• Title/Summary/Keyword: Congenital malformation

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Open heart surgery of Ebstein's anomaly -Report of 12 cases- (Ebstein 기형에 대한 개심술 -12례 보고-)

  • Ahn, Hyuk;Park, Yee-Tae;Rho, Joon-Ryang;Suh, Kyung-Phill;Lee, Yung-Kyoon
    • Journal of Chest Surgery
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    • v.16 no.2
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    • pp.157-163
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    • 1983
  • Ebstein's anomaly is a rare congenital cardiac malformation oand the ideal surgical correction seems controversial at present, and some problems are left unsolved in the surgical correction of this anomaly. Between June 1978 and June 1982, 12 patients with Ebsteins' anomaly underwent corrective open heart surgery at Seoul National University Hospital. Except for one patient, who had no ASD, all had a huge right atrium, secundum type ASD, and definite atrialized right ventricle. Typically, displaced tricuspid valve leaflets were found in all cases, but the degree of displacement and deformity were variable. In the point of NYHA functional classification, five were in class II, six were in class III, and one was in class IV. Ten patients were operated on by tricuspid valve replacement and pication. Two patients were operated on only by plication and annuloplasty techniques, and in all cases, ASD was closed. Postoperatively, four patients suffered from complete A-V block, and two of them died immediately. The remaining two patients took pacemaker generator implantation with good results. The other eight patients were in good condition. Tricuspid valve replacement using tissue valve and plication of the atrialized rght ventricle seems to be a good method of surgical correction for Ebstein's anomaly.

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Simultaneous Repair of Unilateral Cleft Lip and Hard Palate with Vomer Flap : a Case Report (편측성 구순구개열 환자에 있어 구순성형술과 동반한 서골피판법 치험례)

  • Park, Hyong-Wook;Song, In-Seok;Kim, Eu-Gene;Kim, Soo-Ho;Cheon, Kang-Yong;Seo, Byoung-Moo
    • Korean Journal of Cleft Lip And Palate
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    • v.15 no.2
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    • pp.61-68
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    • 2012
  • Cleft lip and palate is the most common congenital facial malformation and has a significant developmental, physical, and psychological impact on those with the deformity and their families. When treating the patients with unilateral cleft lip, many surgeons adopt the rotation advancement flap method originally developed by Millard, or the triangular flap technique developed by Tennison, Randall or the modifications of these techniques. Among these, Millard's rotation advancement flap method has its advantage in designing the flap using the patient's anatomic landmarks. For performing this rotation advancement technique, skillful operation is needed to obtain esthetically satisfactory results. Vomer flap sometimes is used to repair anterior hard palate in complete cleft lip and palate patients. Vomerine tissue is readily available in the vicinity of the palatal defect and elevation of the vomerine flap is relatively simple procedure. In this article, we will introduce the comprehensive vomer flap technique conjunction with primary lip closure and review the comparative studies of the outcome of simultaneous repair of cleft lip and cleft hard palate with Millard's rotation advancement method and vomer flap.

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The Effect of Electromagnetic Fields Shielding on Electromagnetic Fields Decrease in P. T Room (차폐천이 물리치료실 환경내 전자기장 감소에 미치는 효과)

  • Lim Chang-Hun;Kim Jin-Sang
    • The Journal of Korean Physical Therapy
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    • v.12 no.2
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    • pp.69-82
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    • 2000
  • Physical therapists are exposured to radio-and microwave-frequency electromagnetic radiation by operating electrotherapy units. So there is few protection system in physical therapy room. Clinical pathology room and so on where various kins of electromagnetic instruments is used in hospital while protection failities like protection wall or protection glass is being used only in radiological room to reduce the damage of radiation. Acoording to Larsen's survey on female physical therapist in denmark. it was said that the percentage of congenital malfornation was $3.6\%$ and cadiac malformation made up $0.7\%$. It is likely that effect of electromagnetic fields on the result cannot be ruled out. Rita ouellet-Hellstron and Walter F. Steward insisted that the danger of abortion increase in the case of pregnant femeal physical therapist exposured to microwave diathermy. The intention of our study is arousing the necessity of microwave protection in P.T room and finding the proper method for physical therapist safe. The results of this study were as follows: 1. Each electrotherapy units are occurrenced the electromagnetic fields, and specially amply occurrenced in H.P,I.C.T 2 unit operating, M.W.D unit head on parallel, S.W.D unit head on parallel. all electrotherapy units are operating. 2. There were electric fields mount are consideration to species of electrotherapy units(p<.05). 3. There were magnetic fields mount are consideration to species of electrotherapy units(p<.05). 4. There were electric fields mount are consideration to distance of electrotherapy units(p<.05). 7. There were magnetic fields mount are consideration nut to distance of electrotherapy units(p>.05). 8. Before and after protection on magnetic fields mount are consideration to all distance(0m, 0.3m, 1m, 3m, 5m)(p<.05) 9. Before and after protection on electric fields mount are consideration to 0m, 1m, 3m distance(p<.05), and consideration not to 0.3m, 5m distance(p>.05) 10. After protection fellow the each electrotherapy units. distance, intencity to electromagnetic fields are reduced(p<.05).

