Unilateral Coronal Synostosis Research Articles

Intracranial volume in patients with nonsyndromal craniosynostosis

In recent years, comparisons between intracranial volumes (ICVs) in patients with craniosynostosis and healthy patients have given variable results, leading to questions regarding the validity of the normal reference material and the comparability of the measurement techniques. In this study, ICVs in patients with nonsyndromal craniosynostosis without previous surgical intervention were compared with the ICVs of a normal population of European descent determined using the same method for each group. Determination of ICV was based on measuring the area of intersection in each computerized tomography slice. For comparisons the ICV measurements for each patient were standardized with regard to age and sex by expressing them in terms of the standard deviation score. Only the group of boys with metopic synostosis had a tendency toward smaller ICV than did healthy boys (p = 0.04). Partitioning the male metopic data into age groups younger and older than 7 months of age revealed that the younger children had normal ICVs, whereas the older children had, on average, smaller ICVs (p = 0.02). Both the female sagittal synostosis and the male unilateral coronal synostosis groups had larger than normal ICVs, both with a probability value less than 0.001. No evidence was found that the ICVs of patients with nonsyndromal craniosynostosis are smaller than those of normal children, except for boys older than 7 months of age with metopic synostosis. This finding may have implications for the timing of surgical intervention for these patients. The indications are that interventions should be focused less on ICV and more on normalizing craniofacial shape and promoting normal development.

Brain morphology in nonsyndromic unicoronal craniosynostosis

Studies of isolated craniosynostosis have shown biomechanical and biochemical influences on the craniofacial phenotype, resulting from both genetic and epigenetic factors. Much less attention has been directed toward the morphology of the brain, despite the interactive nature of the developing skull and developing brain. The aim of this study is to define the morphology of the brain in nonsyndromic unilateral coronal synostosis (UCS) in order to form more complete hypotheses about the cause of craniosynostosis. Landmark coordinate data were collected from 3D magnetic resonance image reconstructions of the brain in a sample of UCS patients and an age-matched morphologically normal cohort. These data were analyzed using Euclidean distance matrix analysis. The results of our study demonstrate that despite the basic similarity of overall shape of the brain and skull in UCS, the effects of craniosynostosis on the brain are not localized to structures immediately adjacent to the fused suture or to the endocranial surface of the skull. Rather, alterations are observed throughout the volume of the brain, with subcortical structures altered in conjunction with cortical changes. These results indicate that the morphological correlates are different for brain and skull and suggest that there is a large degree of independence in the developmental trajectories of the brain and skull.

Involvement of the Basilar Coronal Ring in Unilateral Coronal Synostosis

Unilateral coronal synostosis denotes one-sided premature fusion of the frontoparietal suture. However, this is only one component of the coronal hemi-ring that includes the contiguous frontoparietal, frontosphenoidal, and frontoethmoidal sutures. Possible fusion of the basilar sutures in unilateral coronal synostosis is poorly documented. The authors retrospectively reviewed the radiographic records of 91 patients with unilateral coronal synostosis who were evaluated in their unit between 1986 and 2003. Fifty-one computed tomography scans were of sufficient quality to adequately assess the basilar sutures. Each suture in the coronal hemi-ring (frontoparietal, frontosphenoid, and sphenoethmoid) was assessed on axial images for evidence of synostosis, extent and location of fusion, and cranial base angulation. The data were analyzed using SAS statistical software (SAS, Cary, N.C.). One frontoparietal ("coronal") suture was fused in all 50 patients. Synostosis was incomplete in 19 patients; specifically, the superior and inferior portions of the frontoparietal suture were patent. Fourteen of the 19 scans showing partial frontoparietal fusion were in infants less than 5 months of age. Scans were grouped according to extent of fusion in the basilar coronal hemi-ring: group I (n = 15), the frontosphenoidal suture was open on the affected side, whereas in group II (n = 36), there was variable synostosis in the lateral portion of the ipsilateral frontosphenoidal suture. Patients in group I were significantly younger than those in group II at the time of computed tomographic study (p < 0.001). Furthermore, group I patients were more likely to have incomplete frontoparietal fusion, as compared with group II (p < 0.001). The frontosphenoidal suture was patent in 100 percent of patients younger than 3 months, in 43 percent of those between 3 and 5 months old, and in 0 percent of patients older than 5 months at the time the scan was obtained. There was also a trend toward a larger cranial base angulation in group II, although this difference was not significant (p = 0.33). Seven patients were syndromic; however, their mean age (207 days) at the time of computed tomography was not statistically different from that of patients without a detectable mutation (188 days), nor did the cranial base angulation differ in this subgroup. The authors conclude that unilateral coronal fusion begins in the middle of the frontoparietal suture and extends superiorly and inferiorly. There also is age-dependent, progressive involvement of the lateral frontosphenoidal suture between 3 and 5 months of age. Nevertheless, synostotic extension into the basilar coronal hemi-ring is not associated with increased cranial base angulation.

