Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “CRANIOSYNOSTOSES”

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.

At least 559 records · Page 31Linked to original sources
PubMed2004

Indication for and surgical outcomes of the distraction method in various types of craniosynostosis. Advantages, disadvantages, and current concepts for surgical strategy in the treatment of craniosynostosis.

BACKGROUND: Various surgical techniques for the treatment of craniosynostosis using distraction devices have been described over the last few years and we have applied these procedures in seven patients with varying types of craniosynostosis. The aim of this report is to clarify the advantages and disadvantages of these surgical methods and to discuss current concepts for the surgical strategy in the treatment of craniosynostosis. MATERIAL AND METHODS: From January 2001 to March 2003, 25 patients with craniosynostosis were examined. Among them, 7 patients, 5 with Apert syndrome, 1 with Crouzon disease, and 1 with multiple-synostosis, underwent surgical treatment using the distraction method with internal distraction devices, according to our treatment strategy for craniosynostosis. All patients underwent preoperative and postoperative evaluations, which included the patient's neurological state, developmental quotient (DQ), and three-dimensional CT (3D-CT). RESULTS: The timing of the procedures undertaken was between the ages of 1 year 5 months and 12 years 6 months (mean age 4 years 11 months). Five patients had received previous treatment and this procedure was used as a secondary operation. Postoperative distraction distances varied from 7 to 20.5 mm (mean distraction distance: 14 mm). Satisfactory cranial volume expansion and aesthetically pleasing morphological states were achieved in all cases. Regarding complications, one patient required re-operation because of dislocation of the device and skin erosion caused by infection around the penetrated wound. Finally, in a second patient a distortion of the device occurred, but no re-operation was needed. CONCLUSION: The advantage of the distraction method is its applicability for Toddler or Elder Children Calvarial Reconstruction to correct cosmetic and functional problems. One disadvantage is the difficulty in using it for Infantile Calvarial Normalization because of thin calvarial bones and the necessity for re-operation to remove the device, which may result in it becoming a "fixation procedure," essentially contraindicated for the fast-developing brain and calvarias. However, the efficacy of this procedure is that the many advantages outweigh the disadvantages as sufficient calvarial expansion and good results using the distraction method, especially in toddler and elder children age groups, can be achieved.

Age Factors↗
PubMed2004

Minimizing blood transfusions in the surgical correction of coronal and metopic craniosynostosis.

OBJECTIVE: The current study was performed to determine the rate of allogeneic blood transfusion for coronal and metopic craniosynostosis surgery done by two experienced pediatric neurosurgeons and to identify factors associated with a need for transfusion. METHODS: First operations for coronal and metopic craniosynostosis correction in children from 1996 to 2002 were reviewed retrospectively. For analysis, the primary endpoint was defined as either the receipt of a blood transfusion or a postoperative Hb level below 70 g/l, even if no blood was transfused. Univariate and multivariate analyses were done to examine the relationship of attaining the primary endpoint to extent of surgery, surgeons, surgery time, preoperative hemoglobin, craniosynostosis type, weight, and age. RESULTS: Thirty-two patients were operated on: 17 with unicoronal; 9 with bicoronal, and 6 with metopic craniosynostosis. Median age at operation was 7.5 months. Blood transfusion rates were 0% for unicoronal, 44% for bicoronal, and 33% for metopic synostosis operations. Logistic regression revealed that the best predictor of having a postoperative hemoglobin level of <70 g/l or receiving a blood transfusion was the extent of surgery (beta=1.59, SE(beta)=0.57). The odds of reaching the primary endpoint for extensive surgery over basic surgery was 4.9 [95%CI=(1.6,15.0)]. Once extent of surgery was accounted for in the model, no other covariates significantly improved the model. CONCLUSIONS: Low transfusion rates were achieved in primary operations for coronal and metopic craniosynostosis using simple intraoperative techniques and by accepting a low postoperative hemoglobin level.

