CT findings in complete premature craniosynostosis.
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Child, Preschool↗
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The frontoorbital advancement--on the use of 3D-CT and the miniplate osteosynthesis.
Between 1987 and 1991 we performed a unilateral or bilateral frontoorbital advancement to correct trigono- or plagiocephaly in 10 children: Three-dimensional CT provides an exact basis for operation planning. Titanium miniplates allow an already primarily rather stable osteosynthesis. The best time for this intervention is the end of the third month of life.
Bone Plates↗
Component bone marker displacements revealed by image-corrected cephalometric analysis. A lateral cephalometric study of the cranium in normal and craniosynostosis--an implant study.
Displacement of bony component anatomy has not been comprehensively described in human cranial development. In this study, tantalum implants were used to define cranial bone position on serial cephalometric surveys. Image correction (ICCA method) was used to eliminate artifactual shift of component markers before serial analysis was used to define implant movement. In addition, applicable normative standards were used to assess all case presentations. Three normal subjects comprised a normal mixed longitudinal sample aged 2 to 84 months. Two plagiocephaly subjects were studied, one from 6 to 77 months and the other from 16 to 44 months of age. Three syndromic craniosynostosis subjects demonstrated both abnormal and normalized growth following craniotomy, from 14 to 45, from 0.5 to 5.5, and from 2 to 75 months of age. A pattern of backward rotation of cranial component anatomy was observed in three normal subjects and two plagiocephaly subjects. The posterior fossa (PF) showed the greatest growth activity, with displacement adjustments throughout the study, and the anterior cranial fossa (ACF) least growth activity, with imperceptible frontal bone movement after age 3 years. After traditional bifrontal craniotomy, an abnormal displacement growth pattern was observed from age 14 to 45 months in the patient with syndromic craniosynostosis (Pfeiffer syndrome). Extensive fronto-parietal "bossing" and grossly deficient movement in the PF were observed. However, after a bifrontal craniotomy that also crossed lambdoid sutures, a normalized pattern of displacement growth was observed in two Apert syndrome patients. These two patients with extensive syndromic craniosynostosis had cranial component pattern adjustments as in the normal and plagiocephalic subjects.
Cephalometry↗
Metopic synostosis: in favour of a "simplified" surgical treatment.
Metopic synostosis is a relatively simple form of craniosynostosis, resulting from premature fusion of the metopic suture. In this pathology different degrees of dysmorphia of the anterior cranial fossa and the presence of associated anomalies of the skull might enable specific subgroups to be identified. Since most functional and cosmetic anomalies benefit from early surgical treatment, over the last few years neurosurgeons have been forced to elaborate less drastic, but nonetheless effective, surgical techniques. In the present report we analyze the surgical results obtained in a series of 62 infants with trigonocephaly operated on within their 1st year of life. Patients were subdivided into two groups (group I: 8 patients; group II: 54 patients) according to the specific dysmorphic characteristics of the frontal bone and anterior cranial fossa, and the presence of compensatory deformities affecting the anterior cranial base and temporo-parietal region. All the patients were treated using one of two relatively simple surgical techniques (procedure A: inversion of two hemifrontal bone flaps--48 cases; procedure B: the "shell" operation--14 cases). Both surgical procedures appeared to be effective, allowing adequate functional and cosmetic correction of the cranial deformity. In patients operated on following procedure B surgical time and blood loss were dramatically reduced. Long-term outcomes were satisfactory in all cases, irrespective of the surgical technique used. In the group II patients, however, progressive normalization of the interorbital distance was constantly observed, suggesting a different degree of stenotic involvement at the level of the anterior cranial base in these patients.
Adolescent↗
Consensus: trigonocephaly.
Trigonocephaly mostly occurs in isolated synostosis of the metopic suture, but 10-20% of patients are affected by complex syndromes, and in 2-5% the condition is familial. Intracranial hypertension has been observed in a minority of cases, but is never severe. Surgery is performed mainly for cosmetic reasons, following the standard principles of craniofacial surgery. Complications are rare and satisfactory cosmetic results are generally obtained. There is little likelihood for recurrent deformity.
Craniosynostoses↗
Procedure of lateral canthal advancement for the treatment of coronal synostosis.
The procedure of lateral canthal advancement has been used at the Hospital for Sick Children, Toronto, since 1973. The patients treated by this technique between 1973 and 1993 consisted of 41 with unilateral coronal synostosis, 13 with trigonocephaly and 29 with craniofacial dysostosis. The results have been excellent in patients with unilateral coronal synostosis and trigonocephaly. Furthermore, 17 of the infants with craniofacial dysostosis needed no further treatment after their initial operation.
