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PubMed1994

[Cranial asymmetries. Reflexions on plagiocephalies. Premature sutural synostosis or extracranial origin?].

The authors relate a clinical and radiological (X Ray and TDM tridimensional) study with an anthropological (dry skulls) study of plagiocephaly. The aim is to find with the aid of a physical examination, some anatomic parameters which permit to differentiate cranial asymmetry with coronal premature synostotic suture from functional deformation with extracranial outset. The term plagiocephaly is used to define forehead asymmetry. It is necessary to study orbital rim, nasal root, malar eminence, ear and chin mid point position. The morphology of the skull is important to look: frontal flattering and also occipital flattering or bulging, contra-lateral frontal and pterional aera bulging. The authors conclude that a single parameter is necessary to differentiate the two kinds of plagiocephaly: the petrous bone position on the flattened frontal side: sagittalisation: fonctionnal plagiocephaly; frontalisation: synostotic plagiocephaly.

Cephalometry↗
PubMed1996

Plagiocephaly: differential diagnosis based on endocranial morphology.

Plagiocephaly is a descriptive term that connotes an asymmetrically oblique or twisted head. Such cranial dysmorphology has a number of etiologies, the most common of which are unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis. Use of the term plagiocephaly in the literature is often ambiguous in that at times it is used inclusively for all etiologies while at other times it is used exclusively as a synonym for unicoronal synostosis. Although differentiation by physical examination among unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis usually is possible for an experienced observer, inexperienced observers often have difficulty making an anatomically accurate diagnosis even with the assistance of conventional skull radiographs. High-resolution CT scans, including three-dimensional osseous surface re-formations, have become a standard element in the evaluation of craniofacial anomalies in many centers. We hypothesized that the three major etiologies of plagiocephaly could be unambiguously differentiated by means of endocranial three-dimensional CT osseous surface re-formations. Archival pretreatment CT data on 15 unicoronal synostosis, 4 unilambdoid synostosis, and 15 plagiocephaly without synostosis patients were reviewed to define, qualitatively and quantitatively, the characteristics of the endocranial base morphologies for each group; in addition to visual dysmorphology specific to each group, there was a statistically significant difference in the angle of deviation from the midlines of the anterior and posterior cranial fossae among unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis. Four radiologists experienced in reading images of craniofacial anomalies were oriented to the group characteristics and then instructed to perform differential diagnosis for each of the 34 patients using only the endocranial three-dimensional CT images. The raters were blind to all other clinical and diagnostic information. The raters correctly diagnosed unicoronal synostosis. Errors were made in differentiation of unilambdoid synostosis and plagiocephaly without synostosis. These errors resulted from the raters' reliance on image inspection rather than quantitation of anteroposterior fossae midline angulation. Such quantitation unambiguously differentiated between unilambdoid synostosis and plagiocephaly without synostosis in the "error" cases. The endocranial base dysmorphology of patients with plagiocephaly is etiology-specific for unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis. Three-dimensional CT endocranial base images can assist differential diagnosis of plagiocephaly.

Cephalometry↗
PubMed1999

Coronal suture response to distraction osteogenesis in rabbits with delayed-onset craniosynostosis.

