SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Cranial synostosis is relatively common; however, premature closure of a facial suture is remarkably rare. We describe an adolescent male with zygomaticotemporal synostosis that manifested as progressive midfacial and orbital asymmetry, angulation of the cranial base, and nasal deviation. To our knowledge, this is the first description of this anomaly in the literature.
Craniofacial growth data from craniosynostotic children have shown that suture immobilization results in predictable restrictions of cranial vault growth in a direction perpendicular to the affected suture and compensatory growth at sutures perpendicular to the affected one. This study tests these predictions by using rabbits with nonsyndromic congenital coronal suture synostosis. Data were collected from 96 rabbits divided into three groups: 42 unaffected litter mate controls, 33 partially synostosed rabbits, and 21 completely synostosed rabbits. Markers were placed bilaterally on either side of the vault sutures at 1.5 weeks of age. Serial radiographs were taken at 1.5, 6, 12, and 18 weeks of age for assessment of growth at the vault sutures and of various cranial landmarks. Results revealed that completely synostosed animals had significantly (p < .05) shorter cranial vaults, reduced growth at the coronal suture, and increased growth at the sagittal, frontal, and squamosal sutures compared with unaffected rabbits. Results also showed that the calvarial growth observed in this craniosynostotic rabbit model closely reflects predicted compensatory patterns seen in human clinical populations and that this rabbit model is valuable for understanding the pathogeneses and craniofacial growth patterns of humans with premature cranial suture synostosis.
OBJECTIVE: The primary purpose of this study was to evaluate the long-term result of an onlay bone graft augmentation of the supraorbital ridge at the time of primary correction of coronal suture synostosis. DESIGN: The study is a retrospective review of 62 consecutive patients treated for coronal synostosis from June 1991 through February 1997. The surgical technique utilized involved a standard bilateral fronto-orbital advancement and calvarial reshaping with the addition of an onlay bone graft in the supraorbital region. SETTING: All patients were treated at a tertiary care craniofacial center. RESULTS AND CONCLUSION: A total of 62 patients were treated by this technique. Fifty patients underwent primary correction as infants (mean age 9.8 months). An additional 12 patients were older (mean age 8.2 years) and were treated for residual deformity having previously undergone correction by another technique. Results with follow-up as long as 7 years demonstrate stable forehead and orbital symmetry. Complications identified by chart review were minimal and not directly attributable to this modification in surgical technique.
Sagittal synostosis is discussed with respect to the variations seen with the deformity. The morphological spectrum ranging from marked frontal bossing to prominent occipital bulging is described. Surgical techniques have been specifically designed for these variants. These techniques provide an immediate correction of scaphocephaly, and achieve a reduction of the specific deformity with morbidity comparable to that associated with conventional operations. The lack of large areas of craniectomy and the avoidance of synthetic materials are cited as additional advantages of these techniques. The importance of altering the surgical approach to the specific clinical problem is underscored. Two illustrative cases of sagittal synostosis variants are described, and recent experience with the modified operative techniques in treating these and similar cases is discussed.
The procedure of lateral canthal advancement was carried out to correct unilateral coronal synostosis in 39 consecutive patients at The Hospital for Sick Children during a 20-year period. Thirty-seven children were available for follow-up study, and results were considered to be good or excellent in 81% of them. Liberation of the supraorbital margin and release of stenotic skull base sutures are crucial for a successful result. The severity of the process and the age of the patient were not restrictive factors in regard to indication for this procedure. Some patients showed a continuous improvement with time. Morbidity was minimal. It is suggested that lateral canthal advancement is the procedure of choice for unilateral coronal synostosis.
Premature closure of the sagittal suture is the most common form of craniosynostosis, but this condition occasionally goes unrecognized until the child is too old to undergo procedures that depend upon continued calvarial growth for success. As the entire calvaria is affected and thus misshapen by sagittal synostosis, late correction involves total calvarial reconstruction. The extensive nature of this undertaking has precluded its utilization despite the presence of significant deformities. Adapting the techniques and experience gained from craniofacial surgery, the authors performed total calvarial reconstruction on nine children with sagittal synostosis and subsequent scaphocephaly diagnosed after the age of 1 year. In each case the goals of shortening the anteroposterior length, widening the biparietal diameter, and reducing frontal and occipital deformities were met. Morbidity consisted of acute blood loss, postoperative hyponatremia, and in one case a residual skull defect. The rationale for this procedure and the techniques utilized are discussed.
