• Archives of Craniofacial Surgery
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• v.10 no.2
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• pp.109-113
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• 2009

Purpose: Materials for ear reconstruction are autogeneous cartilage and artificial implants. Despite their potential for donor site complications, autogeneous cartilage frameworks remain the accepted standard for external ear reconstruction. The purposes of this study were to investigate our ear reconstruction cases for 12 years. Methods: During twelve years from January 1996 to December 2008, 70 patients visited our hospital for ear reconstruction of microtia. Among them, 65 cases used autogenous cartilage frameworks, 3 cases used tissue expander and 2 cases used artificial implants. We investigated sex & age, common site, combined malformation, operation methods & their complications, donor site & their complications, anterior chest wall deformity and aesthetic evaluation. Results: Males were affected more often than females. Male to female ratio was 1.8 : 1. The common site of microtia was right ear (72%). And hemifacial microsomia was the most common associated congenital malformation. Surgical techniques included Tanzer-Ruecker method, Tissue-expander and Artificial framework ($Medpore^{(R)}$). Incidence of complication was higher with Tissue-expander & artificial framework than with Tanzer-Ruecker method. But There are few reports of using Tissue-expander & artificial framework and there was no acceptable statistical difference. And costal cartilage was harvested in ipsilateral side and anterior chest wall deformity reported only 2 cases under 10 year-old patients. Other minor complications reported such as, wound disruption and chest wall scar, but without any serious complications. Aesthetic result was evaluated by surgeons and patients for postoperative satisfaction and clinical evaluation. Conclusion: In ear reconstruction of microtia patient, delicate surgical strategy is important for natural shape and maintenance of postoperative contour. However, many methods were introduced for reconstrucion of microtia, the authors reconstructed an auricle in Tanzer-Ruecker method, Tissue-expander and Artificial famework ($Medpore^{(R)}$) for 70 patients. In our study, we generally chose Tanzer-Ruecker method and this treatment modality was satisfactory for patients and the postoperative result was acceptable for surgeons.

• Journal of Genetic Medicine
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• v.8 no.1
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• pp.67-70
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• 2011

Constitutional trisomy 8 mosaicism (CT8M) is a relatively rare aneuploidy in humans with characteristic phenotypes including typical craniofacial feature (such as deformed skull, prominent forehead, low-set and/or dysplastic ears), skeletal malformation, cardiac anomaly, renal malformation, cryptochidism, varying degree of developemental delay. Due to the extremely variable phenotypic and cytogenetic expression, CT8M has gone undiagnosed in certain patients. We report a 28-year-old women with secondary amenorreha without characteristic CT8M phenotype. Chromosomal analysis showed a CT8M (47,XX,+8[9]/46,XX[41]).

• Archives of Craniofacial Surgery
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• v.11 no.1
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• pp.13-18
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• 2010

Purpose: Nasal defect can be caused by excision of tumor, trauma, inflammation from foreign body reaction. Nose is located in the middle of face and protruded, reconstruction should be done in harmony with size, shape, color, and textures. We report various methods of nasal reconstruction using local flaps. Methods: From March 1998 to July 2008, 36 patients were operated to reconstruct the nasal defects. Causes of the nasal defects were tumor (18 cases), trauma (11 cases), inflammation from foreign body reaction (5 cases) and congenital malformation (2 cases). The sites of the defects were ala (22 cases), nasal tip (8 cases) and dorsum (6 cases). The thickness of the defects was skin only (5 cases), dermis and cartilagenous layer (7 cases) and full-thickness (24 cases). According to the sites and thickness of the defects, various local flaps were used. Most of alar defects were covered by nasolabial flaps or bilobed flaps and the majority of dorsal and tip defects were covered by paramedian forehead flaps. Small defects below $0.25 cm^2$ were covered with composite graft or full-thickness skin graft. Results: The follow-up period was 14 months. Partial flap necrosis was observed in a case, and one case of infection was reported, it was improved by wound revision and antibiotics. Nasal reconstruction with various local flaps could provide satisfactory results in terms of color and texture match. Conclusion: The important factors of nasal reconstruction are the shape of reconstructed nose, color, and texture. Nasolabial flap is appropriate method for alar or columellar reconstruction and nasolabial island flap is suitable for tip defect. The defect located lateral wall could be reconstructed with bilobed flap for natural color and texture. Skin graft should be considered when the defect could not afford to be covered by adjacent local flap. And entire nasal defect or large defect could be reconstructed by paramedian forehead flap.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.4
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• pp.667-672
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• 2003

Rieger syndrome is a rare, autosomal dominant genetic disorder characterized by malformation of the anterior chamber of the eye(goniodysgenesis) coincident with hypodontia. It may also be accompanied by a spectrum of dental, craniofacial and somatic anomalies. Mutations in paired-like homeodomain transcription factor2(PITX2) are associated with the syndrome, and its frequency in the general population has been estimated to be 1 : 200,000. In the present case, the patient, 4 year 7 month-old female, had posterior embryotoxon and polycoria. The maxilla was retrusive in cephalometric radiography. She had congenital missing on #52, #62 and some tooth germs of permanent tooth were not detected in panoramic radiography. The purpose of this paper is to report the dental and craniofacial findings and review the pertinent literature through this case.

• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.54-56
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• 2012

Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.34 no.4
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• pp.460-467
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• 2008

Pierre Robin sequence as a symptom triad of micrognathia, glossoptosis, and cleft palate results in upper airway obstruction and feeding problems. If mild, it is often managed in the prone position. When positional treatment fails, however, surgical intervention such as tongue-lip adhesion, tracheostomy, and mandibular distraction osteogenesis is mandatory to relieve airway obstruction. There has been growing interest in the application of distraction osteogenesis for the management of craniofacial abnormalities. The mandibular distraction osteogenesis to newborns may prevent the airway obstruction, decrease the potential tracheostomy, and reduce the likehood of orthognathic surgery after growth. We experienced an infant with Pierre Robin sequence who showed mandibular hypoplasia, glossoptosis, incomplete cleft palate, intermittent cyanos is, depression of the chest, and respiratory difficulty associated with airway obstruction. We treated the airway obstruction by tongue-lip adhesion at 2 weeks of age, and treated the mandibular retrognathism and depression of the chest byusing internal mandibular distraction osteogenesis at 7 month of age. The mandible moved forwardly, the upper airway space was enlarged, and the antero-posterior distance of the mandible was elongated after the mandibular distraction. Mandibular distraction osteogenesis may be a promising technique to avoid the need of tracheostomy and orthognathic surgery, and to correct airway obstruction in infants with congenital craniofacial malformation.

• 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.5
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• pp.304-309
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• 2019

Background: Treatment for venous malformations of the head and neck includes sclerotherapy, surgical resection, or a combination of both. Surgical resection can remove or reduce the volume of vascular lesions; however, surgery can cause postoperative scarring and potential surgical complications. This study sought to determine the effectiveness of surgery for the treatment of venous malformations of the head and neck. Methods: A retrospective review of the medical records of patients who received surgeries for venous malformations of the head and neck from January 2011 to July 2019 was performed. Using clinical photographs, preoperative and postoperative Doppler ultrasonography, outpatient clinic records, and operation records, the postoperative result and complications were evaluated for each case. Results: Among patients who visited our vascular anomalies clinic, 43 patients (ratio of male to female= 24:19) received surgeries for venous malformations of the head and neck. Twenty-nine patients had undergone surgery only, five patients received sclerotherapy after surgery, and nine patients received surgery after preoperative sclerotherapy. In postoperative evaluations, the result was excellent in 24 patients, good in 18 patients, and poor in one patient. Four patients experienced a recurrence of lesions with lagophthalmos, drooping of the corner of the mouth, partial wound necrosis, and scar widening found in one patient each. Conclusion: Because the head and neck region is the most exposed area in the body, more active implementation of surgical treatments with or without sclerotherapy is essential to reduce the functional and cosmetic impairments associated with venous malformations.

• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.729-735
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• 1996

Cleft lip and palate is the most common malformation in the craniofacial region. The patients with cleft lip and palate have functional problem such as, speech, feeding and respiratory as well as esthetic problem. So, treatment should be done by multidiciplinary team approach. The role of pediatric dentist in the team is advicement for feeding method, guidance of normal growth, caries control and preventive orthodontics. In cleft lip and palate patients, maxillary arch after cheiloplasty is usually collapsed by excessive tension of the scar. This collapse increase the difficulty of later orthodontic treatment. Therefore, the maxillary arch segments should be moved and retaind to normal position as soon as cheiloplasty is done to reduce the need and difficulty of orthodontic treatment. This concept is called by the early orthopedic treatment in cleft lip and palate. Also, this orthopedic appliance works as feeding applince to normal feeding and weight gain We reported two cases of early orthopedic treatment with favorable result in complete bilateral cleft lip and palate patients after cheiloplasty. Patients showed normal weight and their maxillary arch widths were increased.

• Archives of Plastic Surgery
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• v.43 no.2
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• pp.153-159
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• 2016

Background The prenatal ultrasound detection of cleft lip with or without cleft palate (CL/P) and its continuous management in the prenatal, perinatal, and postnatal periods using a multidisciplinary team approach can be beneficial for parents and their infants. In this report, we share our experiences with the prenatal detection of CL/P and the multidisciplinary management of this malformation in our institution's Congenital Disease Center. Methods The multidisciplinary team of the Congenital Disease Center for mothers of children with CL/P is composed of obstetricians, plastic and reconstructive surgeons, pediatricians, and psychiatrists. A total of 11 fetuses were diagnosed with CL/P from March 2009 to December 2013, and their mothers were referred to the Congenital Disease Center of our hospital. When CL/P is suspected in the prenatal ultrasound screening examination, the pregnant woman is referred to our center for further evaluation. Results The abortion rate was 28% (3/11). The concordance rate of the sonographic and final diagnoses was 100%. Ten women (91%) reported that they were satisfied with the multidisciplinary management in our center. Conclusions Although a child with a birth defect is unlikely to be received well, the women whose fetuses were diagnosed with CL/P on prenatal ultrasound screening and who underwent multidisciplinary team management were more likely to decide to continue their pregnancy.