Cases reported "Micrognathism"

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1/21. Treatment planning and biomechanics of distraction osteogenesis from an orthodontic perspective.

    As in traditional combined surgical and orthodontic procedures, the orthodontist has a role in the planning and orthodontic support of patients undergoing distraction osteogenesis. This role includes predistraction assessment of the craniofacial skeleton and occlusal function in addition to planning both the predistraction and postdistraction orthodontic care. Based on careful clinical evaluation, dental study models, photographic analysis, cephalometric evaluation, and evaluation of three-dimensional computed tomographic scans, the orthodontist, in collaboration with the surgeon, plans distraction device placement and the predicted vectors of distraction. Both surgeon and orthodontist closely monitor the patient during the active distraction phase, using intermaxillary elastic traction, sometimes combined with guide planes, bite plates, and stabilization arches, to mold the newly formed bone (regenerate) while optimizing the developing occlusion. Postdistraction change caused by relapse is minimal. growth after mandibular distraction is variable and appears to be dependent on the genetic program of the native bone and the surrounding soft tissue matrix. A significant advantage of distraction osteogenesis is the gradual lengthening of the soft tissues and surrounding functional spaces. Distraction osteogenesis can be applied at an earlier age than traditional orthognathic surgery because the technique is relatively simple and bone grafts are not required for augmentation of the hypoplastic craniofacial skeleton. In this new technique, the surgeon and the orthodontist have become collaborators in a process that gradually alters the magnitude and direction of craniofacial growth.
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keywords = craniofacial
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2/21. Mandibular distraction osteogenesis in a neonate.

    Children with craniofacial anomalies are predisposed to airway obstruction and frequently require airway intervention. tracheotomy is performed when the airway obstruction is severe and refractory to other less invasive interventions. tracheotomy is associated with significant morbidity, and there is a trend noted in the literature toward achieving earlier decannulation by the institution of definitive structural changes to the mandible. Mandibular distraction osteogenesis has been shown to alleviate airway obstruction in the pediatric population. We report a case in which mandibular distraction osteogenesis was successfully carried out in a neonate with acute airway obstruction at birth as a result of combined Pierre Robin sequence and klippel-feil syndrome. After 1 year, the patient still had an adequate airway with tolerable scarring and no neurologic sequelae.
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ranking = 0.33333333333333
keywords = craniofacial
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3/21. Complete bony fusion of the mandible to the zygomatic complex and maxillary tuberosity: case report and review.

    Congenital craniofacial disorders represent approximately 20% of all birth defects. One of these disorders is syngnathia, of which only 24 cases have been reported since 1936. Twenty cases involved fusion of the alveolar processes of the maxilla and mandible. Only four are similar to the presented case, which includes bony fusion of the ascending ramus of the mandible to the zygomatic complex and the posterior part of the maxilla. This case report will present details from the 23rd week of gestation to 8 months of age when the infant underwent the first attempt to free the syngnathia. The literature is discussed and a causative mechanism and new classification are proposed.
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keywords = craniofacial
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4/21. cleft palate in a patient with Williams' syndrome.

    cleft lip or palate has not been reported in the medical literature as a part of Williams' syndrome. We present a patient who had cleft palate among other congenital manifestations. This patient's immediate postnatal period clinically seemed to have a Pierre Robin sequence. With the development of the craniofacial complex, microgenia and micrognathia with glossoptosis gradually became apparent. On further assessment, the patient showed other clinical findings that suggested a syndromic association. This required a complete evaluation to discard other conditions that present with low psychomotor development and distinctive facies, such as Kabuki syndrome or fetal alcohol syndrome. The diagnosis for Williams' syndrome was established based on the clinical features and supported by the fluorescent in situ hybridization test. Williams' syndrome has been described as a rare, congenital disorder characterized by physical and developmental problems. Common features include characteristic "elfin-like" facies, supravalvular aortic stenosis, hypercalcemia, low birth weight, slow weight gain, feeding problems, impulsive and outgoing personality, limited spatial skills and motor control, and intellectual disability. Although individuals with Williams' syndrome may show competence in areas such as language, music, and interpersonal relations, their IQs are usually low and they are considered moderately to mildly retarded.
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ranking = 0.33333333333333
keywords = craniofacial
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5/21. Intracranial migration of halo fixation pins: a complication of using an extraoral distraction device.

