Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency syndrome characterized by recurrent staphylococcal infections in the skin and lungs, with an incidence of less than one case per million persons. Skeletal and connective tissue abnormalities, such as scoliosis, osteoporosis, pathological fractures, and hyperextensive joints, are other manifestations of HIES. However, only one report documents the use of implants to treat spinal deformity caused by HIES, which was discovered following corrective surgery resulting in postoperative infection. In this case report, the authors describe a 16-year-old male with low-back pain and infections of the soft tissue. Radiological findings showed deteriorated kyphotic deformity due to the pathological compression fracture of T-11 with intensive conservative treatment. Anterior and posterior fixation surgery was performed. Thereafter, the patient showed no signs of infection. An investigation was conducted to avoid any postoperative infection.
Naoko Araya, Hiroyuki Inose, Tsuyoshi Kato, Masanori Saito, Satoshi Sumiya, Tsuyoshi Yamada, Toshitaka Yoshii, Shigenori Kawabata, and Atsushi Okawa
Report of 2 cases
Satoru Egawa, Toshitaka Yoshii, Kyohei Sakaki, Hiroyuki Inose, Tsuyoshi Kato, Shigenori Kawabata, Shoji Tomizawa, and Atsushi Okawa
Superficial siderosis (SS) of the CNS is a rare disease caused by repeated hemorrhages in the subarachnoid space. The subsequent deposition of hemosiderin in the brain and spinal cord leads to the progression of neurological deficits. The causes of bleeding include prior intradural surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. Recently, surgical treatment of SS associated with dural defect has been reported. The authors of the present report describe 2 surgically treated SS cases and review the literature on surgically treated SS. The patients had dural defects with fluid-filled collections in the spinal canal. In both cases, the dural defects were successfully closed, and the fluid collection was resolved postoperatively. In one case, the neurological symptoms did not progress postoperatively. In the other case, the patient had long history of SS, and the clinical manifestations partially deteriorated after surgery, despite the successful dural closure.
In previously reported surgically treated cases, the dural defects were closed by sutures, patches, fibrin glue, or muscle/fat grafting. Regardless of the closing method, dural defect closure has been shown to stop CSF leakage and subarachnoid hemorrhaging. Successfully repairing the defect can halt the disease progression in most cases and may improve the symptoms that are associated with CSF hypovolemia. However, the effect of the dural closure may be limited in patients with long histories of SS because of the irreversibility of the neural tissue damage caused by hemosiderin deposition. In patients with SS, it is important to diagnose and repair the dural defect early to minimize the neurological impairments that are associated with dural defects.
Tetsuo Hashiba, Naoya Hashimoto, Shuichi Izumoto, Tsuyoshi Suzuki, Naoki Kagawa, Motohiko Maruno, Amami Kato, and Toshiki Yoshimine
Due to advances in neuroimaging and the increasing use of imaging to screen for brain disease (“brain checkups”), meningiomas are now often detected as an incidental finding. The natural history of these asymptomatic meningiomas remains unclear, however. In this study, the authors investigated the natural history and growth pattern of incidentally detected meningiomas using serial volumetric assessment and regression analysis.
In 70 patients with incidentally discovered meningiomas who underwent follow-up for longer than 1 year, tumor volumes were calculated volumetrically at each follow-up visit, and tumor growth was determined. In patients with tumor growth, regression analysis was performed to determine the pattern of growth.
Forty-four tumors exhibited growth and 26 did not. In a regression analysis, 16 of the tumors that grew followed an exponential growth pattern and 15 exhibited linear growth patterns. The presence of calcification was the only imaging characteristic that significantly distinguished the group with tumor growth from that without, although no radiological characteristics significantly distinguished the exponential growth group from the linear growth group. Two patients with obvious tumor growth underwent surgical removal and the pathological specimens extracted showed a high proliferative potential.
The authors found that incidentally discovered meningiomas did not always follow an exponential growth pattern but often exhibited more complex patterns of growth. Serial monitoring of tumor volumes and regression analysis may reveal the growth pattern of incidental meningiomas and provide information useful for determining treatment strategy.
Morio Matsumoto, Yoshiaki Toyama, Hirotaka Chikuda, Katsushi Takeshita, Tsuyoshi Kato, Shigeo Shindo, Kuniyoshi Abumi, Masahiko Takahata, Yutaka Nohara, Hiroshi Taneichi, Katsuro Tomita, Norio Kawahara, Shiro Imagama, Yukihiro Matsuyama, Masashi Yamazaki, and Akihiko Okawa
The aim of this study was to evaluate the outcomes of fusion surgery in patients with ossification of the posterior longitudinal ligament in the thoracic spine (T-OPLL) and to identify factors significantly related to surgical outcomes.
The study included 76 patients (34 men and 42 women with a mean age of 56.3 years) who underwent fusion surgery for T-OPLL at 7 spine centers during the 5-year period from 2003 to 2007. The authors evaluated the patient demographic data, underlying disease, preoperative comorbidities, history of spinal surgery, radiological findings, surgical methods, surgical outcomes, and complications. Surgical outcomes were assessed using the Japanese Orthopaedic Association (JOA) scale score for thoracic myelopathy (11 points) and the recovery rate.
The mean JOA scale score was 4.6 ± 2.1 points preoperatively and 7.7 ± 2.5 points at the time of the final follow-up examination, yielding a mean recovery rate of 45.4% ± 39.1%. The recovery rates by surgical method were 38.5% ± 37.8% for posterior decompression and fusion, 65.0% ± 35.6% for anterior decompression and fusion via an anterior approach, 28.8% ± 41.2% for anterior decompression via a posterior approach, and 57.5% ± 41.1% for circumferential decompression and fusion. The recovery rate was significantly higher in patients without diabetes mellitus (DM) than in those with DM. One or more complications were experienced by 31 patients (40.8%), including 20 patients with postoperative neurological deterioration, 7 with dural tears, 5 with epidural hematomas, 4 with respiratory complications, and 10 with other complications.
The outcomes of fusion surgery for T-OPLL were favorable. The absence of DM correlated with better outcomes. However, a high rate of complications was associated with the fusion surgery.