• Title/Summary/Keyword: 발육부전

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APERT SYNDROME : A CASE REPORT (Apert syndrome 환자의 제증상에 관한 증례보고)

  • Song, Soo-Bok;Kim, Jung-Wook;Kim, Chong-Chul
    • Journal of the korean academy of Pediatric Dentistry
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    • v.29 no.1
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    • pp.44-50
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    • 2002
  • Apert syndrome is a kind of congenital-acrocephalosyndactyly syndrome which was first reported by Apert in 1906 and characterized by its acrocephaly and syndactyly. Clinical characteristic features are cone-shaped skull morphology due to early fusion of coronal suture, fusion of fingers of hands and toes of feet. It is an autosomal dominant-heritable syndrome. Due to hypo-development of midface region, Apert syndrome patients have a tendency to have ocular proptosis, hypertelorism, maxillary deficiency. High palate and soft palate cleft are common findings in these patients. In general, mandibular growth pattern is normal, but relative maxillary deficiency exaggerates mandibular forward position, so relative mandibular prognathism is inevitable. Narrow maxillary and mandibular dental arch worsen teeth alignment and crowding. Skeletal malocclusion and open bite are also common. This is a case report of a Korean 3 year 1 month male Apert syndrome child referred by department of plastic surgeon for the possibility of orthodontic treatment. General features of Apert syndrome, patient's medical history, radiographic evaluation, clinical examination, orthodontic and surgical treatment planning are discussed in this report.

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A CASE REPORT OF SURGICAL CORRECTION OF NASOMAXILLARY HYPOPLASIA DUE TO CONGENITAL SYPHILIS BY LE FORT II OSTEOTOMY WITH CORONAL APPROACH (선천성 매독에의한 비상악골 부전증환자의 관상두피 접근법에 의한 Le Fort II 골절단술을 이용한 치험례)

  • Um, In-Woong;Kim, Chang-Soo
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.13 no.1
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    • pp.88-94
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    • 1991
  • Maxillofacial deformities are not considered to be a trouble in social life but function. So many maxillofacial plastc surgeons has made efforts to overcome these troubles and bring out more positive life. The proper proportion and shape decide esthetic quality. Lower third of face was consist with lip, cheek, mandibular lower border and mandibular angle. Widening lower third of face give a impression with muscular and recklessness. And lower and wide mandibular angle makes face square shape. Unilateral involvement cause asymmestric face. These face is considered unfavorable, especially in Korea or Japan. We prevent a number of with mandibular angle Bulging which was corrected with mandibular osteotomy or masseter myotomy.

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A Clinical Report of 9 Cases of Congenital Thyroid Dysgenesis (선천성 갑상선 발육이상 9례(例)에 대한 보고)

  • Lee Samuel;Lee Seug-Zae;Lee Hyouk-Jin;Chon Seong-Eun;Park Yoon-Kyu
    • Korean Journal of Head & Neck Oncology
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    • v.10 no.2
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    • pp.206-211
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    • 1994
  • Congenital thyroid dysgenesis including agenesis, hypoplasia and ectopia is the predominant cause of permanent hypothyroidism. Of these, two thirds are due to an ectopic thyroid and about one third to complete thyroid agensis. From Jan. 1981 to Dec. 1992, authors experienced the 9 cases of congenital thyroid dysgenesis. Aberrent thyroid was 4 cases (44.4%), thyroid hemiagenesis with aberrent thyroid was 3 cases(33.3%) and thyroid hemiagenesis was 2 cases(22.2%). The most predominant site of aberrent thyroid is the base of tongue(85.7%). 7 patients(77.8%) revealed euthyroidism and among them, 4 patients showed elevated TSH level. Hypothyroidism was 2 patients (22.2%). 7 cases responded to thyroid suppressive therapy. 2 cases of lingual thyroid which did not responed to thyroid suppressive therapy underwent surgery and they have placed on thyroid suppressive therapy postoperatively.