Recent studies have identified a subpopulation of persons with craniosynostosis who exhibit progressive or delayed-onset synostosis and mild cranial vault deformities. These persons may be good candidates for nonextirpation distraction osteogenesis. The present studies were designed to determine force-displacement parameters and assess the effects of distraction osteogenesis on coronal suture growth and morphologic characteristics in a rabbit model with congenital, delayed-onset craniosynostosis. Data were collected from a total of 178 rabbits: 71 normal controls; 16 normal controls with distraction; 72 with delayed-onset coronal suture synostosis; and 19 with delayed-onset coronal suture synostosis and distraction. At 10 days of age, all rabbits had amalgam markers placed on both sides of the coronal suture. In the force-displacement study, force-displacement distractors were placed across the coronal suture and distracted acutely for 1.0 mm at 42 days of age. Force-displacement curves for the coronal suture were best described by a third-order polynomial regression equation for both normal and synostosed groups. Significant differences (P < 0.05) were found in the mean force necessary to distract a normal suture 1 mm in distance (13.72 kg) compared with a suture with delayed-onset synostosis (48.39 kg). A significant (P < 0.05) relationship was also found between the extent of synostosis and the distractive force in rabbits with delayed-onset synostosis. In the distraction study, internal distractors were fixed across the coronal suture at 25 days of age and percutaneously and intermittently activated at an average of 0.11 mm/day for 42 days (4.54 mm total). Serial radiographs were taken at 10, 25, 42, and 84 days of age. Results revealed that rabbits with delayed-onset synostosis and distraction had significantly (P < 0.01) more coronal suture growth rates compared with rabbits with delayed-onset synostosis and no distraction. Coronal sutures were harvested at 84 days of age for qualitative histologic examination. Normal, distracted coronal sutures showed widened sutural ligaments and thin, active osteogenic fronts. In contrast, distracted coronal sutures from rabbits with delayed-onset synostosis showed narrowed sutural ligaments, thickened and blunt osteogenic fronts, and increased collagen and bony matrix deposition compared with controls. Results suggest that distraction osteogenesis without corticotomy may be a treatment alternative in persons with progressive, delayed-onset synostosis. However, these preliminary data also suggest that distractive forces may accelerate or stimulate osteogenesis differentially in persons with craniosynostosis, possibly through an underlying genetic disorder of bone and cytokine regulation. These differential osteogenic responses to distraction, if validated clinically, will need to be taken into account when planning distraction rate and rhythm protocols for patients with craniosynostosis.

Age of Onset↗
PubMed1996

A long-term study of the donor-site ankle after vascularized fibula grafts in children.

We reviewed the long-term course of the donor-site ankle after vascularized fibula grafts in 13 children. The preventive and therapeutic effects of the tibio-fibular metaphyseal synostosis (T-F synostosis) against valgus ankle deformity, which is one of the postoperative donor-site problems, were evaluated based on three radiologic and clinical parameters. Thirteen patients were divided into two groups: patients with or without simultaneous T-F synostosis when the fibula was taken. Three patients underwent T-F synostosis secondarily after the development of the valgus deformity. Follow-up periods averaged 12.4 years. In the patients with primary T-F synostosis, valgus deformity was only observed in one case. No functional disorder of the ankle joints was observed after T-F synostosis. In the patients without T-F synostosis, all the patients younger than 8 years old showed valgus deformity, in which the tilting angle averaged 6.3 degrees. In the three patients who underwent T-F synostosis secondarily, the tilting angle normalized in cases in which the fixation was performed when the lateral wedging was in a mild stage. There was a statistically significant difference in valgus tilt angle between the two groups (with or without T-F synostosis).

Adolescent↗
PubMed2004

Intracranial pressure changes in craniosynostotic rabbits.

Cranial vault and brain deformities in individuals with craniosynostosis are thought to result, in part, from changes in intracranial pressure, but clinical findings are still inconclusive. The present study describes intracranial pressure changes in a rabbit model with naturally occurring, uncorrected coronal suture synostosis. Longitudinal and cross-sectional intracranial pressure data were collected from 241 New Zealand White rabbits, divided into four groups: normal controls (n = 81); rabbits with delayed-onset coronal suture synostosis (n = 78); rabbits with early-onset unilateral coronal suture synostosis (n = 32); and rabbits with early-onset bilateral coronal suture synostosis (n = 50). Epidural intracranial pressure measurements were obtained at 10, 25, 42, and 84 days of age using a NeuroMonitor microsensor transducer. Normal rabbits and rabbits with delayed-onset coronal suture and early-onset unilateral coronal suture synostosis showed a similar oscillating pattern of age-related changes in normal and head-down intracranial pressure from 10 to 84 days of age. In contrast, rabbits with early-onset bilateral coronal suture synostosis showed markedly elevated normal and head-down intracranial pressure levels from 10 to 25 days and showed a different pattern through 84 days. Results from one-way analysis of variance revealed significant (p < 0.01) group differences only at 25 days of age. Rabbits with early-onset bilateral coronal suture synostosis had significantly (p < 0.05) greater normal and head-down intracranial pressure (by 42 percent) than the other three groups. These results showed differing intracranial pressure compensations in rabbits with uncorrected multiple-suture synostosis compared with normal rabbits or rabbits with uncorrected single-suture synostosis, possibly through progressive cerebral atrophy and decreased intracranial volume, abnormal intracranial vascular patterns and blood volume, and/or differing cranial vault compensatory changes.

