• Title/Summary/Keyword: Midfacial deficiency

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Long-term Orthodontic Treatment and Phonetic Assessment of a Congenital Cleft Lip and Palate Patient (선청성 구순구개열 환자의 장기적인 교정 치료 및 음성언어학적 고찰)

  • Hong, Ji-Suk;Kim, Yoon-Ji;Hwang, Yong-In;Kim, Dae-Sung;Park, Yang-Ho
    • Korean Journal of Cleft Lip And Palate
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    • v.12 no.1
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    • pp.7-20
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    • 2009
  • Orthodontic treatment planning of cleft lip and palate requires consideration of the characteristic features, growth pattern and functional disorders related to cleft lip and palate patients. Tissue deficiencies and constriction of the scar tissue in surgically treated cleft lip and palate results in disturbance of maxillary growth and deficiency of midfacial region with anterior and posterior crossbite. These patients often present congenital missing of teeth, supernumerary teeth, malformed teeth, or ectopic position of teeth, which should be treated by orthodontic treatment by expanding upper arch followed by fixed appliance. Proper use of retainer and continuous follow-up is needed to prevent relapse after orthodontic treatment has finished. Also we have to pay attention to correct speech disorder which is caused by the velopharyngeal insufficiency.

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A Case of Schinzel-Giedion Syndrome (Schinzel-Giedion 증후군 1례)

  • Jeoung Min-Jee;Yim Hyung-Eun;Hong Young-Sook;Lee Joo-Won;Kim Soon-Kyum;Yoo Kee-Hwan
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.57-62
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    • 2004
  • Schinzel-Giedion syndrome is a rare, distinct dysmorphic syndrome characterized by congenital hydronephrosis, skeletal dysplasia, and severe developmental retardation, likely to be inherited as an autosomal recessive trait, but not yet confirmed. This syndrome is characterized by coarse facial features such as midfacial retraction, bulging forehead, short nose with anteverted nostrils, low-set malformed ears, protruding large tongue, and hypertelorism. Skeletal and limb defects, choanal stenosis, simian creases, hypospadias, microphallus, hypertrichosis, and intractable seizures are the frequently associated clinical findings. Urogenital involvement is a major component of the syndrome, and this problem sometimes is associated with nephrocalcinosis and urinary tract infection in the clinical course of the disease. We report a 22 month-old girl with Schinzel-Giedion syndrome complicated by medullary nephrocalcinosis and urinary tract infection due to Klebsiella pneumoniae. This patient had also been suffering from postnatal growth deficiency, intractable seizure, spastic tetraplegia, delayed development and severe mental retardation.

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COMPLEX DISTRACTION OSTEOGENESIS ON HEMIMANDIBULAR HYPOPLASIA : A CASE REPORT (하악과두 골절후 발생한 편측성 하악골 형성부전의 치료로서 복합적 악골 신장술의 임상증례)

  • Oh, Sung-Hwan;Min, Seung-Gi;Kwon, Kyong-Hwan;Koh, Se-Wook;Lee, Kyong-Seuk
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.30 no.3
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    • pp.246-250
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    • 2004
  • Uni- or bilateral mandibular hypoplasia can be associated with various syndromes or is acquired after early traumatic or inflammatory disease in the temporomandibular joint(TMJ). Early treatment is necessary to avoid consequent impairment of midfacial growth. The standard treatment of these malformations consists of the application of bone grafts which can lead to unpredictable growth, but the new procedure of bone lengthening which was presented by McCarthy et al. represents a limited surgical intervention and therefore open up a new perspective of treatment, especially in younger children with severe deformities. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, a tilted occlusal plane, and a short mandible. A 14-years-old boy with facial asymmetry, who was fractured on both condyle and mandibular symphysis before 8 years ago, was treated by mandibular ramus lengthening, symphysial widening and surgically assisted rapid palatal expansion with corticotomy. After allowing 1 week for the healing of the periosteum, the distraction was performed at the rate of 0.5-1.0mm per day for 7 days on maxilla and 14 days on mandible. The device was maintained on maxilla and mandible for 12 weeks following distraction. The difference in ramus and mandibular transverse deficiency were corrected and facial asymmetry was improved with complex distraction osteogenesis.

THE USE OF DISTRACTION OSTEOGENESIS TO TREAT HEMIFACIAL MICROSOMIA: A CASE REPORT (반안면 왜소증 환자에서의 골신장술: 증례보고)

  • Baik, Sung-Mun;Kim, Su-Gwan;Kim, Hak-Kyun;Moon, Seong-Yong
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.33 no.5
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    • pp.559-566
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    • 2007
  • Distraction osteogenesis(DO) is a surgical method of bone formation that involves an osteotomy and sequential stretching of the healing callus by gradual movement and subsequent remodeling. DO is used to correct facial asymmetry, such as in patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip and palate, alveolar defects, and craniofacial deficiency. It is accomplished with the aid of a distraction device, which is secured with screws placed directly into bone, for a predetermined length of time. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, tilted occlusal plane, and short mandible. Early treatment is necessary to avoid subsequent impaired midfacial growth. The standard treatment of these malformations consists of the application of bone grafts, which can lead to unpredictable growth. The new bone-lengthening procedure represents a limited surgical intervention and opens up a new perspective for treatment, especially in younger children with severe deformities. This report describes a case of hemifacial microsomia(Type-II left-sided hemifacial microsomia). The patient, a 10-year-old child, visited our clinic for facial asymmetry correction. He had a hypoplastic mandible, displaced ear lobe, 10 mm canting on the right side, and malocclusion. We planned DO to lengthen the left mandible in conjunction with a Le Fort I osteotomy for decanting and then perform a right intraoral vertical ramus osteotomy(IVRO). Progressive distraction at a rate of 0.5 mm/12 hours was initiated 7 days postoperatively. The duration of DO was 17 days. The consolidation period was 3 months. Satisfactory results were obtained in our case, indicating that DO can be used successfully for functional, aesthetic reconstruction of the mandible. We report a case involving DO in conjunction with orthognathic surgery for correcting mandibular hypoplasia with a review of the literature.