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At least 109 records · Page 6Linked to original sources
PubMed1996

Synostectomy versus complex cranioplasty for the treatment of sagittal synostosis.

Although pediatric neurosurgeons traditionally have been trained to perform a sagittal synostectomy for the treatment of sagittal synostosis, numerous articles advocating modifications of this procedure or more complex cranial vault reconstructions point our the inadequacies of strip craniectomy in providing optimal short- and long-term cosmesis. This review addresses the major arguments for and against performing complex cranioplasties for sagittal synostosis, as well as issues regarding timing of surgery, mortality and morbidity, increased need for blood transfusion, and increased expense. The authors support the contention that for the majority of infants with sagittal synostosis, complex calvarial vault reconstruction provides a better outcome with little increase in operative risk.

Cephalometry↗
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↗
PubMed1998

A rabbit model of human familial, nonsyndromic unicoronal suture synostosis. I. Synostotic onset, pathology, and sutural growth patterns.

Poswillo has stated, "The more severe anomalies of the calvaria, such as plagiocephaly, Crouzon [syndrome], and Apert syndrome still defy explanation, in the absence of an appropriate animal system to study" (p. 207). This two-part study reviews data from a recently developed colony of New Zealand white rabbits with familial, nonsyndromic unilateral coronal suture synostosis. Part 1 presents pathological findings and compensatory sutural growth data from 109 normal rabbits and 82 craniosynostotic rabbits from this colony. Synostotic foci, onset, and progression were described in the calvariae from 102 staged (fetal days 21, 25, 27, 33; term = 30 days) fetuses (39 normal, 63 synostosed). Calvarial suture growth patterns from 10 to 126 days of age were assessed from serial radiographs obtained from 89 rabbits (70 normal rabbits and 19 rabbits with unicoronal suture synostosis) with amalgam bone marker implants. Perinatal results revealed that by fetal day 25 the synostotic focal point in synostotic rabbits consistently originated from the endocortical surface of the calvaria in the middle of the coronal suture at a presumed high-tension, interdigitating zone. Histological analysis revealed hyperostotic osteogenic fronts on the affected side compared with the unaffected side. Postnatal sutural growth data revealed a predictable pattern of plagiocephaly (contralateral coronal sutures growing more than ipsilateral sutures and ipsilateral frontonasal and anterior lambdoidal sutures growing more than contralateral sutures), which resulted in early cranial vault deformities and a double "S" shape torquing towards the affected side. The advantages and disadvantages of these rabbits as a model for human familial, nonsyndromic unicoronal suture synostosis are discussed, especially in light of recent cytokine and genetic findings from human craniosynostotic studies.

Animals↗
PubMed1984

Intracranial changes with unilateral coronal synostosis.

Intracranial changes in two patients with unilateral coronal synostosis were evaluated by computed tomography (CT). Preoperative CT scans showed local compression of the brain by the flattened frontal bone with definite shift of the midline structures, suggesting that unilateral coronal synostosis may be harmful to the growing brain in infants. Postoperative restoration of the intracranial changes led me to draw the conclusion that unilateral coronal synostosis should be treated as early as possible not only for cosmetic reasons but also for the prevention of parenchymal damage.

Brain↗
PubMed2004

The circle of sagittal synostosis surgery.

Sagittal synostosis, the premature closure of the sagittal suture, accounts for more than 50% of all nonsyndromic single-suture synostoses. Although no detrimental neurologic effects can be directly attributed to the synostosis, a number of patients will have relatively increased intracranial pressure. Surgical correction for sagittal synostosis has evolved from simple removal of bone strips to extensive cranial remodeling, all in a attempt to achieve a normal head shape. The lack of outcome measures has limited the surgeon's ability to choose one surgical procedure over another. The use of a cranial helmet for passive molding of the head after surgery is effective when used with limited endoscopic surgery. We present the results of using an extended strip craniectomy combined with long-duration molding helmet therapy and compare it with other reported methods. The results suggest that passive molding with the helmet may have a greater effect on cranial shape than surgery.

Craniosynostoses↗
PubMed2001

Calvarial vault remodeling for sagittal synostosis.

Sagittal synostosis is the most common form of craniosynostosis (i.e., premature fusion of cranial sutures). Sagittal synostosis, which is the premature fusion of the sagittal suture, occurs in 56% to 58% of all reported cases of craniosynostosis. This article describes calvarial vault remodeling, the procedure of choice at Children's Hospital Los Angeles for surgical intervention to correct sagittal synostosis. Perioperative interventions to minimize patients' risks and maximize benefits are explained. A case study is presented to describe the experience of one patient and his family members.

Cranial Sutures↗
PubMed2003

Spring-mediated cranioplasty compared with the modified pi-plasty for sagittal synostosis.