Age Factors↗
PubMed1998

Intracranial volume in craniosynostotic rabbits.

Although craniosynostosis alters brain growth direction resulting in compensatory changes in the neurocranium, it has been suggested that such compensations occur with little reduction in intracranial volume (ICV). This hypothesis was tested in a rabbit model with nonsyndromic, familial coronal suture synostosis. Cross-sectional three-dimensional computed tomographic head scans were obtained from 79 rabbits (25 normal, 28 with delayed-onset synostosis, and 26 with early-onset synostosis) at 25, 42, and 126 days of age. Intracranial contents were reconstructed and indirect ICV was calculated. Results revealed that by 25 days of age the intracranial contents from early-onset synostosed rabbit skulls showed rostral (anterior) constrictions and a "beaten copper" morphology in the parietal and temporal regions compared with the other two groups. These deformities increased in severity with age. Quantitatively, ICV was significantly reduced (P < 0.05) by 7% in rabbits with early-onset synostosis compared with both control rabbits and rabbits with delayed-onset synostosis at 25 days of age. By 126 days of age, ICV in rabbits with synostosis was significantly reduced (p < 0.05) by 11% in early-onset synostosis and by 8% in delayed-onset synostosis compared with normal rabbits. Results suggest that in rabbits with uncorrected craniosynostosis, compensatory changes in the neurocranium were not adequate to allow normal expansion of the neurocapsular matrix. Further research is needed to determine if ICV reduction is correlated with cerebral atrophy or cerebral spinal fluid (i.e., ventricular or subarachnoid) space compression in this model.

Age of Onset↗
PubMed1997

Age related changes in intracranial volume in rabbits with craniosynostosis.

Neurocapsular growth is highly heritable and determines neurocranial form. Although craniosynostosis alters brain growth direction, resulting in compensatory changes in the neurocranium, it is believed that such compensations occur without reduction in intracranial volume. This hypothesis was tested in a rabbit model with nonsyndromic, familial coronal suture synostosis. Skulls of 56 rabbits (20 normals, 20 with delayed onset synostosis, and 16 with complete synostosis) were scanned using three-dimensional computed tomography at 6 and 18 weeks of age. Intracranial contents were reconstructed, and indirect intracranial volume was calculated. Qualitatively, re-formations of intracranial contents from completely synostosed rabbit skulls exhibited the typical "copper beaten" morphology. Quantitatively, intracranial volume was significantly (p < 0.05) reduced in rabbit skulls with complete synostosis compared with both control rabbit skulls and rabbit skulls with delayed onset synostosis at 6 weeks by 11 percent and 14 percent, respectively). By 18 weeks, intracranial volume in rabbit skulls with synostosis was significantly (p < 0.05) reduced (by 12 percent in complete synostosis and 8 percent in delayed onset synostosis) compared with normal rabbits. Results suggest that in rabbits with uncorrected craniosynostosis, compensatory changes in the neurocranium were not adequate to allow normal expansion of the neurocapsular matrix. Further research is needed to determine whether reduction in intracranial volume was a result of neural tissue deficiency or cerebrospinal fluid (i.e., ventricular or subarachnoid) space compression in this model.

Aging↗
PubMed2004

Coronal ring involvement in patients treated for unilateral coronal craniosynostosis.

