• Journal of Korean Neurosurgical Society
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• v.51 no.1
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• pp.31-36
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• 2012

Objective : The purpose of this study was to elucidate the anatomical development of physiologic suture closure processes in infants using three dimensional reconstructed computed tomography (CT). Methods : A consecutive series of 243 infants under 12 months of age who underwent three dimensional CT were included in this study. Four major cranial sutures (sagittal, coronal, lambdoidal and metopic suture) were classified into four suture closure grades (grade 0=no closure along the whole length, grade 1=partial or intermittent closure, grade 2=complete closure with visible suture line, grade 3=complete fusion (ossification) without visible suture line), and measured for its closure degree (suture closure rates; defined as percentage of the length of closed suture line divided by the total length of suture line). Results : Suture closure grade under 12 months of age comprised of grade 0 (n=195, 80.2%), grade 1 (n=24, 9.9%) and grade 2 (n=24, 9.9%) in sagittal sutures, whereas in metopic sutures they were grade 0 (n=61, 25.1%), grade 1 (n=167, 68.7%), grade 2 (n=6, 24%) and grade 3 (n=9, 3.7%). Mean suture closure rates under 12 months of age was 58.8% in metopic sutures, followed by coronal (right : 43.8%, left : 41.1%), lambdoidal (right : 27.2%, left : 25.6%) and sagittal sutures (15.6%), respectively. Conclusion : These quantitative descriptions of cranial suture closure may help understand the process involved in the cranial development of Korean infants.

• Journal of Korean Neurosurgical Society
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• v.59 no.3
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• pp.192-196
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• 2016

Understanding the development of a skull deformity requires an understanding of the normal morphogenesis of the cranium. Craniosynostosis is the premature, pathologic ossification of one or more cranial sutures leading to skull deformities. A review of the English medical literature using textbooks and standard search engines was performed to gather information about the prenatal development and growth of the cranial vault of the neurocranium. A process of morphogenic sequencing begins during prenatal development and growth, continues postnatally, and contributes to the basis for the differential manner of growth of cranial vault bones. This improved knowledge might facilitate comprehension of the pathophysiology of craniosynostosis.

• Imaging Science in Dentistry
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• v.34 no.3
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• pp.165-169
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• 2004

Craniofacial dysostosis is considered to be one of rarely observed syndromes characterized by premature closing of all cranial sutures. The first patient was a 4-year-old male infant who had been complaining of empyema. Clinical findings showed exophthalmos, hypertelorism and facial asymmetry. Conventional radiographs demonstrated abscence of cranial sutures and underdeveloped maxilla. CT scan demonstrated the digital impressions of the inner surface of the cranial vault, enlarged and depressed sella turcica. The second patient was a 2-year-old female infant who had been complaining of facial deformity. Clinical findings showed hypertelorism and underdeveloped maxilla. Radiographs showed premature synostosis of all cranial sutures, depressed and enlarged sella turcica, and hypoplastic maxilla. 3 years after operation, her look improved. However, resurgery may be considered to decreasing intracranial pressure and for correction of facial deformity. Two interesting cases showing ‘cloverleaf’ skulls were presented.

• Archives of Plastic Surgery
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• v.32 no.1
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• pp.29-36
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• 2005

Craniosynostosis is the premature fusion of one or more sutures of either cranial vault or base. Fused sutures may impede normal growth of the calvaria, leading to characteristic skull deformities; Morphological craniosynostosis is classified descriptively. Being craniosynostosis uncorrected the deformity progresses continuously and causes an increase of intracranial pressure. The surgical involvement aims at the expansion of intracranial space as well as satisfactory achievement of craniofacial shape. Early surgical correction in infancy prevents the deformity from the further progression and possible associated complication of high intracranial pressure. A long period of follow-up is essential to asses the outcome of an effectiveness of the surgery. measurement of intracranial volume has been concerned in medical personnel and anthropologists for many years. A reliable and accurate measurements of the intracranial volume facilitates to make a diagnosis and treatment of craniosynostosis. Pre-and postoperative change of intracranial volume was evaluated with 3D CT scanning in 12 cases of craniosynostosis who underwent frontal advancement and total cranial vault remodeling. Increased intracranial volume is attributed to surgical release of craniosynostosis and natural growth. We conceive that the intracranial volume is significantly increased after surgical correction of fused cranial sutures and along with natural growing. A procedure of frontal advancement and total cranial vault remodeling is very useful to correct such a deformity as craniosynostosis. And also 2 cases out of five mentally retarded patients improved remarkably and Forehead retrusion or temporal depression followed in another two cases.

