Analysis of fronto-orbital advancement for Apert, Crouzon, Pfeiffer, and Saethre-Chotzen syndromes

The purposes of this study were (1) to document outcome after primary fronto-orbital advancement for the four major eponymous craniosynostotic syndromes (Apert, Crouzon, Pfeiffer, and Saethre-Chotzen) and (2) to identify factors that might influence need for primary and secondary fronto-orbital advancement or foreheadplasty. Also tested was the hypothesis that coincident sagittal synostosis could modulate brachycephaly and affect whether a primary or secondary frontal operation was necessary. Data were collected on age and indications for initial operation, type of primary and secondary frontal procedures, and concomitant sagittal synostosis. Patients initially managed by subcranial Le Fort III were included in the study group but excluded from analysis of fronto-orbital advancement. Patients treated by monobloc advancement or Le Fort III osteotomies with frontal grafting or Anderl modification were assessed as having had primary fronto-orbital advancement. Minimum time to follow-up was 5 years. A total of 126 patients met inclusion criteria. Lateral photographs were examined to assess preoperative and postoperative sagittal position of supraorbital rims-to-globes. Frontal re-advancement was indicated if the corneal apex was anterior to the supraorbital rim. Foreheadplasty was indicated for unacceptable frontal contour and normal supraorbital rim-to-globe relationship. Primary correction for frontal retrusion was not required in 4 percent of Apert (1 of 25), 16 percent of Crouzon (7 of 44), 6 percent of Pfeiffer (2 of 31), and 19 percent of Saethre-Chotzen (5 of 26) patients. Of those infants who had a primary fronto-orbital advancement, reoperation for either supraorbital retrusion or frontal deformity was necessary in all 16 Apert patients and in 5 of 19 Crouzon (26 percent), 10 of 26 Pfeiffer (38 percent), and 13 of 20 Saethre-Chotzen (65 percent) patients (p < 0.001). Age at initial fronto-orbital advancement did not influence reoperative rate. No correlation was found between concomitant sagittal synostosis and necessity for primary or secondary frontal correction (p = 0.22). In summary, phenotypic diagnosis was determinant for outcome as defined by need for secondary fronto-orbital advancement, foreheadplasty, or both. Apert patients had the highest incidence of reoperation for frontal retrusion or forehead contour. Crouzon and Saethre-Chotzen patients were most likely to express a minor phenotype and not require fronto-orbital correction. Coincident sagittal synostosis did not influence frontal projection, as reflected in need for either primary or secondary frontal advancement.     

Unilateral fusion of the frontosphenoidal suture: a rare cause of synostotic frontal plagiocephaly

Unilateral coronal synostosis is the common appellation for premature, one-sided fusion of the frontoparietal suture-the most common cause of synostotic frontal plagiocephaly. However, frontal asymmetry can also result from isolated fusion across the anterior cranial base without involvement of the frontoparietal suture. This article describes three patients with localized synostosis of the frontosphenoidal suture, the medial extension of the coronal ring. Two patients were initially misdiagnosed as having unilateral coronal synostosis and the other as having deformational frontal plagiocephaly. The patients had variable frontal flattening, with depression and recession of the ipsilateral orbital rim. The nasal root was midline or slightly deviated to the contralateral side. The sagittal position of the ipsilateral malar eminence was slightly retruded in one patient and symmetric in the other two. The auricular position was symmetric in the sagittal plane for all patients. In all three patients, computed tomography examination demonstrated a patent frontoparietal suture and fusion of the frontosphenoidal suture (basilar hemicoronal ring). Two patients had involvement of contiguous sutures: one had fusion extending to the sphenoethmoidal suture and the other's involved part of the sphenozygomatic suture. The sagittal suture was midline in all patients. In summary, synostotic frontal plagiocephaly denotes a relatively broad phenotypic spectrum that includes unilateral coronal synostosis and more isolated fusions in the basilar coronal ring. The physical findings resulting from frontosphenoidal synostosis are unique, yet careful evaluation will minimize confusion with other causes of asymmetric frontal flattening. Proper diagnosis necessitates awareness of this uncommon entity and requires focused computed tomographic assessment.     

