• 제목/요약/키워드: Craniofacial Hypoplasia

검색결과 30건 처리시간 0.032초

반안면 왜소증 환자에서의 골신장술 (DISTRACTION OSTEOGENESIS IN PATIENTS WITH HEMIFACIAL MICROSOMIA)

  • 백진아
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제31권6호
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    • pp.526-531
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    • 2005
  • Distraction osteogenesis is a technique of bone lengthening by gradual movement and subsequent remodeling. Distraction forces applied to bone also create tension in the surrounding soft tissues, distraction histiogenesis. Distraction osteogenesis is used to correct facial asymmetry, such as patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip & palate, alveolar defect and craniofacial deficiency. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. This report describes two cases of hemifacial microsomia(type IIB). In these two cases, distraction osteogenesis was used to correct a facial asymmetry. Two patients underwent unilateral mandibular distraction osteogenesis of ascending ramus of the mandible with extraoral devices. Successful distraction osteogenesis was achieved in the patients with hemifacial microsomia.

랫드에 있어서 배양배자에 대한 Phenytoin의 최기형성 효과 (Teratogenic Effects of Phenytoin on Rat Embryos in Culture)

  • 김종춘;임광현;정문구;노정구
    • Toxicological Research
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    • 제14권3호
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    • pp.357-363
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    • 1998
  • The teratogenic potential of the anticonvulsant drug phenytoin (PHT) has been well documented both in the human and in the experimental animals. However there are few reports on the effects of PHT on embryonic development in rats in vitro. The present study was performed to evaluate the teratogenic effects of PHT using whole-embryo culture system in rats. Sprague-Dawley rat embryos were explanted on gestational day (GD) 9.5 and cultured for 48 hrs in the immediately centrifuged and heat-inactivated rat serum containing 0,25,50, or $100{\mu}g$ PHT/mL. At the end of culture period the embryos were scored for morphological development according to the procedure of Van Maele-Fabry, and their total protein contents were determined. At 100 ${\mu}$g/mL of culture medium. PHT caused significant reduction in developmental score and protein content of embryos and a high incidence morphological abnormalities (100%). Characteristic malformations included altered yolk and embryonic circulation, craniofacial hypoplasia, neural tube schisis, branchial arch defects, abnormal ratation, and limb bud hypoplasia, among others. There were no adverse effects on embryonic growth and development at concentrations of 25 and 50 ${\mu}$g /mL of culture medium. The results indicated that the dysmorphogenic effect of PHT on cultured embryos is due to a direct interference with embryonic development.

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악정형 장치를 이용한 구순구개열 환자의 상악골 전방 견인 (Maxillary Advancement using Distraction Osteogenesis Devices in Cleft Palate Patients)

  • 진임건;신정현;박성수;김성민;명훈;최진영;이종호;정필훈;김명진;황순정
    • 대한구순구개열학회지
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    • 제10권2호
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    • pp.75-80
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    • 2007
  • For the treatment of cleft palate patients, orthognathic surgery has been used by standard protocol. Predictable results have been achieved with standard techniques in slight to moderate maxillary hypoplasia. However, limited advancement and high relapse rate was reported in severe cases. The purpose of the present study was to review the clinical results of distraction osteogenesis in the patients with cleft lip and palate. Distraction osteogenesis has improved results in these patients by allowing soft tissue relaxation and gradual bone generation. Therefore, greater movement of the craniofacial skeleton is possible in severe cases of maxillary retrusion with lower relapse rates. In conclusion, distraction osteogenesis for the advancement of hypoplastic maxilla of cleft patients has shown successful treatment method.

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유년기 안구적출술 및 방사선치료로 인하여 발생된 안와 열성장에 대한 재건 치험례 (PRELIMINARY CASE REPORTS OF RECONSTRUCTION FOR ORBITAL HYPOPLASIA AFTER EYEBALL ENUCLEATION AND IRRADIATION DURING CHILDHOOD)

  • 김훈;최미숙;최성원;홍관석;김성문;임재석;권종진
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제18권1호
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    • pp.17-25
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    • 1996
  • There are many reports of the surgical management for the craniofacial abnormalities arising from the irradiation of the head and face for treatment of childhood cancers. Since the mordern combined-modality theraphy for childhood cancers began in the late 1960s and the early 1970s, recent reports have described the occular, dental and maxillo-facial abnormalities after irradiation in long-term survivors of cancers of the head and face. The resultant deformities may be known to be difficult to reconstruct with surgical techniques. This paper describes the late reconstructive surgery for the unilateral orbital and malar hypoplasia after eyeball enucleation and irradiation during childhood to correct the facial asymmetry and expand the contracted orbital socket into the functional dimension for the retaining eyeball prosthesis with spherical implant. We reports the satisfactory preliminary results from the midfacial osteotomy through the supero-lateral orbital rim and malar bone and the antero-lateral repositioning with the autogenous bone grafting in 26 year-old female patient who will be planned to make the new eyeball prosthesis by the department of ophthalmology.

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크루존씨 병에서 최소침습 절개법 Le Fort III 절골술을 통한 RED II 골 신연술 후 조기 고정 1례 (A Case Report of RED II Distraction Osteogenesis and Early Rigid Fixation by Minimal Invasive Approach Le Fort III Osteotomy in Crouzon's Disease)

  • 김영석;이지나;박병윤
    • 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.

