Comparison of aesthetic outcomes between open and endoscopically treated sagittal craniosynostosis

2021 ◽  
pp. 1-7
Author(s):  
Jacob Lepard ◽  
S. Hassan A. Akbari ◽  
James Mooney ◽  
Anastasia Arynchyna ◽  
Samuel G. McClugage III ◽  
...  
Keyword(s):  
Head Circumference ◽  
Endoscopic Treatment ◽  
Open Repair ◽  
Perioperative Outcomes ◽  
Subjective Rating ◽  
Cephalic Index ◽  
Sagittal Synostosis ◽  
Sagittal Craniosynostosis ◽  
Subjective Assessments ◽  
Z Scores

OBJECTIVE In the last several decades, there has been much debate regarding the ideal treatment for sagittal synostosis. The purpose of this study was to compare perioperative, anthropometric, and subjective assessments of cosmetic outcomes between open and endoscopic management of isolated sagittal synostosis. METHODS At their routine postoperative follow-up, pediatric patients with sagittal craniosynostosis were recruited to undergo digital cranial measurement and standardized photography for objective and subjective assessments of perioperative outcomes. Age-normalized z-scores for cephalic index, head circumference, euryon-euryon diameter (Eu-Eu), and glabella-opisthocranion diameter (G-Op) were calculated for each patient. Faculty surgeons, surgical trainees, nurses, and laypersons were asked to rate the normalcy of craniofacial appearances using a 5-point Likert scale. Outcomes were compared between patients treated with endoscopic correction and those treated with open repair. RESULTS A total of 50 patients were included in the study. Thirty-one had undergone open surgical correction, and 19 had undergone endoscopic treatment. Endoscopic repair involved significantly lower operative time, blood loss, transfusion rate, and hospital length of stay than those with open repair (p < 0.001). There was no significant difference between groups in terms of z-scores for head circumference (p = 0.22), cephalic index (p = 0.25), or Eu-Eu (p = 0.38). Endoscopic treatment was associated with a significantly lower G-Op (p = 0.009). Additionally, the average subjective rating of head shape was higher for endoscopic treatment when corrected for age, gender, and ethnicity (p = 0.02). CONCLUSIONS The study findings suggest that patients who are treated endoscopically may have an overall more normal appearance in skull morphology and cosmesis, although these results are limited by poor reliability.

Endoscopic treatment of combined metopic-sagittal craniosynostosis

2020 ◽  
Vol 26 (2) ◽  
pp. 113-121
Author(s):  
Ema Zubovic ◽  
Gary B. Skolnick ◽  
Sybill D. Naidoo ◽  
Mark Bellanger ◽  
Matthew D. Smyth ◽  
...  
Keyword(s):  
Endoscopic Treatment ◽  
Ct Scans ◽  
Sagittal Synostosis ◽  
Sagittal Craniosynostosis ◽  
Cranial Index ◽  
Helmet Therapy ◽  
The Mean ◽  
Normal Controls ◽  
Cranial Vault Remodeling

OBJECTIVECombined metopic-sagittal craniosynostosis is traditionally treated with open cranial vault remodeling and fronto-orbital advancement, sometimes in multiple operations. Endoscopic treatment of this multisuture synostosis presents a complex challenge for the surgeon and orthotist.METHODSThe authors retrospectively analyzed the preoperative and 1-year postoperative CT scans of 3 patients with combined metopic-sagittal synostosis, all of whom were treated with simultaneous endoscope-assisted craniectomy of the metopic and sagittal sutures followed by helmet therapy. Established anthropometric measurements were applied to assess pre- and postoperative morphology, including cranial index and interfrontal divergence angle (IFDA). Patients’ measurements were compared to those obtained in 18 normal controls.RESULTSTwo boys and one girl underwent endoscope-assisted craniectomy at a mean age of 81 days. The mean preoperative cranial index was 0.70 (vs control mean of 0.82, p = 0.009), corrected postoperatively to a mean of 0.82 (vs control mean of 0.80, p = 0.606). The mean preoperative IFDA was 110.4° (vs control mean of 152.6°, p = 0.017), corrected postoperatively to a mean of 139.1° (vs control mean of 140.3°, p = 0.348). The mean blood loss was 100 mL and the mean length of stay was 1.7 days. No patient underwent reoperation. The mean clinical follow-up was 3.4 years.CONCLUSIONSEndoscope-assisted craniectomy with helmet therapy is a viable single-stage treatment option for combined metopic-sagittal synostosis, providing correction of the stigmata of trigonoscaphocephaly, with normalization of the cranial index and IFDA.