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A CASE REPORT OF SURGICAL CORRECTION OF MIDFACIAL DEFICIENCE BY SUBCRANIAL LE FORT III OSTEOTOMY (두개하 Le Fort III 골절단술에 의한 중안면 발육부전 환자의 치험례)

  • Lee, Sang-Chull;Kim, Yeo-Gab;Ryu, Dong-Mok;Lee, Baek-Soo;Choi, You-Sung
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.18 no.1
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    • pp.69-77
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    • 1996
  • The midfacial deficiency is usually accompanied with congenital craniofacial synostosis, such as Crouzon, Apert, Pfeiffer, Carpenter, Saethre-Chotzen syndrome, and so on. But sometimes isolated midfacial deficiency without cranial malformations may appeared, the cause of which is congenital, hereditary, or secondary to developmental factors, such as infection and trauma to middle face. Since Sir Harold Gillies reposted the first high maxillary osteotomy that alleviated the problems of total midfacial deficiency, the various operative methods were developed by many clinicians, such as Longacre and Tessier. These procedures can enlarge the orbital volume and decreases exorbitism. As middle face was moved forward, these functional, esthetic, and psychologic advantages were resulted from this. This is a case of midfacial deficiency corrected by the subcranial Le Fort Ⅲ osteotomy through only coronal approach.

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DEVELOPMENTAL DISTURBANCES OF SUCCESSIVE PERMANENT TEETH BY TRAUMA TO THE PRIMARY TEETH (유치의 외상에 의한 계승 영구치의 발육 장애)

  • Kim, Ji-Young;Kim, Young-Jin;Kim, Hyun-Jung;Nam, Soon-Hyeun
    • Journal of the korean academy of Pediatric Dentistry
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    • v.37 no.2
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    • pp.260-266
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    • 2010
  • Trauma to the primary teeth may have an effect on the successive permanent teeth. Thus, the treatment plans should be focused on safety of their permanent teeth. The degree of developmental disturbances in those permanent teeth depends upon many factors including the age of the children, developmental conditions of the affected teeth, types and severities of trauma and time interval from the moment of trauma to the initial treatment the patients received. The complications of the effected permanent teeth vary from the mild enamel hypoplasia to the premature finishing of teeth development. In this study, children whose ages were 33 months, 5 years, and 26 months presented the developmental disturbances to their permanent teeth, which resulted from the trauma to the primary teeth: the intrusion, the avulsion, and the subluxation. There showed a mild complication in the case of subluxation resulting in enamel hypoplasia, but, the more severe complication of the root dilacerations occurred in the case of intrusion. Furthermore, the 5-year-old patient whose primary dentition was near in transition period to the permanent one, the more complicated problem such as stop of root development of the permanent teeth was resulted from the avulsion. Thus, one should assume that the types of trauma and the age of the children at the moment of trauma have different effects on those successive permanent teeth. In conclusion, when the trauma that causes changes in the position of primary teeth happened, it is important to arrange an appropriate treatment procedure considering the types of trauma and the developmental conditions of the permanent teeth.

UNERUPTED PRIMARY MOLARS (미맹출 유구치의 치험례)

  • Kang, Sun-Hee;Yang, Young-Sook;Lee, Kwang-Hee
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.2
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    • pp.217-223
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    • 2005
  • The term 'impaction teeth' is used to designate a tooth which remains unerupted in jaw beyond the time at which it should normally be erupted. Most cases of impacted teeth reported in the literature are permanent teeth. The impaction of primary teeth occur rarely whereas impaction of second primary molars is more numerous than all other impactions. Failure of eruption of primary teeth may cause a number of complications, such as interference with development and eruption of successive permanent teeth, malocclusion, cystic change of tooth follicle. The clinican should consider the various treatment option available (a) No treatment and observation, (b) surgical extraction (c) space regainer. Proper treatment plan should be established after thought consideration of impacted tooth and it's relation with successive permanent tooth.