Trisomy 9 Mosaicism 1례 (A Case of Trisomy 9 Mosaicism)

  • 김영옥;박천학;최익선;김현정;조창이;최영륜
    • Clinical and Experimental Pediatrics
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    • 제46권6호
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    • pp.597-601
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    • 2003
  • 저자들은 저출생 체중과 호흡 곤란을 주소로 출생 직후 이송 된 여아에서, 진찰상 안면과 골격계 기형 소견 보여 말초 혈액에서 시행한 염색체 핵형 검사에서 trisomy 9, low level mosaic type으로 진단되었던 1례를 경험하였기에 보고하는 바이다.

증례 보고 : Rieger syndrome (RIEGER SYNDROME : A CASE REPORT)

  • 이홍모;김정욱;장기택;이상훈;한세현;김종철
    • 대한소아치과학회지
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    • 제30권4호
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    • pp.667-672
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    • 2003
  • Rieger syndrome은 눈의 전안방 기형과 치아결손을 특징으로 하는 희귀한 유전질환으로 두개안면 이상과 체성기형을 동반하기도 한다. Rieger syndrome은 상염색체 우성유전(autosomal dominant inheritance)되며, 발생빈도는 약 200,000분의 1이고, Paired-like homeodomain transcription factor2(PITX2)의 변이가 이 질환과 연관이 있는 것으로 보고되고 있다. 본 증례는 Rieger syndrome으로 진단 받은 4세 7개월 된 여아에 대한 것으로, 양안에 다동공증과 후태생환을 보이고, 측모두부계측에서 상악골 열성장이 나타났으며, 상악유측절치와 더불어 다수의 영구치 결손이 방사선 상에서 관찰되었다. 이 증례를 통하여 Rieger syndrome 환아의 구강 및 두개안면의 소견을 관찰하고, 관련 문헌을 고찰하여 다소의 지견을 얻었기에 보고하는 바이다.

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Pierre Robin sequence 환아에서 하악골신장술 (MANDIBULAR DISTRACTION OSTEOGENESIS IN AN INFANT WITH PIERRE ROBIN SEQUENCE: REPORT OF A CASE)

  • 유선열;권준경;김선국
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제34권4호
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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.

외국인에서 발생한 심각한 이차 입술갈림코변형에 대한 한 단계 수술 (One Stage Correction of the Severe Secondary Cleft Lip Nasal Deformities in Foreigners)

  • 김석권;김주찬;박수성;이근철
    • 대한두개안면성형외과학회지
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    • 제12권2호
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    • pp.102-106
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    • 2011
  • Purpose: It is accepted universally that correction of the cleft lip nasal deformity requires multiple stages of surgery. Following primary lip repair in infancy or early childhood, secondary surgery to improve the deformity of the lip and nose is frequently necessary. A suitable surgical procedure to correct the accompanying deformity, such as cleft palate and alveolus, must be carried out at an appropriate age. In developing countries, it is common for patients with cleft lip nasal deformity to present severe secondary deformities in adolescence, because of poor follow-up and inappropriate surgery. Methods: The first patient was a 12 year old Mongolian boy. He presented prominent lip scar, short lip, wide columella, asymmetric nostril, palatal fistula, cleft alveolus, and velopharyngeal incompetence. He underwent cheilorhinoplasty, transpositional flap, alveoloplasty by iliac bone graft, and sphincter pharyngoplasty. On follow-up, a bilateral maxillary hypoplasia and a class III malocclusion developed. He underwent LeFort I osteotomy and maxillary advancement at the age of 16 years. The second patient was an 18 year old Eastern Russian girl. She presented with a deviated nose, right alar base depression, short lip, protrusion on vermilion, large palatal fistula, and severe VPI due to short palate. She underwent the combined procedure of cheilorhinoplasty, corrective rhinoplasty, tongue flap for palatal fistula, and superiorly based pharyngeal flap. And the tongue flap was detached at postoperative 3 weeks. Results: The overall results have been extremely pleasing and satisfactory to patients. There were no postoperative complications. Conclusion: We discovered the one stage operation for radical correction was sufficient procedure to provide excellent clinical outcomes in patients with severe cleft lip nose deformity.

비대칭 우는 얼굴에서 보툴리눔 독소 A를 이용한 치험례 (Correction of Asymmetric Crying Facies with Botulinum Toxin A Injection: A Case Report)

  • 박성오;김민호;송정윤;박지웅;윤병민;최태현;김석화
    • 대한두개안면성형외과학회지
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    • 제12권2호
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    • pp.125-128
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    • 2011
  • Purpose: Asymmetric crying facies is caused by agenesis or hypoplasia of the depressor anguli oris muscle and is often associated various anomalies. Several static and dynamic surgical interventions have been reported, but their effects are unreliable. We report on the successful use of botulinum toxin A in an asymmetric crying facies patient. Methods: A 4-year-old girl presented with a facial asymmetry on crying or smiling. Physical examination revealed that her face had no asymmetry at rest. However, the patient showed characteristic asymmetry when smiling, crying, and with other normal facial movements. Asymmetric crying facies was clinically suspected and the weakness of left depressor anguli oris was present on electrophysiology study. Fifteen units of botulinum toxin type A were injected to the right depressor anguli oris muscle. Results: The patient showed the prominent improvement in the facial symmetry without significant complication and the effect persisted until 3 months post injection. Conclusion: Asymmetric crying facies was treated successfully with botulinum toxin A and this method was easy and noninvasive.