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1/26. Electrical impedance measurements with the CI24M cochlear implant for a child with Mondini dysplasia.

    Electrical impedance measurements can give useful information about the status of individual electrodes of a cochlear implant. Impedances within the normal range (when measured in the common ground mode of stimulation) suggest that current flow occurs in the tissue and fluid of the cochlea. Low impedance measurements may suggest that particular electrodes are short circuiting, whereas high impedances might be due to a broken electrode wire or an electrode only in contact with air. In the case discussed, low impedance measurements were recorded intra-operatively from a child with Mondini dysplasia on electrodes 1-13 of a Nucleus CI24M device. Post-operatively the impedances had returned to the expected range and were comparable with other patients implanted with the CI24M device. Possible reasons for this are discussed. It was thought that impedances were low intra-operatively due to a larger-than-normal proportion of fluid surrounding the electrode array, rather than short circuits occurring along the array, as suggested by the dps7 software.
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2/26. Hip-shelf procedure in the treatment of osteonecrosis of the transpositioned acetabulum after rotational acetabular osteotomy.

    Necrosis of the transpositioned acetabulum after rotational acetabular osteotomy (RAO) is a major complication characteristic of this procedure. This complication, although rare, has been thought difficult to treat. We report a patient with acetabular osteonecrosis and subsequent collapse after RAO that was effectively treated with a shelf operation, providing satisfactory remodeling of the hip joint. A 16-year-old female had undergone RAO for the treatment of developmental acetabular dysplasia. Postoperative radiography showed that the osteotomized acetabular fragment was unusually thin, and that the osteotome entered the hip joint during the surgery. Five months after the RAO, x-rays revealed significant collapse of the transpositioned acetabulum, and femoral head subluxation caused by postoperative osteonecrosis. Seven months after the RAO, the patient underwent a hip-shelf procedure. The remaining acetabular fragment was used in this procedure, according to the Spitzy method. Seven years after the second operation, favorable remodeling of the hip joint was observed; however, early osteoarthritic changes, including slight joint space narrowing, bone sclerosis of the new acetabulum, and bone cysts within the femoral head, were seen.
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3/26. Giant bone island of femur complicating replacement arthroplasty: a report of two cases.

    Bone islands are hamartomatous malformations in the bone that are known to be asymptomatic in nature. Two cases are presented in which the presence of bone islands in the femur led to difficulty with the introduction of a femoral prosthesis. In one case, this led to fracture of the medial part of the cortex, which was fixed with a screw prior to insertion of the prosthesis. In the second case, a window was made in the femur to remove hard bone sufficient for insertion of the prosthesis. This was closed with steel wires prior to insertion of the cemented femoral stem. These cases illustrate the problems that can be encountered during surgery in the presence of bone islands. The surgeon should evaluate the site, size, and location of bone islands during preoperative planning before performing surgery.
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4/26. Presacral meningocele associated with hereditary sacral agenesis and treated surgically: evaluation in three members of the same family.

    OBJECTIVE AND IMPORTANCE: Presacral meningocele in hereditary sacral agenesis is a complex and unusual spinal dysgenetic syndrome. Recognition of the syndromic triad, its natural history, and familial presentation has important practical applications for the management of this disease as well as its complications. CLINICAL PRESENTATION: This report concerns three patients in one family with Currarino syndrome. We detail its clinical presentation, operative management, and outcome and suggest management procedures based on reports in the literature and the results of our surgical techniques, which focus on cases with an "incomplete" triad. INTERVENTION: Three members of the same family, one adult and two children, underwent surgery through the posterior sacral approach tying off the communication between dural sac and anterior meningocele. The adult underwent a second surgical procedure in which a custom-designed surgical technique was used to resolve postoperative cerebrospinal fluid leakage. One of the children underwent an additional posterior sagittal anorectoplasty to remove a presacral teratoma. CONCLUSION: We report a rare occurrence of three familial cases of sacral agenesis accompanied by a presacral mass with various degrees of phenotypic expression and with male dominant transmission. Because of its rarity, the best surgical technique and timing remain an open question especially in cases with incomplete triad syndrome.
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5/26. "The disappearing evoked potentials": a special problem of positioning patients with skeletal dysplasia: case report.

