• Archives of Craniofacial Surgery
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• v.9 no.2
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• pp.85-89
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• 2008

Purpose: Unicoronal synostosis is the craniofacial anomaly caused by premature fusion of unilateral coronal suture. Ipsilateral flattening of the frontal and parietal bones, temporal retrusion with elevation and recession of the supraorbital rim are main clinical features. Compensatory contralateral frontal bossing and deviation of the nasal root and/or chin can also occur. There is a controversy about techniques for surgical correction, however, bilateral approach technique is more effective for correction of deformity. Methods: A 4-year-old patient with unicoronal synostosis had undergone unilateral suturectomy at 28-month-old but fronto-facial deformity had remained and aggravated as she grew older. She had both fronto-facial and endocranial asymmetry. We performed coronal cranial approach and fully exposed affected cranium including supraorbital rim. Anterior 2/3 calvarial reconstruction with bilateral frontal bone osteotomy and fronto-orbital bandeau advancement was performed. Results: Fronto-facial symmetry including fronto-orbital contour, nasal devation was improved. Endocranial twisting was also improved from $158^{\circ}$ to $162^{\circ}$ in CSO(crista gallisella turcica-opisthion) degree. There was no postoperative complications and no need for revision, and facial asymmetry improved at the period of 2 years of follow-up. Conclusion: Bilateral approach with fronto-orbital bandeau remodeling in surgery of unicoronal synostosis looked superior to unilateral approach in achieving better symmetry and preventing recurrence of asymmetry. Remodeling surgery should be tried in patients even at an older age to correct fronto-facial asymmetry.

• Archives of Craniofacial Surgery
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• v.9 no.2
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• pp.105-109
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• 2008

Temporomandibular joint(TMJ) and dental occlusion are important in functional aspect as well as in cosmetic aspect for craniofacial surgery such as orthognathic and traumatic surgery. During these operations, appropriate maxillomandibular fixation(MMF) is especially necessary to conserve or reconstruct, dynamic TMJ and functional occlusion. Arch bar is one of the most popular method to gain proper MMF. Seventeen patients including 5 patients with mandibular fracture, 12 patients with orthognathic surgery(6 patients had relatively normal occlusion, however 6 patients had facial anomalies such as hemifacial microsomia with irregular occlusal plane.) were joined in this study. Arch bar was contoured on the dental cast, which was prepared for model surgery, prior to apply it on the facial anomaly patient. On using pre-contoured arch bar, patients felt better during the procedure and surgeons saved time. Moreover, well-contoured arch bar promises precise transmission of force on the bone, which implies surgeons do not need to worry about the occlusion while fixing bony structures. Authors suggest how to apply arch bar to satisfy basic theories about dental row and occlusion based on the experience. In addition, it is proposed to contour arch bar on the dental impression prior to apply it on the patient who has irregular and complex occlusal plane.

• Archives of Craniofacial Surgery
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• v.17 no.4
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• pp.211-217
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• 2016

Background: Nonsyndromic craniosynostosis is a relatively common craniofacial anomaly and various techniques were introduced to achieve its operative goals. Authors found that by using smaller bone fragments than that used in conventional cranioplasty, sufficiently rigid bone union and effective regeneration capacity could be achieved with better postoperative outcome, only if their stable fixation was ensured. Methods: Through bicoronal incisional approach, involved synostotic cranial bone together with its surrounding areas were removed. The resected bone flap was split into as many pieces as possible. The extent of this 'multi-split osteotomy' depends on the degree of dysmorphology, expectative volume increment after surgery and probable dead space caused by bony gap between bone segments. Rigid interosseous fixation was performed with variable types of absorbable plate and screw. In all cases, the pre-operational three-dimensional computed tomography (3D CT) was checked and brain CT was taken immediately after the surgery. Also about 12 months after the operation, 3D CT was checked again to see postoperative morphology improvement, bone union, regeneration and intracranial volume change. Results: The bony gaps seen in the immediate postoperative brain CT were all improved as seen in the 3D CT after 12 months from the surgery. No small bone fragment resorption was observed. Brain volume increase was found to be made gradually, leaving no case of remaining epidural dead space. Conclusion: We conclude that it is meaningful in presenting a new possibility to be applied to not only nonsyndromic craniosynostosis but also other reconstructive cranial vault surgeries.

• Proceedings of The Korean Cleft Lip And Palate Association
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• 1997.06a
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• pp.29-29
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• 1997

• Archives of Craniofacial Surgery
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• v.21 no.2
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• pp.109-113
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• 2020

Sinus pericranii is a rare vascular anomaly characterized by abnormal venous communication between the inner and outer regions of the cranial cavity. Here, we report a case of sinus pericranii and venous malformations in the right periorbital region of a 2-year-old girl. Radiologic findings showed venous malformations in the right parietal region communicating with the superior sagittal sinus in the intracranial region. There were notable improvements following surgical resection for the abnormal venous lesions and several sclerotherapies. Presence of a bluish and pulsating mass on the scalp, which showed bruit on auscultation, may indicate sinus pericranii, which should be included in the differential diagnosis.