Analysis of Variance↗
PubMed2005

Fetal exposure to sodium valproate associated with Baller-Gerold syndrome: case report and review of the literature.

CASE REPORT: We report three patients with a history of maternal valproate use during pregnancy who presented with a combination of metopic suture synostosis and upper limb malformations, which could be diagnosed as Baller-Gerold syndrome (BGS). The patients underwent surgical treatment for the craniofacial deformity, during which standard frontocranial reconstruction was performed. REVIEW OF THE LITERATURE: Only 32 patients have been reported in the world literature and these cases support the emerging view that BGS is not a distinct syndrome, but should instead be considered to be an heterogeneous phenotype with variable etiology. CONCLUSIONS: Our case series suggests for the first time that fetal sodium valproate exposure may also cause this phenotype.

Craniosynostoses↗
PubMed2005

Age-related changes in lateral ventricle morphology in craniosynostotic rabbits using magnetic resonance imaging.

BACKGROUND AND AIMS: Craniosynostosis occurs in 300-500 per 1,000,000 live births and results in secondary craniofacial, ocular, and intracranial anomalies. Neurologic problems associated with craniosynostosis include changes in intracranial morphology such as dilation of the cerebral ventricles, however, clinical studies are confounded by small sample sizes, heterogenous samples, and lack of age-matched controls. The present study was designed to assess age-related changes in the lateral ventricle volume of the brain in normal rabbits and rabbits with naturally-occurring coronal suture synostosis using serial magnetic resonance imaging. METHODS: Eighteen rabbits (6 wild-type controls, 6 with early-onset [ approximately 21 days gestation], and 6 with delayed-onset [approximately 25 days post-gestation] coronal suture synostosis) had magnetic resonance imaging (MRI) at 10, 25, and 42 days of age. RESULTS: The results demonstrate that rabbits with early-onset synostosis had significantly (p<0.001) dilated and larger lateral ventricles (by 77% at 10 days of age) than wild-type and delayed-onset synostosis rabbits, which progressively worsened by day 42. CONCLUSION: This finding suggests that uncorrected coronal suture synostosis may have early effects on lateral ventricle volume hypertrophy, possibly through obstructed cerebrospinal fluid and/or venous drainage and circulation.

Age Factors↗
PubMed2005

Surgical correction of metopic synostosis.

BACKGROUND: Premature closure of the metopic suture results in deformation of the anterior portion of the calvarium, which can vary from mild to severe. In mild forms, there is only prominent ridging of the metopic suture; more severe forms result in a marked narrowing of the frontal and temporal regions that in turn affects the supraorbital rims and produces hypotelorism. METHODS: The authors retrospectively reviewed 39 consecutive cases of metopic synostosis treated over a 12-year period. RESULTS: The average age at referral was 5 months, with surgery performed at an average age of 7.5 months. Fifteen infants had other congenital anomalies, with eight having synostosis of other sutures. Follow-up ranged from 7 months to 6 years, with an average of 29 months. In three mild cases, burring of the metopic ridge was performed with excellent aesthetic results in all cases. The other 36 patients had significant deformity of the supraorbital ridges and temporal regions, with obvious hypotelorism for over 50% of the time. In these cases, the patients underwent craniofacial reconstruction to normalize their appearance. In addition, the lateral aspect of the sphenoid ridges, including the orbital roof and lateral orbital wall to the infraorbital fissure, was removed to free the cranial base. The average blood loss was under 400 ml and the average hospital stay was 3.6 days. Results were considered good to excellent in all except three cases, which had recurrence of a prominent metopic ridge; two required a second operation after 6 months for burring of this ridge, whereas the third was treated conservatively with an orthotic headband. CONCLUSION: Mild forms of metopic synostosis can be successfully treated with burring of the metopic ridge alone. Severe forms require craniofacial reconstruction and may be associated with other congenital abnormalities, additional synostosis, and developmental delay. In all cases, the operative procedure must be tailored to the nature and severity of the deformity.