The aim of this study was to compare the safety, morphological outcome, and degree of parental satisfaction of the new spring-mediated cranioplasty with those of the modified pi-plasty in the management of sagittal synostosis. Ten patients with non-syndromic sagittal synostosis treated with the spring-mediated cranioplasty were followed prospectively. A control group of 10 sex-matched patients operated on with the modified pi-plasty procedure was chosen. Cephalometric radiographs were obtained preoperatively and postoperatively at 1 year of age. Cephalic index, axial width ratio, length ratio, width ratio and height ratio were used as objective measures of outcome. Parents were sent a questionnaire to obtain a subjective aesthetic assessment of outcome. Significantly less blood replacement was required (p = 0.003), and shorter duration of postoperative anaesthesia (p = 0.030) and postoperative hospital stay (p = 0.013) were found in the spring-mediated cranioplasty group. There were no complications or deaths in either group. Also significant was the inter-group difference in the postoperative change in the height ratio (p = 0.030), the most change being seen in the spring group. The change in the subjective parental aesthetic evaluation of skull shape was significant in both groups. In conclusion, the spring-mediated procedure was morphologically more effective than the modified pi-plasty procedure in the management of sagittal synostosis with the additional benefits of less blood transfusion needed and shorter duration of hospital stay.

Cephalometry↗
PubMed1998

Unilateral lambdoid synostosis: morphological characteristics.

The accurate diagnosis of unilateral lambdoid synostosis versus deformational occipital plagiocephaly has remained a complex and controversial issue in the field of craniofacial surgery. Over the past 30 years, numerous studies have been published describing the treatment for "lambdoid synostosis," the diagnosis having been ascribed despite radiographically patent sutures and pathology specimens displaying fibrous, nonfused suture lines. Over the past 16 years, 204 patients with unilateral occipital plagiocephaly have been seen at our unit, only 2 of whom (approximately 1%) manifested the clinical, radiographic, and pathological features of true lambdoid synostosis. Radiographically, there was evidence of sutural fusion on plain films and two- and three-dimensional computed tomographic scans. Clinical features included a trapezoid head shape as viewed from the vertex, ipsilateral occipitomastoid bossing, contralateral parietal bossing, and anteroinferior ear displacement. Pathology specimens showed bony sutural fusion. These characteristics are contrasted to those of patients with deformational plagiocephaly and those found in other published studies on occipital plagiocephaly.

Craniosynostoses↗
PubMed2006

Quantitative and qualitative assessment of morphology in sagittal synostosis: mid-sagittal vector analysis.

Consensus remains lacking regarding the optimal surgical treatment modality for sagittal synostosis. There is, however, wide agreement that objective analytical methods are required to demonstrate the characteristic morphology of the condition and to substantiate the benefits of specified surgical techniques. Simple calculated anthropomorphic indices, such as the cranial index, are commonly used but fail to provide satisfactory representation of morphology, which is far more complex than can be represented by its simple length-width ratio. Techniques to provide more comprehensive, yet practical, assessment of morphology are needed for analytic purposes. Herein, we introduce vector analysis as an objective, computed tomography (CT)-based morphometric technique for assessment of cranial morphology; this work represents the first application of the technique mid-sagittal vector analysis (MSVA). MSVA is a single plane application that was devised to address dysmorphology in sagittal synostosis. It was our hypothesis that MSVA would quantitatively and qualitatively depict preoperative morphology and postoperative correction in specific regions. Sixteen patients undergoing cranial reshaping surgery for sagittal synostosis were included in the study. All patients underwent routine preoperative and 1 year postoperative CT scans, from which the MSVA was derived. MSVA is a radial vector analysis in which distances to the cranial surface are measured from a single reference point origin in the sagittal plane. Preoperative morphology, characterized by respective vectors, was analyzed in three regions: the frontal, vertex, and occipital regions. Comparison with postoperative paired data was conducted for each patient. The analysis of postoperative change demonstrated (1) decrease in prominence in the frontal and occipital regions, (2) increase in height and forward translation of the vertex, and (3) ability to distinguish and qualify frontal versus occipital bossing and correction thereof. We conclude that the longitudinal differences associated with scaphocephaly are well characterized and differentiated by MSVA. Quantitative and qualitative assessment identifies three relevant regions affected by the condition and its treatment: the frontal, vertex, and occipital regions. The transverse dimension is not addressed in this single plane analysis; a more comprehensive application will require additional planes of analysis and the development of a normative database.

Cephalometry↗
PubMed1993

[Measurement of normal eyeball position and its application for evaluation of exophthalmos in craniofacial synostosis].

