• Korean Journal of Cleft Lip And Palate
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• v.8 no.2
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• pp.71-79
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• 2005

Purpose: Maxillary hypoplasia is a common developmental problem of cleft lip and palate. Fair results with distraction osteogenesis have been reported especially when these patients need a large amount of maxillary advancement, instead of orthognathic surgery. The purpose of this study is to evaluate the clinical results with a relatively new distractor, $TS-MD^{(R)}$ (Trans-sinusoidal maxillary distractor, KLS Martin, Tuttlingen, Germany) which was used for the advancement of the maxilla in the cleft patients. Patients and Method: Distraction osteogenesis using $TS-MD^{(R)}$ was performed for four CLP patients (three males and one female) who had maxillary hypoplasia. All patients were over 16 years old. As three patients showed mandibular prognathism as well, bilateral sagittal split ramus osteotomy for mandibular setback was performed at the same time. After consolidation periods of 4 to 12 weeks, the distraction devices were removed and miniplates were placed for simultaneous internal fixation. Results: Three patients showed a large amount of incisal overbite but one patient did not have sufficient maxillary advancement. Le Fort I osteotomy, maxillary advancement and internal fixation should have been performed for the patient when removing the distraction devices. Different from the $clinician{\box}s$ expectation, the amount of maxillary advancement using $TS-MD^{(R)}$ was not sufficient, although the device has rigid mechanical property. Rotation of maxilla during distraction forward and downward was also observed. Conclusion: Even though the maxillary advancement with $TS-MD^{(R)}$ device could be achieved, the clinical control of some characteristics related with the device was necessary. More clinical studies on $TS-MD^{(R)}$ should be performed.

• Korean Journal of Cleft Lip And Palate
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• v.4 no.2
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• pp.45-50
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• 2001

Recents reports have demonstrated that force and direction is important during mandibular distraction osteogenesis. The purpose of this study was to evaluate the resistant force of internal pterygoid muscles and inquire into relationship between internal pte'Ygoid muscles and cephalometric parameters. Eighty four patients with class III malocclusion underwent bilateral sgittal splitting of ramus with intraoral vertico-sagittal ramal osteotomy. A spring scale were used for measuring for resistence of internal pterygoid mescles after splitting of ramus. Skeletal-dental cephalometric analysis was made following statistic package was used for correlation between resistence and cephalometric parameters. The resistant force of right internal pterygoid muscle was greater than left muscle in Korean with class III malocclusion and the force had a linear regression relationship with facial depth (distance between nasion and gonion). The results suggested that facial depth has significant correlation of the resistance of internal pterygoid muscle (p<0.05).

• Archives of Craniofacial Surgery
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• v.17 no.4
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• pp.222-224
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• 2016

Epidermal cysts are intradermal or subcutaneous cystic tumors that frequently occur in the face, scalp, neck, and body trunk. Acquired cases of epidermal cyst commonly occur as a result of various surgical operations, chronic irritation, or trauma, all of which may trigger the occurrence of the invagination of squamous epithelium. A 57-year-old man presented with a palpable mass $7cm{\times}2cm$ in size in the upper lip. The patient had a 3-year history of wearing a denture to restore missing bilateral maxillary central and lateral incisors, accompanied by inflammatory findings on the buccal mucosa due to chronic lip irritation. The resected oval-shaped cyst had a size of $5.5cm{\times}3.0cm{\times}2.5cm$, and it was an encapsulated mass with a well-defined margin. The histopathology was typical of epidermal cyst. This case of a rare giant upper lip epidermal cyst in a patient wearing a denture may be of interest to clinicians.