The etiopathology of the clinical entity normally referred to as unilateral coronal synostosis is commonly used to connote unilateral fusion of the frontoparietal suture. However, other sutures in the coronal ring may exhibit synostosis concomitant with or independent from frontoparietal synostosis and give rise to similar clinical phenotypes. This study retrospectively analyzes high-resolution computed tomographic data sets to determine patency of sutures within the coronal ring. Computed tomographic scan digital data from 33 infants who subsequently underwent surgical correction of unilateral coronal synostosis were assessed for sutural patency using Analyze imaging software. The frontosphenoidal suture was subdivided into intraorbital frontosphenoidal and extraorbital frontosphenoidal portions, and the patency of the frontoethmoidal suture was also assessed. Patients were sorted into two groups on the basis of the status of their frontosphenoidal sutures: group 1 had patent frontosphenoidal but synostotic frontoparietal sutures (n = 21) and group 2 had both frontosphenoidal and frontoparietal synostoses. Observer reproducibility was tested. The vertical and horizontal dimensions of the bony orbit and the endocranial base deflection angle were measured with the observer blinded with regard to sutural status group. Frontoethmoidal synostosis was not noted in any patients in either group. Two patients had no frontoparietal suture synostosis with isolated intraorbital frontosphenoidal and extraorbital frontosphenoidal suture closures. Suture diagnosis reproducibility was 99 percent. In group 1, the ipsilateral-to-contralateral vertical orbit dimension ratio averaged 1.11, whereas in group 2 it averaged 1.04 (p < 0.05). The ratio of horizontal orbit measurements was not significantly different between groups. In both groups, the endocranial base was deflected ipsilateral to the synostotic frontoparietal suture, with an average angle of 12 degrees in group 1 and 17 degrees in group 2 (p < 0.005). The extent of synostosis along the coronal sutural ring contributes to the dysmorphology of the orbit and the endocranial base deflection in patients whose clinical phenotypic diagnosis is unilateral coronal synostosis.

Child, Preschool↗
PubMed1997

Diagnosis and management of posterior plagiocephaly.

OBJECTIVE: The management of infants with posterior plagiocephaly has been controversial both because of widely differing estimates in the literature of the relative frequencies of true lambdoidal synostosis vs positional molding and because of divergent approaches to treating this problem in different institutions. Based on our experience, we hypothesized that the vast majority of children with posterior plagiocephaly did not have true synostosis and that the cosmetic impairment in such patients could be effectively treated with nonsurgical modalities. METHODS: Between 1992 and 1995, we prospectively applied in 71 infants a consistent management philosophy for these malformations that has incorporated a detailed evaluation of sutural anatomy as the basis for a physiologic approach to treatment. This approach has been directed at distinguishing true synostosis from deformational plagiocephaly and at avoiding surgery for patients with deformational abnormalities by using a combination of nonsurgical modalities to restore normal cranial growth dynamics. All children first underwent skull radiographs to determine whether the lambdoidal sutures were patent. In equivocal cases, computed tomography was also performed. Patients without true synostosis were enrolled on a course of positional therapy. In patients that did not improve after 2 to 3 months, a custom-fitted orthoplastic molding helmet was applied to facilitate passive skull recontouring. RESULTS: Forty children had patent sutures based on skull radiographs, and 29 others, in whom the radiographs were equivocal, had open sutures based on computed tomography, thus establishing the diagnosis of deformational plagiocephaly in 69. Predisposing factors for this deformity included a strong positioning preference during early infancy (n = 67), torticollis (n = 10), prematurity (n = 6), and developmental delay (n = 2). Only two patients had true lambdoidal synostosis; in each case, this was associated with synostosis of the posterior sagittal suture and was managed effectively with cranial reconstructive surgery. Thirty-five patients with deformational plagiocephaly had a dramatic improvement in their cranial contour with positional therapy alone; 34 patients failed to improve and were treated with molding helmets. All but five children, each of whom was more than 6 months old at initial intervention (P < .025), developed a normal or nearly normal head shape with these measures. CONCLUSION: The vast majority of children with posterior plagiocephaly do not have true synostosis and can be effectively managed by nonsurgical means. The impact of positional preference on the development of this process is discussed.

Craniofacial Abnormalities↗
PubMed1998

The differential diagnosis of abnormal head shapes: separating craniosynostosis from positional deformities and normal variants.

