• 제목/요약/키워드: Bilateral cleft

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Trans-sinusoidal maxillary distractor($TS-MD^{(R)}$)를 이용한 구순구개열 환자에서의 상악골 골신장술 (Maxillary Distraction Osteogenesis Using $TS-MD^{(R)}$ (Trans-sinusoidal Maxillary distractor) on Cleft Patients)

  • 팽준영;이일구;명훈;황순정;서병무;최진영;이종호;정필훈;김명진
    • 대한구순구개열학회지
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    • 제8권2호
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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.

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대한악안면성형재건외과학회(KAMPRS)의 베트남 구순구개열 무료 의료 봉사 활동에 관한 보고 (The Report on the Medical Voluntary Activities for Cleft Lip and Palate in Vietnam by the Korean Association of Maxillofacial Plastic and Reconstructive Surgeons)

  • 함태훈;신효근;김종렬;류동목;유선열;김경원;박영욱;정영수
    • 대한구순구개열학회지
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    • 제13권1호
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    • pp.23-28
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    • 2010
  • 대한악안면성형재건외과학회는 2004년도부터 매년 인도적 차원에서 의료 환경이 낙후된 Vietnam의 cleft lip and palate 환자들을 치료하기 위해 무료로 해외 진료 봉사 활동을 해왔다. 초창기 해외 진료 봉사 활동 당시 베트남 의료 기술 발달이 미비하여 준비해야 할 것들이 많았으나 이 후 베트남의 경제 발전과 함께 의료 시설 및 의료 기술이 발달함에 따라 준비 품목 등이 간단명료해지고 현지 지원 및 수술 준비도 원활히 진행되었다. 이러한 무료 의료 봉사 활동을 계기로 개발도상국의 낙후된 의료 시설 및 의료 기술 발전, 의료 혜택을 받지 못한 구순 구개열 환자의 정상적 안모 및 기능 회복, 조건 없는 의료 봉사 활동을 통한 민간외교 효과 및 양국간의 우호 증진 등의 성과가 있었다. 대한악안면성형재건외과학회에서는 2009년도 역시 베트남 National Institute of Odonto-stomatology 에서 11월17일부터 26일까지 cleft lip and palate 무료수술진료단(단장 신효근)을 보내 수술봉사 활동을 펼치고 돌아 왔다. 일반적인 구순구개열 뿐만 아니라 양측성 안면열(Bilateral Facial Cleft), 사경(Torticollis) 등의 희귀 기형을 포함하여 수술을 진행하였으며 총 31례의 수술을 성공적으로 마쳤으며 지난 6년 동안의 성과와 함께 이에 대한 보고를 하고자 한다.

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Congenital Cleft of Anterior Arch and Partial Aplasia of the Posterior Arch of the C1

  • Choi, Ji-Won;Jeong, Je-Hoon;Moon, Seung-Myung;Hwang, Hyung-Sik
    • Journal of Korean Neurosurgical Society
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    • 제49권3호
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    • pp.178-181
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    • 2011
  • Congenital anomalies in arches of the atlas are rare, and are usually discovered incidentally. However, a very rare subgroup of patients with unique radiographic features is predisposed to transient quadriparesis after minor cervical or head trauma, A 46-year-old male presented with a 2-month history of tremor and hyperesthesia of the lower extremities after experiencing a minor head trauma. He said that he had been quadriplegic for about 2 weeks after that trauma. Radiographs of his cervical spine revealed bilateral bony defects of the lateral aspects of the posterior arch of C1 and a midline cleft within the anterior arch of the atlas. A magnetic resonance imaging revealed an increased cord signal at the C2 level on the T2-weighted sagittal image. A posterior, suboccipital midline approach for excision of the remnant posterior tubercle was performed. The patient showed significant improvement of his motor and sensory functions. Since major neurologic deficits can be produced by a minor trauma, it is crucial to recognize this anomaly.

악안면 기형환자에서 내측 익돌근 근력측정 및 의미분석 (Analysis of the resistant muscle force against distraction in the maxilla-facial deformities)

  • 정필훈;홍종락;배용철
    • 대한구순구개열학회지
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    • 제4권2호
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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).

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연구개내근성형술 및 서골피판을 동반한 2개 점막성골막판을 이용한 완전 양측성 구개열 환자의 치험례 (Case Report: Repair of Complete Bilateral Cleft Palate Using Two-Flap Palatoplasty with Intravelar Veloplasty and Vomer Flap)