Child↗
PubMed2005

Skull vault growth in craniosynostosis.

BACKGROUND: Since its first description by Virchow, the principle of abnormal skull growth due to restriction of skull growth at the fused sutures, and the realisation by Moss that the sutures at the skull base are equally affected, have been the main intellectual driving forces behind the majority of cranial expansion procedures performed currently in children with craniosynostosis. CURRENT OBSERVATIONS: Despite original impressions that craniosynostosis leads to craniostenosis, many studies have demonstrated that in the majority of patients with craniosynostosis there is normal skull volume in those over the age of 6 months. In Apert syndrome, skull volume is invariably larger than normal. Some studies have shown that intracranial pressure is independent of intracranial volume, and can exist in the presence of normal volume, or indeed after cranial expansion. These observations imply that cranial expansion procedures create a state of artificially increased skull volume, in the quest to improve appearance and function. FUTURE ADVANCES: This creates a new angle of view through which skull growth abnormalities are seen. It is becoming clearer that in most patients with craniosynostosis, there is regional imbalance of skull growth, which co-exists with a variety of other equally important factors, such as genetic defects, raised intracranial pressure, venous hypertension, and other brain parenchymal anomalies such as hindbrain hernia or hydrocephalus. It is becoming increasingly obvious that the type of surgical treatment currently practised in most cases is conceptually incorrect. Recent modifications such as the use of springs or distraction do not escape from the underlying philosophy of cranial expansion. With that in mind, it is hoped that advances in the fields of genetics and molecular biology will provide treatments for the cause of craniosynostosis rather than the symptomatic relief that surgery offers currently. CONCLUSION: Until then, there is a need to develop better ways of quantifying regional abnormalities of skull growth.

Cranial Sutures↗
PubMed2005

Skull base growth in craniosynostosis.

INTRODUCTION: The interrelationship of skull base growth and craniosynostosis is one that is not often taken into account in the clinical management of patients with craniofacial disorders by neurosurgeons and craniofacial surgeons. Very early on in our medical training we are taught the anatomical differences between the calvarial unit and skull base portion of the skull. The inherent differences in both underlying tissue components and the different growth characteristics of these two uniquely different structures are critical in understanding skull base growth in craniosynostosis and the inherent potential craniofacial growth in these unique children. REVIEW: This paper will review some of the basic anatomy of these zones of growth plus review some of the prevalent theories of the effect of skull base growth on craniosynostosis and the converse. CONCLUSION: While the theories of these growth patterns have yet to be finalized an understanding of their potential influences and abnormal growth patterns remain key to providing a good surgical outcome in surgery for craniosynostosis.

Cranial Sutures↗
PubMed2005

Venous hypertension and craniosynostosis.

INTRODUCTION: This article addresses the relevance of venous hypertension to children born with, in particular, the "syndromic" forms of craniosynostosis. The development of the human cerebral venous system is summarised and the patterns of anomalous venous drainage seen in children with complex forms of craniosynostosis associated with raised intracranial pressure are described. AETIOLOGY: The aetiology of these abnormal patterns is discussed under the general headings of "The constriction theory", "A primary role for FGFR mutations" and "Persistence of the foetal pattern of intracranial venous drainage."Venous hypertension (along with hydrocephalus, cranio-cerebral disproportion and airway obstruction) has an important role in the aetiology of raised intracranial pressure in severely affected children. TREATMENT: The inter-relationship between these factors is explained and then the treatment of those children whose raised intracranial pressure is considered to be due predominantly to venous hypertension is described.

Cerebral Veins↗
PubMed2005

Chiari malformation in craniosynostosis.