The correct differential diagnosis of an abnormal head shape in an infant or a child is vital to the management of this common condition. Establishing the presence of craniosynostosis, which warrants surgical correction, versus non-synostotic causes of head deformity, which do not, is not always straightforward. This paper deals with three groups of abnormal head shape that may cause diagnostic confusion: the spectrum of metopic synostosis; the dolichocephaly of prematurity versus sagittal synostosis; and the differential diagnosis of plagiocephaly. Special emphasis has been placed on the problem of posterior plagiocephaly, in the light of recent evidence demonstrating that lambdoid synostosis has been overdiagnosed. Metopic synostosis presents as a wide spectrum of severity. Although only severe forms of the disorder are corrected surgically, all cases should be monitored for evidence of developmental problems. The dolichocephalic head shape of preterm infants is non-synostotic in origin and is managed nonsurgically. The scaphocephalic head shape resulting from sagittal synostosis requires surgical intervention for correction. Posterior plagiocephaly may be due to unilambdoid synostosis or positional molding, which have very different clinical and imaging features. True lambdoid synostosis is rare. Most cases of posterior plagiocephaly are due to positional molding, which can usually be managed nonsurgically. Regardless of the suture(s) involved, all children with confirmed craniosynostosis should be monitored for increased intracranial pressure and developmental problems.

Bone Diseases↗
PubMed2001

Perioperative transcranial Doppler US to evaluate intracranial compliance in young children undergoing craniosynostosis repair surgery.

PURPOSE: To determine whether measurements with transcranial Doppler ultrasonography (US) of resistive indexes (RIs) of basal cerebral arteries with pressure provocation can be used to identify infants and children with craniosynostosis who have abnormal intracranial compliance and to study the effects of surgery on compliance. MATERIALS AND METHODS: Transcranial Doppler US was performed through the temporal squama, fontanels, and existing skull defects prior to and immediately following cranioplasty. Twenty-four studies were performed in six patients with multisuture synostosis, and 61 studies were performed in 26 patients with single-suture synostosis. Study findings were compared with those of 23 control subjects and were characterized as normal or abnormal on the basis of age-specific normal criteria for RI. RESULTS: In multisuture synostosis, results of six of the nine preoperative transcranial Doppler US studies were abnormal. During postoperative follow-up, three recurrences requiring reoperation occurred, one of which was detected with abnormal transcranial Doppler US findings. In single-suture synostosis, results of seven of the 26 preoperative transcranial Doppler US studies were abnormal, and all occurred in young infants with sagittal and unicoronal synostosis. Immediate effects of surgery were variable. All patients with sagittal synostosis had a significant immediate postoperative increase in RI, which normalized during postoperative follow-up. There was no significant difference in RI between patients with successfully treated craniosynostosis and control subjects. CONCLUSION: Transcranial Doppler US can be used to identify patients with craniosynostosis with decreased intracranial compliance, and it is a suitable noninvasive test to monitor the effects of surgery on compliance.

Child, Preschool↗
PubMed1997

Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients.

OBJECTIVE: Surgery for craniosynostosis has evolved rapidly over the past two decades, with increased emphasis on early, extensive operations. Older published series may not accurately reflect more recent experience. Our study was designed to analyze outcome in a large series of consecutive patients treated recently at a single center. METHODS: We reviewed 250 consecutive patients who underwent surgical treatment of craniosynostosis between January 1, 1987 and December 31, 1992. They were divided into nine groups by suture involvement: sagittal, unilateral coronal, bilateral coronal, unilateral lambdoid, bilateral lambdoid, metopic, multiple suture, the Klee-blattschädel deformity (cloverleaf skull), and acquired craniosynostosis. Outcome was analyzed in terms of residual deformities and irregularities, complications, mortality, as well as the need for additional surgery. RESULTS: There were 157 males (62. 8%) and 93 females (37.2%), with most of the male preponderance accounted for by the large sagittal synostosis group, which consisted of 82 males and 25 females. Median age at first operation was 147 days. A named syndrome was present in 23 patients (9.2%) and was more common than expected with bilateral and unilateral coronal synostosis, the Kleeblattschädel deformity, and multiple suture synostosis. There were two deaths (0.8%), both with Klee-blattschädel patients, and 17 other complications (6.8%). Morbidity and mortality were significantly associated with secondary vs primary operations and syndromic vs nonsyndromic patients. Outcome analysis revealed the best surgical results with metopic synostosis and significantly less good results with the Kleeblattschädel deformity, multiple suture synostosis, and bilateral coronal synostosis. CONCLUSIONS: Using modern surgical techniques, craniosynostosis can be corrected with good outcomes and relatively low morbidity and mortality, particularly for otherwise healthy, nonsyndromic infants.