  • 이의룡;서병무;정필훈
    • 대한구순구개열학회지
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    • 제12권1호
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    • pp.33-40
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    • 2009
  • 구개열이 있으면 언어장애 음식섭취의 어려움, 구개범장근의 기능장애로 인한 이관의 개폐기능부전으로 중이의 액체고임, 부정교합등 여러 문제가 생길 수 있다. 따라서 구개성형술은 갈라진 경구개와 연구개를 막아주며 동적인 연구개를 만들어 주어 충분한 구개인두폐쇄를 하여 정상적 발음을 하는데 그 목표가 있다. 그 외에도 음식물을 정상적으로 섭취할 수 있고 중이염 및 난청을 일으킬 수 있는 기능을 개선시키고 정상적인 교합을 만들어주는데 있다. 위의 목표를 이루기 위해 지금까지 많은 수술 방법이 개발되었고 개선되어 왔다. 하지만 아직도 가장 효과적인 수술방법, 수술시기에 대하여 논쟁거리가 되고있다. 언어를 분명하게 하려면 연구개는 인두벽에 닿기 위해 후상방으로 올라가고 인두의 후벽과 측벽은 올라온 연구개에 닿으려고 수축함으로써 비인두와 구인두 사이의 공간이 좁아지게 됨으로써 가능하다. 따라서 발음이 정확하려면 비인두괄약(nasopharyngeal sphincter)을 합리적으로 만들어주어 비인두와 구인두를 분리해 주어야 한다. 비인두괄약을 조성해 주는 방법에는 구개범거근이 괄약기능을 할수 있도록 연구개내근성형술(intravelar veloplasty)을 시행하여 양편구개범거근을 횡위로 옮겨 연결하여 올림근 걸이(levator muscle sling)을 만드는 방법, 구개 연조직을 후방으로 밀어 구개 길이를 연장하는 방법, 인두 피판술을 하는 방법등이 있다. 구개범거근의 주행방향과 부착이 잘못되어 있는 것으로, 정상에서는 구개범거근이 횡으로 주행하여 연구개의 정중봉선(median raphe)에 부착하는 데 반해 구개열에서는 구개범거근이 전방으로 주행하여 개열 가까이에 있는 구개열 후연과 골선 개열연에 부착되어 있고 구개인두근과 구개수근이 연구개를 그냥 지나쳐직접 구개열 후연에 붙는다. 저자등은 완전 양측성 구개열을 연구개내근성형술 및 서골피판을 동반한 2개 점막성골막판을 이용한 구개성형술로 수술을 시행하여 다소의 지견을 얻었기에 문헌고찰과 함께 보고하는 바이다

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구순열 환자의 이차 구순 성형술 (CORRECTION OF SECONDARY LIP DEFORMITIES IN CLEFT PATIENTS)

  • 김종렬;변준호
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제21권4호
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    • pp.401-406
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    • 1999
  • 편측성 혹은 양측성 구순구개열환자에서 일차 구순 성형술후 나타나는 이차 구순 기형은 세심한 관찰을 통한 문제점에 대한 정확한 인식을 통해 상순, 홍순부, 피부와 근육등의 적절한 교정술로 심미성, 기능적 회복을 이룰 수 있다. 저자들은 이차 구순 기형으로 나타난 구륜근의 부정유합, 휘파람 변형과 부족한 입술등에 대해 적절한 성형술로 심미적, 기능적으로 양호한 결과를 얻었기에 보고하는 바이다.

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부족교정된 일측성 관상봉합 조기유합증 환자의 수술 교정예 (A Case of Surgical Correction of Undercorrected Unicoronal Synostosis)

  • 심형섭;백혜원;변준희
    • 대한두개안면성형외과학회지
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    • 제9권2호
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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.

아가미귀 증후군 1예 (A Case of Branchio-Otic Syndrome)

  • 홍정주;신유섭;김윤태;김철호
    • 대한두경부종양학회지
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    • 제27권1호
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    • pp.84-87
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    • 2011
  • Branchio-otic syndrome(BOS) is a relatively uncommon genetic malformation associated with dysmorphogenesis of the first and second branchial arches and is characterized by branchial fistulae, congenital preauricular fistulae, and anomalies of the pinnae, external, middle, and inner ears, accompanied by hearing loss. Recently, we experienced a case of BOS in a 10 years old female patient and report this case with a review of literature. 10-year-old girl presented with hearing impairment, bilateral preauricular fistula and cervical fistula. The pure tone audiometry revealed that she had 60dB sensorineural hearing loss on right side and 90dB mixed hearing loss on left. Bilateral branchial fistula was found on the neck CT scan and bilateral ossicular and cochlear abnormality combined with enlarged internal auditory canal was noted on the temporal bone CT scan. To investigate the association with EYA1 gene, we performed DNA sequncing with peripheral white blood cell and found the point mutations on Exon 7, 12 and 16 of EYA1 gene. The preauricular fistula and branchial fistula was excised surgically and hearing aid was applied on her left side. There was no sign of fistula recurrence for seven years after the surgery.

Cutaneous Rosai-Dorfman Disease Confused with Vascular Mass

  • Kang, Kwang Rae;Jung, Sung Won;Koh, Sung Hoon
    • 대한두개안면성형외과학회지
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    • 제17권1호
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    • pp.31-34
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    • 2016
  • Rosai-Dorfman disease is a rare histiocytic disorder, clinically characterized by massive, bilateral painless cervical lymphadenopathy with potential for extranodal manifestations. We report a 45-year-old male patient who presented with a slowly growing erythematous nodule of the left chin. The mass appeared non-vascular on computed tomography study, but ultrasonogram was suggestive of a vascular lesion. The lesion was excised with presumptive diagnosis of a hemangioma. However, histopathologic examination of the surgical biopsy revealed histiocytic infiltration with emperipolesis, which was pathognomic for Rosai-Dorfman disease. Additional imaging studies did not reveal lymph node enlargement or other extranodal manifestation. The patient was diagnosed with cutaneous form of the Rosai-Dorfman disease and was discharged home. He remains free of local recurrence at 8 months.

Bilateral congenital alveolar synechiae-a rare cause of trismus

  • Panda, Smriti;Sikka, Kapil;Punj, Jyotsna;Sharma, Suresh C.
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제38권
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    • pp.8.1-8.4
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    • 2016
  • Congenital alveolar synechiae is a rare anomaly mostly presenting in association with cleft palate. Owing to reduced mouth opening, feeding difficulties, and compromised airway in extreme cases along with presentation in early neonatal period, these patients present unique challenges to the surgeon as well as the anesthetist. Here, we discuss the surgical and anesthetic management of this entity in a 12-month-old female child.