INTRODUCTION: Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon's syndrome, 75% with oxycephaly, 50% with Pfeiffer's syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still controversial. DISCUSSION: Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed. Evaluation and management of CM are also discussed. CONCLUSION: Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures. Venous hypertension caused by stenosis of the jugular foramen can also be present in these patients, resulting in intracranial hypertension and/or hydrocephalus. Careful MRI evaluation is recommended for the forms of craniosynostosis at a high risk of developing hindbrain herniation. The selection of posterior cranial vault expansion as the first surgical procedure is advocated. In selected cases, treatment of the posterior cranial deformity by occipital vault remodelling and treatment of the Chiari-like deformity by suboccipital decompression can be carried out using the same surgical procedure.

Arnold-Chiari Malformation↗
PubMed2005

Hydrocephalus in craniosynostosis: a review.

INTRODUCTION: Ventricular dilatation in the presence of primary craniosynostosis is a unique condition with respect to pathogenesis, clinical significance, and morphological appearance. It is rarely observed in nonsyndromic craniosynostosis, and in these cases usually attributable to coincidental disorders. Conversely, it is a common feature of syndromic craniosynostosis, affecting at least 40% of patients with Crouzon's, Pfeiffer's or the Apert syndrome. Shunt-dependent hydrocephalus is predominantly associated with Crouzon or Pfeiffer syndrome while in the Apert syndrome the usual finding is nonprogressive ventriculomegaly which, however, may also occur in some cases of Crouzon syndrome. PATHOGENESIS: The pathogenesis of progressive hydrocephalus remains somewhat obscure, a hypoplastic posterior fossa and a venous outlet occlusion at the skull base being the main causative factors discussed in literature. Ventriculomegaly may reflect primary brain maldevelopment or in some cases even a compensated state of increased cerebrospinal fluid (CSF) outflow resistance. CLINICAL EVALUATION: Clinical evaluation is mainly aimed at identifying progressive hydrocephalus, but diagnosis is hampered by the fact that classical clinical signs may be absent, and that ventricular dilatation will often become evident only after decompressive cranial surgery. Moreover, mild ventriculomegaly may in some cases coexist with intracranial hypertension from craniostenosis. Therefore, careful monitoring of intracranial pressure and ventricular size in the pre- and postoperative period is a diagnostic mainstay. CONCLUSION: In true hydrocephalus ventriculo-peritoneal shunting is currently the single promising mode of treatment.

Cranial Sutures↗
PubMed2005

Intracranial pressure monitoring in children with single suture and complex craniosynostosis: a review.

INTRODUCTION: One third of patients with craniofacial dysostosis syndromes and about 15-20% of children with single suture craniostenosis have a documented increase in intracranial pressure (ICP). The early detection of intracranial hypertension is important in order to reduce the risks for brain development and visual function. However, in children with craniosynostosis, the clinical manifestations of abnormally increased ICP are difficult to detect, as the majority of patients may have neither warning signs nor symptoms for a long period of time. REVIEW: Moreover, data from the literature suggest that neither fundoscopic nor radiological findings are necessarily related to intracranial pressure recordings in this type of pathology. In this context prolonged ICP monitoring seems actually to be the most valuable diagnostic tool. Extradural, subdural, and intraparenchymal devices have been used, and different softwares have been employed for PC storage and analysis of the data obtained. Most recent series consider mean ICP combined with plateaux waves as the most sensitive indicators in the final evaluation of the results. CONCLUSIONS: The analysis of the literature demonstrates a significant difference in the prevalence of raised ICP preoperatively between non-syndromic and syndromic patients. Among the non-syndromic children, a direct relation between the number of sutures involved and raised ICP is documented. The analysis of plateaux waves seems to be particularly important in children with borderline mean ICP values. Persistent postoperative raised ICP has been described in 6-15% of patients with craniofacial dysostosis. It must be related to the multifactorial etiology of increased ICP in these patients, which includes cerebral venous congestion, upper airway obstruction, and hydrocephalus.

