• Clinical and Experimental Pediatrics
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• 제46권4호
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• pp.397-399
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• 2003

저자들은 특이한 얼굴, 근 긴장도 증가, 정신 지체와 혈액학적으로 일시적인 적혈구 이형성증(dyserythropoiesis)의 소견을 동반한 MDS 의증의 소견과 혈소판 감소증, 호산구 증가증을 동반하는 부분 21-monosomy 증후군 1례를 신생아기부터 8세까지 추적 관찰하고 보고한다. 이와 같은 예는 우리나라에서 보고되는 첫 증례라고 사료되어 간단한 문헌 고찰과 함께 보고하는 바이다.

• Archives of Plastic Surgery
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• 제34권1호
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• pp.123-127
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• 2007

Purpose: Rigid external distraction(RED) is a highly effective technique for correction of maxillary hypoplasia in patients with cleft or syndromic craniosynostosis. Despite many advantages of RED, it also has the problem of relapse as the conventional advancement surgery. Bicoronal approach, that is the common approach to gain access to the craniofacial skeleton, had some morbidity, such as hair loss, sensory loss, wide scar and temporal hollowing. We present our clinical experience of RED distraction with minimal invasive approach and early rigid fixation to overcome these disadvantages. Methods: A 27-year-old female patient with Crouzon's disease underwent Le Fort III osteotomy and RED device application through the minimal invasive direct skin incisions. After the latent period of 5 days, distraction was undertaken until proper convexity and advancement were obtained. During the rigid retention period, inflammation occurred on the right cheek, and proper conservative managements were done including continuous irrigation. To maintain the stability of distraction, early rigid fixation was undertaken on the osteotomy sites through another skin incisions. Preoperative and postoperative orthodontic treatments were performed. Serial photographs and cephalometric radiographs were obtained preoperatively, after distraction and 6 months after distraction. Results: The cephalometric analysis demonstrated postoperatively significant advancement of the maxilla and improvement of facial convexity. After 6-month follow-up period, the maxilla was stable in the sagittal plane and no relapse was found. Facial scars were not noticeable and other deformity and morbidity did not occur. Conclusion: This effective and stable technique will be a good alternative for the patients who need large amount of distraction and for adult patients with severe maxillary hypoplasia or syndromic craniosynostosis.

• 대한장애인치과학회지
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• 제12권1호
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• pp.1-5
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• 2016

저자는 과잉치로 인한 불편감을 호소하며 서울대학교치과병원 소아치과에 내원한 2세 7개월의 로비노 증후군 증례를 치료하였기에 문헌 고찰과 함께 보고하는 바이다. 환자의 전신 소견으로는 대두증, 전두부 돌출, 양안격리증, 넓은 안검렬, 들창코, 콧등 함몰, 삼각형의 입술을 포함하는 특이한 안모와 짧은 손,발가락, 소음경을 포함한 잠복고환 및 발달지연이 관찰되었다. 구내 소견으로는 수술받은 구순열과 하악골 저성장, 치아맹출지연, 치은비대, 과잉치가 관찰되었다. 로비노 증후군 환아는 전신마취 하에서 성공적으로 치과 치료가 가능했다. 치과의사는 치과 치료에 의한 스트레스로 인해 근육의 긴장저하, 발달, 호흡, 섭식 등 의 전신적인 상태를 항상 고려해야 한다. 또한, 로비노증후군 환자가 두개 안면부의 이형성과 맹출장애를 가진다는 사실을 알고 접근해야 하며, 환자가 성장함에 따라 적절한 시기에 필요한 치료를 할 수 있도록 주기적인 관찰이 필요하다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제33권5호
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• pp.559-566
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• 2007

Distraction osteogenesis(DO) is a surgical method of bone formation that involves an osteotomy and sequential stretching of the healing callus by gradual movement and subsequent remodeling. DO is used to correct facial asymmetry, such as in patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip and palate, alveolar defects, and craniofacial deficiency. It is accomplished with the aid of a distraction device, which is secured with screws placed directly into bone, for a predetermined length of time. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, tilted occlusal plane, and short mandible. Early treatment is necessary to avoid subsequent impaired midfacial growth. The standard treatment of these malformations consists of the application of bone grafts, which can lead to unpredictable growth. The new bone-lengthening procedure represents a limited surgical intervention and opens up a new perspective for treatment, especially in younger children with severe deformities. This report describes a case of hemifacial microsomia(Type-II left-sided hemifacial microsomia). The patient, a 10-year-old child, visited our clinic for facial asymmetry correction. He had a hypoplastic mandible, displaced ear lobe, 10 mm canting on the right side, and malocclusion. We planned DO to lengthen the left mandible in conjunction with a Le Fort I osteotomy for decanting and then perform a right intraoral vertical ramus osteotomy(IVRO). Progressive distraction at a rate of 0.5 mm/12 hours was initiated 7 days postoperatively. The duration of DO was 17 days. The consolidation period was 3 months. Satisfactory results were obtained in our case, indicating that DO can be used successfully for functional, aesthetic reconstruction of the mandible. We report a case involving DO in conjunction with orthognathic surgery for correcting mandibular hypoplasia with a review of the literature.

• 대한장애인치과학회지
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• 제15권1호
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• pp.79-83
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• 2019

Treacher Collins 증후군(TCS)은 두개안면 발육의 이상을 보이는 상염색체 우성 질환으로, 외이, 중이 및 이소골(auditory ossicle)의 형태이상과 상악골 발육 부전, 후퇴된 하악, 구개열 등의 특징을 보인다. TCS 환아의 경우, 청각 장애로 인한 환아의 불안으로 적절한 협조를 얻기 어렵고, 개구제한과 기도 확보의 어려움으로 인해 전신마취 하에 치과치료를 진행하는 것이 안전하다. 전신마취 시 하악 후퇴 등의 형태학적인 문제로 인하여 삽관의 난이도가 높을 수 있어 주의가 요구되며, 술 후에도 적절한 호흡의 유지를 위해 지속적인 감시가 필요하다. 마지막으로, 보호자로 하여금 구강 위생의 중요성이 강조되어야 한다.