    OBJECTIVE: Distraction osteogenesis is a well-accepted technique in the treatment of patients with hypoplastic craniofacial components. Complications of distraction osteogenesis are well described in the literature. We describe a complication of using an external distraction device in a 9-year-old girl with Pfeiffer. INTERVENTION AND RESULTS: A modified Lefort III osteotomy was performed for maxillary hypoplasia with application of an external distraction halo device by a pediatric neurosurgeon. A postoperative computed tomography (CT) scan showed 0.5-cm skull penetration of the cranial pins. The pins were repositioned and the patient was followed up on a regular basis until discharge from the hospital. At 3-week follow-up, a CT scan of the head showed migration of the pins 1.5 cm intracranially. The halo was removed and repositioned at a different site. No detectable neurological sequelae from the pin penetration were noted. The patient developed cellulitis at the site of the penetration and was admitted to the hospital for a course of intravenous antibiotics. There were no other complications, and the rest of her treatment course proceeded as planned. A review of the literature on complications of halo usage as well as suggestions for their management in association with distraction osteogenesis is described.
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ranking = 0.33333333333333
keywords = craniofacial
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6/21. Trigonomicrocephaly, severe micrognathia, large ears, atrioventricular septal defect, symmetrical cutaneous syndactyly of hands and feet, and multiple cafe-au-lait spots: new acrocraniofacial dysostosis syndrome?

    We report on a patient with a unique constellation of anomalies comprising trigonomicrocephaly, asymmetric severe micrognathia, large ears, atrioventricular septal defect, vertebral anomalies, bilateral cutaneous syndactyly of fingers and toes, unilateral cryptorchidism and multiple cafe-au-lait spots. The mother of the propositus has multiple cafe-au-lait spots. Search of POSSUM and the london Dysmorphology database (LDDB) uncovered no similar case. We think that this patient represents a new acrocraniofacial dysostosis syndrome.
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ranking = 1.6666666666667
keywords = craniofacial
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7/21. Le Fort III distraction osteogenesis of midface-retrusion in a case of Hajdu Cheny syndrome.

    Le Fort III maxillary distraction osteogenesis using the RED system and advancement genioplasty was successfully performed for the midfacial retrusion and to eliminate severe snoring during sleep in a rare case of Hajdu Cheny syndrome. This syndrome is characterized by slowly progressive systemic osseous dysplasia, exhibiting craniofacial disfigurements and other skeletal deformity, but no description is found in a plastic surgical treatment up to now. A rare entity of this syndrome is also presented.
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ranking = 0.33333333333333
keywords = craniofacial
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8/21. The Russell-silver syndrome: a case report and brief review of the literature.

    The Russell-silver syndrome's phenotypic features consist of musculoskeletal abnormalities, genitourinary malformations, craniofacial dysmorphy and cutaneous dyschromia, which is usually reported as cafe au lait spots. We present the first instances of a large, unilateral, achromic patch as an additional cutaneous manifestation of the Russell-silver syndrome.
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ranking = 0.33333333333333
keywords = craniofacial
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9/21. Extensor-tendon hypoplasia and multiple pterygia: Escobar syndrome in a 7-year-old boy.

    Escobar syndrome is a rare condition with autosomal-recessive inheritance, characterised by multiple pterygia, kyphoscoliosis, multiple joint contractures and craniofacial dysmorphisms. A number of other abnormalities are also attributed to the syndrome. Here, we present a case of isolated extrinsic extensor-tendon hypoplasia of the right index finger in a 7-year-old patient with Escobar syndrome. To our knowledge, this has not been previously described in connection with this syndrome.
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ranking = 0.33333333333333
keywords = craniofacial
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10/21. Moulding of the generate to control open bite during mandibular distraction osteogenesis.

    Distraction osteogenesis of the craniofacial skeleton has become a widely accepted, safe, and effective means of craniofacial reconstructive surgery. Despite excellent results in general, there are still some uncertainties related to the procedure, such as development of an anterior open bite (AOB) during mandibular distraction. The aim of this study was to examine whether 'moulding of the generate', i.e. use of intermaxillary elastics during the active distraction phase is possible to close the mandibular plane angle and open bite. Three subjects, 13- and 15-year-old males and a 7-year-old female, underwent mandibular linear and angular bilateral distraction osteogenesis with moulding of the generate. Lateral cephalograms were obtained before the introduction of elastics and following distraction, once the activation was stopped and the patients were ready for the consolidation phase. Conventional cephalometric measurements were used to assess possible changes in the mandibular plane angle and incisor position. Three different anchorage systems (dental, orthopaedic, and skeletal) were used for placement of the intermaxillary elastics. Cephalometric examination showed that the mandibular plane angle was decreased during active distraction osteogenesis with the introduction of elastics and angulation of the distraction device. Depending on the type of elastic anchorage system, smaller or greater amounts of extrusion of the incisors were noted. Moulding of the generate during active distraction can be performed to reduce the mandibular plane angle and open bite. To prevent unwanted dentoalveolar changes from occurring during elastic traction, skeletal rather than dental fixation of the elastics is recommended. Intrusive mechanics may be incorporated into the orthodontic appliances to balance extrusive force by the moulding elastics.
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ranking = 0.66666666666667
keywords = craniofacial
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