Craniosynostoses↗
PubMedDate not supplied

Positional changes of the frontoparietal ossification centers in perinatal craniosynostotic rabbits.

It has been suggested that craniosynostosis is caused by abnormally located ossification centers (i.e., bony tubers) in the developing skull prior to suture formation [Mathijssen et al., 1996, 1997]. The present study was designed to test this hypothesis in a rabbit model of human familial, nonsyndromic coronal suture (CS) synostosis. Calvariae were taken from 99 New Zealand White rabbit perinates (55 normal controls, 15 with delayed-onset CS synostosis, and 29 with bilateral or unilateral CS synostosis), ranging in age from 23 to 34 days postconception (synostosis occurs at approximately 23 days in this model). Frontoparietal, interfrontal, and interparietal ossification center distances were obtained using a Wild microscope with camera lucida attachment and a 2-D computer digitization technique. Linear regression analysis was used to compare age-related changes in the perinatal ossification centers among groups. Results revealed that frontoparietal ossification center regression line slopes had similar start points (24-day intercepts) with significantly (P < 0.05) diverging slopes over time. Normal and delayed-onset ossification center distance increased more rapidly than in synostosed perinates. No significant (P > 0.05) differences were noted in regression line slopes among groups for interparietal or interfrontal ossification center distances. Results demonstrated that, in synostosed perinates, frontoparietal ossification center location was similar to normals around the time of synostosis and became displaced later. These findings suggest that ossification center (i.e., bony tuber) displacement seen in infants with craniosynostosis is probably a secondary and compensatory, postsynostotic change and not a primary causal factor of synostosis in this rabbit model.

Animals↗
PubMed2000

Update on craniofacial surgery: the differential diagnosis of lambdoid synostosis/posterior plagiocephaly.

There has been an evolution of thought on the diagnosis and treatment of posterior plagiocephaly. Synostotic posterior plagiocephaly (lambdoid synostosis) can be diagnosed and differentiated from non-synostotic posterior plagiocephaly (positional molding) based on specific cosmetic and radiologic criteria. The advent of high-resolution three-dimensional CT analysis of cranial morphology combined with meticulous clinical studies of the two major causes of posterior plagiocephaly has added much to our understanding of this skull deformity. Children with lambdoid synostosis have a trapezoid-shaped skull with posterior displacement of their ipsilateral ear, ipsilateral mastoid bossing, contralateral occipital bossing, and a fused lambdoid suture that appears as a ridge. Children with posterior plagiocephaly without lambdoid synostosis have a characteristic parallelogram-shaped skull with anterior displacement of the ipsilateral ear and ipsilateral frontal bossing. This subject is surrounded in controversy because many of the children in the past thought to have lambdoid synostosis probably did not, based in part on the aforementioned specific criteria. This is an important point, as most patients with posterior plagiocephaly without synostosis will improve without surgery. One should thus be appropriately conservative in the selection of patients for surgery. The majority of infants evaluated at craniofacial clinics presumably have posterior plagiocephaly without synostosis and can be successfully treated with frequent head turning, helmet, or band therapy. Patients with clinically and radiologically proven synostotic posterior plagiocephaly and a severe deformity should undergo craniofacial surgery. The technique of biparieto-occipital craniotomy is safe, simple, and delivers a good cosmetic result. Our postoperative photographic evaluations have shown an immediate, aesthetically pleasing change in the contour of the occiput, which tends to improve with time.