• Archives of Craniofacial Surgery
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• v.14 no.2
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• pp.115-118
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• 2013

Craniosynostosis is a congenital anomaly in which cranial sutures close prematurely and restrict skull growth. In this paper, the case of two siblings, a male and a female, who were both diagnosed as craniosynostosis is reported. They underwent corrective osteotomy for cranial vault remodeling. A 22-month-old female infant who was brought to the department of plastic and reconstructive surgery of the authors' hospital was diagnosed with plagiocephaly. At the same time, her 7-month-old brother was diagnosed with brachycephaly. In the case of the female infant, corrective coronal osteotomy and supraorbital bar advancement were performed. Her brother underwent frontal advancement osteotomy using Tessier's tongue in the groove procedure. After the correction of the craniosynostosis, the two patients recovered in several days later, and the results were good in both cases cosmetically and functionally. They showed normal head circumference increasing curves and no symptom of functional disorder in their last follow-up. Isolated or nonsyndromic craniosynostosis is sporadic but mostly autosomal dominant. This paper presents a case of craniosynostosis with a genetic tendency; and although it occurred between siblings, the affected lesions differed. Thus, appropriate diagnosis and management in patients are needed.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.597-601
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• 2003

Trisomy 9 mosaic syndrome is a rarely reported chromosomal abnormality with high incidence of intrauterine growth retardation and perinatal death. Even a baby lives, he has severe mental retardation and significant malformations. The incidence and severity of malformations and mental retardation correlate with the percentage of trisomic cells in the different tissues. The characteristic craniofacial abnormalitis are narrow bifrontal diameter, up-slanted and short palpebral fissures, a prominent nasal bridge with a short root, a prominent lip covering a receding lower lip, low-set, posteriorly rotated, and misshapen ears. Ventricular septal defect is a main cardiac abnormality. Bony hypoplasia and dislocated hips have been frequently reported. Central nervous system, hepatobiliary, gastrointestinal and genitourinary abnormalities also had been reported. The authors report a baby who had characteristic abnormalities of trisomy 9 mosaicism with narrow temples, up-slanted palpebral fissures, a bulbous nose, thin and protruding upper lip, low set and malformed ears, hyperextended wrist and overlapping fingers. Cytogenetic analysis performed to confirm the chromosomal abnormality revealed trisomy 9, low level mosaic type.

• Journal of Genetic Medicine
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• v.7 no.2
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• pp.151-155
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• 2010

Apert syndrome is a rare congenital anomaly characterized by craniofacial malformations and severe symmetrical syndactyly of fingers and toes. This syndrome is caused by a genetic mutation; the S253 mutation is common, though the P253R mutation is not as frequent. Common symptoms include skeletal malformations, poor joint mobility, eye and ear problems, cleft palate, and orthodontic and other dental problems. We report a case of an infant with the common morphological features of Apert syndrome. Interestingly, she was found to have the P253R mutation in FGFR2 exon VIII, which has been less commonly observed in Korea. A brief review of the literature is included.

• Archives of Craniofacial Surgery
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• v.20 no.3
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• pp.170-175
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• 2019

Background: Cryptotia is a congenital anomaly in which the upper part of the retroauricular sulcus is absent and buried underneath the temporal skin. Various surgical techniques have been reported for the correction of cryptotia following Kubo's V-Y plasty in 1933. Conventional methods using a local skin flap, skin grafting, tissue expansion, Z-plasty, and any of these combined approaches can result in skin deficiency of the upper auricle. The aim of this study was to develop a new method that improves cosmetic results and has fewer complications. Methods: This study involved four patients in whom five cryptotia deformities were corrected using V-Y plasty and Z-plasty. After elevation of the flap, acellular dermal matrix (ADM; MegaDerm) that was over 5 mm in thickness was applied to the cephalo-auricular angle and positioned to enhance the projection of the ear. Lastly, the flap was transposed to complete the repair. Results: Between January 2014 and February 2018, cryptotia correction with ADM graft was performed in four patients. None of the patients developed a recurrence of cryptotia, and there were no postoperative complications such as wound infection, seroma formation, and dehiscence. In addition, the procedures resulted in a favorable cosmetic appearance. Conclusion: Based on these findings, i.e., no recurrence and a favorable cosmetic result, when using an ADM graft, it is suggested that this technique could be an alternative method of cryptotia correction. It could also lessen donor-site morbidity when compared to autologous cartilage grafting and be more cost-effective than using cartilage from a cadaver.

• Archives of Craniofacial Surgery
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• v.24 no.5
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• pp.223-229
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• 2023

Background: Preauricular sinus (PAS) is a common congenital anomaly, and complete excision is recommended to prevent recurrence. However, PAS has a high recurrence rate as a result of incomplete removal due to the high variability of the sinus ramifications, making its treatment challenging. In this study, we standardized the surgical procedure to reduce the complications and recurrence rate and compared the postoperative results between the non-standardized and the standardized groups. Methods: This retrospective study included 97 patients (120 ears) who had undergone PAS excision by a single surgeon between October 2014 and September 2022 and underwent at least 6 months of follow-up. After October 2018, all patients were treated using the standardized method, which comprised the use of magnifying glasses, exploration with a lacrimal probe, the use of methylene blue staining, and excision of a piece of surrounding normal tissue and related cartilage in continuity with the specimen. There were 38 patients (45 ears) in the non-standardized group and 59 patients (75 ears) in the standardized group. Results: Recurrence was observed in six of 120 ears, indicating an overall recurrence rate of 5.0%. Recurrence occurred in five ears (11.1%) in the non-standardized group and one ear (1.3%) in the standardized group. The standardized group had a significantly lower recurrence rate (p= 0.027) than the non-standardized group. Conclusion: We defined a standardized sinectomy protocol and used it for the surgical treatment of PAS. With this standardized method, we were able to reduce the rates of complications and recurrence without the use of a long incision.