• Title/Summary/Keyword: 안구돌출증

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CROUZON SYNDROME : CASE REPORT (Crouzon 증후군 환자의 증례보고)

  • Lee, Su-Jin;Kim, Young-Jae;Jang, Ki-Taek;Lee, Sang-Hoon;Kim, Chong-Chul;Hahn, Se-Hyun;Kim, Jung-Wook
    • Journal of the korean academy of Pediatric Dentistry
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    • v.36 no.1
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    • pp.133-138
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    • 2009
  • Crouzon syndrome is a rare disease, first decribed by Crouzon in 1912. This syndrome is cuased by mutations in the FGFR2 gene, which is mapped to chromosome locus 10q25-10q26. The condition occurs in about 1 of every 25,000 birth and is inherited as an autosomal dominant trait. We experienced a case of Crouzon's disease in a 9-year-old-female child. Physical examination revealed craniosynostosis, hypertelorism, exophthalmos, hypoplastic maxilla and a relative mandibular prognathism. The purpose of this study is to report the dental and medical characteristics of the patient and review the literatures of Crouzon syndrome.

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Midfacial distraction osteogenesis of Crouzon syndrome with RED(Rigid External Distraction) system (RED(Rigid External Distraction) system을 이용한 Crouzon syndrome환자의 distraction osteogenesis)

  • Lee, Yang-Ku;Choi, Jeong-Ho;Kim, Seok-Hwa;Baek, Seung-Hak;Chang, Young-Il
    • The korean journal of orthodontics
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    • v.32 no.3 s.92
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    • pp.175-183
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    • 2002
  • Crouzon syndrome Is one of the craniofacial syndromes characterized by craniosynostosis, midfacial hypolpasia and ocular proptosis. Distraction osteogenesis is becoming Important technique to treat craniofacial dyplaisa. It has nuy advantages compared with standard orthognathic surgery. Maxillary distraction osteogenesis after Le Fort III osteotomy with the RED system presents successful maxillary protraction to Crouzon syndrome patient with severe maxillary deficiency. It also allows remarkable improvement of facial esthetics and respiratory functions.

Experience of Single Stage Treatment of Caniosynostosis, Hypertelorism, Exophthalmos Patient (두개조기유합증, 안와격리증, 안구돌출증 환자의 동시 교정 치험례)

  • Chung, Chul Hoon;Eun, Seok Chan;Seo, Dong Kuk;Jo, Woo Sung;Park, Se Hyuck
    • Archives of Plastic Surgery
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    • v.33 no.2
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    • pp.237-240
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    • 2006
  • The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

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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Adenocarcinoma of Meibomian Glands and Moll Glands in the Eyelid of Canine (개의 안검에 존재하는 내검판선 및 외검판선에서 발생한 선암종)

  • Yuan, Dong-wei;Goo, Moon-Jung;Yang, Hai-jie;Hong, Il-Hwa;Ki, Mi-Ran;Park, Jin-Kyu;Lee, Hye-Rim;Yoo, Sung-Eun;Jeong, Kyu-Shik
    • Journal of Life Science
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    • v.18 no.3
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    • pp.414-417
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    • 2008
  • A 4-year-old female beagle with progressive exophthalmos and which had a neoplastic mass with diameter of 1.4 cm in the left lower ocular adnexa. Histologically, the mass was composed of hyper-plastic lobules and tubular structures separated by fibrous septum. The well differentiated sebaceous gland forming various sized lobules, and infiltration of mast cells and mononuclear inflammatory cells were observed. Apical decapitation secretion of these tubular structures with basophilic materials in their lumen showed mild sebaceous gland metaplasia. Immunohistochemical studying, cell groups were positive in ${\alpha}-SMA$ and vimentin. The primary tumor was diagnosed as adenocarcinoma originated from moll gland and meibomian gland of the eyelid, and the infiltrating intraocular neoplasm was diagnosed as a malignant mixed tumor.

Unbalanced Translocations of Chromosome 2 and Chromosome 20 in a Two-Generation Family (2대에 걸쳐 나타난 염색체 2번과 20번의 비균형적 전위 1례)

  • Min, Saeah;Lim, Seonwoong;Kim, Youngsook;Lee, Ohkyung
    • Clinical and Experimental Pediatrics
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    • v.45 no.7
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    • pp.917-922
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    • 2002
  • An unbalanced translocation is frequently the result of inheritance of an unbalanced haploid set from a parent with a balanced translocation. Families in which one parent is a balanced translocation carrier fall into the following classes : Those in which none of the possible abnormal offsprings is viable; Those in which one type of offspring, usually the one with the smaller deletion, is born alive; Those in which two types of abnormal offspring are viable. We report a neonate whose karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),der(20)t(2;20)(q21;p13). She was small for her gestational age and had multiple anomalies such as exophthalmos, corneal opacity, short neck, tongue tie, clinodactyly, atrial septal defect, patent ductus arteriosus and ventriculomegaly. Moreover, her mother's karyotype was 46,XX,der(2)t(2;7)(q21;p21.2),del(16)(q22.1),der(20)t(2;20)(q21;p13) but her father had normal karyotype. The same derivative chrosomes were found between mother and her infant, except for del(16)(q22.1) in her mother and these same unbalanced translocations in a two-generation family are extremely rare.