The Bowstring Canthal Advancement: A New Technique to Correct the Flattened Supraorbital Rim in Unilateral Coronal Synostosis

Patients with unilateral coronal synostosis characteristically have a recessed and flattened supraorbital rim ipsilateral to the fused suture. Despite lateral canthal advancement procedures to correct these anomalies, patients often have a persistent flattened and recessed supraorbital rim after surgery. Current procedures address the pathologic features of the orbital rim only partially by advancing forward a deformed supraorbital rim without correcting the abnormal flattening of the normal rim curvature. The authors describe a technique modification of the supraorbital rim advancement procedure that addresses not only the lack of anterior positioning of it, but also its flattened contour: the bowstring canthal advancement.

Correction of Unilateral Coronal Synostosis Leads to Resolution of Mandibular Asymmetry in Rabbits

Correction of Unilateral Coronal Synostosis Leads to Resolution of Mandibular Asymmetry in Rabbits

Coronal Ring Involvement in Patients Treated for Unilateral Coronal Craniosynostosis

The etiopathology of the clinical entity normally referred to as unilateral coronal synostosis is commonly used to connote unilateral fusion of the frontoparietal suture. However, other sutures in the coronal ring may exhibit synostosis concomitant with or independent from frontoparietal synostosis and give rise to similar clinical phenotypes. This study retrospectively analyzes high-resolution computed tomographic data sets to determine patency of sutures within the coronal ring. Computed tomographic scan digital data from 33 infants who subsequently underwent surgical correction of unilateral coronal synostosis were assessed for sutural patency using Analyze imaging software. The frontosphenoidal suture was subdivided into intraorbital frontosphenoidal and extraorbital frontosphenoidal portions, and the patency of the frontoethmoidal suture was also assessed. Patients were sorted into two groups on the basis of the status of their frontosphenoidal sutures: group 1 had patent frontosphenoidal but synostotic frontoparietal sutures (n = 21) and group 2 had both frontosphenoidal and frontoparietal synostoses. Observer reproducibility was tested. The vertical and horizontal dimensions of the bony orbit and the endocranial base deflection angle were measured with the observer blinded with regard to sutural status group. Frontoethmoidal synostosis was not noted in any patients in either group. Two patients had no frontoparietal suture synostosis with isolated intraorbital frontosphenoidal and extraorbital frontosphenoidal suture closures. Suture diagnosis reproducibility was 99 percent. In group 1, the ipsilateral-to-contralateral vertical orbit dimension ratio averaged 1.11, whereas in group 2 it averaged 1.04 (p < 0.05). The ratio of horizontal orbit measurements was not significantly different between groups. In both groups, the endocranial base was deflected ipsilateral to the synostotic frontoparietal suture, with an average angle of 12 degrees in group 1 and 17 degrees in group 2 (p < 0.005). The extent of synostosis along the coronal sutural ring contributes to the dysmorphology of the orbit and the endocranial base deflection in patients whose clinical phenotypic diagnosis is unilateral coronal synostosis.

