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At least 343 records · Page 19Linked to original sources
PubMed1999

Human NELL-1 expressed in unilateral coronal synostosis.

Surgical correction of unilateral coronal synostosis offers a unique opportunity to examine the molecular differences between an abnormal and a normal cranial suture. We isolated and identified a cDNA fragment whose expression was up-regulated in the premature fusing and fused coronal sutures, as compared with normal coronal sutures. The nucleotide sequence of the full-length cDNA of this gene, human NELL-1, has approximately 61% homology with the chicken Nel gene. Both chicken Nel and human NELL-1 are comprised of six epidermal growth factor-like repeats. The human NELL-1 messages were localized primarily in the mesenchymal cells and osteoblasts at the osteogenic front, along the parasutural bone margins, and within the condensing mesenchymal cells of newly formed bone in sites of premature sutural fusion. Human multiorgan tissue mRNA blot showed that NELL-1 was specifically expressed in fetal brain but not in fetal kidney, liver, or lung. We also showed that Nell-1 was expressed in rat calvarial osteoprogenitor cells and was largely absent in rat tibiae and fibroblast cell cultures. In conclusion, our data suggest that the NELL-1 gene is preferentially expressed in cranial intramembranous bone and neural tissue (both of neural crest cell origin) and is up-regulated during unilateral premature closure of the coronal suture. The precise role of this gene is unknown.

Amino Acid Sequence↗
PubMed1997

Surgical treatment of premature sagittal synostosis.

A series of 50 consecutive children with premature sagittal synostosis is reported. All were treated surgically; 43 were male, 47 were leukodermic and two are siblings. In the pre-operative examination, the head shape, skull measurements and radiologic findings were evaluated; 38 children were operated on before six months of age and 12 of them, between six and 12 months of age. The surgical technique used was a wide biparietal craniectomy. Blood transfusions were occasional, being necessary for only six (12%) children. The children were admitted at the day of surgery and discharged between the second and third post-operative day. No local or general complications were observed and no one died. The aesthetic result was considered good. The altered skull measurements before surgery reached normalization as far as the end of the first year after the treatment. It may be concluded that wide biparietal craniectomy is a procedure of great effectiveness in the treatment of the premature fusions of the sagittal suture.

Craniosynostoses↗
PubMed2004

Unilateral coronal synostosis: a histomorphometric study.

OBJECTIVE: This histomorphometric study compared the open and prematurely fused side of the coronal suture in subjects with unilateral coronal synostosis (UCS). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Bone Density↗
PubMed2006

A case of unilateral coronal synostosis in a child with craniofacial microsomia.

Craniofacial microsomia is a congenital malformation complex associated with a wide array of craniofacial and extracraniofacial anomalies. Frontal plagiocephaly has been shown to occur in approximately 5% to 12% of patients with craniofacial microsomia. The etiology of craniofacial microsomia-associated frontal plagiocephaly is unclear; of the cases described in the literature, all but one had physical findings suggestive of deformational plagiocephaly. In the case with equivocal physical findings, radiographic studies showed no evidence of craniosynostosis. Unlike the above cases, we report a documented case of radiologically-confirmed unilateral coronal synostosis in a child with craniofacial microsomia.

Craniofacial Abnormalities↗
PubMed1994

Asterion region synostosis.

Posterior plagiocephaly historically has been associated with synostosis of the lambdoid suture. The incidence, diagnosis, and modes of treatment for stenosis of the lambdoid suture remain controversial. Commonly, the lambdoid suture is found to be open both on radiographic examination and at the time of surgery. The study reports on nine patients who presented with unilateral posterior plagiocephaly and who were found to have open lambdoid sutures, but a stenosed region of the asterion. The area of involvement included the distal-most lambdoid suture, the parietomastoid, occipitomastoid, and proximal squamosal sutures. Positional molding or torticollis was ruled out in all patients. All the patients showed progressive involvement of the skull base, including anterior shifts of the ipsilateral ear, compensatory ipsilateral frontal bossing and malar protrusion. Stenosis of the asterion was diagnosed with three-dimensional computed tomography scans, corroborated at the time of surgery and confirmed histologically. Surgical correction involved resection of the affected asterion and reconstruction using a bandeau-technique, barrel staves of the occipital bone and bone graft transposition. This approach provided excellent esthetic results in all patients.

Cranial Sutures↗
PubMed1997

Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis.

