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"Wrap around" pedicled muscle flaps for the treatment of recurrent forearm synostosis.

Three proximal and 2 mid shaft recurrent radioulnar synostoses were managed with a "wrap around" vascularized muscle interposition after excision of the heterotopic bone. A proximally pedicled brachioradialis flap was used for the proximal forearm and elbow synostoses, and the flexor carpi ulnaris muscle was used for the midshaft area. Anatomy of the flaps and the technical details of each procedure are thoroughly described. After a mean follow-up period of 8 years (range 3-14 years), there were no recurrences. The functional outcome regarding forearm rotation, elbow motion, and working capacity was highly satisfactory in all 5 patients.

Journal Article↗

Surgical treatment of sagittal synostosis.

A technique for the correction of scaphocephaly is described. It is safe, is technically straightforward, and rapidly corrects the deformity. The results of a standardized operative procedure in 50 patients are reviewed.

Cranial Sutures↗

Experiments of nature: premature unicoronal cranial synostosis in mantled howler monkeys (Alouatta palliata).

In 1960 Adolph Schultz described several cases of plagiocephaly in a collection of mantled howler monkeys (Alouatta palliata) from the forests of Central America. Since then several more specimens have been described. These individuals make up one of the largest samples of nonhuman primates that are affected by craniosynostosis. In this study, we used finite element scaling analysis (FESA) to quantify morphologic differences between adult normal (N = 19) and plagiocephalic female howler monkeys (N = 4). FESA utilizes three dimensional coordinate data to provide detailed information on size and shape differences local to biologic landmarks. The relatively large number of plagiocephalic howler monkeys provides a natural means for examining hypotheses concerning patterns of dysmorphology associated with plagiocephaly. Comparison of our results to observations from the clinical literature reveals similarities in neurocranial and facial morphology among plagiocephalic humans and howler monkeys, but the direction and magnitude of local form differences between howler monkeys and humans indicate species-specific responses to sutural constraint. Few cases of craniosynostosis in nonhuman primates are known. The number of plagiocephalic howler monkeys suggests these monkeys may be particularly susceptible to some defect that leads to premature suture closure. If this is the case, then howler monkeys may be a good model to study the cause(s) of craniosynostosis.

Alouatta↗

Multiple suture synostosis and increased intracranial pressure following repair of single suture, nonsyndromal craniosynostosis.

OBJECTIVE: Increased intracranial pressure, frequently associated with closure of multiple cranial sutures, has been reported to occur in 36% of cases following correction of syndromal craniosynostosis. Although much less common, multiple suture closure may occur following repair of single suture, nonsyndromal craniosynostosis and we present cases that concern two such children. RESULTS: Two children with nonsyndromal craniosynostosis, one metopic and one left-coronal, underwent fronto-orbital advancement at age 3 months. At age 19 months and at age 5 years, respectively, both patients re-presented with headaches, decrease in head circumference percentile, and acceptable cosmetic outcome. Both had computerized tomographic evidence of multiple closed cranial sutures and increased intracranial pressure (ICP) (determined by monitoring). Both patients improved following a cranial expansion procedure. CONCLUSION: Delayed closure of multiple sutures and resultant increased ICP may occur following correction of nonsyndromal, single suture craniosynostosis. This may be more likely when the initial suture is contiguous with the facial sutures. Children should be followed for many years following craniosynostosis repair with cranial, neurologic, and possibly funduscopic examinations as well as head circumference measurements to detect delayed closure of cranial sutures.

Age Factors↗

Craniofacial growth in premature craniofacial synostosis.

Serial cephalometric radiographs of 55 patients with different types of premature craniofacial synostoses were analysed. The aim of the study was to answer the following four questions in an effort to provide a better rational for classification, treatment, and possibly add insight into the mechanisms controlling craniofacial growth. (1) Is the shape of the skull syndrome specific. (2) How does the craniofacial complex grow. (3) What is the effect of craniectomy on the growth of the cranium. (4) Can roentgencephalometry provide a rational basis for planning reconstructive surgery. The results were presented as answers to each of the questions restated under each type of craniosynostosis. i.e. oxycephaly, scaphocephaly, plagiocephaly, trigonocephaly, Crouzon syndrome, and Apert syndrome.

Adult↗

Treatment of premature sutural synostosis.

Premature sutural synostoses are currently being treated at the Craniofacial Center at the Department of Plastic Surgery, University of Göteborg. A leading principle has been to release tethered parts of the skull and the facial skeleton in order to let the expanding brain mould the skeletal parts to an acceptable position. This can be achieved within the first year of life. When operating in later stages, the skeletal parts are wired in a semi-static position by a single osteosynthesis, still allowing expansion by the growing brain.

Craniofacial Dysostosis↗

Cranio-facial surgery for cranio-synostosis.

Cranio-facial principles have been applied to the treatment of craniosynostosis by the senior author since 1973, and original techniques have been developed for the treatment of oxycephaly, trigonocephaly, plagiocephaly brachycephaly and scaphocephaly; 156 patients have been operated upon. There is now enough follow-up material to establish that the good morphological results obtained initially are maintained through growth, and that there is a definite improvement in affected facial structures when early correction has been performed. The functional results have also been very satisfactory and reoperation for insufficient release has been rare. A multidisciplinary approach is highly recommended, and this type of surgery should be performed in a pediatric neuro-surgical environment.

Adolescent↗

Lambdoid synostosis and occipital plagiocephaly: clinical decision rules for surgical intervention.

Lambdoid craniosynostosis has been regarded as one of the least common categories of premature fusion of the cranial sutures, yet reports have suggested the incidence may be increasing. To guide treatment decisions, the authors describe a set of rules based on radiographic indicators and clinical assessment in the child. Experience suggests that children can have abnormal-appearing cranial sutures with normal neurological status and normal-appearing sutures with neurological deficits or marked cerebral compression. Early evaluation and follow-up treatment is essential for children with suspected craniosynostosis.

Journal Article↗

A new surgical approach to the treatment of coronal synostosis.

The authors described a surgical technique for the treatment of unilateral coronal synstosis, which they have carried out successfully in 16 children. Frontal craniotomy, removal of the lesser wing of the sphenoid, orbital unroofing, and removal of the entire orbital rim are recommended as essential elements of carnio-orbital reconstruction in the treatment of plagiocephaly.

Craniosynostoses↗

Multiple suture synostosis, macrocephaly, and intracranial hypertension in a child with alpha-D-mannosidase deficiency. Case report.

The authors present an unusual case in which increased intracranial pressure developed because of multiple-suture craniosynostosis and megaloencephaly in a child with a previously undiagnosed lysosomal storage disease, alpha-D-mannosidase deficiency. This 3-year-old boy presented with a history of frequent naps, headaches, florid papilledema, enlarged head (> 95th percentile), elevated opening pressure by lumbar puncture, a "beaten copper" appearance on skull radiographs, and no hydrocephalus. Multiple synostectomies were performed. Postoperatively, the child's headaches and papilledema resolved and his level of physical activity increased dramatically. The authors discuss the paradoxical presentation of prematurely fused sutures and macrocrania in light of this lysosomal storage disease and its subsequent management.

Child, Preschool↗