1/11. Anaesthetic management of two paediatric patients with Hecht-Beals syndrome.We undertook the anaesthetic management of two children with Hecht-Beals syndrome for orthopaedic surgery under general anaesthesia. Both patients had arachnodactyly, kyphoscoliosis, and multiple congenital joint contractures, but limited mandible excursion was not obvious preoperatively in either, although mental retardation made it difficult for them to cooperate with mouth examination. They had no apparent difficulties with their mouths in daily activities. The anaesthesia records of one patient showed that intubation had been difficult in an earlier procedure. The other patient also had a history of difficult intubation, with slight tearing of the corners of her mouth during an intubation procedure. During slow induction of general anaesthesia with sevoflurane, face mask ventilation was easily performed. We attempted to visualize the larynx under anaesthesia with muscle relaxation, but we were unsuccessful because of the limited mouth opening. After several trials, blind oral intubations were fortunately successful in both patients. There were no postoperative problems with the airway.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
2/11. The cerebro-costo-mandibular syndrome: 9-year follow-up of a case.Cerebro-costo-mandibular syndrome (CCMS) is a rare multiple congenital anomaly with a low survival rate. There are few reports of long-term survival in this condition. We describe the findings and management of a 9-year-old survivor of CCMS, outline the importance of early intervention and multidisciplinary team approach. The child presented in the neonatal period in respiratory distress with classical features of the syndrome. Aggressive initial respiratory management was later followed up with an integrated multidisciplinary team approach. He has been carefully followed up for nine years now, illustrating well, the course of the syndrome.- - - - - - - - - - ranking = 2keywords = tea (Clic here for more details about this article) |
3/11. Partial lumbosacral kyphosis reduction, decompression, and posterior lumbosacral transfixation in high-grade isthmic spondylolisthesis: clinical and radiographic results in six patients.BACKGROUND: In an attempt to increase fusion while decreasing the neurologic risk associated with complete reduction of high-grade spondylolisthesis, the authors have used a technique of partial lumbosacral kyphosis reduction, posterior decompression, and pedicle screw transfixation of the lumbosacral junction. OBJECTIVE: To determine if this technique is effective in treatment of high-grade spondylolisthesis. STUDY DESIGN: A retrospective review of six patients with high-grade spondylolisthesis treated by this technique was performed. There were four female patients (ages 16 years [n=2], 23 years [n=1], and 29 years [n=1]) and two male patients (both 13 years of age) with spondylolisthesis ranging from Grade IV to Grade V. All patients presented with pain and radiculopathy. After surgery the patients were evaluated for resolution of symptoms, sagittal alignment, fusion, and satisfaction. The radiographic measurements included the slip angle, the percentage slip, and the sacral inclination. An SRS outcome score was also obtained on all six patients to evaluate postoperative outcome, in terms of pain control, self-image perception, and return to function. RESULTS: The average length of follow-up was 42.6 months (range 24-60 months). All patients evidenced solid fusion by the 6-month follow-up (based on oblique radiographs showing lateral bridging bone masses). The slip angle was improved from 62 degrees to 28 degrees (P < 0.5), whereas there was no significant improvement in the percentage slip or the sacral inclination (89-80% and 28-37 degrees, respectively). No progression of the slip angle or percentage slip was noted on the follow-up radiographs. Complications included two intraoperative dural tears that were identified and repaired. There were no neurologic complications. The SRS outcome instrument demonstrated good postoperative pain control, function, self-image, and satisfaction in all patients. CONCLUSION: In high-grade spondylolisthesis, this posterior approach is safe and effective in obtaining a solid arthrodesis, restoring sagittal balance, and improving function. These results reinforce the impression that it is the partial reduction of the slip angle, not the percentage slip, in high-grade spondylolisthesis that is important in obtaining optimal results.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
4/11. Intra-articular fracture of the knee with spondyloepiphyseal dysplasia congenita: successful result of open reduction and internal fixation.In spondyloepiphyseal dysplasia congenita (SEDC), since the cartilage is congenitally abnormal, functional recovery of an intra-articular fracture is uncertain even with surgical treatment. We report a 29-year-old Japanese woman with SEDC whose left knee injury (intercondylar femur fracture and tibial plateau fracture) was surgically reduced and fixed. Although special care was required during the operation for associated atlantoaxial instability and cardiopulmonary suppression due to severe thoracolumbar kyphoscoliosis as well as osteopenia, she had neither restriction of knee motion nor pain at follow-up 2 years and 4 months after surgery. Therefore, although the situation involving fractures in a patient with SEDC is complicated, we believe the main problem to be solved is whether the risk-related kyphoscoliosis and atlantoaxial instability can be managed or not. Fractures themselves can be treated based on the principles used for patients without SEDC.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