OBJECT: A recurrent point mutation in the fibroblast growth factor receptor 3 gene that converts proline 250 into arginine has been reported recently in cases of apparently nonsyndromic coronal craniosynostosis. The goal of the present study was to examine the phenotype of patients in whom this mutation was present, to determine the prevalence of the condition, and to assess the functional and the morphological outcome of the surgically treated patients. METHODS: A DNA analysis was performed in 103 children suffering from apparently isolated coronal synostosis, 41 of whom had bilateral and 62 of whom had unilateral disease. There were 31 boys and 72 girls in the study group. Sixty cases were sporadic and 43 were familial; the 43 familial cases arose in 33 unrelated families. The mutation was found in seven (12%) of 60 sporadic cases and in 24 (73%) of the 33 families. The functional and morphological results were assessed in all surgically treated patients who had at least 1 year of follow up and who were at least 3 years of age at the time of assessment. A comparison was made between patients with the mutation and those without. CONCLUSIONS: The most typical presentation was seen in girls and consisted of a bicoronal synostosis resulting in a severe brachycephaly associated with mild hypertelorism and marked bulging of the temporal fossae, which resulted in a huge enlargement of the upper part of the face. The most frequently associated extracranial anomaly was brachydactyly, identified either clinically or radiologically. Based on the proportion of bilateral and unilateral coronal synostoses, the present data indicate that the mutation is associated with more severe cases and that girls with the mutation are more severely affected than boys. The functional and morphological results were worse in patients in whom the mutation was present as compared with those in whom it was not.
OBJECT: As public concern about the risks of blood transfusions increased in the mid-1990s, avoidance of transfusions became a goal of surgery for sagittal synostosis. This study was performed to confirm a hypothesized reduction in transfusion rates in recent years and to identify factors associated with both the need for transfusion and low postoperative levels of hemoglobin. METHODS: Sagittal synostosis operations performed in children between 1986 and 1999 were reviewed retrospectively. Patients underwent a minimum of vertex strip craniectomy and parietal craniectomies. There were 118 patients whose median age at surgery was 4.2 months. The primary end point for analysis was defined as either the receipt of a blood transfusion or a postoperative level of hemoglobin less than 70 g/L. Forty-two percent of patients (95% confidence interval [CI] 31-52%) treated before 1996 and 11% of patients (95% CI 0-23%) treated from 1996 onward received blood. The reduction in the blood transfusion rate in later years was, in part, related to the acceptance of a lower postoperative hemoglobin level, often below 70 g/L. A univariate analysis showed that the only patient or surgical factors that correlated with reaching the primary end point in a statistically significant manner were the year of surgery and the extent of surgery. A logistic regression of the age and weight of the child, length of surgery time (from skin opening to skin closure), preoperative hemoglobin level, extent of surgery, and surgeon against the primary end point revealed that the best predictor of the need for a blood transfusion or the presence of a postoperative hemoglobin level lower than 70 g/L was the extent of surgery (beta = 1.4, standard error of the beta statistic = 0.44). Once the extent of surgery was accounted for in the model, no other covariates significantly improved the model. Techniques implemented to minimize blood loss since 1995 included the following: use of the Colorado needle for scalp incision, selection of the Midas Rex craniotome for cranial cuts, and application of microfibrillar collagen. Postoperative hemoglobin was allowed to decrease to 60 g/L if the child was stable hemodynamically, before blood was administered. There were no cardiovascular, wound healing, or infectious complications, and no surgeries were repeated for cosmetic reasons. CONCLUSIONS: Low blood transfusion rates were achieved using simple intraoperative techniques and by accepting a low level of postoperative hemoglobin.
BACKGROUND: Surgical treatment of syndromal craniofacial synostosis consists of combined neurosurgical and maxillofacial reconstructions of the neurocranium and midface. Patients often need several operations if they are to achieve a functionally and cosmetically good result. This is, in part, due to limitations in the amount of "acute" correction possible peroperatively with conventional surgical technique. MATERIAL AND METHODS: In recent years, a new method called distraction osteogenesis has been developed, by which the skeletal elements are gradually advanced. The method is based upon a process whereby the reparative callus formed between the surfaces of two bone segments is subjected to traction. This leads to new bone formation parallel to the vector of distraction. RESULTS: Distraction osteogenesis allows for greater mobilizations, hence the promise of fewer reoperations. INTERPRETATION: The method seems to be a valuable treatment modality in syndromal craniofacial synostosis, where major fronto-orbital, midface or maxillary reconstructions are often called for.