    STUDY DESIGN: A retrospective study of 3 patients with skeletal dysplasia, who had a loss of the evoked potentials during prone positioning before spine surgical intervention. OBJECTIVES: To bring attention to the potential hazard of neurologic compromise during the positioning of patients with skeletal dysplasia for spine surgery. Recommendations are suggested to prevent the disappearance of intraoperative evoked potentials and, therefore, possible neural injury in these patients. SUMMARY OF BACKGROUND DATA: In a very few published cases, loss or attenuation of monitored potentials has been observed at the time of initial patient positioning. Although patients with skeletal dysplasia might be considered particularly vulnerable to spinal cord injury caused by malpositioning of the head and neck, to our knowledge, no association with lost evoked potentials has previously been described. methods: Intraoperative transcranial electrical motor-evoked potential and/or somatosensory evoked potential baseline studies were performed after induction in the supine position. These studies were repeated as soon as practicable, after intubation and, again, after the patients were turned prone. The neurophysiologist informed the surgeon that evoked potential change in latency or amplitude met warning criteria. Alteration in the surgical plan resulted in successful spinal surgery in these cases. RESULTS: In case No. 1, repositioning of the head in flexion was sufficient to return the evoked potentials to normal. In the other two cases, attempts to reposition the patients prone failed, and the procedures were abandoned. In case No. 2, four months after the initial surgery, a halo cast for immobilization and craniocervical decompression were needed before the corrective cervical spine surgery, and in case No. 3, two steps were taken after the initial surgery: 1) trial positioning awake on the surgical table before surgery; and 2) awake postintubation prone positioning on the actual surgery day. CONCLUSIONS: patients with skeletal dysplasia are susceptible to serious neurologic misadventure when turned to a prone position. Neurophysiologic and/or clinical monitoring of patient positioning should be undertaken, and a plan of intervention, should loss of signal or function occur, must be implemented.
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6/26. magnetic resonance imaging in the evaluation of partial growth arrest after physeal injuries in children.

    The precise delineation of the size, shape, and location of an osseous bridge is a critical step in the management of children who have a partial growth arrest of the epiphyseal plate. Five children between the ages of ten and fourteen years were diagnosed, with the aid of conventional roentgenograms, as having a partial growth arrest. Magnetic resonance-imaging studies were carried out to determine the exact size, shape, and location of the osseous bridge. The information derived from the imaging studies was essential for the determination of the appropriate treatment and for the planning and undertaking of any operative intervention.
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7/26. Computerized tomographic analysis of orbital hypertelorism repair: spatial relationship of the globe and the bony orbit.

    Computerized tomographic scans provide a new means of evaluating the spatial and geometric relationships between the movement of the bony orbit and its soft tissue contents (the globe and extraocular muscles) [1, 12]. Preoperative and postoperative computerized tomographic scans were analyzed in four patients to explore these relationships. Measurement of the changes in distance between the globes correlated most closely with the change in the distance between the lateral orbital walls; resection of medial (inter-orbital) bone provides space into which the globe is translocated. The medial rectus muscle may be bowed across the medial wall osteotomy line, creating a functional shortening of the muscle; this finding may explain the esotropia that is commonly seen after this procedure [2, 3]. These observations should have a direct impact on the understanding and planning of orbital hypertelorism correction.
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8/26. hearing loss due to cranio-metaphysial dysplasia.

    Craniometaphysial dysplasia is a rare cause of conductive hearing loss. It is a genetic disorder of bone due to decreased osteoclastic activity in the endosteal and periosteal regions of craniofacial and long bones. The characteristic temporal bone abnormalities are attic fixation, abnormally shaped ossicles and obliterated foramina ovale. Computerised tomography is helpful in predicting operative findings; surgery should be reserved for those with a severe hearing loss.
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9/26. Simultaneous correction of pelvic obliquity, frontal plane, and sagittal plane deformities in neuromuscular scoliosis using a unit rod with segmental sublaminar wires: a preliminary report.

    Ten patients with neuromuscular scoliosis and pelvic obliquity had segmental spinal instrumentation using a unit Luque rod with sublaminar wires and fixation into the pelvis. Nine of the 10 patients also had anterior spinal fusion without instrumentation before the posterior procedure. Average preoperative pelvic obliquity was 42 degrees which was corrected to 6 degrees (82% correction). Average preoperative scoliosis was 92 degrees, which was corrected to 16 degrees (81% correction). Complications included a wound hematoma in one patient and a superficial wound dehiscence in another. There have been no pseudarthroses or hardware failures to date. Excellent correction of the pelvic obliquity and the spinal curve in neuromuscular scoliosis can be obtained with use of a unit rod and without use of anterior instrumentation.
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10/26. The anaesthetic management of an infant with frontometaphyseal dysplasia (Gorlin-Cohen syndrome).

    We describe an infant with frontometaphyseal dysplasia, who presented to us twice for anaesthesia for orthopaedic surgery. These patients have facial asymmetry, mandibular hypoplasia, bradycardia, restrictive lung disease, primary pulmonary hypertension, skeletal abnormalities and difficult endotracheal intubation. The patient also showed laryngeal stridor because of laryngomalacia, vocal cord paralysis and subglottic stenosis. light premedication along with atropine, ECG and blood pressure monitoring, gradual inhalational induction and intubation of the spontaneously breathing patient, careful positioning and postoperative CPAP are recommended.
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