5/11. Surgical management of the spinal deformity in ehlers-danlos syndrome type VI.Five patients with ehlers-danlos syndrome type VI were surgically treated for their spinal deformities. All of them were female. All but one had a double-major thoracic and lumbar curve. One had a mild scoliotic curve but severe thoracic lordosis. Anterior discectomy and fusion and posterior instrumentation was performed in a single stage in two patients, while two had anterior and posterior surgery performed in two stages. The remaining patient underwent posterior surgery only, because of the relative mildness of the deformity and adequate flexibility. Two serious vascular complications were confronted. One patient experienced avulsion of segmental arteries and rupture of iliac artery and vein during anterior surgery. Another patient had avulsion of superior gluteal artery, which happened during subperiosteal dissection to harvest iliac bone graft. Fortunately, we did not see any temporary or permanent neurological complications. The spinal fusions appeared solid radiographically, with no implant failure and loss of correction after an average follow-up of 4 years.- - - - - - - - - - ranking = 2keywords = tea (Clic here for more details about this article) |
6/11. Transient kyphotic deformity of the thoracolumbar junction resulting from a large abdominal cyst: a case report.BACKGROUND CONTEXT: Pronounced kyphosis of the thoracolumbar junction is a common orthopedic problem in adolescents and may require prolonged bracing therapy or correction spondylodesis. PURPOSE: To describe a case where a kyphotic deformity was related to gynecological instead of spine pathology. STUDY DESIGN: Case report. methods: A 17-year-old girl presented with a structural hyperkyphosis of the thoracolumbar spine and radiographic changes of the involved vertebral end plates. RESULTS: The thoracolumbar hyperkyphosis appeared to have evolved from a massive intra-abdominal ovarian cyst. Endoscopic paracentesis of the cyst resulted in a complete regression of the hyperkyphosis. CONCLUSIONS: A hyperkyphosis is not always related to spine pathology, and other potential causes must be excluded before bracing therapy is initiated.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
7/11. Gowers intrasyringal hemorrhage. Case report and review of the literature.The concept of hemorrhage in a preexisting syringomyelic cavity was first described by Gowers in 1904. Since its first description only 13 cases have been reported. The aims of this report are to describe a new case, bring this entity to wider attention, and summarize the existing literature on the subject. This 36-year-old woman presented with progressive gait disturbance and unsteadiness. physical examination revealed incomplete quadriparesis, predominantly on the left side, and hypesthesia below C-7. magnetic resonance imaging revealed hematomyelia characterized by a heterogeneous hyperintense signal within the central cervical cord. A liquefied well-limited hematoma was evacuated. The postoperative course was uneventful; a near-complete recovery was observed at the 7-year follow-up examination. Most cases of intrasyringal hemorrhage (ISH) have occurred in syringomyelic cavities associated with scoliosis or Chiari malformation Type I. Although there is no specific clinical picture associated with this entity, it can be characterized by three neurological forms: 1) sudden onset or rapid development of signs and symptoms, 2) acute worsening of symptoms that may improve but leaving greater neurological dysfunction than before the previous episode, and 3) ISH may initiate progressive deterioration in a patient with known syringomyelia. Intrasyringal bleeding is most probably caused by a sudden dilation of the syringomyelic cavity, which may provoke rupture of the intrasyringal vessels by an acute distension of the accompanying strands. magnetic resonance imaging is the most accurate diagnostic modality, and recognition of ISH can lead to early, safe, and efficient surgical treatment.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
8/11. Normal MPS excretion, but dermatan sulphaturia, combined with a mild Maroteaux-Lamy phenotype.A mildly affected Maroteaux-Lamy patient is described. Electrophoretic separation of acid mucopolysaccharides (MPS) in the urine showed an increased excretion of dermatan sulphate in spite of a normal total excretion of MPS.- - - - - - - - - - ranking = 5keywords = tea (Clic here for more details about this article) |
9/11. laminectomy or laminotomy?After laminectomy in infants and children vertebral deformities often develop. To prevent these the author applied the reanchoring of the dissected arches and ligaments (laminotomy) with success in four children. The details of the procedure and the reanchoring of the vertebral arches are described. This treatment modality should be favourized instead of the destructive laminectomy in this age group in the majority of cases.- - - - - - - - - - ranking = 1keywords = tea (Clic here for more details about this article) |
10/11. Compressive myelopathy associated with type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome).spinal cord compression with resultant myelopathy is a frequent occurrence in patients with mucopolysaccharidoses. Etiological factors include developmental abnormalities of the cervical spine and infiltration of the dura mater by the accumulated products of mucopolyssacharide metabolism. Compression at the thoracolumbar junction is rare, but was found in a child with the characteristic physical and biochemical stigmata of the Maroteaux-Lamy syndrome (mucopolysaccharidosis vi). An anterolateral approach to remove the compressing bony elements resulted in symptomatic improvement. Careful radiological evaluation is required so that all surgical options can be considered. patients with metabolic storage diseases and the capacity for normal intellectual function warrant aggressive surgical care to optimize neurological function.- - - - - - - - - - ranking = 5keywords = tea (Clic here for more details about this article) |
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