Hybrid of Distraction Osteogenesis Unilateral Frontal Distraction and Supraorbital Reshaping in Correction of Unilateral Coronal Synostosis

Unicoronal synostotic plagiocephaly is routinely treated by intracranial wide frontal and bilateral supraorbital reshaping. Recent advancement of distraction osteogenesis in craniofacial surgery has extended to patients with craniosynostosis. Although a controversy remains between conventional osteotomy and reshaping and application of the distraction technique in surgical treatment of craniosynostosis, there have been several positive clinical reports on distraction techniques for nonsyndromic and syndromic craniosynostosis. Unicoronal distraction applied successfully to a case of frontal plagiocephaly has been described. The authors report a procedure: hybrid of unilateral frontal distraction and supraorbital reshaping on the affected side for frontal synostotic plagiocephaly. This procedure was conducted on four typical unicoronal synostotic plagiocephaly cases (patient age range, 9-14 months; all patients female) with successful results. No particular complications were encountered in any of the four cases with a follow-up period that ranged from 18 to 53 months.

American society of maxillofacial surgeons outcome study: preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis.

The purpose of this study was to prospectively determine the neurodevelopmental effects associated with single-suture, nonsyndromic craniosynostosis before and after surgery. Children diagnosed with single-suture craniosynostosis were evaluated by a psychologist using the Bayley Scales of Infant Development-Second Edition (BSID-II) within 2 months before and again 1 year after surgical correction. The BSID-II is a widely used measure of infant cognitive and motor development. The scale consists of three parts, the Mental Developmental Index (MDI), the Psychomotor Developmental Index (PDI), and the Behavior Rating Scale. The MDI and PDI yield age-standard scores (mean, 100; SD, 16). The children ranged in age from 2.5 to 10 months at the time of the craniofacial reconstruction (average age, 5.9 months). Metopic synostosis was diagnosed in 23 percent, sagittal synostosis in 45 percent, and unilateral coronal synostosis in 32 percent of patients. Twenty-two patients were evaluated preoperatively, of whom 15 patients were evaluated postoperatively. Mean baseline BSID-II scores revealed a mild delay in mental and motor scores (MDI, 82.3; PDI, 79.5). Mean postoperative BSID-II scores still revealed a mild delay in mental scores but significantly improved motor scores (MDI, 79.3; PDI, 89.3). Of the 15 children, four (27 percent) had BSID-II evaluations that were in the average range for all scales and nine infants (60 percent) had at least one MDI or PDI score in the significantly delayed range (<70). Among children with single-suture nonsyndromic craniosynostosis, mean Bayley scores indicated mild baseline deficits in both mental and motor scores. After surgical treatment, improvement was seen in the motor scale. It appears from this sample that neurodevelopmental abnormalities may be present in children with single-suture synostosis, and some may persist at 1 year of follow-up.

820 Endoscope-assisted Fronto-orbital and Cranial Osteotomies

INTRODUCTION: An operative approach has been developed that incorporates fronto-orbital and cranial osteotomies to achieve immediate correction using specially designed bioresorbable plates and screws. Postoperative helmet/band therapy may be eliminated or its duration shortened using these techniques. METHODS: Forty-two patients (age, 2 to 8 mo) with sagittal (n = 25), metopic (n = 11), unicoronal (n = 5), or lambdoidal synostosis (n = 1) underwent endoscopic craniosynostosis repair with complex osteotomies performed through two or three small incisions in the scalp and/or upper eyelids. Suturectomy was performed in concert with wedge osteotomies, outfracture of parietal plates, reduction of cranial length, and stabilization with resorbable plates and screws for patients with sagittal synostosis, whereas unilateral or bilateral fronto-orbital advancement was performed for children with unilateral coronal (UCS) and metopic synostosis, respectively. Specially designed bioresorbable plates were used for fixation when appropriate. RESULTS: Follow-up has ranged from 1 month to 2 years. One patient developed bleeding in the recovery room that required transfusion. One patient with UCS required conversion to the open procedure. One patient with UCS will require reoperation. All patients were discharged by hospital Day 3. Two patients with sagittal synostosis had a small protrusion on the vertex of the cranium despite postoperative helmet therapy. Satisfactory to excellent aesthetic results were obtained in the remaining cases. In five of the children, helmets were not used. The others were placed in postoperative helmets for 6 to 12 weeks. CONCLUSION: Preliminary results are encouraging. More follow-up is necessary to determine selection criteria and critically evaluate outcomes. With more experience and refinement of instrumentation, minimally invasive repair will become an important tool in surgical management of craniosynostosis.

Unilateral coronal synostosis: a histomorphometric study.

