Babette S. Saltzman

Making the Diagnosis: Metopic Ridge Versus Metopic Craniosynostosis

Introduction The metopic suture is the only calvarial suture which normally closes during infancy. Upon closure, a palpable and visible ridge often forms which can be confused with metopic craniosynostosis. Metopic ridging (MR) is treated nonsurgically while metopic craniosynostosis (MCS) is treated surgically. Differentiating between the two is paramount; however, consensus is lacking about where a clear diagnostic threshold lies. The goal of this study is to describe the physical examination and CT scan characteristics which may help to differentiate between physiological closure of the metopic suture with ridging (MR) and MCS. Methods A retrospective chart review of all patients seen at Seattle Children’s Hospital between 2004 and 2009 with the diagnosis of either MCS or MR (n = 282) was performed. Physical examination characteristics described by diagnosing practitioners were analyzed. Clinical photos were assessed by 3 expert raters to determine the importance of these characteristics. CT scan findings were abstracted and compared between the two diagnoses. Results The “classic” triad of narrow forehead, biparietal widening, and hypotelorism was present in only 14% of patients with MCS. Ninety-eight percent of patients in both groups had a palpable metopic ridge. The photographic finding of narrow forehead and pterional constriction was present in all patients with MCS, but only in 11.2% and 2.8% of patients with MR. On CT scan, the presence of 3 or more MCS findings was diagnostic of MCS in 96% of patients. Patients with MCS were more likely to present before 6 months of age (66% vs. 32%). Conclusions Patients with MCS tend to present earlier than those with MR. Upon physical examination, the relationship between the lateral frontal bone and the lateral orbit is important in distinguishing between the two diagnoses. A CT scan can be helpful in making the diagnosis not to confirm a closed suture but to identify 3 or more MCS characteristics.

Reliability of nasolabial anthropometric measures using three-dimensional stereophotogrammetry in infants with unrepaired unilateral cleft lip.

Background: Surgical and orthodontic treatment of unilateral cleft lip +/– palate can produce dramatic changes in nasolabial form; however, the lack of ideal methods with which to objectively document three-dimensional form limits the ability to assess treatment outcomes. The purpose of this study was to determine the reliability of three-dimensional stereophotogrammetry for anthropometric assessment of the unilateral cleft lip +/– palate deformity in infants before cleft lip repair. Methods: Preoperative three-dimensional images were acquired from 26 consecutive patients with unrepaired unilateral cleft lip +/– palate. Three raters performed indirect anthropometry on each image on two separate occasions, with at least 1 week between rating sessions. One rater performed direct measurements on participants before surgery while in the operating room. Twenty-six linear and angular measurements were considered, and intrarater, interrater, and intermethod reliability were assessed. Results: Regarding intrarater and interrater reliability, most measurements had Pearson coefficients greater than 0.75, mean differences less than 0.8 mm, and mean proportional differences less than 0.1. For measurements involving vermilion height, nostril remnants, or Cupid’s bow width, Pearson coefficients ranged from 0.3 to 0.75, mean differences ranged from 0.4 to 0.9 mm, and mean proportional differences ranged from 0.1 to 0.3. Regarding intermethod reliability, correlation coefficients ranged from 0.4 to 0.75 for most measurements. The mean differences for nose and lip measurements were less than 1 mm and between 0.8 and 1.3 mm, respectively. Conclusion: Three-dimensional stereophotogrammetry provides a reliable method for many anthropometric measurements of nasolabial form in infants with unrepaired unilateral cleft lip +/– palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.

Measuring Symmetry in Children With Unrepaired Cleft Lip: Defining a Standard for the Three-Dimensional Midfacial Reference Plane

Objective Quantitative measures of facial form to evaluate treatment outcomes for cleft lip (CL) are currently limited. Computer-based analysis of three-dimensional (3D) images provides an opportunity for efficient and objective analysis. The purpose of this study was to define a computer-based standard of identifying the 3D midfacial reference plane of the face in children with unrepaired cleft lip for measurement of facial symmetry. Participants The 3D images of 50 subjects (35 with unilateral CL, 10 with bilateral CL, five controls) were included in this study. Interventions Five methods of defining a midfacial plane were applied to each image, including two human-based (Direct Placement, Manual Landmark) and three computer-based (Mirror, Deformation, Learning) methods. Main Outcome Measure Six blinded raters (three cleft surgeons, two craniofacial pediatricians, and one craniofacial researcher) independently ranked and rated the accuracy of the defined planes. Results Among computer-based methods, the Deformation method performed significantly better than the others. Although human-based methods performed best, there was no significant difference compared with the Deformation method. The average correlation coefficient among raters was .4; however, it was .7 and .9 when the angular difference between planes was greater than 6° and 8°, respectively. Conclusions Raters can agree on the 3D midfacial reference plane in children with unrepaired CL using digital surface mesh. The Deformation method performed best among computer-based methods evaluated and can be considered a useful tool to carry out automated measurements of facial symmetry in children with unrepaired cleft lip.

Clinical characteristics and surgical decision making for infants with metopic craniosynostosis in conjunction with other congenital anomalies.

Background: Metopic craniosynostosis can occur in isolation or in conjunction with other congenital anomalies. The surgical decision making and outcomes between these 2 groups are analyzed. Methods: A retrospective review of all children evaluated in the craniofacial clinic at Seattle Children’s Hospital for metopic craniosynostosis between 2004 and 2009 was performed. Physical examination and CT scan characteristics were analyzed as were the treatment decisions and surgical outcomes. Results: From 2004 to 2009, 282 patients were evaluated and 100 were determined to have metopic craniosynostosis. Of these, 19 patients were found to have additional congenital anomalies. Review of these patients’ CT scans revealed 13 with classic trigonencephaly, 3 with microcephaly, and 3 with narrow frontal bones, abnormal orbits, and small anterior fossa. Patients (90%) with isolated metopic craniosynostosis underwent cranial vault expansion, whereas only 63% of the complex group did so. The complex metopic group had a longer hospital stay (5 d vs 3.4 d), more intraoperative complications, and required more repeat surgery. Conclusion: Patients with metopic craniosynostosis and additional anomalies require special consideration when deciding upon surgical intervention and should be cared for by a multidisciplinary team to address their additional needs.