Craniofacial Dysostosis↗
Ocular findings in children operated on for plagiocephaly and trigonocephaly.
Clinical examination of patients affected by plagiocephaly or trigonocephaly reveals evident malformation of the orbits, and the ocular repercussions are pronounced when children are operated on at an advanced age. Since it is generally accepted that binocular vision is fully developed by approximately 6 months of age, a late correction of plagiocephalic or trigonocephalic skull deformities may be an obstacle to the development of normal visual function. For the present report we investigated astigmatism and strabismus in 53 children, 39 of whom were operated on for plagiocephaly and 14 for trigonocephaly. Traction on the ocular globe induced by the bony deformation caused by the craniosynostosis may explain astigmatism and strabismus.
Astigmatism↗
Treatment of cloverleaf skull syndrome.
Up to a few years ago, patients with cloverleaf skull deformity underwent partial surgery to relieve intracranial hypertension with poor functional and aesthetic results, often leading to relapses and reoperations, both in our own experience and in that of other authors. As of 1990, however, we started to use the technique described by Persing et al. to resolve the complex cloverleaf malformation in a single definitive procedure. Five patients, whose ages ranged from 2 months to 5 years, achieved satisfactory results both as regards relief from intracranial hypertension and preservation of visual acuity, and from the aesthetic viewpoint. Surgical approaches and their modifications are described.
Cephalometry↗
Management of secondary turricephaly in craniofacial surgery.
In children with syndromic craniofacial disorders, such as Crouzon and Apert syndromes, who are managed surgically, a difficult problem that can occur is secondary turricephaly. One of the more widely accepted theories as to why this deformity occurs is that a lack of skull base growth results from fusion of the basal and facial sutures. Despite initial adequate forehead and orbital bandeau advancement, many of these patients require subsequent procedures, which do not always correct the characteristics deformity. We have identified a subset of 11 syndromic children who developed this characteristic deformity of turricephaly after primary reconstruction, 6 of whom required either secondary or tertiary procedures. Only 5 patients had a good outcome with a mean follow up of 4.5 years (range 1-8 years). Our surgical methods, and our rationale for the timing of surgery are discussed, and the literature on the management of this problem is reviewed.
Acrocephalosyndactylia↗
Lambdoid stenosis (posterior plagiocephaly) and craniofacial asymmetry: long-term outcomes.
We recently reviewed our series of craniofacial cases involving lambdoid stenosis (posterior plagiocephaly) and positional deformation. We now have 22 cases (who underwent surgery) with greater than 1 year follow up (range 1-7 years). We were impressed by the potential severity of the craniofacial deformity that can occur in what is often considered a positional deformation caused either by intrauterine conditions or by postnatal positioning. To decide which children were candidates for craniofacial reconstruction, we reviewed our cases and determined what we considered were appropriate criteria for craniofacial surgery. Among the 22 patients selected for surgery, 16 patients had changes in facial characteristics secondary to skull base and petrous ridge deformation, which often led to mandibular malalignment. In addition, 11 children were found to have developed scolosis of the face, similar to that seen in anterior plagiocephaly. The long-term severity of these selected craniofacial deformities may be markedly reduced if these children are operated on early with craniofacial reconstruction techniques. The removal of the lambdoid sutures, which appears to apply a twisting or torque effect on the facial structures and skull base, assisted in the children's further normal cranial development. In the children that were operated on before 1 year of age, most of the facial asymmetry was corrected. In addition, we observed that several of the children showed correction of the malignment of the ears, a common finding in these cases. Three-dimensional reconstructions have been extremely helpful in documenting the abnormalities of the skull base and sutural patterns and in planning the surgical approaches. All the patients had either a Marchac transposition or a bandeau/forehead type reconstruction. There were no long-term complications, and the esthetic results were considered good to excellent.
Cephalometry↗
Posterior skull surgery in craniosynostosis.