Cerebrospinal Fluid Shunts↗
PubMed2005

Positional skull deformities in children: skull deformation without synostosis.

BACKGROUND: Patients with craniosynostosis are readily diagnosed by clinical and neuroimaging findings. Surgical treatment is indicated for preventing neurological deficits and for correcting esthetically unacceptable head deformities. In recent years, we have witnessed a progressive number of neurosurgical consultations for abnormal head shapes unrelated to premature fusion of the cranial sutures, especially of positional plagiocephaly. There have been descriptions of abnormalities in the cerebrospinal fluid (CSF) spaces in children with craniosynostosis. OBJECTIVES: The aim of the present study was to investigate the role of the changes of the CSF spaces in the development of positional skull deformities in children. PATIENTS AND METHODS: The authors reviewed demographic, clinical, and neuroimaging data of 23 patients assessed for some form of nonsynostotic skull deformity (group A). The results were compared with those of a simultaneous group of nine infants diagnosed with benign extracerebral collections of fluid (group B). RESULTS: The study group was composed of 11 boys and 12 girls, aged 3 years or younger. Seventeen children had plagiocephaly, four scaphocephaly, and two brachycephaly. Sixteen children (15 with plagiocephaly and 1 with brachycephaly) exhibited enlarged subarachnoid CSF spaces. In group B, the boy/girl ratio was of 7:2. Infants in group A presented at an older age (mean 12.7 months) than group B (mean age at presentation of 7.17 months). Children with benign extracerebral fluid collections were born with a head circumference (HC) greater than infants with positional skull deformities (p=0.005). The percentile of the children's HC at consultation was also larger for children of group B (p=0.03). The form of clinical presentation differed between the two groups. Most infants of group A were seen because of a type of head deformity, and children in group B were studied for macrocephaly. Long-term follow-up assessment showed better outcomes for patients in group B than for children of group A in regard to regression of initial symptoms (p=0.03). CONCLUSIONS: Most positional head deformities appear to be related with the children's positioning for sleeping. We have not confirmed macrocephaly as a contributing factor for positional deformities. The distribution of extracerebral CSF and the presence of abnormal collections of fluid in children with positional head deformities do not seem to be related with the findings of pericerebral CSF encountered in children with benign extracerebral collections of fluid. In our view, brain pulsations, transmitted to these accumulations of CSF, play an important part in the development of the infants' skull deformities (p=0.02). The findings of enlarged CSF spaces in children with nonsynostotic skull deformation constituted an age-related event, as these collections tended to disappear as the children grew older (p=0.04).

Cerebral Ventricles↗
PubMed2005

Diagnosis of isolated sagittal synostosis: are radiographic studies necessary?

AIMS AND OBJECTIVES: Isolated sagittal synostosis can be diagnosed easily on clinical grounds. This study was designed to determine if children could be operated on based solely on a clinical diagnosis or whether such an approach would result in any potentially unnecessary surgeries. MATERIALS AND METHODS: Records of 114 consecutive children operated on for isolated nonsyndromic sagittal synostosis over a 14-year period (1987-2000) were reviewed to see whether the clinical findings were in concordance with the intraoperative findings and histopathology of the sagittal suture. RESULTS: The age at surgery ranged from 1.9 to 81.3 months (median 4.3 months). Preoperative skull radiographs were done in 78 children and computer tomography (CT) scans of the head in 12 children. One hundred percent of the skull radiographs and 83.3% of the CT scans were completed prior to referring the children to a pediatric neurosurgeon. CT scans confirmed the diagnosis in all patients in whom it was available. For the rest, pathology reports and/or operative records were available for 108 (94.7%) children and were confirmatory for sagittal craniosynostosis in all. CONCLUSIONS: Accurate diagnosis of isolated sagittal synostosis can be made clinically, and operative correction can proceed without a need for radiological investigations, unless the clinical features are not completely typical. This approach could result in significantly reduced radiation exposure for the developing brain and could provide economic benefits to health care providers.