• Archives of Craniofacial Surgery
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• v.21 no.2
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• pp.106-108
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• 2020

Premature fusion of one or other of the minor sutures can subtly influence the shape of the human skull. Although infrequently reported or not clinically recognized, it can such contribute to a variety of craniofacial dysmorphisms. We herein report a case of late presenting, isolated bilateral synostosis of the squamosal suture dysmorphologies whose presentation mimics aspects of sagittal synostosis.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.2
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• pp.217-228
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• 2003

Co-ordinate growth of the brain and skull is achieved through a series of tissue interactions between the developing brain, the growing bones of the skull and the sutures that unite the bones. Craniosynostosis, the premature fusion of cranial sutures, presumably involves disturbance of these interactions. Bmp2, one of bone morphogenetic proteins (Bmps), is involved in the regulation of the shapes of individual bones and the relative proportions of the skeleton. Mutations in the homeobox gene Msx2, known as a downstream gene of Bmp, cause Boston-type human craniosynostosis. The phenotype of Dlx5 homozygote mutant mouse presents craniofacial abnormalities including a delayed ossification of calvarial bone. These facts suggest important roles of Bmp2, Msx2 and Dlx5 genes in the cranial bone growth and suture morphogenesis. To elucidate the function of these molecules in the early morphogenesis of mouse cranial sutures, we first analyzed by in situ hybridization the expression of Bmp2(E15-18), Msx2 and Dlx5 genes in the developing sagittal suture of calvaria during the embryonic stage. Bmp2 mRNA was intensely expressed in the osteogenic fronts and also at the low level in the periosteum of parietal bones during embryonic stage, Msx2 mRNA was intensely expressed in the sutural mesenchyme and mildly expressed in the dura mater during the embryonic stage. Dlx5 mRNA was intensely expressed osteogenic fronts and parietal bones. To further examine the role of Bmp signaling in cranial suture, we did in vitro experiments in E15.5 mouse calvarial explants. Interestingly, implantation of Bmp2-soaked beads onto the osteogenic fronts after 48 hours organ culture resulted in the increase of the tissue thickness and cell number around Bmp2 beads, compared to BSA control beads. In addition Bmp2 induced etopic expressions of Msx2 and Dlx5 genes. On the other hand, overexpression of FGF2 did not induce the expression of Msx2 and Dlx5. Taken together, these data indicate that Bmp2 signaling molecule has a important role in regulating the cranial bone growth and early morphogenesis of cranial suture. We also suggest that Bmp signaling is involved in all the stages of osteogenesis of cranial bones and the maintenance of cranial suture by regulating Msx2 and Dlx5 genes, and that Msx2 and Dlx5 genes are specific transcription factors of Bmp signaling pathway.

• Archives of Craniofacial Surgery
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• v.15 no.2
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• pp.47-52
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• 2014

Background: Management of positional plagiocephaly by wearing a cranial molding helmet has become a matter of growing medical interest. Some research studies reported that starting helmet therapy early (age 5 to 6 months) is important and leads to a significantly better outcome in a shorter treatment time. The aim of the present study was to evaluate the effectiveness of cranial remodeling treatment with wearing helmet for older infants (${\geq}18$ months). Methods: We conducted a retrospective study of 27 infants with positional plagiocephaly without synostosis, who were started from 2008 to 2012. Every child underwent a computerized tomography (CT) before starting helmet therapy to exclude synostosis of the cranial sutures and had CT performed once again after satisfactory completion of therapy. Anthropometric measurements were taken on using spreading calipers in every child. The treatment effect was compared using cranial vault asymmetry (CVA) and the cranial vault asymmetry index (CVAI), which were obtained from diagonal measurements before and after therapy. Results: The discrepancy of CVA and CVAI of all the patients significantly decreased after cranial molding helmet treatment in older infants (${\geq}18$ months) 7.6 mm from 15.6 mm to 8 mm and 4.51% from 9.42% to 4.91%. Six patients had confirmed successful outcome, and all subjects were good compliance patients. The treatment lasted an average of 16.4 months, was well tolerated, and had no complication. Additionally, the rate of the successful treatment (final CVA ${\leq}5mm$) significantly decreased when the wearing time per was shorter. Conclusion: This study showed that treatment by cranial remodeling orthosis was effective if the patient could wear the helmet longer and treatment duration was somewhat longer than in younger patients, well tolerated in older infants and had no morbidity. This therapeutic option is available and indicated in these older infants before other cranial remodeling surgery.