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DENIAL CHARACTERISTICS OF PSEUDOHYPOPARATHYROIDISM : CASE REPORT (가성부갑상선기능저하증 환아의 구강내 증상에 관한 증례보고)

  • Chang, Ji-Young;Lee, Jae-Ho;Choi, Byung-Jai;Lee, Jong-Gap
    • Journal of the korean academy of Pediatric Dentistry
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    • v.26 no.3
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    • pp.486-491
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    • 1999
  • Hypoparathyroidism has abnormally decreased secretion of parathyroid hormon which responds to the blood calcium level. Wherease, Pseudohypoparathyroidism has normal activity of thyroid hormon, but end-organs, such as urinary tract and osteoclast, do not respond to parathyroid hormon. The cause of this disease is due to the mutation of Guanine stimulating(Gs) protein regulating Gs gene, which is the receptor to this hormon. Pseudohypoparathyroidism is usually noted before 20 years old on average of 8-9 years old. The clinical features of this disease includes delayed growth and development, round face, obesity, soft tissue calcification, ectopic ossification, shortening of metacarpals and metatarsals by epiphyseal closure in advance of age. The mutation of Gs gene which are found in brain, endocrine organs, and chondrocytes is the cause of those features. Reaction to Glucagon, gonadal hormon, and thyroid stimulating hormon is not expected in both cases. The common dental manifestations include enamel hypoplasia, delayed eruption, agenesis of tooth, hypodontia, dysplastic short roots, widened pulpal space, microdontia, intrapulpal calcification, and malocclusion are also often reported. This case which is diagnosed to Pseudohypoparathyroidism showed short and under-developed root of permanent troth, delayed eruption, and non-eruption of premolars and molars. And morphogenesis imperfecta of first and second premolars were also found.

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Surgical Correction of Tetralogy of Fallot in Adults over 20 Years of Age (20세이상 성인 팔로사징후의 완전교정술에 관한 임상적 고찰)

  • 류완준;강종렬;조창욱;김정철;구본일;이홍섭;김창호;우건화;이신영
    • Journal of Chest Surgery
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    • v.29 no.11
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    • pp.1197-1201
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    • 1996
  • From October, 1987 to December, 1995, nine patients underwent total correction of tetralogy of Fallot in adults over 20 years of age. There were 5 male and 4 female patients aged from 22 to 42 years(mean, 29.6 years). Three patients were in New York Heart Association(NYHA) functional class II, and 6 patients in class III. The hemoglobin values ranged from 10.8 to 20.7 gm/㎗ (average, l5.6gm/㎗). The preoperative clinical features were as follows: cyanosis, 8 patients; dyspnea on exertion, 6 patients; clubbing of fingers, 5 patients; frequent upper respiratory infection, 3 patients. At the operation both infundibular and valvular stenosis were present in all patients. Reconstruction of right ventricular outflow tract(RVOT) using Goretex was required in 7 patients, and transannular patching with Goretex in 2 pateints. Left pulmonary angioplasty with pericardium was done in 2 patients. No hospital deaths occurred. Four of 9 patients(44.4%) had postoperative low cardiac output syndrome, and postoperative bleeding in 5. One patient required reoperation due to residual ventricular septal defect and tricuspid regurgitation 3 months after the first operation. The mean follow-up period was 25 months, range 11 to 77 months. All was asymptomatic and in NYHA class I. We suggest that advanced age is not contraindication to surgery in tetralogy of Fallot, and tetralogy of Fallot in adults could be operated on due to low mortality.

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Modified Norwood Procedure without Circulatory Arrest and Myocardial Ischemia - Report of 2 cases - (완전순환정지와 심근허혈 없이 시행한 변형 Norwood 술식 - 2 례 보고 -)

  • 백만종;김웅한;전양빈;김수철;공준혁;류재욱;오삼세;나찬영;김양민
    • Journal of Chest Surgery
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    • v.34 no.7
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    • pp.547-551
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    • 2001
  • The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.

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