OBJECTIVE: To analyze the efficacy and complications of the surgical correction of exophthalmos in craniofacial synostosis. METHODS: Three different procedures were used in exophthalmos patients with different ages. In patients aged 1 - 3 years old, the fronto-orbital advancing osteotomy to deepen the upper part of orbital cavity was employed. In patients aged 4 - 15 years old, Le Fort III osteotomy and distraction osteogenesis were selected. In patients aged 16 years old or more, Le Fort III osteotomy or monobloc craniofacial osteotomy with immediately advancement of the midface segments were selected. RESULTS: Good results were achieved for all 18 patients. The proptosis reduced 7.8 mm postoperatively. The depth of the skull base increased 8.2 mm and inferior orbit margin was advanced 7.8 mm as compared with the preoperative measurements. The angle between the maxilla and skull base (SNA) increased 9 degree. All of these measurements indicated that the proptosis and craniofacial contouring were approached to the normal situation after surgical intervention. CONCLUSION: Both immediate advancement and gradual distraction after frontal, orbital, and maxillar osteotomy to enlarge the orbital cavities are the best approaches for the treatment of exophthalmos in craniofacial synostosis.
OBJECTIVE: To study the technique of total calvaria reconstruction for sagittal synostosis. METHODS: 11 children with sagittal synostosis were treated. The David II procedure was used to reconstruct the calvarial gap in 6 cases; Osteotomies with reversal change was performed on 2 cases; Plum-plasty of the calvaria was performed on 3 cases. RESULTS: The 11 cases were cured with satisfactory head shape. CONCLUSIONS: The David II procedure is applicable for patient of 1 approximately 3 months. The technique of floating skull flap plasty and plum-plasty of the calvaria can be performed on patients older than 3 months.
This article reports a retrospective quantitative (cephalic index) evaluation of the effect that two different operations for treatment of scaphocephaly secondary to sagittal synostosis have on cranial shape. Combining our results with those of others, the trend is clear: if the objective of surgical intervention for isolated nonsyndromal sagittal synostosis is normalization of the calcarial width to length ratio, a more extensive procedure than simple or extended craniectomy is required.
Involvement of the lambdoid suture appears to be somewhat different from that of other cranial sutures because the ability to tell whether this suture is fused is often impossible by clinical examination or imaging studies, with the findings at operation often variable irrespective of the degree of the deformity. Thus, it is best to think in terms of functional unilambdoid synostosis. The diagnostic evaluation is limited to visual inspection alone in most cases. The indication for surgical intervention is the degree of the deformity. Over the past 15 years I have undergone a transition as to the operative management of functional unilambdoid synostosis, starting initially with a strip craniectomy, followed by removal of the flattened portion of the calvarium to removal of the entire posterior portion of the calvarium, including the bone at the cranial base. Parietal asymmetry as a more extensive approach has uniformly produced better cosmetic results without significant increase in morbidity. In infants less than 3 to 4 months of age the bone removed is not replaced, whereas in those older than 4 months of age the bone from the two sides is reversed, rotated, trimmed, and shaped to reach symmetry. Treatment of bilateral lambdoid involvement is the same as when one lambdoid suture is functionally synostosed, although asymmetry is not a consideration. In a nonsyndromic form, operative intervention allows for increase in the anteroposterior diameter of the calvarium, with eventual reduction of the biparietal widening and vertical orientation to the calvarium if performed early enough.(ABSTRACT TRUNCATED AT 250 WORDS)
The purposes of this study were (1) to evaluate the histologic differences between synostotic versus deformational suture abnormalities and (2) to correlate these histologic findings with anatomic and three-dimensional computed tomographic (CT) scans. We examined three infants with premature metopic synostosis; one infant also had microcephaly trisomy 13 and curious overriding of the coronal sutures. The three-dimensional CT scans demonstrated obliteration of the metopic suture inferiorly. Histologic sections of this suture showed complete bony stenosis. The same pattern was found in all three infants, including the two infants with trigonocephaly who did not have trisomy 13 or microcephaly. In the trisomy 13 infant, the overlapped inferior coronal suture was obliterated on CT examination. However, histologic sections in this region showed a merging of bone; there was no synostosis. In summary, three-dimensional CT re-formation correlated with metopic suture histology. "Stenotic" fusion existed in all infants with trigonocephaly, those with normal and abnormal karyotypes, with and without microcephaly. However, three-dimensional CT re-formation of the trisomic infant showed opacification of the coronal suture in the areas of greatest overlap, whereas histology revealed a curious bone remodeling pattern, possibly a precursor to "deformational" craniosynostosis.
