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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↗

Sagittal synostosis: results of surgical treatment in 210 patients.

From 1977 to 1996, 210 patients suffering from scaphocephaly, have been operated on in our institutions. The surgical technique depended on the patients age. Single wide sagittal synostectomy was performed in 155 babies aged less than 3 months, obtaining good cosmetic results. Patients older than 3 months needed more complex and aggressive procedures to achieve similar results. The conclusions is that sagittal suturectomy is the proper treatment in younger patients under 3 months of age, and that a greater effort must be made to diagnose and treat these patients early.

Anthropometry↗

Cranial reshaping employing distraction and contraction in the treatment of sagittal synostosis.

We treated four patients with scaphocephaly using a combination of distraction and contraction techniques and achieved satisfactory results. Radial osteotomies in the frontal and occipital bones flattened these abnormal bossing bones and accelerated the disappearance of bony bumps created by distraction. This technique facilitates the achievement of the desired shape of the skull through fine adjustments of the distraction and contraction devices.

Craniosynostoses↗

Effect of quadriceps exercise on synostosis following tibial osteotomy with internal fixation: a finite element simulation.

OBJECTIVE: To clarify the influence of quadriceps muscle exercises on the apposed bony surfaces of the tibia after a high tibial osteotomy. The study was designed so that the instability at the osteotomy could be evaluated by a finite element simulation. BACKGROUND: A detailed knowledge of the effects of quadriceps exercises on the healing tibia may lead to a solid rationale for exercise prescription. METHODS: Using a three-dimensional finite-element method, we calculated displacement at the site of surgical repair of a tibia with an osteotomy and fixation plate attached. Construction of the finite element model of the tibia and subsequent calculations were done with the aid of commercial software. Boundary and loading conditions were calculated for two situations: a straight leg raising exercise and a full knee extension beginning from 90 degrees of knee flexion. RESULTS: In the straight leg raising exercise, the surgically cut surfaces were apposed tightly, whether or not an external load was added. For the knee extension exercise, on the other hand, separation occurred when the lower leg began at a full vertical orientation of 90 degrees of knee flexion, but as the lower leg diverged from the vertical orientation, compression began to come into play and progressively increased. CONCLUSIONS: A position of 90 degrees of knee flexion with the lower leg vertical should be considered hazardous to the integrity of the surgically cut and apposed surfaces. RELEVANCE: Currently, the basis for deciding the timing and intensity of exercise is simply clinical experience. A more detailed knowledge of the effects of quadriceps exercise on the healing tibia may lead to a more solid rationale for exercise prescription in the postoperative care of a high tibial osteotomy.

Biomechanical Phenomena↗

Ocular abnormalities associated with unilateral coronal synostosis.

Forty-five patients with premature fusion of one coronal suture were treated between June 1985 and June 1991. Thirty-three patients were evaluated with pre- and postoperative photographs, computed tomographic scans, and serial radiographs and were assessed by the ophthalmological and plastic surgical services before and after surgery. The patients' mean age was 11.5 +/- 3.2 months at the time of surgery. Two patients (6%) were noted to have facial abnormalities preoperatively. Seventeen patients (52%) exhibited a disorder of extraocular muscle function preoperatively. After craniofacial repair, 21% of patients exhibited a distinct change from their preoperative ocular examination. A substantial majority of patients with premature fusion of the unilateral coronal suture (72%) will present with an abnormal examination postoperatively. In addition, a significant percentage of these patients will exhibit a change in ophthalmological status after craniofacial surgery; therefore, surgery on the extraocular musculature should, in general, be deferred until after bony repositioning.

Cohort Studies↗

Intracranial hypertension in a patient with craniofacial synostosis and patent sutures.

Craniosynostosis is often associated with restrictive cranial vault deformities, diminished intracranial volume, and intracranial hypertension. Advances in imaging techniques have provided a method of intracranial volume analysis that has demonstrated a more complicated relationship between craniofacial abnormalities and elevated intracranial pressures. Studies have confirmed a decrease in intracranial volume in the presence of craniosynostosis, but this association is not found in every patient. We report an unusual case of elevated intracranial pressure in a patient with the clinical stigmata of Crouzon's syndrome but with patent cranial vault sutures.

Cranial Sutures↗

Oblique facial clefting associated with unicoronal synostosis.

Oblique facial clefts are said to account for 0.25% of all clefts. Craniosynostosis is not known to be associated with oblique facial clefting, although craniofrontonasal dysplasia is well described. Craniosynostosis and oblique facial clefting presenting together in the same patient is a rare event. We present such a case, and detail the treatment plan to allow simultaneous correction of both deformities.

Child, Preschool↗

Complications associated with gradual cranial vault distraction osteogenesis for the treatment of craniofacial synostosis.

Distraction osteogenesis has become a standard technique for craniomaxillofacial reconstruction. The authors performed gradual cranial vault distraction osteogenesis in 19 patients with craniosynostosis to study the outcome and complications of this procedure. Postoperative infections developed around the shaft puncture wounds in four patients, including one who required surgical removal of the device. Advanced bone was deformed in one patient. In another, the expansion device was exposed, resulting in a postoperative scar. Despite these complications, the cranium was successfully expanded in all patients.

Child↗

Distraction osteogenesis of the cranial vault for the treatment of craniofacial synostosis.