Child↗
PubMed2006

Emotional and psychological impact of delayed craniosynostosis repair.

INTRODUCTION: Among children with craniosynostosis, there exists an interesting dynamic involving parents' preconceptions of craniosynostosis and its repair, influenced in large part by differing cultural perspectives. In a time in which we are understanding how critical a child's early formative years are in influencing his/her emotional and psychological development, the authors describe one medical aspect involved in that dynamic process. MATERIALS AND METHODS: The authors reviewed their cumulative experience at the Children's Hospital San Diego between January 2000 and June 2004 and identified nine children with significant craniofacial deformities and, for one reason or another, had delayed surgical repair. DISCUSSION: The authors have found that by age 6, parents will often bring their children back to their physician and insist on surgical correction. The significant motivating factor in most of these cases stems from teasing by classmates with respect to head shape. In this manuscript, we report and discuss some of the emotional and psychological issues associated with delayed craniosynostosis repair. Often times, these issues are overlooked or underemphasized in the overall surgical care of such patients.

Adaptation, Psychological↗
PubMed2006

Distraction vs remodeling surgery for craniosynostosis.

OBJECTS: We designed several distraction devices and applied these instruments in 14 patients with varying types of craniosynostosis. The aim of this report is to clarify the advantages and disadvantages of these surgical methods and to discuss current concepts for the surgical strategy in the treatment of craniosynostosis. METHODS: From January 2000 to July 2005, 28 patients with craniosynostosis were retrospectively analyzed. Surgical treatment was performed on 14 patients using the distraction method with internal distraction devices that we designed, in which 5 patients had plagiocephaly, 3 brachycephaly, and 6 scaphocephaly. All patients underwent preoperative and postoperative evaluations, which included the patient's neurological state, and three-dimensional CT. RESULTS: With distraction devices, the time required for the surgery could be shortened almost 3 1/3 h; the bleeding during the surgery was decreased with reduced requirement of more than 200 ml of blood transfusion as compared with remodeling surgery. Postoperatively achieved distraction distances varied from 30.0 to 47.5 mm (mean, 42.99 mm). The average increased volume percent of cranium in distraction surgery group was 20.9% (range, -11.5 to 58.9%) after full distraction. CONCLUSION: With distraction surgery, satisfactory cranial volume expansion and aesthetically pleasing morphological states were achieved in all cases, and the efficacy was statistically significantly high as compared with remodeling method.

Adolescent↗
PubMed2006

Neurodevelopment of children with single suture craniosynostosis: a review.

INTRODUCTION: Rates of neurocognitive risk range from 35-50% of school-aged children with isolated single suture craniosynostosis (SSC). It has been hypothesized that early surgical intervention to release suture fusion reduces risk for increased intracranial pressure (ICP) and the corresponding risk to neurodevelopment. However, studies assessing children with SSC have been inconsistent in finding an association between neurocognitive development, age of surgery, and ICP. REVIEW: SSC produces notable distortion of the cranial vault and underlying brain mass. Although a linear relationship between skull distortion, ICP, and neurocognitive deficits has generally been assumed, recent studies have postulated an interactive process between the skull and developing brain that results in neuroanatomical changes that are not limited to areas directly beneath the fused suture. The specific neuropsychological deficits identified in children with SSC including problems with attention and planning, processing speed, visual spatial skills, language, reading, and spelling may be related to the anatomic differences that persist after correction of suture fusion. CONCLUSIONS: Available literature on neurocognitive development of children with SSC is suggestive of mild but persistent neuropsychological deficits, which become more significant as cognitive demands increase at school age. Anatomical studies of children without SSC are beginning to identify particular groups of brain structures that if disrupted or malformed, may be associated with specific cognitive deficits. Controlled research investigating the relationship between persistent anatomical changes and neurocognitive functioning of school-aged children with SSC is needed.

Adolescent↗