The degree of normalization of the shape of the skull was studied in 12 craniosynostosis patients who had been treated with different types of craniotomies in infancy. Three diagnoses were represented: isolated bicoronal synostosis; Crouzon's syndrome; and Apert's syndrome; there were four subjects in each group. Selected cranial dimensions were measured on roentgencephalograms taken in infancy and at follow-up examinations at 2 to 3 years of age. The measurements were compared with control values obtained from patients with cleft lip. Cranial dimensions in the synostosis patients seldom corresponded well with the values of the controls, either before or after surgery. Cranial height, in particular, was increased, especially in patients with Crouzon's or Apert's syndrome. This resulted in modulus values above the control mean for 11 of the 12 patients at the follow-up examinations.
The results on X-ray densitometric studies of the hand bones in adolescent and young people at the age of 10--17 years (369 persons in all) are presented in the work. Processes of mineralization, ossification and synostosis, as the investigation has demonstrated, are closely connected with each other. From the beginning of ossification in the pisiform and sesamoid bones of the first metacarpophalageal joint (11--12 years of age) up to the completion of synostosis in short tubular bones of the hand (15--16 years of age), a decrease of mineral salts is noted in osseous tissue. Hence, accumulation of mineral salts in skeleton of children and adolescent persons does not occur smoothly, but rather distinctly reflects those functional changes which take place in the adolescent organism when the genital glands begin their increased activity.
As the second of a two-part series, 76 patients with pansynostosis and craniofacial synostosis syndromes were retrospectively analyzed. Diagnoses included pansynostosis (7), craniofrontonasal dysplasia (8), and Apert (24), Crouzon (15), and Pfeiffer (15) syndromes. All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 6.1 months). Twenty-eight patients (36.8 percent) required a secondary cranial vault operation (mean age 28.4 months). Additionally, a major tertiary procedure was necessary in 5 patients to deal with persistent unacceptable craniofacial form. To address the associated finding of midface hypoplasia, 64.8 percent (n = 35) of patients underwent Le Fort III midface advancement or had that procedure recommended for them. The remainder were awaiting appropriate age for this reconstruction. The more extensive pathologic involvement of the pansynostosis and craniofacial syndrome group is illustrated. As compared with the isolated craniofacial synostosis group previously reported, the incidence of major secondary procedures (36.8 versus 13.5 percent), perioperative complications (11.3 versus 5.0 percent), follow-up complications (44.7 versus 7.7 percent), hydrocephalus (42.1 versus 3.9 percent), shunt placement (22.4 versus 1.0 percent), and seizures (11.8 versus 2.9 percent) was significantly increased. Complex problems including those of increased intracranial pressure, airway obstruction, and recurrent turricephaly or cranial vault maldevelopment are repeatedly encountered. In addition, that early fronto-orbital advancement-cranial vault remodeling failed to promote midface development and hypoplasia of this region is almost a consistent finding in the craniofacial syndromic group. The average length of postoperative follow-up was 6 years. According to the classification of Whitaker et al., which assesses surgical results, 73.7 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. An algorithmic approach to the treatment of all patients with craniosynostosis is presented utilizing early surgical intervention as the key element.
Sagittal synostosis comprises a spectrum of deformities caused by premature fusion of the sagittal suture. The treatment of sagittal synostosis is dependent on the age of the patient and the characteristics of the presenting deformity. Immediate correction of the scaphocephalic deformity is the goal of operative treatment. The operative procedures proposed involve whole-vault cranial reshaping, active shortening of the anteroposterior axis, and widening of the mediolateral dimension of the skull leading to a more normal, "rounded" skull shape.