scholarly journals Regression of cephalic index following endoscopic repair of sagittal synostosis

2019 ◽  
Vol 23 (1) ◽  
pp. 54-60
Author(s):  
Nicholas A. Pickersgill ◽  
Gary B. Skolnick ◽  
Sybill D. Naidoo ◽  
Matthew D. Smyth ◽  
Kamlesh B. Patel
Keyword(s):  
Time Course ◽  
Strong Positive Correlation ◽  
Cephalic Index ◽  
Sagittal Synostosis ◽  
Endoscopic Repair ◽  
Future Studies ◽  
Helmet Therapy ◽  
Significant Regression ◽  
Strip Craniectomy

OBJECTIVEMetrics used to quantify preoperative severity and postoperative outcomes for patients with sagittal synostosis include cephalic index (CI), the well-known standard, and the recently described adjusted cephalic index (aCI), which accounts for altered euryon location. This study tracks the time course of these measures following endoscopic repair with orthotic helmet therapy. The authors hypothesize that CI and aCI show significant regression following endoscope-assisted repair.METHODSCT scans or 3D photographs of patients with nonsyndromic sagittal synostosis treated before 6 months of age by endoscope-assisted strip craniectomy and postoperative helmet therapy (n = 41) were reviewed retrospectively at three time points (preoperatively, 0–2 months after helmeting, and > 24 months postoperatively). The CI and aCI were measured at each time point.RESULTSMean CI and aCI increased from 71.8 to 78.2 and 62.7 to 72.4, respectively, during helmet treatment (p < 0.001). At final follow-up, mean CI and aCI had regressed significantly from 78.2 to 76.5 and 72.4 to 69.7, respectively (p < 0.001). The CI regressed in 33 of 41 cases (80%) and aCI in 39 of 41 cases (95%). The authors observed a mean loss of 31% of improvement in aCI achieved through treatment. A strong, positive correlation existed between CI and aCI (R = 0.88).CONCLUSIONSRegression following endoscope-assisted strip craniectomy with postoperative helmet therapy commonly occurs in patients with sagittal synostosis. Future studies are required to determine whether duration of helmet therapy or modifications in helmet design affect regression.

Comparing Cephalic Index and Midsagittal Vector Analysis in Assessing Morphology in Sagittal Synostosis: A CT-Based Morphometric Analysis

2018 ◽  
Vol 56 (7) ◽  
pp. 944-952
Author(s):  
Brian Y. Hong ◽  
Emily S. Ho ◽  
Elizabeth Zellner ◽  
John H. Phillips ◽  
Christopher R. Forrest
Keyword(s):  
Discriminant Validity ◽  
Rating Scale ◽  
Numeric Rating Scale ◽  
Vector Analysis ◽  
Control Group ◽  
Global Rating ◽  
Cephalic Index ◽  
Sagittal Synostosis ◽  
Ct Evaluation ◽  
The Difference

Introduction: Assessment of cranial dysmorphism in sagittal synostosis is often subjective but objective measures can be applied. These include cephalic index (CI) and midsagittal vector analysis (MSVA). Objective: To assess discriminant validity, construct validity, and responsiveness of CI and MSVA measured from computed tomography (CT) in patients with sagittal synostosis. Methods: Patients with nonsyndromic isolated sagittal synostosis with complete preoperative (n = 30) and postoperative (n = 13) CT data were included. Age-matched control group (n = 24) comprised of normocephalic patients who underwent CT for reasons related to trauma. Outcome Measures: Retrospective CT evaluation of CI and MSVA was conducted and correlated with a dysmorphism numeric rating scale (D-NRS) that measured surgeon-rated severity of sagittal synostosis. Responsiveness of CI and MSVA was evaluated using dysmorphism global rating of change (D-GRC). Results: Thirty patients with sagittal synostosis were demographically similar to 24 normocephalic patients. The difference in CI and MSVA was statistically significant between normocephalic and scaphocephalic patients. Cephalic index had a good correlation with D-NRS ( r = −0.665, ρ = −0.667), but not with MSVA ( r = 0.250, ρ = 0.203). Change in CI ( r = 0.738, ρ = 0.657) was well correlated with D-GRC, but not with MSVA ( r = −0.409, ρ = −0.301). Conclusion: Cephalic index appears to quantify the severity of sagittal synostosis better than MSVA. Cephalic index also has better responsiveness than MSVA to measure a reduction in severity of disease; however, MSVA is a better descriptive craniometric measurement. Midsagittal vector analysis was able to quantify the shift in morphology in sagittal synostosis following surgical treatment.

