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Combined Metopic and Sagittal Craniosynostosis: Is It Worse Than Sagittal Synostosis Alone?

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Combined Metopic and Sagittal Craniosynostosis: Is It Worse Than Sagittal Synostosis Alone? Neurosurg Focus 31 (2):E2, 2011 Combined metopic and sagittal craniosynostosis: is it worse than sagittal synostosis alone? JORDAN S. TERNER, B.A.,1 ROBERTO TRAVIESO, B.A.,1 SU-SHIN LEE, M.D.,2 ANTONIO J. FORTE, M.D.,1 ANUP PATEL, M.D., M.B.A.,1 AND JOHN A. PERSING, M.D.1 1Section of Plastic Surgery, Yale School of Medicine, Yale University, New Haven, Connecticut; and 2Department of Surgery, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan, Republic of China Object. Combined metopic and sagittal craniosynostosis is a common variant of the nonsyndromic, multiple- suture synostoses. It is unknown whether this combined form causes reduced intracranial volume (ICV) and poten- tially 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 sag- ittal 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 sagit- tal 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. (DOI: 10.3171/2011.6.FOCUS11100) KEY WORDS • craniosynostosis • metopic • sagittal • volume AGITTAL craniosynostosis is the most common of the mity may appear less severe due to the metopic synos- single-suture synostoses, with an incidence between tosis–induced inability to develop compensatory frontal 1 in 2000 and 1 in 5000 live births. Reported rates bossing. In truth however, this combined form may be Sof metopic synostosis vary between 1 in 10,000 and 1 in less benign than sagittal synostosis alone due to greater 100,000 live births, and the incidence of this synostosis is restriction on the growing brain (Fig. 1). on the rise. While premature fusion of the sagittal suture In the normal pediatric skull, ICV, BTV, and CSF results in scaphocephaly, characterized by biparietal nar- volume continually increase through early childhood. rowing, frontal bossing, and occipital prominences, iso- Yet, this increase is most rapid during the first 2.5 years lated metopic synostosis results in the well-recognized of life, allowing the brain to reach more than 80% of its frontal bone deformity, trigonocephaly. The combination adult size in this period. Untreated craniosynostosis may of these 2 forms of craniosynostosis yields a skull shape lead to an inhibition of brain growth and in some cases an that is long and narrow (scaphocephaly), but the defor- increase in intracranial pressure. It has been previously demonstrated that infants with sagittal craniosynostosis Abbreviations used in this paper: BTV = brain tissue volume; have higher intracranial pressure and an increased inci- HSD = Honestly Significant Difference; ICV = intracranial volume; dence of learning disabilities.10,13 VV = ventricle volume. Of note, the surgical technique for correcting these Neurosurg Focus / Volume 31 / August 2011 1 Unauthenticated | Downloaded 09/24/21 08:24 PM UTC J. S. Terner et al. FIG. 1. A–C: Frontal (A), lateral (B), and birds-eye (C) views of a patient with isolated sagittal craniosynostosis showing promi- nent frontal bossing and scaphocephaly with superimposed deformational plagiocephaly. D and E: Frontal (D) and lateral (E) views of a patient with combined sagittal and metopic craniosynostosis showing a deceivingly less severe–appearing deformity. conditions can vary significantly. In scaphocephaly, a Methods multitude of techniques have been employed. Strip cra- Patient Population niectomies are often performed either as open surgical procedures or endoscopically.8 However, concerns over Between November 1998 and October 2006, 77 con- lack of complete and early correction of the restrictive secutive children with previously untreated sagittal syn- nature of the deformity have led to more extensive pro- ostosis underwent whole-vault cranioplasty at Yale–New cedures being used. These include the modified pi-plasty Haven Hospital. These children presented with scapho- and more comprehensive cranioplasty.3,6 In trigonocepha- cephaly and a palpable ridge along the sagittal plane. Pre- ly, strip craniectomy or bifrontal craniotomy with orbital operative CT scans were obtained to confirm the diagno- rim advancement have been used to correct for the lack of sis of sagittal craniosynostosis and determine the patency lateral frontal orbital projection. of other calvarial sutures. Fifty-three patients (15 female, Previous studies by Posnick et al.,12 Hill et al.,7 and 38 male) had pre- and postoperative CT scans available Lee et al.9 demonstrated normal or increased preopera- for review. tive mean ICV (relative to controls) in patients with sagit- Based on both radiographic data (omega sign present tal synostosis who were older than 6 months of age. The in the metopic area) and direct observation at the time of degree of difference appears to be age dependent.7,9,12 The surgery, a subset of these patients was identified as hav- older the patient, the greater the increase in ICV com- ing concurrent metopic and sagittal synostosis. Because pared with normal values. the metopic suture normally closes at approximately 9 Currently, no data exist regarding ICV in patients months of age, only patients who were 9 months of age or with combined metopic and sagittal synostosis. It is also younger were included in this study. unknown whether timing of surgery, specifically early After applying these criteria, 16 patients (7 female, 9 surgical intervention, or alternatively, a different surgical male) with combined metopic and sagittal synostosis (the technique may prove beneficial in these patients. Intracra- M/S group), and 19 (5 female, 14 male) with sagittal syn- nial volume measurements may aid in this determination ostosis alone (the S group), were included in the analysis. and can be calculated noninvasively using CT scans. Such Additionally, data from age- and sex-matched controls techniques have already been employed to measure ICV (39 children) from the same institution, were compared in patients with isolated sagittal synostosis. Using these with these groups; the control data had been obtained us- CT-based techniques, we sought to clarify the volumetric ing the same data acquisition methodology as was used in changes associated with the combined form of metopic the synostosis cases. Available data included preoperative and sagittal craniosynostosis versus isolated sagittal syn- ICV, BTV, CSF space, and VV. Data were analyzed using ostosis and compare these to each other as well as to val- a combination of R (www.R-project.org) and Microsoft ues obtained in specific age- and sex-matched controls, Excel software. The research protocol was approved by who were evaluated using the same methods at the same the Human Investigation Committee at Yale University institution. School of Medicine. 2 Neurosurg Focus / Volume 31 / August 2011 Unauthenticated | Downloaded 09/24/21 08:24 PM UTC Combined metopic and sagittal craniosynostosis Computed Tomography and Volume Measurement (controls: 16 girls and 23 boys) under the age of 9 months. All patients underwent axial CT scanning on a Light- Data acquired included preoperative ICV, BTV, CSF space, speed-16 CT scanner (General Electric) with a 3-mm and VV. Data were analyzed using a combination of R, pitch and slice thickness at Yale–New Haven Hospital. JMP (SAS Institute, Inc.), and Excel software. Some patients underwent CT scanning at a higher resolu- Preoperative ICV tion preoperatively with a 1.25-mm pitch and slice thick- ness. Volume determination was based on measuring the A 1-way ANOVA was conducted to compare the pre- area in each CT section. operative ICV of the patients in the S group with that of The ImageJ software package, downloaded from the the patients in the M/S group. The results of this initial Internet (National Institutes of Health, http://rsb.info.nih. test were significant (p = 0.029), and the differences be- gov/ij/download.html) was used to measure the CT im- tween the 3 groups (S, M/S, and control) were then ex- age. The ImageJ software read the CT data and calibrated plored using the Tukey HSD test. This subsequent step the images automatically. The threshold was set at the in- showed a trend for both the S and M/S groups to have, tensity that corresponded to the interface between brain on average, smaller preoperative ICV than controls (p = tissue and the signal void caused by the dense bone of 0.086, p = 0.065, respectively).
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