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Development of Nurse's Handover Standards between Hospital Units using SBAR (SBAR를 이용한 간호단위간 인수인계 표준항목 개발에 관한 연구)

  • Kim, Eun Man;Yu, Mi;Ko, Ji Woon
    • Journal of Korean Clinical Nursing Research
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    • v.21 no.1
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    • pp.127-142
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    • 2015
  • Purpose: The purpose of this study was to develop the adapted standard items of situation, background, assessment, recommendations (SBAR) processing for handover between nursing units in Korean hospitals and evaluate the validity and relevance of the standard items. Methods: A delphi method with 33 experts was used to evaluate content validity of the standard items. Then, 1,175 nurses working in general hospitals of more than 500 beds were recruited to evaluate the validity and relevance of the standard items for clinical implication. Results: Content validity was higher than 0.8. The highest scores for relevance among items in handover standards were state of consciousness in the assessment domain for a ward to a ward transfer ($3.82{\pm}0.40$), for a ward to an ICU ($3.85{\pm}0.38$), an ICU to a ward ($3.81{\pm}0.39$) and an ER to a ward ($3.85{\pm}0.37$). Congenital malformation was the highest relevance score for handover from a delivery room to a neonatal unit ($3.91{\pm}0.30$). Conclusion: This study evaluated validity and relevance of the essential contents for handover standards between units to improve communication quality among nurses. The findings of this study should also be applied in clinical nursing areas and the quality of information and effectiveness of usage of the standard should be evaluated.

A STUDY ON THE CRANIOFACIAL GROWTH AND DEVELOPMENT IN KOREAN EMBRYOS AND FETUSES (한국인 태아의 악안면 성장 발육에 관한연구)

  • Kim, Cheol-Soo;Lee, Suk-Keun;Yang, Won-Sik
    • The korean journal of orthodontics
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    • v.20 no.3 s.32
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    • pp.427-446
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    • 1990
  • The objective of this study was to understand the major changes of craniofacial dimensions and spatial growth pattern during the late embryonic and fetal period of human fetures. This study was performed with the selective materials of normal fetuses received from the Registry of Congenital Malformation of Seoul National University Hospital. The specimens consisted of nineteen embryos and sixty-six fetuses. The photomicrographs from mid-segittal sections of embryos were used for angular measurement, and the lateral cephalograms taken with soft X-ray were also measured in liners and angular aspects. All of the anatomical landmarks for the tracing of the photomicrographs and cephalograms were referred to the previous reports on literature. The sequential changes of prenatal craniofacial dimensions and agles were analysed statistically and discussed on the focus about the developmental growth directions of human ore-facial structure arised from heterogeneous origins. The results are as follows, 1) Cranial base angle was almost formed at about 6 weeks old embryos with the average angle of $127.4{\pm}6.33^{\circ}$ (n=3) and it was almost constant onwards. 2) The linear increase rates of anterior cranial base length and anterior facial height exceeded those of the posterior cranial base length and posterior facial height, and the maxilla grows more rapidly on the horizontal dimension than the vertical dmension during the fetal period. 3) The angular relationship between the anterior cranial base and palatal plane decreasedslightly during the fetal period, disclosing $11^{\circ}$ at 12th week gestation and $5^{\circ}$ at 41th weeks gestation. 4) Genial angle was maintained almost constantly at about $130^{\circ}$ during the fetal period from 12 weeks to 41 weeks of gestation.