Maxillary changes and occlusal traits in crania with artificial fronto-occipital deformation

Artificial fronto-occipital deformation of the cranial vault was typical of pre-Columbian cultures in the central Andean coastal regions. We have studied the influence of this deformation on maxillary and mandibular morphology. Measurements were performed on 86 adult Ancon skulls with anteroposterior deformation. Undeformed skulls from the area of Makatampu (n = 52) were used as the control group. To explore the influence of the deformity on occlusion, the skulls were categorized using the Angle classification and the alignment of the interincisor midline. In the group of deformed skulls, there was an increase in lateral growth of the vault and of the base of the skull (P < 0.001), giving rise to a greater interpterygoid width of the maxilla (P < 0.001), and an increase in the transverse diameter of the palatal vault. The mandible presented an increase in the length of the rami (P < 0.001) and in the intercondylar width, with no alteration of mandibular length. The deformed skulls had normal (class I) occlusion, with no displacement of the midline. The difference in the asymmetry index between the two groups was not statistically significant. Artificial fronto-occipital deformation of the cranial vault provoked compensatory lateral expansion of the base that was correlated with the transverse development of the maxilla and mandible. Occlusion and sagittal intermaxillary position were not affected by the cranial deformity. These results provide evidence of the integration between the neurocranium and the viscerocranium in craniofacial development, and support the hypothesis of a compensatory effect of function.     

Sutural effects of fronto-occipital cranial modification

Maya adult crania from the site of Lamanai, Belize provide a retrospective means of examining growth processes in the cranial vault. The Lamanai population practiced fronto-occipital deformation which is found to be significantly associated with premature sagittal synostosis and wormian bones of the lambdoidal suture. The undeformed members of the population also exhibit an abnormally high frequency of sagittal synostosis, but a significantly lower frequency than the deformed sample. It is suggested that the deforming apparatus creates tensile forces on the sagittal suture during the peak period of growth of the parietals, and that these forces might induce an adaptive response important in producing premature sagittal synostosis. The undeformed sample may have an increased congenital risk of sagittal synostosis created by their natural brachycephalic morphology in utero. The frequency patterning of wormian bones suggests a mixture of genetic and environmental causes in which tensile forces may also play a role. © 1996 Wiley-Liss, Inc.     

Preoperative anthropometric dysmorphology in metopic synostosis

Anthropometric identification of dysmorphology in craniofacial anomalies, including the craniosynostoses, provides invaluable assistance in clinical diagnosis as well as offering a technique for interpreting possible deformities in skeletal remains. Premature closure of the metopic suture is a rare form of craniosynostosis, representing about 4% of clinically diagnosed synostoses. Accompanying this closure are defects of the head and face, particularly the upper face and orbits. To identify quantitatively the craniofacial dysmorphology associated with metopic synostosis, 50 patients with a diagnosis of primary (nonsyndromal) metopic synostosis were examined using a battery of 24 anthropometric measurements from which 11 proportion indices were calculated. The data were compared to sex- and age-matched normal standards and converted to standard (Z) scores before being analyzed using Student's t-test. The data indicate a complex pattern of dysmorphology arising from the synostosis which affects the upper face and orbits as well as the cranial vault. The entire fronto-orbito-zygomatic complex is narrowed, and vertex is reduced. There is compensatory sagittal and transverse growth of the posterior neurocranium and compensatory vertical and sagittal growth of the upper face. There are statistically significant differences in the pattern of dysmorphology between patients presenting prior to 6 months of age and those older but no significant differences between sexes. Am J Phys Anthropol 103:341–351, 1997. © 1997 Wiley-Liss, Inc.     

The reversal exchange technique of total calvarial reconstruction for sagittal synostosis

