Imaging of malignant infantile osteopetrosis before and after bone marrow transplantation.

BACKGROUND: Malignant infantile osteopetrosis (MIOP) is a sclerosing bone disease caused by absence or defective function of osteoclasts. Since these are of haemopoietic origin, the disease can be cured by allogeneic stem-cell transplantation, but there are no detailed studies of radiological follow-up of these procedures. OBJECTIVE: To investigate the radiological findings at presentation and follow-up in children undergoing bone marrow transplantation (BMT) for MIOP. MATERIALS AND METHODS: Examination of the records and imaging studies of nine paediatric patients undergoing BMT for MIOP during 1988-2000. RESULTS: Presentation findings included characteristic features such as fractures, subperiosteal new bone formation and rachitic appearances. Five children engrafted successfully, allowing assessment of the nature and speed of resolution of radiological features after transplantation. CONCLUSIONS: Radiological improvement was apparent within 2 months of successful engraftment with almost complete resolution of abnormalities after 1 year. Studies in two children who are, respectively, 58 and 83 months post-transplant show complete resolution of all bone changes.

Imaging in haematopoietic stem cell transplantation.

Haematopoietic stem cell transplantation (SCT) is used to treat a wide range of malignant and non-malignant haematological conditions, solid malignancies, and metabolic and autoimmune diseases. Although imaging has a limited role before SCT, it is important after transplantation when it may support the clinical diagnosis of a variety of complications. It may also be used to monitor the effect of therapy and to detect recurrence of the underlying disease if the transplant is unsuccessful. We present a pictorial review of the imaging of patients who have undergone SCT, based upon 15 years experience in a large unit performing both adult and paediatric transplants.

The role of magnetic resonance imaging in the preoperative assessment of anorectal anomalies.

The role of magnetic resonance imaging (MRI) compared to other investigations in providing information relevant to and affecting surgical management in patients with anorectal anomalies (ARA) was studied in two groups of patients. In group 1 a pilot study was done in 9 patients with colostomy for high ARA awaiting posterior sagittal anorectoplasty. These patients had MRI, plain radiographs, a distal loopogram through the mucous fistula, ultrasound scans of the spinal cord and urinary tract, and a micturating cystourethrogram. The level of bowel and spinal-cord termination was correctly determined in all patients by MRI and conventional investigations. The presence or absence of a fistula, urological abnormalities, and bony abnormalities of the pelvis and spine were better visualized by conventional investigations than with MRI. The striated-muscle complex (SMC) was well-visualized by MRI, the thickness accurately graded, and the findings confirmed at operation. The information on muscle thickness and the presence or absence of a fistula was interesting, but played no part in decisions made about surgical management, and the operative technique was not altered. In group 2 the patients were much older and had MRI for persistent problems, mainly soiling; 6 had low lesions and 6 had high lesions. Three patients with low lesions were found to have intraspinal abnormalities on MRI; 4 were found to have malpositioned bowel in the SMC, which was again accurately visualized and graded. Therefore, in 7/12 patients of this group a surgically correctable abnormality was found. We conclude that MRI has no role as a primary investigation in patients with high ARA, but it is useful if there are suspected spinal-cord problems or persistent problems after definitive surgery.

Are routine chest radiographs prior to angiography of any value?

Chest radiographs are frequently requested prior to diagnostic angiography, though there is no published evidence of their clinical utility. This study was undertaken to evaluate their contribution to patient management. The routine chest radiographs obtained prior to peripheral and coronary angiography in 240 patients were prospectively reviewed for abnormalities likely to affect management. Two hundred and twenty (91.7%) examinations were performed, of which 164 were obtained within 24 h of angiography. Previous radiographs were available in 154 patients (64.2%). One hundred and sixteen radiographs were normal. There were 117 abnormalities on the radiographs of 104 patients, mainly cardiac enlargement and heart failure. No angiogram was postponed or cancelled because of abnormalities detected on a routine radiograph, although radiographic findings led to a change in the volume of contrast medium injected into dilated aortic roots in 10 patients undergoing cardiac catheterization. Pre-angiography radiographs had no effect on the practice of peripheral angiography. In only one patient were further investigations and therapy instigated because of findings, but even in this case these findings were present on previous studies. We conclude that routine pre-angiography chest radiographs are not necessary in the absence of specific clinical indications.