OBJECTIVE: The current study compared the mental and psychomotor development of infants with nonsyndromic sagittal synostosis (SS) with a demographically matched comparison group without congenital defects. Within the SS group, we tested the hypothesis that age of cranial release would be inversely correlated with mental development. DESIGN: The design was prospective and longitudinal. Participants were assessed at 4, 12, and 24 months of age. SETTING: The study was conducted in a craniofacial clinic at an urban children's hospital. PARTICIPANTS: Participants were 19 infants with SS (consecutive craniofacial program referrals) and 19 demographically matched comparison infants recruited from the community. One infant with SS did not attend the 24-month assessment. MAIN OUTCOME MEASURES: Mental and Psychomotor Indices from the Bayley Scales of Infant Development were the primary outcome measures. Subdomains of development were created using Kohen-Raz scoring procedures. All measures were determined a priori. RESULTS: Repeated-measures MANOVAs revealed no statistically significant differences in the developmental trajectories of the two groups. None of the SS group infants received Mental Development Index (MDI) scores in the mentally retarded or borderline range of intellectual functioning (i.e., below 78). An inverse correlation (r = -.30) was found between the age at surgery and Bayley growth curve coefficients; however, this association was not statistically significant (p = .10, one-tailed). CONCLUSIONS: Results are consistent with previous studies of the mental and psychomotor development of infants with nonsyndromic craniosynostoses in relation to normative test data. The relation between surgery age and developmental outcome merits further study in a larger sample with a greater range of surgery ages.

Age Factors↗
PubMed2001

Correction of coronal suture synostosis using suture and dura mater allografts in rabbits with familial craniosynostosis.

OBJECTIVE: Resynostosis following surgical correction of craniosynostosis is a common clinical correlate. Recent studies suggest that the dura mater is necessary to maintain suture patency. It has also been hypothesized that dura mater from synostotic individuals may provide aberrant biochemical signals to the osteogenic fronts of the calvaria, which result in premature suture fusion and subsequent resynostosis following surgery. This study was designed to test this hypothesis by surgically manipulating the coronal suture and dura mater in rabbits with familial craniosynostosis to prevent postsurgical resynostosis. DESIGN: Craniofacial growth and histomorphometric data were collected from 129 rabbits: 72 normal controls and 57 rabbits with bilateral coronal suture synostosis (15 unoperated on controls; 13 surgical controls; 9 dura mater transplant only; 10 suture transplant only; and 10 suture and dura mater transplant). At 10 days of age, all rabbits had radiopaque amalgam markers placed on either side of the coronal, frontonasal, and anterior lambdoidal sutures. At 25 days of age, 42 synostosed rabbits had a 3 to 5-mm wide coronal suturectomy. Coronal sutures and/or underlying dura mater allografts were harvested from same-aged, wild-type, isohistogenic control rabbits and transplanted onto the dura mater of synostosed host rabbits. Serial radiographs were taken at 10, 25, 42, and 84 days of age, and the suturectomy sites were harvested at 84 days of age in 44 rabbits and serially sectioned for histomorphometric examination. RESULTS: Results revealed that cranial vault growth was significantly (p < .05) improved following surgical release of the fused coronal suture compared with synostosed rabbits who were not operated on but was still significantly different (p < .05) from that of normal control rabbits. By 84 days of age, significant (p < .05) differences were noted in calvarial suture marker separation, cranial vault shape indices, and cranial base angles between rabbits with and without dura mater allografts, probably as a result of resynostosis of the suturectomy site or suture-only allografts. Qualitative histological examination revealed that at 84 days of age rabbits with suture and dura allografts had patent coronal sutures, suture-only allografts had fused coronal sutures with extensive endosteal hyperostosis, dura mater-only allografts had some new bone in the suturectomy site that resembled rudimentary osteogenic fronts, and suturectomy controls had extensive endosteal bone formation and resynostosis of the suturectomy site. Significantly (p < .05) more bone was found in the suturectomy sites of rabbits without dura mater allografts compared with rabbits with dura mater allografts. CONCLUSIONS: Results support the initial hypothesis that normal dura mater allografts will maintain suture or suturectomy site patency and allow unrestricted craniofacial growth. However, it is still unclear whether the dura mater from normal rabbits was providing biochemical signals to the transplanted sutures or suturectomy sites or simply acting as a barrier to prevent abnormal biochemical signals from the dura mater of synostosed rabbits from reaching the calvaria. The clinical and therapeutic implications of these procedures are discussed.

