• 대한소아치과학회지
• /
• 제30권4호
• /
• pp.667-672
• /
• 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.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제22권1호
• /
• pp.63-69
• /
• 2000

Le fort II osteotomy is much useful technique to correct the midfacial hypoplasia including nasomaxillary complex especilly in patient with dish face appearance. Not in simple orthognathic surgery but in Le Fort II osteotomy, the standardization of prognostic value is essential in treatment planning to achieve satisfactory postoperative results. According to pervious reports, the ratio of soft tissue change to hard tissue movements varies as to different surgical methods and different facial regions. But there are few report about the ratio of soft tissue change to hard tissue movement following Le Fort II osteotomy. So we tried to develop standarized soft tissue surgical treatment objective. We have followed up 16 patients, who had received Le Fort II osteotomy by one operator from 1990 to 1996, one year postoperatively. In cephalometrics, we used Frankfort line as horizontal reference line, and vertical reference line as one drawn from Sella to horizontal line perpendicularly. The landmarks are G to soft tissue G, N on reference line to soft tissue N, ANS to Pn and A to Sn. The results are as follows. 1. The value of soft tissue change to hard tissue movement showed positive correlation, having statistical significancy at G, N2, N3 point. 2. At G, N2, N3 point, the ratio of soft tissue change to hard tissue movement was 0.51, 0.98 and 0.80 respectively and showed statistical significancy, while at N1, ANS, A point, that was 0.72, 0.49 and 0.26 but didn't showed statistical significance. 3. This result shows much the same change of the soft tissue change to hard tissue movement on the upper nasomaxilla, and less the same change on the lower maxilla and so the Le Fort II osteotomy can be recommended as a reliable effective operation method for correction of nasomaxillary retrusion.

• Journal of Plant Biology
• /
• 제3권2호
• /
• pp.29-34
• /
• 1960

From the view point of phytopathological anatomy, the author has tried to study the effect of the shoot cluster disease virus on the internal structure of vascular tissues of chinese date tree (Ziziphus jujuba var. inermis Rehd.) comparing healthy checks and diseased plants. The materials were collected at the several sites, Kumgock-Ri, Masuc-Ri, Kyungi-Do, and near the campus of Korea University and around the area of Chongam-Dong, Seoul City, from August 15th to September 5th 1959. The leaf materials of healthy and diseased plants are fixed and aspirated in two kinds of killing solutions, formalin-acetic acid alcohol solution and Craf III solution. Sections were cut at 5-10$\mu$ thickness and stained with the double staining reagents of safranin and fast green. In this experiment the author has observed that there are marked structural changes in the infected plants in contrast of healthy checks. As figures 3-7 show that the following characteric changes have taken place on infected plants: 1) the arrangement of irregularly developed sieve elements in phloem, 2) the degeneration of phloem elements, 3) the irregular arrangement of epidermis in mid-vein, 4) more necrosis is observed among the parenchymatous cells, 5) abundant accumulatin of starch grains in parenchymatous cells, . In contrast to the above irregularities caused by the virus disease, the healthy checks appear normal structures as shown in figures 1 and 2. In adding to the all features noted above, the author could also observe an interesting feature that the xylem elements in mid-vein vascular bundle tissues are considerably disorganized to show the unspecialized vessel elements, the irregularly arranged xylem elements. However, this kind of irregularities which occur in xylem under the virus infection has not been reported previously. The features noted on the internal structure of vascular bundle under the condition of infection by the shoot cluster disease on chinese date trees appear to be more or less closely similar to the symptoms of the bunchy-top of banana and the yellow dwarf disease of barley in respect to the fact that whether phloem necrosis takes place as a primary symptom or a secondary symptom. In all these disease, primary histological changes of hypoplasia and hypertrophy are preceeded by the necrosis of phloem.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제13권1호
• /
• pp.88-94
• /
• 1991