This histomorphometric study compared the open and prematurely fused side of the coronal suture in subjects with unilateral coronal synostosis (UCS). Sutures and parasutural bone were obtained from seven subjects with nonsyndromic UCS during operative correction at 3 to 24 months of age. Histological and cellular analyses were performed for the affected and open sutures. Specimens were examined by light and polarizing microscopy. Sutural patterns, osseous morphology, calvarial thickness, tartrate-resistant acid phosphatase (TRAP)-positive cells, and marrow spaces were evaluated histomorphologically, qualitatively, and semiquantitatively. Histomorphometry was performed to determine total projected area of marrow space as a percentage of unit area, total number of TRAP-positive cells per specimen, and perisutural cranial thickness. Polarizing microscopy showed that affected sutures were composed of more lamellar bone than the normal sutures. By light microscopy, the clinically fused sutures were 1.7-fold thicker (p <.02), had twofold larger marrow spaces (p <.0006), and contained sixfold more TRAP-positive osteoclasts in marrow spaces near the suture (p <.04) than the normal sutures. Quantitative analysis of the normal sutures revealed that calvarial thickness was greater with age and that there was an inverse correlation between medullary area and age. For the affected sutures, there was also an age-related increase in calvarial thickness. There were also trends for age-related declines in numbers of osteoclasts in both open and affected sides. These results question the hypothesis that defective osteoclastic activity is pivotal in the pathogenesis of UCS and support the hypothesis that this condition results from abnormally active bony remodeling.

Molecular analysis of patients with synostotic frontal plagiocephaly (unilateral coronal synostosis).

Mutations in genes known to be responsible for most of the recognizable syndromes associated with bilateral coronal synostosis can be detected by molecular testing. The genetic alterations that could cause unilateral coronal synostosis are more elusive. It is recognized that FGFR and TWIST mutations can give rise to either bilateral or unilateral coronal synostosis, even in the same family. The authors undertook a prospective study of patients presenting with synostotic frontal plagiocephaly (unilateral coronal synostosis) to Children's Hospital Boston during the period from 1997 to 2000. Mutational analysis was performed on all patients and on selected parents whenever familial transmission was suspected. Intraoperative anthropometry was used in an effort to differentiate those patients in whom a mutation was detected from those in whom it was not. The anthropometric measures included bilateral sagittal orbital-globe distance, inter medial canthal distance, and nasal angulation. Macrocephaly and palpebral angulation were also considered possible determinants. There was a 2:1 female preponderance in 47 patients with synostotic frontal plagiocephaly. Mutations were found in eight of 47 patients: two patients with different single-amino-acid changes in FGFR2, three patients with FGFR3 Pro250Arg, and three patients with TWIST mutations. Another patient had craniofrontonasal syndrome for which a causative locus has been mapped to chromosome X, although molecular testing is not yet available. Two features were strongly associated with a detectable mutation in patients with synostotic frontal plagiocephaly: asymmetrical brachycephaly (retrusion of both supraorbital rims) and orbital hypertelorism. Other abnormalities in the craniofacial region and extremities were clues to a particular mutation in FGFR2, FGFR3, TWIST, or the X-linked mutation. Neither macrocephaly nor degree of nasal angulation nor relative vertical position of the lateral canthi correlated with mutational detection. An additional four patients in this study had either unilateral or bilateral coronal synostosis in an immediate relative and had anthropometric findings that predicted a mutation, and yet no genetic alteration was found. This suggests either that the authors' screening methods were not sufficiently sensitive or that perhaps there are other unknown pathogenic loci. Nevertheless, molecular testing is recommended for infants who have unilateral coronal synostosis, particularly if there are the anthropometric findings highlighted in this study or an otherwise suspicious feature in the child or a parent. Infants with either an identified or a suspected mutation usually need bilateral asymmetric advancement of the bandeau and may be more likely to require frontal revision in childhood.

Facial scoliosis due to sternocleidomastoid torticollis: a cephalometric analysis

Facial scoliosis can be caused by either the plagiocephaly which arises from unilateral coronal suture synostosis, or by the effects of sternocleidomastoid torticollis. Despite publications which have presented the key clinical and radiographic craniofacial differentiating features, confusion between the two still persists. This report presents the essential cephalometric features found in sternocleidomastoid torticollis, which may be applied to confirm the diagnosis in those cases which present late, and which do not exhibit the characteristic features of synostosis.