Analysis of Variance↗
PubMed1994

Sleep apnea and sleep-related breathing disorders in patients with craniofacial synostosis.

Seventeen patients with craniofacial synostosis (CFS) have been treated at the Department of Plastic and Reconstructive Surgery at Osaka Medical College during the past 10 years. Six patients were thoroughly evaluated at the Department of Otolaryngology by polysomnography (PSG), cephalometric X-ray and nasopharyngoscopy during sleep. In 4 of the patients PSG showed obstructive sleep apnea syndromes (OSAS). Heavy snoring without apnea and paradoxical respiration were noted in the other 2 patients. Thus, all of the patients had sleep-related breathing disorders (SRBD). It is assumed that the incidence of SRBD in CFS is high. Cephalometric analysis and profilogram showed maxillomandibular hypoplasia in each patient, and it was assumed that the main cause of SRBD in CFS was stenosis of the upper airway tract caused by maxillo-mandibular hypoplasia. Nasopharyngoscopy was performed during sleep in 2 patients with OSAS secondary to CFS. One patient with adenotonsillar hypertrophy had nasopharyngeal obstruction and another patient whose posterior airway space (PAS) on cephalometric radiograph was 3 mm (normal value: 11 mm) had obstruction at the tongue base.

Acrocephalosyndactylia↗
PubMed1984

Alternative techniques in the treatment of unilateral coronal synostosis.

Premature closure of one coronal suture results in bilateral abnormalities. There is always ipsilateral flatness of the orbital rim, and contralateral frontal bossing is often found. The authors have employed three operative techniques for correction of unilateral coronal synostosis: frontal bone overlay, lateral canthal advancement, and the tongue-in-groove procedure. The choice of operative technique depends upon the exact deformity to be corrected. The authors believe that altering the relations between the bone and dura by techniques such as radical remodeling and dural plication may improve the results of surgical correction of craniosynostosis.

Craniosynostoses↗
PubMed1987

Late surgical treatment of unilateral coronal synostosis using methyl methacrylate.

Three techniques combining the shaping of calvarial and facial bone with onlay of methyl methacrylate are presented for use in the late treatment of unilateral coronal synostosis deformities. The procedures described are suggested as possible alternatives to extensive bone repositioning procedures. They have the advantage of being quicker and are therefore potentially safer operations. Acrylic is malleable and does not resorb; thus, permanent superior esthetic results may be achieved. The two most serious risks when using this technique are infection and limitation of growth. The risk of infection may be reduced by attaching the acrylic implant securely to surrounding bone, under sterile conditions, beneath well-vascularized skin. Growth limitation may be obviated by not placing acrylic across sutures in children with enlarging skulls.

Adult↗
PubMed1990

Treatment of bilateral coronal synostosis in infancy: a holistic approach.

Bilateral coronal synostosis often results in a turribrachycephalic skull shape. Reduction of skull height and elongation of the anteroposterior axis of the skull while preserving normal cerebral function are the major therapeutic goals. A surgical technique is described which can successfully accomplish these goals in a single operative procedure.

Craniosynostoses↗
PubMed1995

Chronic tonsillar herniation in Crouzon's and Apert's syndromes: the role of premature synostosis of the lambdoid suture.

The incidence of chronic tonsillar herniation (CTH) was evaluated with magnetic resonance imaging in 44 patients with Crouzon's syndrome and 51 with Apert's syndrome; the incidence was 72.7% in Crouzon's syndrome and 1.9% in Apert's syndrome. All the patients with Crouzon's syndrome and progressive hydrocephalus had CTH, but of 32 individuals with Crouzon's syndrome and CTH, only 15 had progressive hydrocephalus. Five patients with Apert's syndrome were treated for progressive hydrocephalus; none had CTH. The patterns of suture closure in these two groups of patients were studied, and significant differences in coronal, sagittal, and lambdoid sutures were found between patients with Crouzon's and Apert's syndromes. In Crouzon's syndrome, significant differences in the pattern of lambdoid suture closure were found between the groups with and without CTH; in the group with CTH, the lambdoid closure appeared earlier. The authors propose that the high incidence of individuals with CTH who have Crouzon's syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.

