The Experts below are selected from a list of 18 Experts worldwide ranked by ideXlab platform
Alessandro Segna - One of the best experts on this subject based on the ideXlab platform.
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Spondylocostal Dysostosis (Jarcho-Levin Syndrome) in an Adult Patient with Consanguineous Parents, in Long-Term Follow-Up.
World neurosurgery, 2018Co-Authors: Oriela Rustemi, Giacomo Beggio, Alessandro SegnaAbstract:A 24-year-old woman presented to neurosurgical consultation for chronic back pain. The patient was long term in wheelchair for vertebral deformity. She was the third child of first-degree consanguineous parents. The 2 older brothers had also vertebral Malformations. The radiological images showed butterfly vertebra, vertebral fusion, hemivertebrae, scoliosis, and Rib Malformation. The patient was in follow-up for restrictive lung disease. Motor evoked potentials and lower limb electromyography were normal. We recommended conservative treatment for the back pain with antalgic and physical therapy. Diagnosis of spondylocostal dysostosis, or Jarcho-Levin syndrome, was made based on radiological features. Radiological mages are pathognomonic. Spondylocostal dysostosis is a rare hereditary disorder associated with multiple vertebral and Rib anomalies. The entity is distinct from spondylothoracic dysostosis, which has a higher mortality due to respiratory complications. The patient was not compliant for genetic familiar counseling. At 12-year follow-up, the patient was in periodic respiratory and motor rehabilitation therapy.
Oriela Rustemi - One of the best experts on this subject based on the ideXlab platform.
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Spondylocostal Dysostosis (Jarcho-Levin Syndrome) in an Adult Patient with Consanguineous Parents, in Long-Term Follow-Up.
World neurosurgery, 2018Co-Authors: Oriela Rustemi, Giacomo Beggio, Alessandro SegnaAbstract:A 24-year-old woman presented to neurosurgical consultation for chronic back pain. The patient was long term in wheelchair for vertebral deformity. She was the third child of first-degree consanguineous parents. The 2 older brothers had also vertebral Malformations. The radiological images showed butterfly vertebra, vertebral fusion, hemivertebrae, scoliosis, and Rib Malformation. The patient was in follow-up for restrictive lung disease. Motor evoked potentials and lower limb electromyography were normal. We recommended conservative treatment for the back pain with antalgic and physical therapy. Diagnosis of spondylocostal dysostosis, or Jarcho-Levin syndrome, was made based on radiological features. Radiological mages are pathognomonic. Spondylocostal dysostosis is a rare hereditary disorder associated with multiple vertebral and Rib anomalies. The entity is distinct from spondylothoracic dysostosis, which has a higher mortality due to respiratory complications. The patient was not compliant for genetic familiar counseling. At 12-year follow-up, the patient was in periodic respiratory and motor rehabilitation therapy.
Bernward Passlick - One of the best experts on this subject based on the ideXlab platform.
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Venous thoracic outlet syndrome caused by a congenital Rib Malformation
Interactive cardiovascular and thoracic surgery, 2012Co-Authors: Andreas Kirschbaum, E. Palade, Zoltan Csatari, Bernward PasslickAbstract:Venous thoracic outlet syndrome (VTOS) represents a rare disorder. Hypertrophy of the anterior scalene musculature is the cause of the compression syndrome in most cases. To our knowledge, we descRibe the first reported case worldwide of a venous compression syndrome caused by a congenital Malformation of the 1st and 2nd Ribs. Treatment by transaxillary partial Rib resection was necessary and a very good postoperative result was achieved.
Zeng Jin-ji - One of the best experts on this subject based on the ideXlab platform.
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16 multi-slice CT three-dimensional and multiplanar reconstruction for evaluation of pediatric congenital scoliosis
Chinese journal of radiology, 2006Co-Authors: Zeng Jin-jiAbstract:Objective Our study is to use of 16 MSCT three-dimensional images and multiplanar reconstruction images in the preoperative investigation of patients with congenital scoliosis,to study its technical advantage and work out surgical plan.Methods Twenty-seven pediatric patients with congenital scoliosis processing between April to October 2004 were reviewed, including 13 boys and 14 girls. X-ray plain film and sixteen multi-slice CT examination on curved/standard multiplanar reconstruction and three-dimensional computed tomographic imaging may offer many potential advantages for defining congenital spine anomalies liable to cause progression of scoliosis, including visualization of the deformity in any plane, from any angle, with the overlying structures subtracted. Results Ten patients had segmentation defects, 6 patients underwent formation defects, 11 patients had complex, unclassifiable anomalies. The patients of Rib deformity were found in 15 patients,the most prominent part of the Rib cage deformity was at the same level as the most rotated vertebra in 7 patients; 8 patients had vertebral anomalies accompanied with diastematomyelie, including 6 patients with uncompleted or completed bony spur. In 19 of 27 cases, the multiplanar reconstruction and three-dimensional images allowed identification of unrecognized Malformations and completely evaluated the degree of scoliosis,during conventional X-ray images and axial CT images, including volume 3D imaging evaluated approximately classification and modality of complex anomalies in 11 cases ,which were unclassifiable Malformation in 7 cases and unsegmented bar with contralateral hemivertebrae; 4 children had segmentation defects revealed unilateral unsegmented bar (3 cases) and bilateral block vertebra (1 case) in volume 3D reconstruction images; 2 children were found occultation hemivertebrae which were not been discovered during conventional X-ray images and axial CT images; and 2 children were revaluated the amount of hemivertebrae. The curved multiplanar reconstruction images in all cases were best for defining the spinal canal and spinal medulla, and showed clearly the modality and developed way of bony spur, even the relationship with spinal cord in 6 cases. The standard multiplanar reformatted images in 4 cases were helpful in showing occipital-C1-C2 anatomy and malformatiom, especially the junction of skull and cervix Malformation. Conclusion Our data suggest that 16 MSCT three-dimensional images and multiplanar reconstruction images appears to be most useful tool in the patients with congenital scoliosis in which the anomalies are complex, multiple, or obscured by deformity and Rib Malformation. The reformatted images of MSCT is markedly superior to conventional X-ray images and axial CT images when used to evaluate the progressive spinal curvatures and work out surgical plan.
Giacomo Beggio - One of the best experts on this subject based on the ideXlab platform.
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Spondylocostal Dysostosis (Jarcho-Levin Syndrome) in an Adult Patient with Consanguineous Parents, in Long-Term Follow-Up.
World neurosurgery, 2018Co-Authors: Oriela Rustemi, Giacomo Beggio, Alessandro SegnaAbstract:A 24-year-old woman presented to neurosurgical consultation for chronic back pain. The patient was long term in wheelchair for vertebral deformity. She was the third child of first-degree consanguineous parents. The 2 older brothers had also vertebral Malformations. The radiological images showed butterfly vertebra, vertebral fusion, hemivertebrae, scoliosis, and Rib Malformation. The patient was in follow-up for restrictive lung disease. Motor evoked potentials and lower limb electromyography were normal. We recommended conservative treatment for the back pain with antalgic and physical therapy. Diagnosis of spondylocostal dysostosis, or Jarcho-Levin syndrome, was made based on radiological features. Radiological mages are pathognomonic. Spondylocostal dysostosis is a rare hereditary disorder associated with multiple vertebral and Rib anomalies. The entity is distinct from spondylothoracic dysostosis, which has a higher mortality due to respiratory complications. The patient was not compliant for genetic familiar counseling. At 12-year follow-up, the patient was in periodic respiratory and motor rehabilitation therapy.