The role of total calvarial reconstruction in the treatment of sagittal synostosis remains controversial, especially in patients younger than 1 year of age. The purpose of this study was to prospectively evaluate the efficacy of a single surgical technique for total calvarial reconstruction (the reversal exchange technique) in patients younger than 1 year of age who had a radiographically confirmed diagnosis of sagittal synostosis. Twenty-three consecutive patients underwent the reversal exchange technique of total calvarial reconstruction at a median age of 3 months (age range, 6 weeks to 10 months). Quantitative assessments were performed on the basis of preoperative and postoperative (minimum, 6 months) measurements of the cephalic index (cranial width/cranial length x 100) taken from three-dimensional computed tomography scans, which were obtained in 18 of 23 patients. Aesthetic assessments were performed on the basis of the grading of preoperative and postoperative photographs, obtained in 17 of 23 patients, by three independent raters who were blinded as to the surgical technique. The mean preoperative cephalic index was 65.0, and the mean postoperative index was 76.4, yielding a mean improvement of 11.4 (17.5 percent). By photographic evaluation, 12 of 17 patients (70.6 percent) were classified as having a normal head shape (grade 4) and five of 17 (29.4 percent) as having minor residual deformities (grade 3). No patients were identified as having significant residual deformities (grades 1 or 2). There were two intraoperative complications and one postoperative complication, none of which resulted in permanent morbidity. It was concluded that the reversal exchange technique of total calvarial reconstruction provided significant improvement in head shape on the basis of quantitative measurements (cephalic index) and independent evaluations of aesthetic improvement.     

Trigonocephaly: refinements in reconstruction. Experience with 33 patients

Thirty-three consecutive patients with metopic synostosis treated between January of 1980 and June of 1984 are presented. All patients underwent craniofacial reconstruction by an interdisciplinary team utilizing a detailed remodeling of the supraorbital rims and forehead. Advantages of the current modifications are discussed, as well as an analysis of the results. We believe this represents a significant advance over previous methods not only because of the superior cosmetic results achieved, but also because of its safety and reproducibility. Reoperation rate was less than 10 percent overall and was necessary only in patients with complex cranial vault abnormalities. Mean follow-up was 22.8 months, with no deaths, infections, or damage to visual or cerebral function. Postoperative head circumference demonstrated an immediate increase followed by a normal growth curve.     

Total cranial vault reconstruction for parietal encephalocele

A 3-year-old boy underwent skull reconstruction in order to reduce a parietal encephalocele. The reduction of the encephalocele was accomplished fully and safely while simultaneously correcting a concomitant turriscaphocephaly skull-shape irregularity. A combination of the modified prone position and cranial reconstruction using barrel stave expansion of the basal vault was employed.     

Osseous expansion of the cranial vault by craniotasis.

A study of cranial vault lengthening using a custom expandable fixation-distraction (craniotatic) appliance was performed in the young-adult rabbit model. Ten 24-week-old rabbits underwent circumferential suturectomy plus expansion (expanded group), 10 underwent circumferential suturectomy without expansion (sham control group), and 10 served as normal controls. The appliance was lengthened at a rate of 2.5 mm per week for 5 weeks. Serial lateral cephalometry, comparative dry-skull anthropometric measurements, and histologic examinations were performed. The expanded group demonstrated a significantly longer skull, cranial vault, anterior cranial base, posterior face, and orbit as compared with the control groups (p less than 0.05). Callus bone filled the distracted suturectomy and united the frontofacial complex to the posterior cranium. In conclusion, skull lengthening by distraction osteogenesis is possible in the rabbit model and offers a new technique for future investigation in the treatment of coronal synostosis.     

Photographic assessment of head shape following sagittal synostosis surgery.

A photographic assessment of the head shape of infants who had undergone surgical correction of sagittal synostosis was performed to determine (a) whether this subset could be delineated from an age-matched normal subpopulation and (b) whether two operative procedures differed in achieving normalization of head shape. This retrospective study included 8 patients who underwent extended strip craniectomy, 12 patients who underwent subtotal calvarectomy and cranial vault remodeling, and 12 age-matched subjects with no calvarial abnormality, for a total of 32 subjects. Criteria for inclusion in this study included surgery for sagittal synostosis within the first year of life and postoperative photographs at ages 4 to 8 years (mean, 4.5 years). Each set of images (frontal and lateral profile) were ranked from most to least normal by five lay observers and four professional observers. The rankings were analyzed with statistics designed for ordinal data. Differences in ranking between treatment groups were examined with Kruskal-Wallis rank sums tests. Mean ranks were calculated for lay and professional observers in an attempt to produce simpler and more generalizable results; these means were also analyzed using statistics designed for ordinal data. There was no statistical difference in the ranks of infants who had undergone a surgical correction and the normal subpopulation. In the mean rankings of the lay observers, the normal groups had the highest score mean (15.6), the group with extended strip craniectomy was second (16.0), and the subtotal calvarectomy with calvarial remodeling group was last (17.8) (p = 0.84). In the mean rankings of the professional observers, the normal groups again had the highest score mean (15.8), the subtotal calvarectomy group was second (15.9), and the extended craniectomy group was last (18.6) (p = 0.77). These results suggest that children who have undergone correction of sagittal synostosis in infancy are indistinguishable from their peers, on the basis of fully haired head shape on frontal and lateral photographs, when they begin primary school, irrespective of the type of calvarial surgery.     