Acrocephalosyndactylia↗
PubMed1996

Outcome analysis of 85 patients undergoing the pi procedure for correction of sagittal synostosis.

The authors present a retrospective review of their experience with 85 cases using the pi procedure to correct sagittal synostosis. A male preponderance of four to one was recognized in this group. Sixty-five infants underwent computerized tomography scanning of the head prior to surgery; these scans revealed unexpected intracranial pathology in 5% of cases. Surgical complications included three intraoperative dural lacerations. All children received blood transfusions with no complications. Cosmetic outcomes were excellent in 53%, good in 43%, and poor in 4% of cases. One patient required reoperation. All poor outcomes were in infants who were younger than 8 weeks of age at the time of surgery and who underwent a "reverse pi" procedure. Most of the excellent outcomes occurred in infants who were between 3 and 6 months of age at the time of surgery. Although more extensive than strip craniectomy, the pi procedure can be accomplished with minimal morbidity. In the authors' opinion, the pi procedure provides better immediate and long-term cosmetic results than synostectomy alone.

Age Distribution↗
PubMedDate not supplied

Visual loss in patients with craniofacial synostosis.

We reviewed the ocular findings in patients with cranial stenosis, who were seen in the Craniofacial Clinic at The Children's Hospital of Philadelphia, to determine the prevalence, pattern, and etiology of visual loss. There were 15 patients with Apert syndrome, 25 patients with Crouzon syndrome, and 18 patients with other forms of craniofacial synostoses. Visual loss occurred in 17 eyes of 11 patients with Apert syndrome, 14 eyes of 11 patients with Crouzon syndrome, and 12 eyes of 8 patients with other craniofacial synostosis syndromes. Refractive errors and strabismus were present in the majority of these patients. Amblyopia due to strabismus, ametropia, or ptosis was the major reason for visual loss in all three groups of patients. Structural abnormalities contributed to loss of vision in six eyes.

Craniofacial Dysostosis↗
PubMed2003

Speech, language, and cognitive development in children with isolated sagittal synostosis.

This study investigated the occurrence, nature, and severity of speech, language, and cognitive impairment in 76 children (61 males, 15 females) with isolated sagittal synostosis (ISS) aged 9 months to 15 years 7 months. There was no increased prevalence of global cognitive impairment in the group but there was a high prevalence rate of speech and/or language impairment with 28 (37%) displaying impairment of whom 20 (71%) had moderate or severe impairments that fulfilled the criteria for specific impairments. Prevalence rates were only increased for children over two years of age. Expressive language impairment occurred most frequently. Raised intracranial pressure, peri-neonatal risk factors, otitis media, or being operated were not associated with impairment. Surgery at a later age and a family history of speech and language impairment were both associated with impairments but numbers were small. The findings suggest that children with ISS are at an increased risk of developing speech and language impairment.

Adolescent↗
PubMed2003

Single suture synostosis and intracranial hypertension.

It is difficult to determine the incidence and importance of intracranial hypertension in children with single suture synostosis. A review of the literature suggested that some number of affected children have high intracranial pressure. The incidence of cognitive disability also appears to be high in this population. Currently, it cannot be proven that the intracranial hypertension seen in these children causes the cognitive dysfunction or that, for the group as a whole, surgical intervention alters cognitive outcome.

Child↗
PubMed1991

Unicoronal synostosis. A surgical intervention.

Computer-assisted medical imaging technology provides useful tools for the in vivo study of congenital craniofacial deformities. Using these tools, a standardized operation for infants with nonsyndromal unicoronal synostosis has been shown to consistently normalize orbital dimensions and decrease exocranial base angulation. Normalization of endocranial base angulation is less consistent. The plasticity of the infant's cranial base after cranial vault surgery suggests a locus of the primary pathology at a site other than the cranial base.

Craniosynostoses↗
PubMed1990

[Dolicocephaly and premature synostosis of the sagittal suture].

Guided by the literature historical, racial, genetical and diagnostical aspects, likewise associated syndromes and malformations of dolichocephaly and premature sagittal synostosis are represented, also own clinical, roentgenological, pneum- and electroencephalographic findings of 57 patients. Vascular disturbances of brain were striking, further emotional lability and tendency for relapsing depressions. Psychosocial problems of patients with scaphocephaly are discussed in detail and from that a conditional indication for cosmetic correction is asserted.

Cephalometry↗