• Korean Journal of Cleft Lip And Palate
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• v.3 no.1
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• pp.23-31
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• 2000

Objective: When an alveolar cleft is too large to close with adjacent mucobuccal flaps or large secondary fistula following a primary bilateral palatoplasty exists, a one-stage procedure for bone grafting becomes challenging. In such a case, we used the tongue flap to repair the fistula and cleft alveolus in the first stage, and bone grafting to the cleft defect was performed in the second stage several months later. The purpose of this paper is to report our experiences with the use of an anteriorly-based Y-shaped tongue flap to fit the palatal and labial alveolar defects and the ultimate result of the bone graft. Patients: A series of 14 patients underwent surgery of this type from January 1994 to December 1998.The average age of the patients was 15.8 years old (range: 5 to 28 years old). The mean period of follow-up following the 2nd stage bone raft operation was 45.9 months (range: 9 to 68 months). In nine of the 14 cases, the long-fork type of a Yshaped tongue flap was used for extended coverage of the labial side alveolar defects with the palatal fistula in the remaining cases the short-forked design was used. Results: All cases demonstrated a good clinical result after the initial repair of cleft alveolus and palatal fistula. There was no fistula recurrence, although Partial necrosis of distal margin in long-forked tongue flap was occurred in one case. Furthermore, the bone graft, which was performed an average of 8 months after the tongue flap repair, was always successful. Occasionally, the transferred tongue tissue was bulging and interfering with the hygienic care of nearby teeth; however, these problems were able to be solved with proper contour-pasty performed afterwards. No donor site complications such as sensory disturbance, change in taste, limitations in tongue movement, normal speech impairments or tongue disfigurement were encountered. Conclusion: This two-stage reconstruction of a bilateral cleft alveolus using a Y-shaped tongue flap and iliac bone graft was very successful. It may be indicated for a bilateral cleft alveolus patient where the direct closure of the cleft defect with adjacent tissue or the buccal flap is not easy due to scarred fibrotic mucosa and/or accompanied residual palatal fistula.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.234-238
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• 2003

Fanconi syndrome is a generalized functional disorder of the proximal tubule of the kidney and is characterized by aminoaciduria, glycosuria, hyperphosphaturia, dehydration, rickets, and growth failure. Nephrocalcinosis and hypercalciuria are rare manifestations of Fanconi syndrome. There is no case report of Fanconi syndrome complicated with nephrocalcinosis and hypercalciuria in Korea. A 6-year-old boy presented with genu valgum and waddling gaits for about 3 years. There was no family history of renal disease and his physical examination was normal except for genu valgum and corrected cleft lip and palate. Laboratory investigations showed generalized aminoaciduria, glycosuria, hyperphosphaturia, hypercalciuria, and low-molecular weight proteinuria including ${\beta}$-microglobulin. Serum 25-OH vitamin $D_3$ was within the normal range, and $1,25-(OH)_2$ vitamin $D_3$ was elevated. Bilateral renal medullary hyperechogenicity was demonstrated by ultrasonography. Analysis of the CLCN5 gene revealed no mutation. Here we describe a boy with Fanconi syndrome complicated with nophrocalcinosis and discuss the differential diagnosis.

• The korean journal of orthodontics
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• v.46 no.6
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• pp.395-408
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• 2016

We report a case involving a young female patient with severe mandibular retrognathism accompanied by mandibular condylar deformity that was effectively treated with Le Fort I osteotomy and two genioplasty procedures. At 9 years and 9 months of age, she was diagnosed with Angle Class III malocclusion, a skeletal Class II jaw relationship, an anterior crossbite, congenital absence of some teeth, and a left-sided cleft lip and palate. Although the anterior crossbite and narrow maxillary arch were corrected by interceptive orthodontic treatment, severe mandibular hypogrowth resulted in unexpectedly severe mandibular retrognathism after growth completion. Moreover, bilateral condylar deformities were observed, and we suspected progressive condylar resorption (PCR). There was a high risk of further condylar resorption with mandibular advancement surgery; therefore, Le Fort I osteotomy with two genioplasty procedures was performed to achieve counterclockwise rotation of the mandible and avoid ingravescence of the condylar deformities. The total duration of active treatment was 42 months. The maxilla was impacted by 7.0 mm and 5.0 mm in the incisor and molar regions, respectively, while the pogonion was advanced by 18.0 mm. This significantly resolved both skeletal disharmony and malocclusion. Furthermore, the hyoid bone was advanced, the pharyngeal airway space was increased, and the morphology of the mandibular condyle was maintained. At the 30-month follow-up examination, the patient exhibited a satisfactory facial profile. The findings from our case suggest that severe mandibular retrognathism with condylar deformities can be effectively treated without surgical mandibular advancement, thus decreasing the risk of PCR.

