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1/42. Neuro-ophthalmic complication after maxillary surgery.

    We report an unusual case of ophthalmoplegia after maxillofacial surgery. A thirteen year old girl with unilateral left cleft lip and palate underwent maxillary advancement by distraction osteogenesis. Postoperatively she developed diplopia. The diplopia was not due to orbital lesions, most often seen after maxillofacial surgery, but to a haemorrhage posterior to the cavernous sinus. ( info)

2/42. Facio-genito-popliteal syndrome presenting with bilateral choanal atresia and maxillary hypoplasia.

    The most classic characteristics of facio-genito-popliteal syndrome are cleft lip and/or palate, anomalies of the external genitalia and popliteal pterygium. A case of facio-genito-popliteal syndrome with bilateral choanal atresia and maxillary hypoplasia is presented. No previous report of such an association was found in the literature. ( info)

3/42. Assessment of patients for orthognathic surgery.

    Rapid advances in orthognathic surgery now allow the clinician to treat severe dentofacial deformities that were once only manageable by orthodontic camouflage. These cases were often compromised with unacceptable facial esthetics and unstable occlusal results. Over the past 25 years, there have been numerous improvements in technology and the surgical management of dentofacial deformities. These progressions now allow more predictable surgical outcomes, which ensure patient satisfaction. Not all patients are candidates for surgical treatment; therefore, patient assessment and selection remains paramount in the process of diagnosing and treatment planning for this type of irreversible treatment. The inclusion of patients in the decision-making process increases their awareness and acceptance of the final result. The past three decades indicate an increased usage of orthodontic treatment by both children and adults. Patient demographic profiles for severe occlusal and facial characteristics are presented in an effort to understand the epidemiological factors of malocclusion and predict the population's need for this service. ( info)

4/42. Endoscopic treatment of sinonasal disease in patients who have had orthognathic surgery.

    Certain skeletofacial patterns may be predisposed to aggravated sinonasal disease postoperatively. These may include, but are not limited to, facial skeletal asymmetries with high septal deviations and those with obstructive nasal respiration and mouth breathing that leads to skeletal growth disturbances such as vertical maxillary hyperplasia and apertognathism. These sinonasal diseases may partly be the result of osteomeatal blockage by pre-existing structures, or synechial shelves and webs blocking normal maxillary antral mucosal flow. The use of nasal antral windows placed anteriorly in the lateral nasal wall at the time of downfracture LeFort (Hosaka window) do not seem to benefit the drainage of the maxillary antrum. This is because physiological flow often bypasses this region. If patients present postoperatively with new sinonasal disease or the aggravation of pre-existing symptoms, evaluation by both endoscopically assisted intranasal and axially and coronal computed tomography (CT) is recommended. Functional endoscopic sinus surgery by the minimally invasive Messerklinger technique, combined with intranasal use of laser-assisted turbinoplasty and soft tissue lysis, have been successfully used for most of these patients. Because the anatomical positioning of the midfacial structure can potentially affect patients with a predisposition to sinonasal physiological disturbances, consideration should be given to preoperative evaluation and discussion of potential consequences. ( info)

5/42. Multiple odontogenic keratocysts in mental retardation-overgrowth (Simpson-Golabi-Behmel) syndrome.

    We report on a 10-year-old boy with mental retardation-overgrowth (Simpson-Golabi-Behmel) syndrome. The child had the typical clinical features including, postnatal overgrowth, mental retardation, and a characteristic facial appearance. He was admitted for treatment of multiple mandibular and maxillary cysts. Histopathological examination of the cyst tissue showed keratinized epithelium. Odontogenic keratocysts may have to be added to the typical features of this syndrome. ( info)

6/42. Bimaxillary orthognathic surgery in a patient with long face: a case report.

    The primary characteristics of long face patients are gummy smile and/or anterior open bite. Consequently, correction of esthetic and functional problems are especially important for long face patients. Since orthodontic therapy alone is not sufficient to solve the problem, orthognathic surgical approach is indicated for these patients. In this report, orthognathic surgical therapy of a severe long face patient with similar findings was presented. Following clinical and cephalometric examination and preoperative orthodontic therapy, a Le Fort I osteotomy, a bilateral sagittal split osteotomy, and vertical and horizontal reduction genioplasties were performed. Alternative surgical therapies, complications, and the effects on the upper respiratory tract are also discussed. ( info)

7/42. Distraction osteogenesis of the maxilla and midface using a subcutaneous device: report of four cases.