Skull base expansion: craniofacial effects

In order to determine what effect the anterior cranial base has on the developing craniofacial skeleton, mechanical expansion of the growth of one segment of the anterior cranial base was performed. New Zealand white rabbits were used for this study. A sham-treated group (n = 16) underwent implantation of dental amalgam markers to either side of the frontonasal, coronal, and lambdoid sutures at 9 days of age to serve as markers of vault growth. The experimental group (n = 7) underwent the same marker placement at 9 days of age, but, in addition, at 30 days of age these animals underwent placement of a mechanical spring, unilaterally, at the frontosphenoid suture. A second control group (n = 8) underwent the same exposure of the frontosphenoid suture, but the spring was laid only on the surface of the bone. All animals were followed by radiographic cephalometry at 9, 30, 60, and 90 days of age. The experimental group demonstrated statistically significant expansion of the cranial base and ipsilateral coronal suture. The midface skeletal dimensions were unchanged by spring distraction of the cranial base. These findings indicate that cranial base sutural growth can be manipulated mechanically and that growth changes can be attained secondarily in the cranial vault skeleton.     

Craniofacial suture stenosis: morphologic effects

Craniofacial anomalies, such as Apert's and Crouzon's syndromes, are presumed to be related to premature growth arrest of cranial base growth sites. However, premature growth arrest at cranial vault sutures in animals appears to play a causative role in the development of cranial deformities characteristic of single-suture, or simple, craniosynostosis in humans. To study the possible causative role of cranial vault and other (interface) suture stenoses on the development of craniofacial deformity, a vault suture and an interface suture between the cranial vault and facial skeleton were simultaneously immobilized. Thirty-one New Zealand White rabbits at 9 days of age underwent implantation of dental amalgam growth markers adjacent to cranial vault and facial sutures. In the experimental group (n = 15), methylcyanoacrylate adhesive was applied over the coronal (vault) and frontonasal (interface suture between vault and facial skeleton) sutures to immobilize them. The remaining 16 animals served as sham-treated controls. All animals underwent serial radiographic cephalometry to document growth effects in the cranial vault, cranial base, and facial skeleton. Application of adhesive resulted in statistically significant (p less than 0.05) reduction in growth at the coronal and frontonasal sutures. This was accompanied by an overall significant reduction in neurocranial vault length during the first 30 days of development.(ABSTRACT TRUNCATED AT 250 WORDS)     

Endoscopic craniectomy for early correction of craniosynostosis

Twelve patients between 0.4 and 7.8 months of age were treated by an endoscopic approach to strip craniectomy. Nine patients had sagittal suture involvement. Two patients had a single unilateral lambdoid suture synostosis, and one patient had a combination of a right coronal synostosis and a metopic synostosis. Postoperatively, all patients were placed in cranial remodeling helmets and the results showed that the estimated blood loss ranged from 5 cc to 150 cc, with blood transfusion required in only one patient. All patients were discharged from the hospital by day 2, and all patients had an improvement in their cranial head shape. The specific technique of using the endoscope to aid in performing a strip craniectomy will be discussed. Nine endoscopically treated patients with the diagnosis of sagittal suture synostosis were compared with nine patients treated by using the Marchac remodeling techniques. The mean operative time (1.6 hours versus 3.5 hours), estimated blood loss (43 cc versus 168 cc), hospital costs ($11,671 versus $36,685), and length of stay (1.16 days versus 5.1 days) were less by using the endoscopic technique. All nine patients treated by using the Marchac technique required a blood transfusion, whereas only one patient was transfused in the endoscopically treated group.     