The chronologic changes in eyeball position in relation to bony landmarks were studied by CT scans. The results were applied to the preoperative evaluation in 16 craniofacial synostosis patients. Ten subjects from each age group were selected from more than 2000 subjects who underwent CT scanning of the skull without any brain and skull diseases. These age groups ranged from birth to 20 years of age. The following four distances were measured on the CT section incorporating lenses: (1) the distance from the dorsum sellae to the lenses, (2) the distance between the lateral orbital rim and the lenses, (3) the distance from the nasal root to the lenses, and (4) the width between the lenses. These distances also were measured in 16 craniofacial synostosis patients preoperatively, and the results were compared with those of the healthy individuals in the same age group. The distances of the lenses from the nasal root and from the lateral orbital rim were larger than in the healthy individuals in almost all craniofacial synostosis patients. However, the distance from the dorsum sellae to lenses was the same as that in healthy individuals on the whole.

Adolescent↗
PubMed1996

Late correction of sagittal synostosis in children.

Twelve patients with sagittal synostosis underwent correction between 8 months and 8 years of age. The patients underwent expansion in the parietal region of 4 cm and had a reduction in length of 3 cm following dural plication. Average follow-up was 2.2 +/- 0.8 years (mean +/- SD). While optimal treatment for sagittal synostosis may be obtained in the first few months of life, late correction of sagittal synostosis may be obtained in a safe and effective manner.

Age Factors↗
PubMed1999

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.

Craniosynostoses↗
PubMed2002

Delayed cranial vault reconstruction for sagittal synostosis in older children: an algorithm for tailoring the reconstructive approach to the craniofacial deformity.

An algorithm for the management of sagittal synostosis in older children who underwent delayed cranial vault reconstruction is presented. This algorithm tailors the surgical approach to the specific craniofacial deformity present in each case. The scaphocephalic deformity characteristic of sagittal synostosis varies significantly when presentation is delayed beyond the first year of life, the time during which reconstruction is usually performed. Sixteen patients with sagittal synostosis who presented after 12 months of age, and were a mean of 3.2 years of age at the time of cranial vault reconstruction, were reviewed. Four patients demonstrated preoperative symptoms and objective findings indicative of increased intracranial pressure, including frequent headaches and emesis, papilledema, or digital markings on computed tomographic scan. Each of the 16 patients underwent either (1) single-stage total vault reconstruction with or without concomitant fronto-orbital expansion; (2) two-stage total vault reconstruction with anterior two-thirds vault expansion followed by transverse occipital expansion and recession a mean of 8.7 months later; or (3) anterior two-thirds vault reconstruction with or without fronto-orbital expansion. In each case, the extent of the scaphocephalic deformity determined the procedure used. The presence of severe frontal bossing associated with transverse restriction of the orbitotemporal region was an indication for fronto-orbital expansion in addition to vault reconstruction, whereas significant occipital protrusion was an indication for transverse posterior vault expansion and recession in addition to anterior two-thirds vault reconstruction. Excellent aesthetic results were obtained in all cases regardless of the type of reconstruction performed. However, it is essential that the extent of the deformity be carefully evaluated preoperatively to permit selection of the appropriate technique for reconstruction.

Algorithms↗
PubMed2003

Predicting the risk of reoperation in metopic synostosis: a quantitative CT scan analysis.

Children with metopic synostosis have a well-described clinical picture of trigonocephaly, often with hypotelorbitism. The craniofacial disorder itself is well recognized; however, objective prognostic factors for predicting the risk of reoperation are not well known. In 39 children with metopic synostosis, measurements of the cranial length, cranial width, anterior intercoronal distance, anterior interorbital distance (intercanthal distance), lateral orbital distance, and interzygomatic buttress distance were taken from preoperative computed tomographic (CT) scans and were normalized relative to each child's age. To separate overall facial hypoplasia from regional hypoplasia, a ratio of intercanthal distance to interzygomatic buttress distance was determined. These prognostic factors were analyzed with respect to reoperation rate. A stepwise logistic regression analysis was used to determine the interrelationships between the prognostic factors. Twenty-eight percent of the children underwent reoperation (N=11, 1 total reoperation and 10 minor recontouring). All of the reoperations occurred in children with a decreased intercanthal distance (p=0.30). The ratio of intercanthal distance to midfacial width was related to reoperation rate, with those children who had a ratio < or =0.80 having a reoperation rate of 44% (8 of 18 total children with an intercanthal-to-zygomatic ratio < or =0.8, p=0.07). This relationship was significant in children younger than the age of 12 months (6 of 13 total children with an intercanthal-to-zygomatic ratio < or =0.8, 46% reoperation rate, p=0.006). This study suggests that preoperative CT measurements can be used as a means of risk stratification in outcome analyses of the surgical treatment of craniosynostosis. In children treated for metopic suture synostosis, a foreshortened intercanthal distance compared with the interzygomatic buttress distance was related to reoperation rate, especially in children younger than 12 months of age.

Cranial Sutures↗
PubMed1981

Treatment of coronal and metopic synostosis: 107 cases.