Craniofacial Abnormalities↗
PubMed1986

[Morphology and classification of cleft hands].

It is the intention of this study to present a more profound investigation of the morphology of cleft hands and to provide a new classification based on the results of that investigation. After a short review of the literature, which shows the different opinions regarding heredity, pathogenesis and classification of cleft hand, the authors demonstrate their own patients with 35 cleft hands: The deformities were mostly bilateral and associated with cleft feet. In unilateral cases the right side was more common. Males were in the majority. This paper puts emphasis on the analysis of X-ray morphology. The authors are able to demonstrate that the cleft hand shows several peculiarities which have not been yet sufficiently respected. It was found out, that, apart from aplasia of the bones and soft tissue, synostosis is often the origin of clefting. In 40% of our cases the cleft was caused exclusively by synostosis, in a further 34% it derived partly from synostosis of the phalanges and the metacarpal bones. In the carpus we found osseous deformities surprisingly often, a feature which has hardly been mentioned in former studies. Among the numerous associated malformations emphasis must be placed on the osseous syndactylies and the central polydactylies, because they are closely related to the cleft hand as shown by Ogino. 18 of our own cases belong to this group. Our investigations lead us to the following classification: Cleft hand type 1: Cleft hands with osseous defects (aplasias) Cleft hand type 2: Cleft hands with synostosis Cleft hand type 3: Cleft hands with aplasias and synostosis Hands with central polydactyly and synostosis as preforms of the cleft hand could be classified in type 4. These phenomena form the beginning of the teratological row towards the completely developed cleft hand. In combination with Blauth's distribution of cleft hands, who distinguished the median and medio-lateral form (1976, 1978) this new classification enables each cleft hand to be placed into one of the different types, which are analysed: Type 1 mostly shows a medio-lateral form, is always combined with cleft feet and shows heredity in 50% of the cases. It cannot be classified by the Ogino method. Type 2 mostly shows a median form, is not frequently combined with cleft feet, heredity occurs in one third of the cases. This type can very often be classified by the Ogino method. Type 3 varies from case to case because of the different items of defects.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed2000

Occipital plagiocephaly.

The diagnosis of 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 management and treatment for 'posterior plagiocephaly', 'plagiocephaly without synostosis', 'deformational plagiocephaly' and 'occipital plagiocephaly', with surgical 'correction' being chosen as the primary modality of treatment irrespective of the patency status of the lambdoid sutures. Two hundred and four patients with unilateral occipital plagiocephaly have been seen at the Australian Craniofacial Unit over the past 16 years. Each patient was evaluated by a craniofacial surgeon, paediatric neurosurgeon and paediatric geneticist. All children underwent plain radiographs of the skull to define the sutural anatomy. In those patients where the sutural anatomy was equivocal, 2-D and 3-D CT scans were performed. Only two of the 204 patients (approximately 1%) manifested the clinical, radiographic and pathological features of true unilambdoid synostosis. There was radiographic evidence of sutural fusion on plain films, 2-D and 3-D CT scans. Pathology specimens showed bony sutural fusion. Two hundred and two patients presented with unilateral occipital deformities and patent sutures on radiography. These patients with occipital plagiocephaly in the absence of true synostosis were initially managed conservatively (head positioning, and physiotherapy in those patients with torticollis). Those patients who underwent surgical correction in infancy (21/204) included patients with severe plagiocephaly not responding to conservative therapy (19/204) and the two patients with true unilambdoid synostosis (2/204).One hundred and ninety-one of the total patients (94%) were noted by their parents to have acceptable improvement in their head shape. Thirteen patients were seen within the past year and are too early to assess. Two surgical patients (one fronto-orbital advancement, one occipital craniectomy) and one patient followed conservatively were judged by their parents to be without notable improvement. In our series it is apparent that the majority of cases of occipital plagiocephaly are not secondary to true synostosis and can be managed by conservative positional measures.

Cranial Sutures↗
PubMed2002

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

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

Craniosynostoses↗