华北人欧洲人和澳洲土著人的头骨厚度

The thickness of the cranial vault at the midline on the mid-frontal squama, pre-bregmatic einence, frontal at bregma, parietal at vertex, occipital at lambda and the external occipita1 protuberance was recorded in 40 male and 7 female Northern Chinese crania, 47 male and 52 female Australian Aboriginal crania and 13 male European crania using specially nodified vernier calipers. Comparison of vault thickness data obtained through direct measurement with those obtained fron lateral radiographs indicated that direct measurenent provided consistently more accurate results.
Male and fermale samples were processed separately so that the extent of sexbased variation could be examined.Student's t test was used to compare the sample means and the percentage of sexual dimorphism for each dimension was calculated according to Garn et al, (1964).The possibility of an allometric association between the thickness of the bones within the cranial vault, size of the cranial vault and stature was examined using Spearman's rank correlation coefficient and the Australian Aboriginal sample.
All but one of the mean thickness dimensions in the Australian Aboriginal male sample is significantly greater than the Northern Chinese and European means. The female results support those obtained with the males.In both males and females thickness at the external occipital protuberance, in all of the populations examined,did not correlate highly with that obtained from other parts of the cranial vault.This reflects the high degree of morphological variation in the position of the internal occipital protuberance and its influence on cranial vault thickness dimensions recorded at the external occipital protuberance.The European and Northern Chinese samples have similar cranial vault thickness dimensions. The Spearman's rank correlation coefficient matrix scores provide sone support for a biological association between vault thickness and overall cranial size. However, there appears to be little support for an association between stature and cranial vault thickness. The difference between the male and female mean vault thickness dimensions were significant at bregma, vertex and the external occipital protuberance in Australian Aboriginals and lambda and the external occipital protuberance in Northern Chinese. Some caution is needed in the interpretation of the Northern Chinese female data as the sample is extremely small.
Evidence of trauma, supressed fractures, is extremely common on the vaults of Australian Aboriginal crania from southern and central Australia. Traditionally Australian Aboriginals, males and females, involved in agressive dispute will use a substantial wooden implement and strike to the head of thir opponent(Meggitt 1962).The injuries that result from this are more common in females than in male. This form of social interaction must have rigorously selected against those individuals with thinner bones in their cranial vaults. To a large degree this may explain the greatly thickened vaults in Australian Aboriginals relative to Europeans and Northern Chines.This may also provide a clue to the factors resulting in the development of marked cranial vault thickness in Homo erectus.
     

Maxillary volume growth in craniosynostosis

Craniosynostosis, and in particular, craniofacial dysostosis, exhibits abnormalities of the nasomaxillary complex in form, position, and development. The aim of this study was to quantitatively assess the volumetric maxillary abnormality in patients at the time of initial diagnosis of craniosynostosis and to make comparisons with a "normal" reference range for maxillary volumes throughout childhood. The technique of segmentation was applied to preoperative computed tomographic head scans obtained in 31 children (14 boys, 17 girls), between 1 and 34 months of age (mean, 11.06 months), who underwent cranial expansion surgery for craniosynostosis affecting the coronal suture complex. Maxillary volumes were plotted against age for the first 3 years of life and were compared with a healthy population. There was no statistical difference between the two sexes for mean maxillary volume. The mean maxillary volumes for the entire group were statistically smaller than the norm (p = 0.046, linear regression with age as a covariable), but there was no statistical difference among the four different groups of coronal synostosis (unilateral coronal, nonsyndromic bilateral coronal, nonsyndromic complex pansynostosis, syndromic bilateral coronal synostosis) (p = 0.407, one-way analysis of variance). On graphic data analysis, the maxillary volume was smaller than the norm in craniosynostotic children who presented in the first few months of life. However, by 7 months of age in nonsyndromic bilateral coronal synostosis and by 17 months of age in syndromic bilateral coronal synostosis, the maxillary volumes had increased toward the norm. This implies that the effect of the craniosynostotic process on the midface structures is present from birth and parallels the effect on the cranial vault sutures.     

Effects of fronto-occipital artificial cranial vault modification on the cranial base and face.

