• Title/Summary/Keyword: congenital hypoplasia

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Surgical Experiences of Shone's Syndrome (숀 증후군의 외과적 치료)

  • Won, Tae-Hui;Lee, Jeong-Ryeol;Kim, Yong-Jin;No, Jun-Ryang
    • Journal of Chest Surgery
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    • v.30 no.9
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    • pp.862-868
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    • 1997
  • Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0$\pm$31.0 months ranged from 2 months to 1 1 years. The congenital mitral, stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have beeli followed from 11 months to 12 years(mean follow-up 6.7 $\pm$ 3.6 years). There was no late death and the New York HeArt Association activity level was class I for all patients. We conclude that a food lone-term outcome can be expected by proper surgical treatment tailred to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.

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CLINICAL STUDY OF AUGMENTATION MALARPLASTY WITH POROUS POLYETHYLENE (Porous polyethylene을 이용한 관골증대술의 임상연구)

  • Kook, Min-Suk;An, Jin-Suk;Kim, Young-Joon;Park, Hong-Ju;Oh, Hee-Kyun
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.30 no.3
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    • pp.283-291
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    • 2008
  • The malar mound defines the contour of the lateral face between the inferior orbital rim and the mandible, and hypoplasia or asymmetry of this region is readily noticeable. A flat, hypoplastic malar eminence can make the face blunt and wearisome, which contributes to a premature aged appearance. Patients with congenital or traumatic flattening of the malar eminence can obtain esthetic improvement with implants. Indications for placement of malar implants to improve the appearance of subtle flattening or to enhance the esthetic harmony of a patient's face have been suggested in several studies. Many augmentation materials, such as silicone, proplast, polyamide, and porous polyethylene implants have been used. Many methods of localization have been described, the key to proper placement of the implants lies in a through understanding of the esthetics of the malar mound. From August 2001 to June 2007, 12 patients with malar depression who visited the Department of Oral and Maxillofacial Surgery, Chonnam National University Hospital were treated by augmentation malarplasty with Porous polyethylene. The location and amount of augmentation are determined by preoperative interview, physical examinations, facial models and radiographic findings. 12 patients were satisfied with the results of augmentation malarplasty and severe complications were not occurred.

AXENFELD-RIEGER SYNDROME WITH DENTAL PROBLEM : A CASE REPORT (Axenfeld-Rieger Syndrome 환자의 증례보고)

  • Seong, Min-Kyoung;Shin, Teo-Jeon;Kim, Young-Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Kim, Chong-Chul;Hyun, Hong-Keun
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.8 no.2
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    • pp.113-117
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    • 2012
  • Axenfeld-Rieger syndrome (ARS) is a rare disorder with variable morphology characterized by malformations of the anterior segment of the eye such as iris hypoplasia, iridocorneal adhesions, corectopia and polycoria. Craniofacial and dental anomalies are also regularly reported in connection with ARS. Dental features include hypodontia in primary and permanent dentition, microdontia, short roots and abnormally shaped teeth. The patient in this case is a 14-year-old girl who has visited Seoul National Dental Hospital in 2002 for the first time. A panorama was taken on the day of her first visit, and total absence of maxillary anterior dentition and mandibular premolars were observed. The patient was on a treatment schedule by the department of ophthalmology at Seoul National Hospital for a morphological dyplasia of the pupil and she was diagnosed with ARS. Periodic panoramic observations were performed followingly, and she was finally diagnosed of congenital missing of maxillary anteriors and mandibular second premolars. In 2009, composite resin build-ups of maxillary primary central incisors were performed, and she has been on her regular follow-ups until now.

CLEIDOCRANIAL DYSPLASIA : REPORT OF A CASE (쇄골두개 이형성증 환자에 관한 증례보고)

  • Lee, Yeon-Ho;Yoo, Seung-Hoon;Kim, Jong-Soo
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.4
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    • pp.703-708
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    • 2005
  • Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

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DENTAL TREATMENT IN A PATIENT WITH PIERRE ROBIN SYNDROME UNDER GENERAL ANESTHESIA : A CASE REPORT (피에르 로빈 증후군 환아의 전신마취 하 치아우식 치료 증례 보고)

  • Ryu, Jiyeon;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Young Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Kim, Chong-Chul;Lee, Sang-Hoon
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.12 no.2
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    • pp.87-91
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    • 2016
  • Pierre Robin syndrome (PRS) is characterized by the triad of congenital mandibular hypoplasia, glossoptosis and cleft palate. Infant PRS patients are frequently suffering from upper airway obstruction, gastroesophageal reflux and growth retardation caused by above mentioned problems. We report a dental caries treatment of 3-year old girl with Pierre Robin syndrome with multiple caries. The cause of multiple caries was mainly presumed as patient's eating habit caused by her general condition. She had some feeding problems and had history of gastric tube. She was still using milk bottle and took more than an hour to finish a meal. The treatment was performed under general anesthesia considering patient's condition; mild autism, poor cooperation and respiratory problem due to micrognathia. Severely affected upper incisors were treated with pulp treatment and restored with zirconia crown for esthetic purpose. Lower incisors were treated with pulp treatment and restored with composite resin. Upper right first primary molar was restored with stainless steel crown and other primary molars were treated with composite resin. There were no postoperative complications. According to her parents, the patient's compliance to oral hygiene management was greatly improved after the treatment since she was very pleased with the esthetic result and highly motivated by her looks. The treatment without sedation or general anesthesia would be possible once the airway is improved as the mandible grows.

Mid-term Follow-Up Results of Cryopreserved Valved Conduit in RVOT Reconstruction (우심실 유출로에 사용된 냉동 동종 판막도관의 중기성적)

  • 장윤희;전태국;민호기;한일용;성기익;이영탁;박계현;박표원
    • Journal of Chest Surgery
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    • v.36 no.6
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    • pp.384-390
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    • 2003
  • Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.

Clinical Evaluation of Patients with Unilateral Non-functioning Kidney on $^{99m}Tc-DMSA$ scan ($^{99m}Tc-DMSA$ 스캔에서 일측성으로 동위원소 섭취가 안되는 환아들에 대한 임상적 고찰)

  • Bae Hyun-Chul;Kim Hyun-Young;Kim Pyung-Kil;Han Sang-Won
    • Childhood Kidney Diseases
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    • v.1 no.1
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    • pp.31-37
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    • 1997
  • The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.

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