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A CASE REPORT ON PRIMARY INCISOR TREATMENT USING OPEN-FACE STAINLESS STEEL CROWN (Open-face Stainless Steel Crown을 이용한 유전치의 치료에 대한 증례보고)

  • Kim, Jong-Min;Choi, Hyung-Jun;Lee, Jae-Ho;Choi, Byung-Jae
    • Journal of the korean academy of Pediatric Dentistry
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    • v.24 no.4
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    • pp.781-787
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    • 1997
  • In spite of the improvements of the techniques in the field of preventive dentistry, many children still present with extensive destruction of primary anterior teeth. Not only the practioner must consider the pulp state of the primary incisor, but also restore the form, function and esthetics of the tooth. Restorative treatment of primary incisor tooth requires durability, retention and esthetics. Stainless steel crowns used in restoring primary anterior teeth is retentive and durable in comparison with the composite resin, celluloid crown. But they are not esthetic. To enhance the esthetics of the anterior stainless steel crown without reducing its superior retention, an open-face stainless steel crown has been suggested. Several authors have suggested cutting away the labial portion of the stainless steel crown and placing the composite resin in that area. By following this technique, the practioner can prepare a retentive, durable, and esthetic restoration for primary teeth which have suffered from extensive loss of teeth structure. In addition, the single missing primary anterior teeth can be successfully restored by soldering the stainless steel crown together. Open-face stainless steel crown is indicated in the areas of large interproximal lesions involving incisal edge, crown fracture with pulp exposure and congenital malformation of the teeth. By this technique, the practioner can restore primary anterior teeth successfully regardless of the amount of remaining tooth structure, bruxism habit and presence of attrition. In this case, rampant caries with extensive loss of tooth structure and single missing of primary anterior tooth hart been successfully treated with open-face stainless steel crown.

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

  • Lee, Hong-Mo;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Hahn, Se-Hyun;Kim, Chong-Chul
    • Journal of the korean academy of Pediatric Dentistry
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    • v.30 no.4
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    • pp.667-672
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    • 2003
  • Rieger syndrome is a rare, autosomal dominant genetic disorder characterized by malformation of the anterior chamber of the eye(goniodysgenesis) coincident with hypodontia. It may also be accompanied by a spectrum of dental, craniofacial and somatic anomalies. Mutations in paired-like homeodomain transcription factor2(PITX2) are associated with the syndrome, and its frequency in the general population has been estimated to be 1 : 200,000. In the present case, the patient, 4 year 7 month-old female, had posterior embryotoxon and polycoria. The maxilla was retrusive in cephalometric radiography. She had congenital missing on #52, #62 and some tooth germs of permanent tooth were not detected in panoramic radiography. The purpose of this paper is to report the dental and craniofacial findings and review the pertinent literature through this case.

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Dandy-Walker Syndrome with Submucous Cleft Palate: A Case Report (댄디워커증후군에 동반된 점막 밑 입천장갈림증 치험례)

  • Kwang, Bum Sik;Na, Young Cheon
    • Archives of Craniofacial Surgery
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    • v.13 no.1
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    • pp.54-56
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    • 2012
  • Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

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

  • Ryu, Sun-Youl;Kwon, Jun-Kyong;Kim, Sun-Kook
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.34 no.4
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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.

Tongue-Lip Adhesion Using an Alveolar Protector Appliance for Management of Pierre Robin Sequence (피에르 로빈 연속증의 치료로써 치조 보호 장치를 이용한 혀-하순 유착술)

  • Lee, Jang-Won;Park, Beyoung-Yun
    • Archives of Plastic Surgery
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    • v.38 no.4
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    • pp.547-551
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    • 2011
  • Purpose: Pierre Robin sequence is a congenital malformation in which micrognathia causes glossoptosis and airway obstruction. If conservative treatment fails, surgical procedures such as tongue-lip adhesion can be performed. However, this procedure remains a subject of debate, with favorable results being countered by reports of complications. To overcome the above limitations, we revised the traditional method of tongue-lip adhesion using an alveolar protector. Methods: Between 1992 and 2011, a total of eight patients were identified with Pierre Robin sequence and were treated with tongue-lip adhesion. Two of these eight tongue-lip adhesion procedures were performed with an alveolar protector. The operative technique for tongue-lip adhesion was similar to that described in other published reports. The alveolar protector was inserted between the ventral surface of the tip of the tongue and the lower labial sulcus. Results: Tongue-lip adhesion failed in two patients because of wound dehiscence. The primary surgical success rate was 66.7%. In the two tongue-lip adhesion procedures performed with the alveolar protector, we observed no postoperative complications. Conclusion: Resistance to traction of the tongue can be encountered with nonunionized symphysis menti, causing loosening of the traction suture through the symphysis menti. This can lead to backward positioning of tongue, resulting in dehiscence of tongue lip adhesion. The alveolar protector is a good adjunct to tongue-lip adhesion because this method avoids postoperative loosening of the traction suture and wound dehiscence. It is a simple and effective auxiliary method that yields functional improvement.