Search

STUDY DESIGN.: Literature review. OBJECTIVE.: To present a comprehensive overview of spinal involvement in patients with [beta]-thalassemia, aiming to orient the spinal surgeon to these potentially disabling complications. SUMMARY OF BACKGROUND DATA.: [beta]-thalassemia, an inherited disorder of hemoglobin synthesis, is the most common monogenetic disease worldwide. Patients with thalassemia major (TM) suffer a severe anemia that requires regular blood transfusions for survival and iron chelation therapy to control transfusional iron overload. Thalassemia intermedia is a phenotype of thalassemia with a milder course and anemia, wherein most patients remain transfusion independent. Spinal involvement related to disease course and treatment is common in patients with thalassemia syndromes, yet it has not been constructively reviewed in the literature. METHODS.: Potentially relevant studies were identified from an electronic search of MEDLINE (1966 to the second week of May 2010). RESULTS.: In patients with TM, genetic and acquired risk factors lead to osteoporosis, pathologic fractures of the spine, and back pain. Osteoporosis in TM patients is progressive; thus, early diagnosis and treatment are recommended. Bisphosphonates are relatively safe and effective in this patient population. Characteristic intervertebral disc degeneration is also seen in patients with TM who have evidence of severe iron overload or those who receive the subcutaneous iron chelator deferoxamine. Spinal asymmetry and overt scoliosis are common in patients with TM. The prognosis seems favorable, with many patients showing spontaneous resolution without the need for intervention. In patients with thalassemia intermedia, ineffective erythropoiesis drives extramedullary hematopoietic tissue formation, which is mostly evident on magnetic resonance imaging. Paraspinal involvement is of greatest concern because of the associated spinal cord compression. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin-inducing agents that decrease the hematopoietic drive. CONCLUSION.: Current knowledge supports that spinal involvement in patients with [beta]-thalassemia is common and diverse yet still requires further prospective evaluation. [ABSTRACT FROM AUTHOR]

Ineffective erythropoiesis in patients with thalassemia intermedia drives extramedullary hematopoietic tumor formation in several parts of the body. Paraspinal involvement has received increasing attention due to the associated morbidity secondary to spinal cord compression. Although the history and physical examination may help narrow the differential diagnosis, radiographic imaging remains essential to confirm the existence of hematopoietic tissue. Characteristic appearance has been observed mainly on magnetic resonance imaging. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management scheme remains controversial. Until large prospective trials evaluate the efficacy and safety of the available treatment options, both in single and in combination therapy, an individualized approach should be entertained. [ABSTRACT FROM AUTHOR]

Study Design: Cross-sectional validation and reliability assessment study of Arabic version of Scoliosis Research Society-22 (SRS-22r) Questionnaire., Objective: To develop and validate the Arabic version of the SRS-22r questionnaire., Summary of Background Data: The diagnosis and treatment of adolescent idiopathic scoliosis may influence patient quality of life. SRS-22r is an internationally validated questionnaire used to assess function/activity, pain, self-image, and mental health of patients with scoliosis. It has been translated into several languages but not into Arabic language. Therefore, a valid health-related quality-of-life outcome questionnaire for patients with spinal deformity is still lacking in Arabic language., Methods: The English version of SRS-22r questionnaire was translated, back-translated, and culturally adapted to Arabic language. Then, 81 patients with idiopathic adolescent scoliosis were allocated randomly into either the reliability testing group (group 1) or the validity testing group (group 2). Group 1 patients completed Arabic version of SRS-22r questionnaire twice with 1-week interval in-between. Cronbach α and intraclass correlation coefficient were measured to determine internal consistency and temporal reliability. Group 2 patients completed the Arabic version of SRS-22r questionnaire and the previously validated Arabic version of 36-Item Short Form Health Survey (Short Form-36) questionnaire concurrently, and Pearson correlation coefficient was obtained to assess validity., Results: Content analysis, internal consistency reliability, test/retest reproducibility (intraclass correlation coefficient range: 0.82-0.90), and test of concurrent validity showed satisfactory results. Function/activity and satisfaction with management domains had a lower Cronbach α (0.58 and 0.44, respectively, vs. 0.71-0.85 range for others). Self-image/appearance and satisfaction with management had a lower correlation with domains of the 36-Item Short Form Health Survey., Conclusion: An Arabic version of the SRS-22r questionnaire has been developed and validated. This questionnaire will aid health care workers and researchers in evaluation of patient perception of the deformity, satisfaction with treatment, and quality of life in Arabic-speaking populations., Level of Evidence: 3.

Objective: To report our experience with 3 cases of vertebral sarcoidosis (VS) and review the available literature., Methods: We retrospectively analyzed 3 patients with VS, with special emphasis on radiologic imaging. The literature was reviewed using the MEDLINE database., Results: In 2 cases, VS was the first manifestation of sarcoidosis. Severe pain was present in all patients. Chest radiographs showed normal results. Therapy with corticosteroids and calcitonin relieved the pain. In 2 patients, the pathologic vertebral magnetic resonance imaging abnormalities normalized with treatment., Conclusions: Although bone lesions in sarcoidosis may occur throughout the entire skeleton, axial involvement is rare. VS may be the initial presentation of the disease, and pain frequently is present. This condition is usually responsive to glucocorticoids. Magnetic resonance imaging may be helpful in monitoring the response to treatment., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Thoracotomy is used to approach and treat anterior spinal lesions arising from various causes. Between 1990 and 1997, we treated 56 patients (40 men and 16 women) between 14 and 67 years old (mean 38.4). All had spinal lesions that were impossible or difficult to reach by a posterior approach. Thirty-one (55.3%) had suffered spinal damage, 8 (14.3%) had spinal deformities, 7 (12.5%) had metastatic tumors, 5 (8.9%) had herniated discs, 4 (7.1%) had Pott's disease and 1 (1.8%) had osteolysis at D6. Thoracotomy was left-sided in 35 cases (62.5%) and right-sided in 19 (33.9%). Video-assisted thoracoscopy was used twice (3.6%). The level of incision was based on the site of the lesion, and the pleural cavity was opened in all cases except one. The posterolateral pleuro-diaphragmatic fold was dissected and the diaphragm opened for retroperitoneal access in 37 cases (66.1%) of thoracolumbar disease. Orthopedic treatment consisted of autologous bone grafts in all cases and placement of a Kaneda splint in 32 cases (57.1%). One patient had to undergo surgery a second time due to inappropriate placement of the vertebral splint. Pneumothorax occurred in one patient after removal of pleural drains. The incision became infected in one patient, and one case of ileal paralysis was observed. Overall, morbidity was 7.1%. We conclude that thoracotomy offers a good alternative approach to spinal lesions. Results are good and morbidity low.