This paper presents a personal series of 107 children with synostosis, 53 unicoronal, 27 bicoronal, and 28 metopic cases (1 child had both metopic and bicoronal synostosis). In 16 patients, other vault sutures later closed partially, some requiring operation. All of the patients underwent surgical treatment without mortality or notable morbidity. Nineteen patients showed mild or moderate mental retardation; other defects included cleft palate, epicanthic folds, strabismus, and syndactyly. Three patients with unicoronal closure had a second operation about 5 years after the initial craniectomy, and 2 required a third procedure. Cranioplasty was done in 10 patients for persisting skull defects. The recent results have been satisfactory in most cases, probably because of more precise diagnosis and improved surgical techniques. It is concluded that craniofacial operations for synostosis should be as extensive as necessary, and if subsequent revision or cranioplasty is appropriate it should be done.

Child, Preschool↗
PubMed1986

Surgical approaches for the correction of metopic synostosis.

Premature closure of the metopic suture results in a deformity ranging from a minor variation to a severe cosmetic deformity. The three principal abnormalities comprising metopic synostosis are trigonocephaly secondary to the restriction of growth of both frontal bones, deficient lateral supraorbital rims, and hypotelorism. Seventeen of 18 patients evaluated for metopic synostosis had surgical correction of their anomalies. For a minor degree of prominence at the metopic suture, a bicoronal flap followed by shaping at the suture with a shaping burr was sufficient and yielded favorable cosmetic results. A more extensive procedure for cosmetic resolution of trigonocephaly and hypotelorism was required for those patients with more pronounced deformities. In patients with moderate to severe metopic synostosis, the following procedures were important in achieving excellent cosmetic results: a bicoronal subgaleal flap down to the supraorbital rims with preservation of continuity of pericranium with an intact periorbita; bifrontal craniotomy with complete removal of the metopic suture; dural plication in midline to achieve an immediate aesthetically pleasing contour; removal or remodeling of the supraorbital rims and nasion with replacement of the remodeled frontal bone anteriorly in order to rebuild the orbital rim and release the supraorbital bar from the anterior cranial base; and securement of the bifrontal bones anteriorly and laterally, but not posteriorly, to allow further anterior and lateral displacement of the supralateral orbital margin.

Craniosynostoses↗
PubMed1994

Development of a strain of rabbits with congenital simple nonsyndromic coronal suture synostosis. Part I: Breeding demographics, inheritance pattern, and craniofacial anomalies.

The lack of an animal model of congenital coronal suture (CS) synostosis has prompted the widespread use of an experimental rabbit model using adhesive immobilization of the CS. Such postnatal models have helped make significant scientific contributions but may still not fully represent all aspects of the human congenital condition. In the March 1993 issue of The Cleft Palate-Craniofacial Journal we reported a female rabbit born in our laboratory with complete bilateral CS synostosis. This follow-up study presents our attempts to breed this animal and establish a strain of craniosynostotic rabbits. To date, we have accomplished 10 back- and intercrosses with these animals and have produced a total of 71 live offspring; 10 animals exhibited complete nonsyndromic unilateral (plagiocephalic) or bilateral (brachycephalic) CS synostotic deformities at birth, and 19 animals exhibited partial CS synostosis that showed more than 75% growth retardation across the CS (well below the 95% confidence interval for normals). Results revealed that gestational time and litter size averages were consistent with those reported for the strain, although the average litter size decreased with increased inbreeding. By 1.5 weeks of age the completely synostosed animals already exhibited brachycephalic cranial vaults and midfacial hypoplasia compared to unaffected siblings. Initial pedigree analysis suggested an autosomal dominant inheritance pattern with incomplete penetrance and variable expressivity. The development of such a congenital rabbit model may prove useful in helping to understand the etiopathogenesis of this condition in human populations.

Animals↗
PubMed2001

The faith of a coronal suture grafted onto midline synostosis inducing dura and deprived from tensile stress.

OBJECTIVE: To discuss possible reasons for the synostosis of a coronal suture that was transplanted onto synostosis inducing dura in a scaphocephalic human cranium. DESIGN: Case report. SETTING: Supraregional teaching hospital, center for craniofacial anomalies. PATIENT: A bathmocephalic boy, followed from age 7(1/2) to 26 months. INTERVENTION: Radical synostosectomy, radial osteotomies in the parietal bone with outward fracturing of the barrel staves, and left-sided coronal suture transplantation onto the midline was undertaken at the age of 11 months. METHODS: Computer tomography and clinical follow-up. RESULTS: The sutural graft, initially deprived from tensile stress and quickly exposed to the anomalous dura, turned synostotic in one year. CONCLUSIONS: Both cell signaling and biomechanical theories on calvarial morphogenesis, sutural development, and synostosis can apply. An animal experiment is recommended to test which hypothesis prevails.

Bone Screws↗