1/6. Cranial vault distraction: its illusionary effect and limitation.BACKGROUND: Distraction osteogenesis has some advantages, such as less hazardous complications, less scarring, and fewer bone defects. However, it has not been fully accepted yet because of a unidirectional expansion along the distraction device. Because cranial expansion is limited by scalp tension and soft-tissue scarring, undercorrection of the cranium and relapse of the vault deformities have occasionally been seen on long-term follow-up. These patients also had so much bone defect that the donor bone was inadequate for immediate revisions, and dissection under the scalp was complicated. methods: The authors used distraction osteogenesis to treat 12 cases of craniosynostosis. Five patients were syndromic (two cases of Pfeiffer's syndrome, two cases of Crouzon's disease, and one case of Apert's syndrome), and seven were nonsyndromic (four cases of brachycephaly, two cases of scaphocephaly, and one case of plagiocephaly). RESULTS: After only unidirectional expansion without vertical reduction, the shape of the cranium was satisfactorily improved, which might have been an illusion caused by the relative reduction attributed to the elongation by the distraction osteogenesis and, in brachycephaly, alteration of the patient's head position resulting from inclination of the facial plane by forehead advancement. However, in scaphocephaly, the unique deformity in the occipital and frontal regions remained after simple distraction. Distraction osteogenesis should be applied for a narrow cranium because the bone defect and scalp closure could pose problems. Radical reshaping should be used in cases of sufficient donor bone and scalp closure. Although additional surgery is unavoidable for device removal, minor revisions can be performed for revision of the irregularity at that time. CONCLUSION: Except for prolonged treatment, based on minimally invasive operations, distraction cranioplasty might be applied extensively in cases of craniosynostosis.- - - - - - - - - - ranking = 1keywords = plagiocephaly (Clic here for more details about this article) |
2/6. Experience of surgical treatment for craniofrontonasal dysplasia.We present a case of a 5-year-old girl diagnosed as having craniofrontonasal dysplasia (CFND), which was first reported by Cohen in 1979. CFND is very rare and reports concerning this syndrome have never been found in japan. In our case, frontal plagiocephaly, third degree orbital hypertelorism and clefting nasal tip coexisted. At 10 months after birth when she visited our hospital, signs of craniostenosis were not recognized and cosmetic improvement was considered the main purpose of the treatment. We performed supraorbital bar reshaping, ethmoidectomy and orbitotomy for reconstruction in one-stage at the age of five. The operation produced marked improvement in her exotropia before entering a primary school. Past reports dealing with abnormalities of the central nervous system in this syndrome are few, but agenesis of the splenium was noted in our case. This paper is a report of our findings together with some discussions in reference to the literature.- - - - - - - - - - ranking = 1keywords = plagiocephaly (Clic here for more details about this article) |
3/6. Cephalometric radiography and computed tomography in infants undergoing major craniofacial surgery--a comparison.Craniofacial growth after surgery in children is not completely understood. We have therefore formed a programme for pre- and postoperative studies with both roentgencephalometric- and computed tomographic investigations. This regimen results in overlapping information. Thus, an assessment of the advantages and disadvantages of the two methods was made and exemplified in four patients. In our experience both cephalometry and CT are essential for the diagnosis, surgical planning and follow-up of surgically treated children with craniofacial anomalies. To minimize the radiation doses and the diagnostic procedures which in these cases often includes general anesthesia, patients with isolated, asymmetrical conditions such as plagiocephaly, might be followed with CT only, since roentgencephalometry can not add much information.- - - - - - - - - - ranking = 1keywords = plagiocephaly (Clic here for more details about this article) |
4/6. Craniofacial growth in plagiocephaly and Crouzon syndrome.Very little information is available in the literature regarding the morphology and growth of the cranial base, the orbits and the jaws, in patients with premature craniosynostosis. The present report deals with the craniofacial morphology and growth in plagiocephaly and Crouzon syndrome. For each anomaly a dry skull was compared with a clinical case followed longitudinally with roentgencephalometry. Metallic implants in the jaws were employed in one case. Cranial base involvement was demonstrated for both types of premature craniosynostosis. In Crouzon syndrome the cartilagenous cranial base was affected. Marked compensatory and dysplastic growth changes were observed in the facial skeleton in both anomalies. The methodological problems related to roentgencephalometric examination of patients with plagiocephaly are discussed, and a roetgencephalometric method for evaluation of exophthalmos in patients with Crouzon syndrome is presented.- - - - - - - - - - ranking = 6keywords = plagiocephaly (Clic here for more details about this article) |
5/6. 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.- - - - - - - - - - ranking = 1keywords = plagiocephaly (Clic here for more details about this article) |
6/6. A novel mutation (a886g) in exon 5 of FGFR2 in members of a family with Crouzon phenotype and plagiocephaly.We identified a novel mutation in members of a family with signs of Crouzon syndrome and plagiocephaly. In affected members of the family an A-->G transition was found at position 886 in exon 5 of the fibroblast growth factor receptor 2 (FGFR2) gene. The base change results in the replacement of a lysine by glutamic acid in Ig-like loop III of FGFR2. The unusual finding of plagiocephaly in these Crouzon patients may either be the result of the type of mutation or because of genetic and environmental factors that affect the phenotype in addition to the mutated FGF receptor.- - - - - - - - - - ranking = 6keywords = plagiocephaly (Clic here for more details about this article) |