scholarly journals Improved cephalic index following early cranial vault remodeling in patients with isolated nonsyndromic sagittal synostosis

2021 ◽  
Vol 50 (4) ◽  
pp. E7
Author(s):  
Arvid Frostell ◽  
Maryam Haghighi ◽  
Jiri Bartek ◽  
Ulrika Sandvik ◽  
Bengt Gustavsson ◽  
...  
Keyword(s):  
Mixed Model ◽  
Linear Mixed Model ◽  
Cranial Vault ◽  
Hospital Length Of Stay ◽  
Cephalic Index ◽  
Sagittal Synostosis ◽  
The Mean ◽  
Cranial Vault Remodeling

OBJECTIVE Isolated nonsyndromic sagittal synostosis (SS) is the most common form of craniosynostosis in children, accounting for approximately 60% of all craniosynostoses. The typical cranial measurement used to define and follow SS is the cephalic index (CI). Several surgical techniques have been suggested, but agreement on type and timing of surgery is lacking. This study aimed to evaluate the authors’ institutional experience of surgically treating SS using a modified subtotal cranial vault remodeling technique in a population-based cohort. Special attention was directed toward the effect of patient age at time of surgery on long-term CI outcome. METHODS A retrospective analysis was conducted on all patients with isolated nonsyndromic SS who were surgically treated from 2003 to 2011. Data from electronic medical records were gathered. Eighty-two patients with SS were identified, 77 fulfilled inclusion criteria, and 72 had sufficient follow-up data and were included. CI during follow-up after surgery was investigated with ANOVA and a linear mixed model. RESULTS In total, 72 patients were analyzed, consisting of 16 females (22%) and 56 males (78%). The mean ± SD age at surgery was 4.1 ± 3.1 months. Blood transfusions were received by 81% of patients (26% intraoperatively, 64% postoperatively, 9% both). The mean ± SD time in the pediatric ICU was 1.1 ± 0.25 days, and the mean ± SD total hospital length of stay was 4.6 ± 2.0 days. No patient required reoperation. The mean ± SD CI increased from 69 ± 3 to 87 ± 5 for patients who underwent surgery before 45 days of age. Surgery resulted in a larger increase in CI for patients who underwent surgery at a younger age compared with older patients (p < 0.05, Tukey’s HSD test). In the comparison of patients who underwent surgery before 45 days of age with patients who underwent surgery at 45–90, 90–180, and more than 180 days of age, the linear mixed model estimated a long-term loss of CI of 3.0, 5.5, and 7.4 points, respectively. CONCLUSIONS The modified subtotal cranial vault remodeling technique used in this study significantly improved CI in patients with SS. The best results were achieved when surgery was performed early in life.

Combined metopic and sagittal craniosynostosis: is it worse than sagittal synostosis alone?

2011 ◽  
Vol 31 (2) ◽  
pp. E2 ◽  
Author(s):  
Jordan S. Terner ◽  
Roberto Travieso ◽  
Su-shin Lee ◽  
Antonio J. Forte ◽  
Anup Patel ◽  
...  
Keyword(s):  
Brain Tissue ◽  
Brain Volume ◽  
Ventricular Volume ◽  
Brain Dysfunction ◽  
Volume Reduction ◽  
Intracranial Volume ◽  
Age Group ◽  
Sagittal Synostosis ◽  
Sagittal Craniosynostosis ◽  
Patient Groups

Object Combined metopic and sagittal craniosynostosis is a common variant of the nonsyndromic, multiplesuture synostoses. It is unknown whether this combined form causes reduced intracranial volume (ICV) and potentially more brain dysfunction than sagittal synostosis alone. This study is a volumetric comparison of these 2 forms of craniosynostosis. Methods The authors conducted a retrospective chart and CT review of 36 cases of isolated sagittal synostosis or combined metopic and sagittal synostosis, involving patients seen between 1998 and 2006. Values were obtained for the intracranial compartment, brain tissue, CSF space, and ventricular volumes. Patients with craniosynostosis were then compared on these measures to 39 age- and sex-matched controls. Results In patients with isolated sagittal synostosis and in those with combined metopic and sagittal synostosis, there was a trend toward smaller ICV than in controls (p < 0.1). In female patients older than 4.5 months of age, there was also a trend toward smaller ICV in patients with the combined form than in those with sagittal synostosis alone (p < 0.1), and the ICV of patients with the combined form was significantly smaller than the volume in controls in the same age group (p < 0.05). Brain tissue volume was significantly smaller in both patient groups than in controls (p < 0.05). Ventricular volume was significantly increased (compared with controls) only in the patients with isolated sagittal synostosis who were younger than 4.5 months of age (p < 0.05). Overall CSF space, however, was significantly larger in both patient groups in patients younger than 4.5 months of age (p < 0.05). Conclusions These findings raise concerns about intracranial and brain volume reduction in patients with sagittal and combined metopic and sagittal synostoses and the possibility that this volume reduction may be associated with brain dysfunction. Because the ICV reduction is greater in combined metopic and sagittal synostosis in patients older than 4.5 months of age than in sagittal synostosis in this age group, the potential for brain dysfunction may be particularly true for these younger infants.

