Cases reported "Acrocephalosyndactylia"

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1/21. Monobloc and midface distraction osteogenesis in pediatric patients with severe syndromal craniosynostosis.

    We present 2 children with severe syndromal craniosynostosis who were in need of urgent midface advancement surgery due to recurrent ocular dislocations (Pfeiffer's syndrome type II) or severe upper respiratory obstruction (Crouzon's syndrome). They were operated using distraction osteogenesis, with gradual midface or monobloc advancements. In the Pfeiffer patient, a maxillary distraction of 25 mm achieved effective cessation of ocular dislocations, whereas a 23-mm monobloc advancement in the Crouzon patient achieved cessation of nocturnal arterial desaturations. No major postoperative complications were recorded. Distraction osteogenesis has become a versatile and safe technique that allows for large skeletal advancements.
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2/21. 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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3/21. Roentgencephalometric studies of the premature craniofacial synostoses: report of a family with the Saethre-Chotzen syndrome.

    Five affected individuals in 4 generations of a family with acrocephalosyndactyly (McKusick ACS Type III; Saethre-Chotzen syndrome) are reported. Serial roentgencephalometric data obtained pre- and postoperatively on the proband were compared with similar measurements on the affected mother and maternal uncle, both of whom have not had operative corrections, and the proband's unaffected older brother. Similarity in skull form among the affected individuals was demonstrated. head circumference as an index of cranial growth or intracranial capacity was misleading in assessing the intellectual potential of the affected adults. In contrast, cranial capacity, as measured by the modulus, was found to be more reliable.
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4/21. Early failure of absorbable plating in a patient with syndromic brachycephaly.

    The use of bioabsorbable plating systems for rigid fixation after cranial remodeling surgery has become the standard of care in the treatment of syndromic and nonsyndromic craniosynostoses at pediatric craniofacial centers in north america. The advantages of these absorbable plating systems over metallic plates and screws have been well documented. The absorbable plates have been used with remarkable safety and efficacy for more than 15 years. An unusual case of repeated early failure of an absorbable plating system in a patient with syndromic brachycephaly complicated by the development of hydrocephalus is reported. The reoperative rates for cranial remodeling surgery at our institution and the possible causes for this failure are discussed.
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5/21. Subcranial facial bipartition osteotomy with glabellar reverse V-shaped and temporal approaches instead of the bicoronal approach.

    patients with Apert syndrome show hypertelorism and midfacial hypoplasia, and their features are significantly improved through facial bipartition surgery. In addition, because patients with Apert syndrome demonstrate cranial deformity as well as other deformities, they require multiple surgical interventions throughout their development. We present herein a girl with Apert syndrome for whom subcranial facial bipartition was performed. We could not use the coronal approach because she had a terribly cicatricial scalp and wide calvarial defects caused by previous cranial surgeries carried out at another institution. Therefore, we used the glabellar reverse V-shaped approach and temporal approaches in place of the standard approach. She enjoyed a complication-free intraoperative and postoperative course, and left the hospital on postoperative day 10. Her midfacial segment was repositioned 6 mm anteriorly and 12 mm inferiorly on the cephalometric measurement. Her interpupillary distance was altered from 83 mm preoperatively to 76 mm postoperatively. The reverse V-shaped glabellar approach permits more relaxation of the nasal skin and nasal augmentation that is more reliable, although an inconspicuous scar remains in the prominent area. The temporal approach is also useful, enabling easy zygomatic arch osteotomy and secure pterygo-maxillary separation for pterygo-maxillary separation through an oral approach, allowing chiseling toward the skull base.
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ranking = 5
keywords = operative
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6/21. Dual segmental distraction osteogenesis of the midface in a patient with Apert syndrome.

    OBJECTIVE: To present orthodontic treatment combined with dual segmental distraction osteogenesis in a patient with Apert syndrome. PATIENT: A 15-year-old boy exhibited severe midfacial hypoplasia with retruded and hypoplastic maxilla and anterior open bite. The patient was treated with a rigid external distraction II system for distraction osteogenesis, a preadjusted edgewise appliance, and a modified maxillary protraction headgear. The concave profile with midfacial hypoplasia was improved. A tight occlusal relationship between maxillary and mandibular teeth was achieved. Postoperative treatment results have been stable for 1 year.
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7/21. An operation for syndactyly, and its results.

    We describe our method of operation for syndactyly repair and our results in 62 patients (74 hands, 143 commissures), all treated more than two years ago. The operation was performed on patients aged from 17 months to two years (except those with acrosyndactyly). Postoperative web formation was not seen in any of these patients.
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keywords = operative
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8/21. Three dimensional reconstruction in coronal synostosis: pre and post operative appearances.

    Three dimensional computed tomography (CT) is a relatively new method of arranging the elements of CT slices into a morphological image with a multi dimensional appearance. It is of particular use in craniofacial deformities. The following report shows its use in the pre and post operative assessment of frontal skull deformity in three infants with coronal synostosis.
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ranking = 5
keywords = operative
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9/21. airway obstruction and sleep apnea in children with craniofacial anomalies.

    Children with severe craniofacial anomalies and breathing problems are rare, and the accumulated experience of their treatment is limited. LeFort III midface advancements have been tried by many craniofacial teams, but no consensus has yet been reached as to the effectiveness of this procedure. In this report of seven patients with craniofacial malformations and severe breathing problems, three had a LeFort II midface advancement, one had release of bilateral temporomandibular joint ankylosis, and two had tonsillectomies. Two patients without a tracheostomy suffocated, four had a long-term tracheostomy, and one was cured by a unilateral tonsillectomy. It was concluded that LeFort III midface advancement is ineffective in these types of cases without a very stable postoperative retention, and it was suggested that all patients with severe craniofacial anomalies and breathing problems, regardless of their planned subsequent treatment, should have a tracheostomy as an initial measure.
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keywords = operative
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10/21. body image and the process of reconstructive surgery.

    Newer craniofacial operative techniques produce rapid changes in objective appearance and permit the study of body-image change. In 21 patients with either Crouzon's disease or Alpert's syndrome, a four-factor model of body-image development was applied that emphasizes cognitive growth, perception of body stimuli, stimuli from the environment in the form of comparison, and the response from others. Before a child is of school age, he has substantially defined his body image, therefore corrective surgery must be considered earlier. Although surgical intervention may produce significant objective physical change, there is not a correspondingly rapid change in body image. Four phases in the modification of body image are (1) the decision to undergo surgery, (2) the operative experience, (3) the immediate postoperative period, and (4) the reintegration stage. Recognition of this phasic process will help integrate care of these patients.
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