Intracranial pressure changes in craniosynostotic rabbits.

Cranial vault and brain deformities in individuals with craniosynostosis are thought to result, in part, from changes in intracranial pressure, but clinical findings are still inconclusive. The present study describes intracranial pressure changes in a rabbit model with naturally occurring, uncorrected coronal suture synostosis. Longitudinal and cross-sectional intracranial pressure data were collected from 241 New Zealand White rabbits, divided into four groups: normal controls (n = 81); rabbits with delayed-onset coronal suture synostosis (n = 78); rabbits with early-onset unilateral coronal suture synostosis (n = 32); and rabbits with early-onset bilateral coronal suture synostosis (n = 50). Epidural intracranial pressure measurements were obtained at 10, 25, 42, and 84 days of age using a NeuroMonitor microsensor transducer. Normal rabbits and rabbits with delayed-onset coronal suture and early-onset unilateral coronal suture synostosis showed a similar oscillating pattern of age-related changes in normal and head-down intracranial pressure from 10 to 84 days of age. In contrast, rabbits with early-onset bilateral coronal suture synostosis showed markedly elevated normal and head-down intracranial pressure levels from 10 to 25 days and showed a different pattern through 84 days. Results from one-way analysis of variance revealed significant (p < 0.01) group differences only at 25 days of age. Rabbits with early-onset bilateral coronal suture synostosis had significantly (p < 0.05) greater normal and head-down intracranial pressure (by 42 percent) than the other three groups. These results showed differing intracranial pressure compensations in rabbits with uncorrected multiple-suture synostosis compared with normal rabbits or rabbits with uncorrected single-suture synostosis, possibly through progressive cerebral atrophy and decreased intracranial volume, abnormal intracranial vascular patterns and blood volume, and/or differing cranial vault compensatory changes.

Differential diagnosis of the trapezoid-shaped head.

To recognize several conditions that result in a trapezoid head shape and review and contrast their various physical findings. A detailed review of all patients seen in the Craniofacial Clinic at the Children's Hospital and Regional Medical Center in Seattle, Washington, over a 10-year period from 1991 to 2001, with the diagnosis of craniosynostosis and plagiocephaly was performed. During this period, 690 patients had a surgical correction of craniosynostosis, and 1537 patients had posterior plagiocephaly. The shape of the head when viewed from the vertex position in an axial plane can be a significant diagnostic aid when evaluating a patient with plagiocephaly. Positional molding causes the vast majority of plagiocephaly. This deformational change results in a parallelogram-shaped head. A much more rare cause of plagiocephaly is lambdoid synostosis. With premature fusion of one of the lambdoid sutures, the head takes on a very characteristic trapezoid shape when viewed from the vertex. Unilateral coronal synostosis that occurs on the same side as either posterior positional molding or unilateral lambdoid synostosis will also result in the trapezoid head shape. Furthermore, on the rare occasion when anterior and posterior deformational plagiocephaly occurs on the same side, the trapezoid head shape may be the consequence. The choice of appropriate treatment modality requires systematic evaluation of the patient with a trapezoid-shaped head to determine the etiology of the deformation.

Anthropometric study of synostotic frontal plagiocephaly: before and after fronto-orbital advancement with correction of nasal angulation.