In 1984, two young infants with unusual "clover-leaf" patterns of skull deformity were treated by posterior skull-releasing surgery that dramatically improved their overall skull shape, to the extent that further operative intervention was not required. This focused our attention on the posterior skull and its role in craniosynostosis. In cases of multi-suture craniosynostosis and craniofacial syndromes severely raised intracranial pressure is frequent, demanding early surgery. One of the problems identified with such surgery undertaken before 6 months of age is recurrent craniosynostosis needing later re-operation. This occurred in 15 (5%) of 275 patients treated between 1978 and 1994. Since 1986, in the presence of significant raised intracranial pressure it has been our policy to do an initial posterior skull release or decompression. This takes the pressure of the growing brain away from the orbits, allowing us to defer fronto-orbital advancement until the age of 12 months or later. Three patients managed in this way completely avoided anterior surgery, while in another 9 patients re-operation for recurrent anterior deformity has not been required. The exception to this policy has been the presence of severe exorbitism posing a threat to vision. Under these circumstances early fronto-orbital advancement is mandatory, and an additional posterior skull release may be helpful later. Debate continues especially on the management of unilateral lambdoid synostosis. The recent increase in positional posterior plagiocephaly. possibly related to supine nursing of newborns, has emphasised the need to differentiate between a fixed deformity, which might require surgical correction, and positional moulding of the occiput, which improves spontaneously. This paper reports our experience with 22 patients treated by posterior skull surgery, either alone or as an additional procedure, which we believe has a definitive role in the management of craniosynostosis.
Adolescent↗
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↗
Terminal 7p deletion and 1;7 translocation associated with craniosynostosis.
A female infant with presumptive deletion of the 7p2 region and an unusual translocation between a part of the short arm of chromosome 1 and a deleted chromosome 7 is described. The patient showed congenital craniosynostosis of the coronal and metopic sutures; marked turricephaly; hypotelorism; deeply cleft palate; shallow orbits with prominent bulging eyes; a depressed nasal bridge; anteverted nostrils; short hands with broad thin fingers and elongated thumbs; a mild talipes calcaneovalgus deformity of the feet; a systolic murmur due to a small VSD; and psychomotor retardation. The child died of bronchopneumonia at 10 weeks of age. The parents are chromosomally normal.
Abnormalities, Multiple↗
Craniosynostosis in hyper-IgE-syndrome.
A 9-year-old boy with hyperimmunoglobulin-E-syndrome (HIE) and craniosynostosis is reported. Premature fusion of the sagittal and lambdoid suture led to scaphocephaly. A partial optic atrophy without clinical signs of raised intracranial pressure was observed. This is the fourth reported case of craniosynostosis in HIE. Bone anomalies like osteoporosis are frequent findings in HIE. Apart from their clinical impact they could be related to factors involved in the pathogenesis of HIE, such as impairment of chemotaxis in tissues or monocyte differentiation.
Child↗
Osteoglophonic dysplasia: a new case.
A new case of osteoglophonic dysplasia is described in a Portuguese boy. The clinical and radiographic features are similar to the five previously described cases. The boy died suddenly at 10 months of age.
Abnormalities, Multiple↗
Ear morphology in Treacher Collins', Apert's, and Crouzon's syndromes.
Size, proportions, level, inclination and shape of the ears were assessed by anthrompometric methods in eight patients with Treacher Collins' syndrome, eleven with Apert's, and 25 with Crouzon's syndrome. The ears were most severely damaged in Treacher Collins' syndrome, in which they were microtic or disproportionally long; many were low-set and with great inclination. All patients with Apert's syndrome had low-set ears and a tendency to disproportion, with widening and small inclination of the longitudinal axis. The ears were least affected in Crouzon's syndrome; in two-thirds of the patients there were mild growth variations leading to disproportion in width (wide ears), or low-set ears, or both.
Acrocephalosyndactylia↗
Studies of malformation syndromes of man XXXIX: a craniosynostosis-craniofacial dysostosis syndrome with mental retardation and other malformations: "craniofacial dyssynostosis".
We report clinical findings in 2 sisters and 5 sporadic cases with a "new" type of craniosynostosis/craniofacial dysostosis and shortness of stature. Premature closure of lambdoid sutures and posterior part of sagittal suture causes a posteriorly narrow, dolichocephalic skull with small, flat or bulging occiput and protuberance of the forehead; disturbance of the growth of basal skull structures leads to craniofacial dysostosis and (secondary) anomalies of the face. In one patient the coronal suture was also involved. One of the patients had a congenital heart defect. Four untreated patients had mental retardation; 3 had craniosynostectomy with more or less normal psychomotor development afterwards. Some patients had hydrocephalus and 1 had a brain malformation (agenesis of the corpus callosum with presumed interventricular lipoma). The observation of sisters with the same condition suggests autosomal recessive inheritance. This etiologic hypothesis is supported by the fact that 4 of 7 patients are of Spanish, Mexican, or Puerto Rican ancestry; this population probably has a rather high gene frequency and the trait should be relatively common in areas occupied by this population and their descendents. The condition has been designated craniofacial dyssynostosis.
Abnormalities, Multiple↗
Sagittal synostosis--clinical significance?
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Cerebral Palsy↗