Le Fort II 골절단술 및 전방 이동술은 비상악골의 형성 부전증이나 짧은 코, 또는 상악골에 대비하여 상대적인 하악 전돌증 환자에 있어 고려 대상이 되어 왔다. 이러한 비상악골 형성 부전증의 원인으로는 외상, 토순 또는 구개열로 인한 발육부전, 선천성 매독증과 같은 감염 증상 등을 들 수 있다. Le Fort II 골절단술 및 전방 이동술은 1973년 Henderson 과 Jackson 에 의해 처음으로 보고되었고, 그 후 1980년 Steinhauser, Kinnebrew 등에 의해 변화되어 왔다. Le Fort II 골절단술에 있어서의 관상 절개술은 paranasal incision에 비해 안면부 술후 반흔이 남지 않아 심미적으로 우수하며, 두부의 상부 1/2까지 노출이 가능하여 수술시야가 좋으며, 안면 신경 손상 및 lacrimal apparatus 손상의 위험이 적다는 장점 이 외에도 nasofronatal osteotomy site와 pterygomaxiliary osteotomy site를 위한 bone graft의 donor site로서 skull bone을 immediate로 사용할 수 있다는 장점이 있다. 본 교실에서는 선천성 매독의 후유증의 하나인 비상악골 형성부전증을 가진 23세의 여자 환자에 있어서 관상 절개술을 통한 Le Fort II 골절단술을 통한 전하방 이동 및 하악지의 시상골 절단술을 시행하여 양호한 결과를 얻었기에 이에 보고하는 바이다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제34권4호
• /
• pp.460-467
• /
• 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.

• 대한구순구개열학회지
• /
• 제11권2호
• /
• pp.71-75
• /
• 2008

The outcome of primary surgery for cleft lip is judged by its effects on the quality of oro-facial function and development. Many surgical techniques have been tried to obtain better results, however, Delaire introduced a technique of functional closure of the lip and nose, based on the findings of no true hypoplasia in the tissues either side of the cleft. In a seven-month-old Asian male patient with unilateral incomplete cleft lip, we carried out the primary closure by modified Delaire's technique. With no alveolar bone graft, the vertical incision on the nasal base was omitted in this patient because of his acceptable symmetry of nose. Also, a small Z plasty was added on the non-cleft side. The V-shaped incisions, whose notch was located on each side of the red vermilion, were designed and beveled incisions were performed for the rehabilitation of lip length and thickness, considering the postoperative wound contracture. We assured that this modification of Delaire's technique could be applied for various cases of primary closure of incomplete cleft lip.

• 대한소아치과학회지
• /
• 제37권1호
• /
• pp.124-129
• /
• 2010

Seckel 증후군은 상염색체 열성 유전질환으로 태내에서의 성장지연에 따른 출생 후의 소인증, 심한 소두증을 나타내며, 큰 눈과 돌출된 코, 후퇴된 하악을 동반한 새 모양의 안모가 특징적이다. 다른 임상증상으로는 관절의 결함, 내반족, 드문드문 난모발, 비뇨생식기의 이형성, 정신지체, 혈액학적 이상 등이 나타날 수 있다. Seckel 증후군의 구강 내 소견으로는 부정교합, 법랑질 저형성증, 일부 치아의 선천적 결손, 우상치 등이 나타날 수 있다. 본 증례는 Seckel 증후군을 보이는 두 증례에서 발육부전, 새 모양의 안모, 비뇨기과적 기형, 치아의 저형성과 부정교합 등 다양한 전신증상과 구강 내 증상들을 보였기에 이를 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제39권4호
• /
• pp.317-319
• /
• 2006

대동맥궁의 형성 저하증과 비정형 대동맥궁 축착을 가진 한자에서 정중 흉골 절개술과 좌측 개흉술하에서 인공 심폐 바이패스 없이 상행 대동맥-하행 대동맥간 우회로 조성술을 시행하여 좋은 결과를 얻었기에 보고하는 바이다. 상행 대동맥하행 대동맥간 우회로 조성술은 복잡한 대동맥궁 재건술이 필요한 경우에 다른 술식으로서 고려될 수 있을 것이다.

• 대한두개안면성형외과학회지
• /
• 제12권2호
• /
• pp.102-106
• /
• 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.

• 대한두개안면성형외과학회지
• /
• 제12권2호
• /
• pp.125-128
• /
• 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.