Pregnancy Experiences in the Woman With Mild Hyperphenylalaninemia

PEDIATRICS ◽  
2003 ◽  
Vol 112 (Supplement_4) ◽  
pp. 1548-1552
Author(s):  
Harvey L. Levy ◽  
Susan E. Waisbren ◽  
Flemming Güttler ◽  
William B. Hanley ◽  
Reuben Matalon ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Head Circumference ◽  
Congenital Heart ◽  
Collaborative Study ◽  
Dietary Therapy ◽  
Maternal Phenylketonuria ◽  
Phenylalanine Level ◽  
Live Births ◽  
Normal Limits ◽  
Z Scores

Objective. A major issue in maternal phenylketonuria (MPKU) has been whether maternal non-PKU mild hyperphenylalaninemia (MHP) is teratogenic. Such untreated pregnancies and their outcomes are presented on this report. Methods. Enrolled pregnancies in which the untreated prepregnancy assigned phenylalanine level (APL) was no more than 600 μmol/L were included in the Maternal PKU Collaborative Study and were followed according to protocol. Results. Forty-eight enrolled women with non-PKU MHP had mean APL 408 ± 114 μmol/L. They had a total of 58 pregnancies that resulted in live births. Fifty were untreated. Maternal phenylalanine (Phe) levels in the untreated pregnancies decreased during pregnancy for average Phe exposure of 270 ± 84 μmol/L, virtually identical to the level of 269 ± 136 μmol/L in the 8 treated pregnancies. Birth measurements in the 50 offspring from untreated pregnancies were within normal limits with z scores of −0.25 for weight, 0.28 for length, and −0.63 for head circumference, although birth head circumference was negatively correlated with maternal APL (r = −0.30). Only 1 offspring had congenital heart disease. Offspring IQ was 102 ± 15 compared with 96 ± 14 in the mothers with untreated pregnancies and with 109 ± 21 in control offspring. Conclusion. Maternal non-PKU MHP no more than 600 μmol/L does not require dietary therapy. The naturally lower Phe level during pregnancy seems to protect against teratogenesis.

scholarly journals Ellis—van Creveld Syndrome with Sagittal Craniosynostosis

2015 ◽  
Vol 8 (2) ◽  
pp. 132-135 ◽  
Author(s):  
Andrew S. Fischer ◽  
William M. Weathers ◽  
Erik M. Wolfswinkel ◽  
Robert J. Bollo ◽  
Larry H. Hollier ◽  
...  
Keyword(s):  
Clinical Features ◽  
Ectodermal Dysplasia ◽  
Sagittal Suture ◽  
Head Shape ◽  
Rare Disorders ◽  
Sagittal Synostosis ◽  
Cardiac Anomalies ◽  
Sagittal Craniosynostosis ◽  
Ellis Van Creveld Syndrome ◽  
Cranial Vault Remodeling

Ellis—van Creveld syndrome (EVC) is a rare disorder (the incidence is estimated at around 7/1,000,000) characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia, and cardiac anomalies. Sagittal synostosis is characterized by a dolichocephalic head shape resulting from premature fusion of the sagittal suture. Both are rare disorders, which have never been reported together. We present a case of EVC and sagittal synostosis. We report the clinical features of a Hispanic boy with EVC and sagittal craniosynostosis who underwent cranial vault remodeling. The presentation of this patient is gone over in detail. A never before reported case of EVC and sagittal synostosis is presented in detail.

scholarly journals Spring-mediated sagittal craniosynostosis treatment at the Children’s Hospital of Philadelphia: technical notes and literature review

2015 ◽  
Vol 38 (5) ◽  
pp. E7 ◽  
Author(s):  
Leopold Arko ◽  
Jordan W. Swanson ◽  
Tamara M. Fierst ◽  
Rosemary E. Henn ◽  
Daniel Chang ◽  
...  
Keyword(s):  
Literature Review ◽  
Blood Loss ◽  
Sagittal Synostosis ◽  
Data Set ◽  
Ultrasonic Scalpel ◽  
Children's Hospital ◽  
Sagittal Craniosynostosis ◽  
Institutional Data ◽  
Cranial Index ◽  
Children’S Hospital

