomy and barrel stave osteotomies versus narrow vertex suturectomy

OBJECTIVE Endoscope-assisted methods for treatment of craniosynostosis have reported benefits over open calvarial vault reconstruction. In this paper, the authors evaluated 2 methods for endoscope-assisted correction of sagittal synostosis: wide vertex suturectomy and barrel stave osteotomies (WVS+BSO) and narrow vertex suturectomy (NVS). METHODS The authors evaluated patients with nonsyndromic sagittal synostosis treated with either wide vertex suturectomy (4–6 cm) and barrel stave osteotomies (WVS+BSO) or narrow vertex suturectomy (NVS) (approximately 2 cm) between October 2006 and July 2013. Prospectively collected data included patient age, sex, operative time, estimated blood loss (EBL), postoperative hemoglobin level, number of transfusions, complications, and cephalic index. Fourteen patients in the NVS group were age matched to 14 patients in the WVS+BSO group. Descriptive statistics were calculated, and Student t-tests were used to compare prospectively obtained data from the WVS+BSO group with t...

Comparison of Direct and Digital Measures of Cranial Vault Asymmetry for Assessment of Plagiocephaly.

Abstract Measurement of cranial vault asymmetry (CVA) is a common feature in the treatment of patients with deformational plagiocephaly (DP). In many cases, this measure is the primary marker of improvement. CVA is typically measured with calipers and is subject to interrater variability. There is little research comparing results of calipers with those of three-dimensional (3D) photogrammetry. Fifty nine visits were made by 51 children previously diagnosed with DP. Thirty eight were male and 13 were female. Thirty one of the visits included a 3D photograph. Direct measures were obtained by 2 experienced anthropometrists and included head length, width, circumference, and CVA. Their results were compared to digital measures including measures unobtainable with calipers, asymmetry of head circumference and global asymmetry. The interrater reliability of all caliper measures was excellent (intraclass correlation coefficients > 0.94). Caliper and digital measures of length, width, cephalic index, and circumference were strongly correlated (R 2 > 0.90). There was a consistent bias, caliper measures being 1 to 4 mm shorter than their digital analogues. Caliper measured CVA was highly correlated (R 2 > 0.90) with the directly corresponding digital measures. It was poorly correlated with measures of overall hemispheric asymmetry (R 2 < 0.10). The cranial measurements of children with DP taken independently by 2 experienced anthropometrists showed excellent interrater reliability. Caliper measures are consistently smaller than the digital measures, presumably due to pressure of the calipers and/or the use of skullcaps during photography. Like circumference and other assessments, cranial vault asymmetry measures correlate well with their analogous digital measurements.

Analysis of digital measures of cranial vault asymmetry for assessment of plagiocephaly.

Background Deformational plagiocephaly (DP) is an asymmetry of the skull caused by extrinsic compression. With the advent of the Back to Sleep campaign, DP is of increased interest to parents and healthcare providers. Traditionally, this asymmetry has been assessed by caliper measurements. However, little consensus exists as to which caliper measures (linear) give the most accurate representation of DP. The purposes of this study are to examine different measures and to determine which have the greatest validity in the assessment of skull asymmetry. Methods Six linear measures were assessed using 31 three-dimensional photographs of 26 children diagnosed with DP. These measurements were compared with more encompassing measures: a head perimeter measurement, a global three-dimensional measure of hemispheric asymmetry, and the radial symmetry index. These 3 broader measures were used to determine which linear, caliper-style measurements most accurately reflect overall DP. Results Intrarater reliability of the measurements varied widely (intraclass correlation coefficients from 0.42 to 0.99). Correlations between the measures also varied widely (0.10 < r < 0.95). The linear measure that best correlated with the inclusive measures of asymmetry was FZ-EU, the distance from the frontozygomaticus to the contralateral eurion (r ≥ 0.90). Conclusions These data introduce 2 digital measures that might serve as standards against which linear measures of asymmetry may be tested: Global asymmetry and head perimeter measures were strongly correlated to each other. When these 2 parameters were compared against linear measures, FZ-EU was noted to be the best linear measure of asymmetry based on these benchmarks.

Effects of open and endoscopic surgery on skull growth and calvarial vault volumes in sagittal synostosis.

BackgroundThere have been conflicting reports on how sagittal synostosis affects cranial vault volume (CVV) and which surgical approach best normalizes skull volume. In this study, we compared CVV and cranial index (CI) of children with sagittal synostosis (before and after surgery) with those of control subjects. We also compared the effect of repair type on surgical outcome. MethodsComputed tomography scans of 32 children with sagittal synostosis and 61 age- and sex-matched control subjects were evaluated using previously validated segmentation software for CVV and CI. Sixteen cases underwent open surgery, and 16 underwent endoscopic surgery. Twenty-seven cases had both preoperative and postoperative scans. ResultsAge of subjects at computed tomography scan ranged from 1 to 9 months preoperatively and 15 to 25 months postoperatively. Mean age difference between cases and matched control subjects was 5 days. The mean CVV of cases preoperatively was nonsignificantly (17 mL) smaller than that of control subjects (P = 0.51). The mean CVV of postoperative children was nonsignificantly (24 mL) larger than that of control subjects (P = 0.51). Adjusting for age and sex, there was no significant difference in CVV between open and endoscopic cases postoperatively (&bgr; = 48 mL, P = 0.31). The mean CI increased 12% in both groups. There was no significant difference in mean postoperative CI (P = 0.18) between the 2 groups. ConclusionsPreoperatively, children with sagittal synostosis have no significant difference in CVV compared with control subjects. Type of surgery does not seem to affect CI and CVV 1 year postoperatively. Both open and endoscopic procedures result in CVVs similar to control subjects.