Surgical correction of synostotic frontal plagiocephaly (unilateral coronal synostosis) focuses on the asymmetry of the forehead and orbits. However, there is controversy regarding whether nasal angulation should be addressed during primary fronto-orbital advancement in infancy. This prospective study was undertaken to answer that question. Preoperative and postoperative anthropometric measurements were obtained for 19 infants with nonsyndromic synostotic frontal plagiocephaly. The measurements included nasal angulation, nasion-to-endocanthion distance, nasion-to-exocanthion distance, and exocanthion-to-tragion distance. All patients underwent bilateral parallelogrammatic fronto-orbital correction. Closing wedge nasal ostectomy was performed for group I (n = 14) and was not performed for group II (n = 5). The average age at the time of follow-up assessments was 3 years 8 months (range, 1 to 14 years) in group I and 5 years 5 months (range, 2 to 15 years) in group II. A statistically significant change was observed for patients who underwent primary correction of nasal angulation; the change correlated with improved naso-orbital symmetry, as judged with nasion-to-endocanthion and nasion-to-exocanthion measurements (p < 0.01 and p < 0.05, respectively). Group I patients exhibited an average preoperative nasal angulation of 9.15 +/- 0.8 degrees that decreased to 3.1 +/- 0.6 degrees postoperatively (p < 0.01). Group II patients exhibited an average preoperative nasal angulation of 6.4 +/- 0.7 degrees that was unchanged postoperatively at 7.2 +/- 1 degrees. The improvement in nasal angulation in group I was particularly striking because the patients in group II exhibited, on average, a lesser degree of preoperative nasal deviation (p < 0.01). This prospective comparison of fronto-orbital correction of synostotic frontal plagiocephaly with and without nasal correction confirmed an earlier study and demonstrated that angulation of the nasal pyramid does not self-correct within 5 years after traditional bilateral fronto-orbital repair. Closing wedge nasal ostectomy results in improved nasal angulation and naso-orbital symmetry, without evidence of distortion or inhibition of nasal growth.

Effects of a child with a craniofacial anomaly on stability of the parental relationship.

The purpose of this study was to determine rates of divorce in parents of children with various types of craniofacial anomalies and to analyze possible confounding factors. A 29-question survey was sent to parents of all children evaluated in the Craniofacial Centre between 1992 and 1997. Parents were questioned regarding pre- and postnatal marital stability, whether the child's facial anomaly contributed to divorce, and involvement in the child's welfare. Using deformational posterior plagiocephaly as a control group, rates of divorce vs. non-divorce were compared for craniofacial anomalies, categorized as asymmetric (hemifacial microsomia, unilateral coronal synostosis, cleft lip, cleft lip/palate) or symmetric (syndromic-craniosynostosis, orbital hypertelorism, Treacher Collins syndrome). Major anomalies (hemifacial microsomia, craniosynostosis, orbital hypertelorism, Treacher Collins syndrome) were also compared to minor anomalies (cleft lip, cleft lip/palate). Surveys were sent to both parents in 412 families; 403 surveys were returned; and the results were evaluated in 275 families (67%). Frequency analysis demonstrated an overall divorce rate of 6.8% and 4.9% separation. Anomalies associated with the highest rate of divorce were hemifacial microsomia (24.0%), syndromic craniosynostosis (12.2%), and cleft lip/palate (6.8%). 79% of non-divorced couples reported a strong prenatal relationship, whereas 59% of divorced couples reported a problematic relationship. Following birth of the affected child, 47% of non-divorced couples responded that the bonds became stronger and 41% of divorced couples thought the relationship worsened. Two-sided Fisher exact test comparing control vs. all other anomalies showed significance (p=.030) for rates of divorce. Separation of anomalies into asymmetric vs. symmetric and major vs. minor categories demonstrated no significant difference in divorce rate (p>.05). The mother was more likely to become a child's primary caregiver following estrangement.

Changes in orbital volume during childhood in cases of craniosynostosis.

Controversy remains concerning the timing of frontoorbital advancement (FOA) surgery performed for craniosynostosis. Reduced orbital volume and degree of exorbitism are often cited as reasons for early surgical intervention. To date, however, little attention has been given to orbital volume and its changes during the first few years of life as an indicator of orbital growth in children with craniosynostosis. Knowledge of orbital volume and growth patterns in individuals with craniosynostosis and those with normal cranial structures will enable surgeons to refine both the type and timing of surgical intervention required, thus obtaining the optimum outcome for their patients. Using the procedure of segmentation, orbital volumes in 50 children with various forms of craniosynostosis were measured on preoperative computerized tomography scans. Changes in average volume that occur with increasing age were calculated and compared with a model of normal orbital volume growth. At presentation the children with craniosynostosis ranged in age from 1 to 29 months, with 82% of them within the 1st year of life. Several interesting observations emerged from this study. Excluding patients with unilateral coronal synostosis, there was no difference between orbital volumes measured on the right and left sides, allowing mean orbital volume measurements to be used for comparative purposes. Although children with craniosynostosis begin life with significantly smaller orbital volumes, overall normal mean volumes for both sexes are attained by 13 months of age, with volumes approaching normal by 6 months of age in male infants and by 8 months of age in female infants. Changes in orbital volume associated with age generally appear to be similar in most forms of craniosynostosis. There appears to be no significant difference in changes in orbital volume between children with syndromic or nonsyndromic forms of bicoronal synostosis. Orbital volume is significantly reduced on the ipsilateral affected side in cases of unicoronal synostosis in comparison with the contalateral side, but it is not significantly lower than that of normal. Finally, FOA surgery appears to restore normal growth of orbital volume. The results of this study indicate that the underlying mechanism leading to craniosynostosis and restriction of orbital volume "burns out" and begins to lose its major effects within the first few months of life. It would appear that FOA surgery should be delayed until the end of the second half of the 1st year of life, thus maximizing the effects of accelerated normal orbital growth and reducing the risks of relapse.