• Archives of Plastic Surgery
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• v.38 no.5
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• pp.683-686
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• 2011

Purpose: Craniosynostosis of three or more cranial sutures was not common. "Mercedes Benz pattern," named by Moore1 was a rare form of craniosynostosis and had an atypical pattern of premature closure of cranial suture. It was not reported in Republic of Korea. We report this case with literature review. Methods: A 13-months-old male patient visited our clinic due to exophthalmos. He showed normal developmental course. Other neurological tests were normal but he was Crouzon syndrome patient. CT scans showed bilateral lambdoid and posterior sagittal sutures were fused and the length of the skull was extended. Cranioplasty with pi craniotomy & Barrel-Stave osteotomy and recombination of the bone flap was performed. Results: The patient was discharged after post operative 10 days without any complications. In follow up visit after 2.7 years, he was in good state without recurrence and functional abnormality of skull. Conclusion: This was the first case of Mercedes Benz pattern craniosynostosis with Crouzon syndrome in Korea. This type of craniosynostosis has to be considered differently from single type of craniosynostosis or typical syndromic craniosynostosis clinically and surgically.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.4
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• pp.652-663
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• 1999

Craniosynostosis, the premature fusion of cranial sutures, presumably involves disturbance of the interactions between different tissues within the cranial sutures. Interestingly, point mutaions in the genes encoding for the fibroblast growth factor receptors(FGFRs), especially FGFR2, cause various types of human craniosynostosis syndromes. To elucidate the function of these genes in the early morphogenesis of mouse cranial sutures, we first analyzed by in situ hybridization the expression of FGFR2(BEK) and osteopontin, an early marker of osteogenic differentiation, in the sagittal suture of calvaria during embryonic(E15-E18) and postnatal stage(P1-P3). FGFR2(BEK) was intensely expressed in the osteogenic fronts, whose cells undergo differentiation into osteoprogenitor cells that ultimately lay down the bone matrix. Osteopontin was expressed throughout the parietal bones excluding the osteogenic fronts, the periphery of the parietal bones. To further examine the role of FGF-mediated FGFR signaling in cranial suture, we did in vitro experiments in E15.5 mouse calvarial explants. Interestingly, implantation of FGF2 soaked beads onto both the osteogenic fronts and mid-mesenchyme of sagittal suture after 36 hours organ culture resulted in the increase of the tissue thickness and cell number around FGF2 beads, moreover FGF4-soaked beads implanted onto the osteogenic fronts stimulated suture closure due to an accelerated bone growth, compared to FGF4 beads placed onto mid-mesenchyme of sagittal suture and BSA control beads. In addition FGF2 induced the ectopic expression of osteopontin and Msx1 genes. Taken together, these data indicate that FGF-mediated FGFR signaling has a important role in regulating the cranial bone growth and maintenance of cranial suture, and suggest that FGF-mediated FGFR signaling is involved in regulating the balance between the cell proliferation and differentiation through inducing the expression of osteopontin and Msx1 genes.

• Journal of Korean Neurosurgical Society
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• v.59 no.3
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• pp.187-191
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• 2016

Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures. It leads not only to secondary distortion of skull shape but to various complications including neurologic, ophthalmic and respiratory dysfunction. Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. Both environmental factors and genetic factors are associated with development of craniosynostosis. Nonsyndromic craniosynostosis accounts for more than 70% of all cases. Syndromic craniosynostosis with a certain genetic cause is more likely to involve multiple sutures or bilateral coronal sutures. FGFR2, FGFR3, FGFR1, TWIST1 and EFNB1 genes are major causative genes of genetic syndromes associated with craniosynostosis. Although most of syndromic craniosynostosis show autosomal dominant inheritance, approximately half of patients are de novo cases. Apert syndrome, Pfeiffer syndrome, Crouzon syndrome, and Antley-Bixler syndrome are related to mutations in FGFR family (especially in FGFR2), and mutations in FGFRs can be overlapped between different syndromes. Saethre-Chotzen syndrome, Muenke syndrome, and craniofrontonasal syndrome are representative disorders showing isolated coronal suture involvement. Compared to the other types of craniosynostosis, single gene mutations can be more frequently detected, in one-third of coronal synostosis patients. Molecular diagnosis can be helpful to provide adequate genetic counseling and guidance for patients with syndromic craniosynostosis.