The infection of irido-like virus in cultured turbot (양식산 터봇, Scophthalmus maximus에서의 irido-like virus 감염)

  • Kim, Wi-Sik;Kim, Gi-Hong;Kim, Chun-Seop;Kim, Yeong-Jin;Jeong, Seong-Ju;Jeong, Tae-Seong;;;O, Myeong-Ju
    • Journal of fish pathology
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    • v.16 no.3
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    • pp.153-159
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    • 2003
  • The high mortality of cultured juvenile turbot, Scophthalmus maximus occurred in Gochang on June, 2003. The diseased fish was lethargic with reduced feed intake. Grossly, these fish showed pale body, abdominal extension and exophthalmia. The dominant internal gross features of diseased fish were severely enlarged spleen, pale gills and/ or liver. Diseased fish histologically showed basophilic enlarged cells in the kidney, spleen, gill, heart, stomach, intestine, liver, pancreas and adipose tissue. Transmission electron microscopy (TEM) reveled hexagonal virions in the cytoplasm of necrotic cells. The viral particles lead a central electron-dense core and an electron translucent zone, and were 136-159 nm in diameter. These results suggest that the virus belonging to the iridoviridae was responsible for the mortality of cultured juvenile turbot.

A Case of Mucocele in the Left Frontal Sinus (전두동 점액낭종)

  • 박병옥;노영식;소장영;김영길;김선우
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1981.05a
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    • pp.13.2-13
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    • 1981
  • Authors was experienced a case of mucocele in the left frontal sinus. A 24-years-old Korean male soldier was admitted to C.A.F.G.H. on 16th May 1980, with chief complaints of dull headache, exophthalmos, visual disturbance and intermittent epistaxis on the left side. On physical examination, left turbinates and septum were revealed hyperemic middle turbinate with blood tinged spots and deviated slightly to right side, and felt round, smooth, rubbery painless swelling within the orbit at the left inner canthus. The left orbital contents was displaced laterally producing proptosis with diplopia. On the radiological examination, Caldwell and Water's view showed hazy density in medial side of left orbital and left frontal sinus. Tomography of orbit showed hazy increased mass density with rather sharply defined outer margin of left bony orbit probably due to compressive erosion. The case was treated with surgical removal of the Lynch frontal approach, so present this case with a brief review of the literature.

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A Case of Congenital Progressive Bilateral Convergent & Divergent Strabismus with Unilateral Exophthalmus in Holstein Cattle (선천성 진행성 양측성 내외측성 사시 및 편측성 안구돌출증 홀스타인 1예)

  • Jung, Young-Hun;Hur, Tai-Young;Choe, Chang-Yong;Kang, Seog-Jin;Lee, Hyun-June;Ki, Kwang-Seok;Park, Yung-Sang;Suh, Guk-Hyun;Kim, Jong-Taek
    • Journal of Veterinary Clinics
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    • v.29 no.4
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    • pp.344-347
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    • 2012
  • Bilateral convergent strabismus with exophthalmus(BCSE) is an eye disorder affecting many cattle breeds worldwide. BCSE is the most common in cattle at various types of strabismus. Divergent strabismus is of relatively low incidence than convergent strabismus. This report is the to shown a case of convergent and divergent strabismus with unilateral in the exophthalmus in a heifer Holstein cow. A female Holstein calf born with congenital progressive divergent strabismus in right eye and convergent strabismus in left eye was tested. Ocular and blood examination, and activity were checked from the first week, 8 month, and 16 month after birth. The ocular examination includes general inspection, fixation reflex and menace response. The general inspection is checking the degree of deviation of both eyes from the normal visual axis, which was determined by the amount of sclera permanently visible in the temporal corner of the eye. The stage is divided into 4 stages depending on the degree of deviation. The right eye shown in stage 4 continues after birth and the left eye shown stage 2, stage 3 and stage 4 at 1 week, 8 month and 16 month after birth, respectively. In fixation reflex and Menace response, both eye balls showed a normal response at 1 week, 8 month and 16 month after birth. Blood count and serum chemistry test were performed, but a specific factor was not detected outside the reference range.