Combination of Congenital Myogenic Torticollis and Plagiocephaly Caused by Unilateral Coronal Synostosis in Association with Infantile Spasm and Contralateral Hemiplegia as a Result of Encephalitis

Combination of Congenital Myogenic Torticollis and Plagiocephaly Caused by Unilateral Coronal Synostosis in Association with Infantile Spasm and Contralateral Hemiplegia as a Result of Encephalitis

The craniofacial anomalies archive at St. Louis Children's Hospital: 20 years of craniofacial imaging experience.

This article describes how the Craniofacial Imaging Laboratory at the Cleft Palate and Craniofacial Deformities Institute, St. Louis Children's Hospital, Washington University Medical Center, has developed an electronic archive for the storage of computed tomography image digital data that is independent of scanner hardware and independent of units of storage media (i.e., floppy disks and optical disks). The archive represents one of the largest repositories of high-quality computed tomography data of children with craniofacial deformities in the world. Archiving reconstructed image data is essential for comparative imaging, surgical simulation, quantitative analysis, and use with solid model fabrication (e.g., stereolithography). One tertiary craniofacial center's experience in the establishment and maintenance of such an archive through three generations of storage technology is reported. The current archive is housed on an external 35-GB hard drive attached to a Windows-based desktop server. Data in the archive were categorized by specific demographics into groups of patients, number of scans, and diagnoses. The Craniofacial Imaging Laboratory archive currently contains computed tomography image digital data for 1827 individual scans. The earliest scan was done in 1980; the most recently stored scan for the purposes of this report occurred in May of 2000. The average number of scans archived per complete year was 94, with a range of 59 to 138. Of the 1827 total scans, 74 percent could be classified into specific diagnostic categories. The majority of the archive (55 percent) is composed of the following five diagnoses: sagittal synostosis (17 percent), unilateral coronal synostosis (11 percent), hemifacial microsomia (10 percent), plagiocephaly without synostosis (10 percent), and metopic synostosis (7 percent). Storage of computed tomography image data in a digital archive currently allows for continuous upgrading of image display and analysis and facilitates longitudinal and cross-sectional studies, both intramural and extramural. Internet access for clinical and research purposes is feasible, but contingent on protection of patient confidentiality. The future of digital imaging regarding craniofacial computed tomography scan storage and processing is also discussed.

Endocranial Vascular Patterns in a Familial Rabbit Model of Coronal Suture Synostosis

Abstract Objective: The present study investigates the potential relationship between craniosynostosis and any changes in endocranial vasculature. The hypothesis that crania from rabbits with familial, nonsyndromic coronal suture synostosis and crania from rabbits with experimental immobilization of the coronal suture are associated with altered form of the middle meningeal vessels and dural venous sinuses is tested. Design: Silicone rubber endocasts from 14 adult New Zealand white rabbits (Oryctolagus cuniculus) with familial nonsyndromic coronal suture synostosis (five with bilateral coronal suture synostosis and nine with unilateral coronal suture synostosis) were made to assess middle meningeal vessel and dural venous sinus form. For comparative purposes, endocasts were made from 25 rabbits with normal, patent coronal sutures and 10 rabbits with experimental immobilization of the coronal suture. Impressions of the dural venous sinuses were assessed for depth and width. The area of the confluens of sin...