Rachid Haidar

Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia

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.

Bone disease and skeletal complications in patients with β thalassemia major

Increased survival in patients with β thalassemia major (TM) allowed for several complications of the disease and its treatment to manifest, one of which is bone disease. Osteoporosis in this patient population results from a variety of genetic and acquired factors. Early diagnosis and prevention are essential and several measures have been evaluated for management including bisphosphonates. Fracture prevalence in TM patients seems to be clustered in mid adulthood, and is related to vitamin D deficiency and low bone mineral density. Fracture healing in patients with TM does not seem to be different from that in normal individuals. Bone and joint pain are a common manifestation of the underlying pathophysiology or may be related to iron chelator intake. Intervertebral disc changes are seen in patients who are heavily iron overloaded or those who are chelated with deferoxamine. Spinal deformity is common in TM, yet the prognosis is benign with spontaneous resolution frequently observed. Further research is warranted to evaluate the mechanisms, clinical implications, and optimal management of bone disease in this patient population.

SIMULTANEOUS OPEN REDUCTION AND SALTER INNOMINATE OSTEOTOMY FOR DEVELOPMENTAL DYSPLASIA OF THE HIP

We have studied retrospectively 37 hips in 36 children at an average of 91 months after simultaneous open reduction and Salter innominate osteotomy for developmental hip dysplasia. At the latest review 97.3% were clinically and 83.8% radiologically good or excellent. In three hips (8%) there were signs of avascular necrosis, but only one had been symptomatic. There were no cases of recurrent posterior displacement.

Duration of post-surgical antibiotics in chronic osteomyelitis: empiric or evidence-based?

Chronic osteomyelitis is a relatively common infection and is often a lifelong disease. Traditionally, osteomyelitis has been treated with 4-6 weeks of parenteral antibiotics after definitive debridement surgery. Antibiotic-impregnated cement beads have also been used as adjuvant therapy for chronic osteomyelitis. However, this time frame of antibiotic treatment has no documented superiority over other time intervals, and there is no evidence that prolonged parenteral antibiotics will penetrate the necrotic bone. There is no solid evidence in the medical literature to support the continuous use of long duration antibiotic treatment for chronic osteomyelitis. A small number of comparative trials on the treatment of chronic osteomyelitis have been published. Also, the type of surgical procedures practiced in the past in treating chronic osteomyelitis and the lack of effective muscle flap application might have contributed to the prolonged antibiotic treatment. And although the surgical approach to the treatment of chronic osteomyelitis has advanced markedly, still the same duration of antibiotic treatment is adopted. In this review we question the continuous and traditional use of long-term antibiotic treatment for chronic osteomyelitis in spite of the advances in surgical treatment using flaps. The medical literature, including studies in animals and humans, was searched for evidence to support the use of short courses of antibiotics. We hope this review will provoke the initiation of animal studies and clinical trials assessing the use of short courses of antibiotics for chronic osteomyelitis.

Propionibacterium acnes causing delayed postoperative spine infection: review.

Abstract Propionibacterium acnes was previously considered a contaminant and its role in spine infection has been understated. Although rare, such infections are present especially after instrumented spine surgery. They are usually delayed and hard to diagnose. Delayed infection should be suspected in patients with pain and a history of spine surgery. In this article, we review the diagnostic challenges associated with delayed P. acnes infection after spine surgery, guided by relevant studies in the literature. The medical databases of PubMed, Medline and Embase were searched for the literature on delayed spine infections and osteomyelitis following spinal procedures. The medical literature was reviewed for articles published between 1955 and 2008. Our review of the literature revealed 13 cases of P. acnes osteomyelitis following surgical procedures involving the spine. We also present a clinical case of delayed P. acnes osteomyelitis following discectomy and fusion with instrumentation.

Seven‐year experience of using Repiphysis® expandable prosthesis in children with bone tumors

Results of the use of the non‐invasive expandable endoprosthetic device Repiphysis® in limb salvage surgery for skeletally immature children with bone cancer have been promising.

The spine in β-thalassemia syndromes.

Study Design. Literature review. Objective. To present a comprehensive overview of spinal involvement in patients with &bgr;-thalassemia, aiming to orient the spinal surgeon to these potentially disabling complications. Summary of Background Data. &bgr;-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 &bgr;-thalassemia is common and diverse yet still requires further prospective evaluation.

Complete cleft sternum and congenital heart disease: review of the literature.

Cleft sternum is a rare malformation due to failure of sternal fusion. It forms a distinct entity from the thoracic subtype of ectopia cordis where there is extra mediastinal displacement of the heart with various degrees of sternal clefting. The intrinsic cardiac anomalies contribute to a very high neonatal mortality rate.1 Isolated cleft of the sternum which is extremely rare has not been commonly associated with cardiac anomalies and usually has a very good prognosis. We reviewed a total of 35 cases of isolated complete cleft sternum (CCS) published since 1900, with their associated anomalies. We report, to the best of our knowledge, a newborn male with complete cleft sternum and a previously unreported association of cardiac malformation consisting of mesocardia, double outlet right ventricle (DORV), large subarterial ventricular septal defect (VSD) and pulmonary hypertension.

Hip Fractures in Lebanese Patients: Determinants and Prognosis

Hip fractures are the most costly of osteoporotic fractures, but little is known about their epidemiology in the Middle East. Hip fracture patients and controls with osteoarthritis admitted to our institution from 1992 to 2002 were studied. Information on gender, age, type of fracture, comorbid conditions, and medications use was obtained. The mean age for hip fracture patients (n = 274) was 72.1(8.5) yr, and for controls (n = 112), it was 71.1(4.4) yr, two-thirds of fractures occurred in women. Fractures were 59% intertrochanteric, 34% femoral neck, and 7% subtrochanteric, with no gender differences. Hip fracture patients were more likely to have had a prior fracture and to suffer from neurological, gastrointestinal, or renal comorbidities, as compared to controls. Less than 10% of hip fracture patients received any therapy for osteoporosis, either on admission or discharge. In a subset of patients with follow-up, the mortality rate was 47% in subjects with hip fracture, and most deaths occurred within the first year postoperatively. Gender but not fracture type affected mortality. Lebanese patients with hip fractures are younger, more likely to sustain intertrochanteric fractures, and experience higher mortality than Western counterparts. Few subjects received osteoporosis therapy. This study carries important public health implications on the management of hip fracture in subjects from Lebanon and, possibly, the Middle East.

Practical approach to the child presenting with back pain

Back pain may be the presenting symptom of many children attending to pediatric health care settings. As such, awareness to the common etiologies of back pain in this subgroup of patients remains essential as it guides appropriate diagnosis. Although several clues may be derived from the child’s history and physical examination, imaging techniques may be required to confirm the underlying diagnosis. This review summarizes the most commonly encountered causes of back pain in children and highlights diagnostic approaches that will ensure early diagnosis and intervention for a more favorable outcome.