    The use of distraction osteogenesis in the hypoplastic maxilla and midface is still controversial. Since the beginning of 1998, 25 patients have been treated with osteodistraction techniques for various reasons. Among them were four patients who were treated by high LeFort I osteotomies and insertion of a newly developed subcutaneous distraction device in the malar region. Distraction osteogenesis was successful in all four cases resulting in a mean sagittal bone gain of 12.0 mm (range 7-14) at the level of distractor fixation. All patients were kept under orthodontic supervision during osteodistraction. The final occlusal relation was satisfactory. Cephalometric measurements after distraction showed an anterior rotational movement of the midface region. As the question of relapse and further growth is still not clear, Delaire masks are used to stabilize the surgical result after removal of the distractor. The importance of long-term follow-up is stressed. ( info)

8/42. Bilateral Tessier no. 4 facial cleft with left eye anophthalmos: a case report.

    Craniofacial clefts are very rare and manifest in a variety of patterns. Tessier classified these clefts in 1973 and numbered them 0 to 14. Tessier No. 4 Facial cleft is a rare variant of craniofacial cleft. Not more than 50 cases are reported in world literature, amongst which only 5 cases are true bilateral in nature. However, combinations of two different variants are not very uncommon. A case of male Indian child aged 4 years with a true bilateral Tessier No. 4 Facial cleft is reported. He also had anophthalmos of the left eye. This is probably the first case in which true bilateral Tessier No. 4 Facial cleft with anophthalmos of one eye is noticed. ( info)

9/42. prenatal diagnosis of partial monosomy 18p(18p11.2-->pter) and trisomy 21q(21q22.3-->qter) with alobar holoprosencephaly and premaxillary agenesis.

    A prenatal diagnosis of partial monosomy 18p(18p11.2-->pter) and trisomy 21q(21q22.3-->qter) in a fetus with alobar holoprosencephaly (HPE) and premaxillary agenesis (PMA) but without the classical down syndrome phenotype is reported. A 27-year-old primigravida woman was referred for genetic counselling at 21 weeks' gestation due to sonographic findings of craniofacial abnormalities. Level II ultrasonograms manifested alobar HPE and median orofacial cleft. cytogenetic analysis and fluorescence in situ hybridization (FISH) on cells obtained from amniocentesis revealed partial monosomy 18p and a cryptic duplication of 21q,46,XY,der(18)t(18;21)(p11.2;q22.3), resulting from a maternal t(18;21) reciprocal translocation. The breakpoints were ascertained by molecular genetic analysis. The pregnancy was terminated. autopsy showed alobar HPE with PMA, pituitary dysplasia, clinodactyly and classical 18p deletion phenotype but without the presence of major typical phenotypic features of down syndrome. The phenotype of this antenatally diagnosed case is compared with those observed in six previously reported cases with monosomy 18p due to 18;21 translocation. The present study is the first report of concomitant deletion of HPE critical region of chromosome 18p11.3 and cryptic duplication of a small segment of distal chromosome 21q22.3 outside down syndrome critical region. The present study shows that cytogenetic analyses are important in detecting chromosomal aberrations in pregnancies with prenatally detected craniofacial abnormalities, and adjunctive molecular investigations are useful in elucidating the genetic pathogenesis of dysmorphism. ( info)

10/42. Associated orthodontic, surgical and hematological management of Cooley s anemia. Report of a case.

    Cooley s anaemia or b-thalassaemia or mediterranean anaemia is a blood disease characterised by malformation of the skull and long bones, which confers a typical appearance on the patient. The complete development of facial abnormalities can be prevented by an intensive blood transfusion programme or by bone-marrow-transplantation. At the present time these therapeutic strategies would be able to help these patients grow and develop, live a prolonged life and avoid bad surgical RESULTS. The aim of this study was to evaluate the feasibility of orthodontic and maxillofacial surgical corrective treatment associated with an appropriate transfusion therapy in a b-thalassemic patient. The patient enrolled in the study was affected by major b-thalassemia and diagnosis was performed as an infant. She was referred to our centre at puberty for dento-maxillofacial disorders. Clinical, haematological and radiographic evaluation permitted a complete diagnosis. She received a combined haematological, orthodontic and maxillofacial surgical treatment. Controls for evaluating the statement of results obtained were performed at different times after the end of the therapy and have shown that the therapeutic objectives had been achieved and maintained. At the present time, complete diagnostic and therapeutic haematological strategies cannot be carried out on a large scale, especially in countries where health resources are limited. This objective reason, associated with possible low patient compliance, explains why we still encounter severe facial deformities resulting from erythroid hyperplasia. Our results suggest that this facial disfigurement requires surgical and orthodontic correction by consolidated surgical-orthodontic techniques performed according to the appropriate anaemia therapy. Although this is a preliminary study, initial encouraging results show no relapse three years after the end of the therapy. ( info)
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