Artificial reshaping of the cranial vault has been practiced by many human groups and provides a natural experiment in which the relationships of neurocranial, cranial base, and facial growth can be investigated. We test the hypothesis that fronto-occipital artificial reshaping of the neurocranial vault results in specific changes in the cranial base and face. Fronto-occipital reshaping results from the application of pads or a cradle board which constrains cranial vault growth, limiting growth between the frontal and occipital and allowing compensatory growth of the parietals in a mediolateral direction. Two skeletal series including both normal and artificially modified crania are analyzed, a prehistoric Peruvian Ancon sample (47 normal, 64 modified crania) and a Songish Indian sample from British Columbia (6 normal, 4 modified). Three-dimensional coordinates of 53 landmarks were measured with a diagraph and used to form 9 finite elements as a prelude to finite element scaling analysis. Finite element scaling was used to compare average normal and modified crania and the results were evaluated for statistical significance using a bootstrap test. Fronto-occipitally reshaped Ancon crania are significantly different from normal in the vault, cranial base, and face. The vault is compressed along an anterior-superior to posterior-inferior axis and expanded along a mediolateral axis in modified individuals. The cranial base is wider and shallower in the modified crania and the face is foreshortened and wider with the anterior orbital rim moving inferior and posterior towards the cranial base. The Songish crania display a different modification of the vault and face, indicating that important differences may exist in the morphological effects of fronto-occipital reshaping from one group to another.     

Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive?

Twenty-eight craniomaxillofacial fibrous dysplasia patients were treated as early as the symptoms occurred. The principles of surgical treatment were based on the zones of involvement: total excision of dysplastic bone of fronto-orbital, zygoma, and upper maxillary origin (zone 1) and bone reconstruction primarily; conservative excision on hair-bearing skull (zone 2), central cranial base (zone 3), and tooth-bearing bones (zone 4); and optic canal decompression on patients with orbital dysplasia and decreasing visual acuity. Patients were followed for 1 to 11 years (average 5.3 years). No recurrence or invasion of the fibrous dysplasia into the grafted bone was seen. One patient had orthognathic maxillary osteotomy on the reconstructed maxilla 6 years after initial reconstruction. Five of 19 patients with alveolar dysplasia had a recurrence and were reshaped. One patient had mandibular sagittal osteotomies to set back the prognathic, fibrous dysplasic mandible after three attempts at conservative shaving. Another patient with mandibular fibrous dysplasia had recurrence with pain and a hemimandibulectomy with successful immediate free vascularized iliac bone graft reconstruction.     

Segmental bone and cartilage reconstruction of major nasal dorsal defects

This article describes the results of segmental bone and cartilage reconstruction of significant nasal dorsal defects. Solid bone graft reconstructions frequently lead to an unnatural hardness of the nasal tip. Rib cartilage reconstructions are pliable and soft but are a problem because they easily undergo warpage. The operation is performed using the open approach. Outer cranial bone graft is used for the bone component and extends at least two-thirds of the length of the dorsum. It is secured in place with a compression screw and a Kirschner wire. The cartilage component consists of an abbreviated L strut constructed of septal or conchal cartilage. It is slotted into the cranial bone in a tongue-in-groove manner and is sutured to it through a drill hole in the bone. The dorsal profile is completed with a single cartilage onlay graft or multiple sagittal cartilage grafts secured to the sides of the L strut. Twelve patients underwent segmental reconstruction of nasal deformities. Within this group, five patients underwent secondary rhinoplasty, five underwent posttraumatic rhinoplasty, and two underwent nose augmentation for Oriental features. There were seven men and five women. In all cases, good nasal tip mobility was maintained, and the nasal tips were soft. The interface between the bone graft and cartilage graftwas well camouflaged. The two did not separate. This procedure follows the principle of replacing lost tissue with like materials.     

Skull expansion in experimental craniosynostosis

Bilateral coronal suture immobilization was performed in 9-day-old rabbits to simulate the brachycephaly deformity characteristic of coronal synostosis. Growth abnormalities were documented by serial radiographic cephalometry. A "corrective" linear craniectomy procedure was performed on the rabbits with an immobilized coronal suture at 60 days of age. The degree of amelioration of the induced abnormalities by the surgery in these animals was compared with a similar group of animals that had the same surgery plus supplemental implantation of a spring expansion device at the coronal suture craniectomy site. The mean marker separation at the coronal suture was significantly greater (5.61 mm +/- 0.52 SE versus 2.53 mm +/- 0.38 SE; p less than 0.05) following spring expander implantation than with linear craniectomy alone. Similarly, induced anterior cranial base shortening and distortion of craniofacial cephalometrics were more significantly improved by the additional use of the spring expansion device.