From January 2000 to December 2001, six patients with craniosynostosis were treated. Involved sutures were coronal sutures in three patients, coronal and metopic sutures in one patient, multiple sutures (brachycephaly and oxycephaly) in one patient, and multiple sutures with a cloverleaf skull deformity in one patient. The age distribution of the patients was 4 months to 3 years. Four were male, and two were female. A frontal craniotomy was performed in four patients with brachycephaly. In one patient with brachycephaly, the osteotomies were made across the nasofrontal junction, across the roof of the orbit, and along the lateral orbital wall. In one patient with a cloverleaf skull deformity, a frontal bone osteotomy was first performed 1 cm above the roof of the orbit. A supraorbital frontal bar was then made across the nasofrontal junction, across the roof of the orbit, and down to the lateral orbital wall. The frontal bone flap was repositioned to the supraorbital bar using absorbable miniplates and screws. Distraction was started 3 to 7 days after the operation at a distraction rate of 1 mm/d. The real duration of the first operation was 90 to 120 minutes, and the second operation to remove the device took 40 to 50 minutes to perform. The distracted length was 15 to 25 mm. The consolidation period was 3 to 5 weeks. The follow-up period was 6 months to 1 year. Postoperative three-dimensional computed tomography demonstrated reossification at the bone flap and advancement of the fronto-orbital area. After surgery, the cranial volume increased 22.7% on average compared with before surgery. The mean ratio of the anteroposterior length to the transverse length of the cranial vault was changed from 0.96 before surgery to 1.04 after surgery. In conclusion, the advantages of distraction osteogenesis of the cranial vault are that it offers a less invasive technique, a shorter operation time, easy care, and postoperative safety as a result of minimal dissection of the dura. Disadvantages are the limited possibility of initial reshaping and the necessity of one more operation for device removal.

Cephalometry↗

Treatment of nonsyndromic bilateral coronal synostosis using a multiple bone flap rotation-reposition technique.

Brachycephaly is the result of premature fusion of the bilateral coronal suture. Various surgical procedures have been devised to manage brachycephaly, but there is no standard surgical method in brachycephalic treatment. Suboptimal results may be related to the tendency of misdirected bone growth of the remaining cranium in brachycephaly. Therefore, the abnormal growth vectors should be corrected toward a more normal configuration. Between 1997 and 2001, three nonsyndromic brachycephalic patients were treated using the described procedure. The cranial remodeling procedure used consists of supraorbital bar advancement and the rotation-reposition of multiple frontoparietal bone flaps. The frontoparietal bone was cut into seven segments consisting of two upper frontal, two lower frontal, one anterior parietal (T-shaped), and two posterior parietal segments. Each lower frontal segment was rotated 180 degrees and transposed with the corresponding contralateral upper frontal segment. Each upper frontal segment was then transposed to the upper margin of the supraorbital bar. The posterior margin of the frontal segment was not fixed (floating forehead). Anterior parietal and two posterior parietal segments were trimmed and repositioned to the original position. The postoperative follow-up period ranged from 17 to 52 months (mean = 30 months). The cranial configuration obtained after the operation was considerably improved, approaching a normal shape in all cases. The mean length of frontal advancement was 11 mm (range: 7-15 mm). The preoperative mean value of the cephalic index was markedly raised to 104.7 (range: 100-111). The postoperative (at last follow-up) mean value of the cephalic index was 89.2 (range: 86.3-94.3). A better skull contour could be obtained easily by converting abnormally directed long upward and transverse dimensions to short anteroposteriorly directed dimensions. Cranial remodeling using repositioned multiple bone flaps can produce excellent results functionally and esthetically in brachycephaly.

Cephalometry↗

Hybrid of distraction osteogenesis unilateral frontal distraction and supraorbital reshaping in correction of unilateral coronal synostosis.

Unicoronal synostotic plagiocephaly is routinely treated by intracranial wide frontal and bilateral supraorbital reshaping. Recent advancement of distraction osteogenesis in craniofacial surgery has extended to patients with craniosynostosis. Although a controversy remains between conventional osteotomy and reshaping and application of the distraction technique in surgical treatment of craniosynostosis, there have been several positive clinical reports on distraction techniques for nonsyndromic and syndromic craniosynostosis. Unicoronal distraction applied successfully to a case of frontal plagiocephaly has been described. The authors report a procedure: hybrid of unilateral frontal distraction and supraorbital reshaping on the affected side for frontal synostotic plagiocephaly. This procedure was conducted on four typical unicoronal synostotic plagiocephaly cases (patient age range, 9-14 months; all patients female) with successful results. No particular complications were encountered in any of the four cases with a follow-up period that ranged from 18 to 53 months.

Craniosynostoses↗

Early fibular bypass procedures (tibiofibular synostosis) for massive bone loss in war injuries.

The techniques of fibular bypass grafting for massive tibial bone loss have been well described over the past 50 years. These techniques have generally been used as salvage procedures in post-traumatic civilian patients with chronic sepsis, or for congenital pseudoarthrosis. We present the experience of the Orthopaedic Department of the Hadassah University Hospital in successfully treating massive tibial bone loss by early fibular bypass grafting in four war injuries, where such definitive procedures would have been avoided in the past.

Adolescent↗