• Archives of Craniofacial Surgery
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• v.17 no.1
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• pp.20-24
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• 2016

Background: Midface concavity is a relatively common facial feature in East Asian populations. Paranasal augmentation is becoming an increasingly popular procedure for patients with mild concavity and normal occlusion. In this study, we evaluate clinical outcomes following a series of paranasal augmentation. Methods: A retrospective review was performed for patients with Class I occlusion who had undergone bilateral paranasal augmentation using custom-made silicone implants, between October 2005 and September 2013. Patient charts were reviewed for demographic information, concomitant operations, and postoperative complications. Preoperative and postoperative (1-month) photographs were used to evaluate operative outcome. Results: The review identified a total of 93 patients meeting study criteria. Overall, aesthetic outcomes were satisfactory. Five-millimeter thick silicone implant was used in 81 cases, and the mean augmentation was 4.26 mm for this thickness. Among the 93 patients, 2 patients required immediate implant removal due to discomfort. An additional 3 patients experienced implant migration without any extrusion. Nine patients complained of transient paresthesia, which had resolved by 2 weeks. There were no cases of hematoma or infection. All patients reported improvement in their lateral profile and were pleased at follow-up. Complications that arose postoperatively included 9 cases of numbness in the upper lip and 3 cases of implant migration. All cases yielded satisfactory results without persisting complications. Sensations were fully restored postoperatively after 1 to 2 weeks. Conclusion: Paranasal augmentation with custom-made silicone implants is a simple, safe, and inexpensive method that can readily improve the lateral profile of a patient with normal occlusion. When combined with other aesthetic procedures, paranasal augmentation can synergistically improve outcome and lead to greater patient satisfaction.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.5
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• pp.422-427
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• 2004

Treacher Collins syndrome is inherited as an autosomal dominant trait with variable penetrance. It shows a marked variability even in the same family. This syndrome is developmental defect affecting the branchial arches. It is not usually associated with acute respiratory distress, but has symptoms of microtia, hypoplastic zygomatic bones, hypoplastic mandibular rami, and bilateral coloboma. It usually requires an emergency operation immediately after the birth. We experienced an infant with Treacher Collins syndrome who showed retrognathia, glossoptosis, microtia, and cleft palate. Intermittent cyanosis, depression of the chest, respiratory difficulty associated with airway obstruction, and swallowing difficulty were also observed. To relieve severe upper airway obstruction caused by retrognathia and glossoptosis, we simultaneously performed tongue-lip adhesion and subperiosteal release of the floor of the mouth. The respiratory and swallowing difficulties were relieved and the tongue repositioned anteriorly. We report the present case with a review of the literature.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.492-498
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• 1999

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.3
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• pp.374-380
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• 2010

Treacher Collins syndrome(TCS) is a rare, incurable condition occurring in approximately 1 of 25,000 to 50,000 births. It may occur as a spontaneous mutation out of genetically normal parents or it may be manifested as an autosomal dominant pattern. TCS is characterized by facial deformities such as, underdevelopment of the maxilla, mandible and zygoma, malocclusion, bilateral deformities of auricles, antimongoloid slant of the palpebral fissures. The syndrome is often associated with cleft lip and palate, ear malformations and hearing loss, short stature, and anomalies of the heart and skeleton. Respiratory difficulty associated with air way obstruction may also be observed, and there is considerable difficulty in airway management during general anesthesia. It is necessary that dentists provide safe dental treatments and guidelines to TCS patients by providing adequate understanding about the characteristics of the syndrome and proper ways of managements. The purpose of this study is to report the dental and medical characteristics of the patient who visited the Department of Pediatric Dentistry, Yonsei University for multiple dental caries treatment and to review the literatures of TCS.