OBJECT Sagittal craniosynostosis has been treated using both cranial remodeling techniques and modification of the sagittal strip craniectomy. A more recent technique is to implant springs in conjunction with a suturectomy to transversely expand the parietal bones to accommodate the growing brain. In this paper the authors describe and evaluate several modifications to the spring-mediated cranioplasty (SMC) technique, most notably use of an ultrasonic scalpel to limit dural dissection and maximize opening of the stenosed suture by placement of multiple spring devices. In addition, the literature is reviewed comparing SMC to other surgical treatments of sagittal synostosis. METHODS The authors retrospectively reviewed patients who presented to the Children’s Hospital of Philadelphia with a diagnosis of sagittal synostosis from August 2011 to November 2014. A pooled data set was created to compare our institutional data to previously published work. A comprehensive literature review was performed of all previous studies describing the SMC technique, as well as other techniques for sagittal synostosis correction. RESULTS Twenty-two patients underwent SMC at our institution during the study period. Patients were 4.2 months of age on average, had a mean blood loss of 56.3 ml, and average intensive care unit and total hospital stays of 29.5 hours and 2.2 days, respectively. The cranial index was corrected to an average of 73.7 (SD 5.2) for patients who received long-term radiological follow-up. When comparing the authors’ institutional data to pooled SMC data, blood loss and length of stay were both significantly less (p = 0.005 and p < 0.001, respectively), but the preoperative cranial index was significantly larger (p = 0.01). A review of the SMC technique compared with other techniques to actively expand the skull of patients with sagittal synostosis demonstrated that SMC can be performed at a significantly earlier age compared with cranial vault reconstruction (CVR). CONCLUSIONS The authors found that their institutional modifications of the SMC technique were safe and effective in correcting the cranial index. In addition, this technique can be performed at a younger age than CVRs. SMC, therefore, has the potential to maximize the cognitive benefits of early intervention, with lower morbidity than the traditional CVR.

Spring-mediated cranioplasty versus endoscopic strip craniectomy for sagittal craniosynostosis

2021 ◽  
pp. 1-9
Author(s):  
Shih-Shan Lang ◽  
Petra M. Meier ◽  
William Z. Paden ◽  
Phillip B. Storm ◽  
Gregory G. Heuer ◽  
...  
Keyword(s):  
Length Of Stay ◽  
Hospital Length ◽  
Hospital Length Of Stay ◽  
Perioperative Outcomes ◽  
Sagittal Craniosynostosis ◽  
Icu Length Of Stay ◽  
Data Points ◽  
Incident Rate ◽  
Invasive Techniques ◽  
Strip Craniectomy

OBJECTIVE Endoscopic strip craniectomy (ESC) and spring-mediated cranioplasty (SMC) are two minimally invasive techniques for treating sagittal craniosynostosis in early infancy. Data comparing the perioperative outcomes of these two techniques are sparse. Here, the authors hypothesized that outcomes would be similar between patients undergoing SMC and those undergoing ESC and conducted a study using the multicenter Pediatric Craniofacial Surgery Perioperative Registry (PCSPR). METHODS The PCSPR was queried for infants under the age of 6 months who had undergone SMC or ESC for sagittal synostosis. SMC patients were propensity score matched 1:2 with ESC patients on age and weight. Primary outcomes were transfusion-free hospital course, intensive care unit (ICU) admission, ICU length of stay (LOS), and hospital length of stay (HLOS). The authors also obtained data points regarding spring removal. Comparisons of outcomes between matched groups were performed with multivariable regression models. RESULTS The query returned data from 676 infants who had undergone procedures from June 2012 through September 2019, comprising 580 ESC infants from 32 centers and 96 SMC infants from 5 centers. Ninety-six SMC patients were matched to 192 ESC patients. There was no difference in transfusion-free hospital course between the two groups (adjusted odds ratio [aOR] 0.78, 95% CI 0.45–1.35). SMC patients were more likely to be admitted to the ICU (aOR 7.50, 95% CI 3.75–14.99) and had longer ICU LOSs (incident rate ratio [IRR] 1.42, 95% CI 1.37–1.48) and HLOSs (IRR 1.28, 95% CI 1.17–1.39). CONCLUSIONS In this multicenter study of ESC and SMC, the authors found similar transfusion-free hospital courses; however, SMC infants had longer ICU LOSs and HLOSs. A trial comparing longer-term outcomes in SMC versus ESC would further define the roles of these two approaches in the management of sagittal craniosynostosis.

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