Progressive diaphyseal dysplasia: evaluation of corticosteroid therapy.

Progressive diaphyseal dysplasia is characterized clinically by crippling leg pain, fatigue, headache, poor appetite, muscle weakness, and waddling gait. Twelve affected patients, aged 2 years 4 months to 40 years, were treated with intermittent courses of low doses of prednisone given in a single dose on alternate mornings for periods ranging from 6 months to 10 years. The average initial dose of prednisone was 0.6 mg/kg/d, and average maintenance dose was 0.3 mg/kg/d. Relief of all crippling symptoms was achieved in all patients. No untoward serious side effects have been observed, and the growth of children was not slowed. However, corticosteroid therapy should be restricted to patients suffering from crippling pain. The mechanism through which steroids act remains undefined.

Progressive diaphyseal dysplasia: genetics and clinical and radiologic manifestations.

Progressive diaphyseal dysplasia was found in a three-generation family including 13 affected individuals, the largest family reported to date. Our study confirms that progressive diaphyseal dysplasia, also known as Engelmann's or Camurati-Engelmann disease, is an autosomal dominant disorder with variable osseous and muscular manifestations. Disease distribution among patients, within a given patient, or even in individual bones is unpredictable. The femur is the most commonly and severely affected bone and hence most useful for radiographic screening of possible patients. Radiographs provide a meaningful assessment of disease activity and extent. The severity of symptoms is generally proportionate to severity of involvement shown by roentgenography. Exophthalmos due to osteosclerotic dysplasia of the skull occurred in more than half of the patients with progressive diaphyseal dysplasia. Twelve-year follow-up of this family, with affected individuals ranging in age from 6 months to 12 years, indicates that progressive diaphyseal dysplasia may progress or become quiescent and be remarkably inactive despite advanced osteosclerosis and structural deformity.

Splenic echinococcal cyst burrowing into left pleural space.

We describe a rare case of hydatid cyst in the spleen which communicated to the left pleural cavity. The patient presented with respiratory distress, characterized by nonproductive cough and dyspnea. The difficulties in diagnosis, using standard laboratory and radiologic techniques, were overcome by the use of ultrasound examination diagnosing both the cysts and the supra-diaphragmatic extension.

Application of a bedside whole blood D-dimer assay in the diagnosis of deep vein thrombosis.

Cross-linked fibrin degradation products have been used to detect venous thrombosis. While the sensitivity of plasma D-dimer measured by ELISA in the diagnosis of deep vein thrombosis (DVT) is high, the utility of ELISA methods is limited in a clinical setting. This study analysed the diagnostic value of a rapid D-dimer assay performed on whole blood samples (SimpliRed D-dimer) compared with latex and ELISA in 86 patients suspected of having DVT. SimpliRed D-dimer was positive in 47/50 of patients with DVT established by Doppler ultrasound (DU; sensitivity 94%). SimpliRed D-dimer was positive in 35/37 of patients with proximal DVT, nine out of nine with popliteal DVT and three out of four with superficial thrombophlebitis. The specificity of SimpliRed D-dimer in the diagnosis of DVT was 61%. The sensitivity of the SimpliRed D-dimer assay was at least comparable with the ELISA (87%) and superior to the latex assay (80%). The positive predictive value (77%), the negative predictive value (88%) and the overall accuracy (80%) of the SimpliRed assay were better than the ELISA and latex methods. It is concluded that SimpliRed D-dimer is a rapid useful assay for screening of patients suspected of having deep vein thrombosis.

Duplex sonography in the diagnosis of portal vein thrombosis.

Portal vein thrombosis was readily diagnosed by pulsed duplex Doppler sonography in five patients. The cases represent different stages in the development of the thrombotic event, from partial or complete occlusion by an echogenic intraluminal thrombus to cavernous transformation of the portal vein. The unique ability of duplex scanning to demonstrate abnormal vascular structures and flow pattern is emphasised. This method should be the technique of choice in the diagnosis of portal vein thrombosis.

Non-functioning islet cell tumours of the pancreas: a review of radiological literature and a report on two cases.

Two cases of endocrine inactive malignant islet cell tumors diagnosed by angiography are presented. The angiographic features are described and the differential diagnosis of the tumor and its metastases are discussed. Radiological literature of the past decade on this subject is reviewed.

Late changes in veins after deep venous thrombosis: ultrasonographic findings.

In a prospective trial, 43 patients (45 legs) were re-examined by high resolution and continuous-wave Doppler ultrasonography 3-32 months after an acute episode of deep venous thrombosis (DVT). The veins were abnormal in 31 legs (69%). Abnormal veins were either narrowed with a highly echogenic lumen (thought to represent persistent occlusion) or with a relatively sonolucent lumen and thickened walls (thought to represent recanalized veins). Collateral veins were present. Some compression by probe could be achieved in partially occluded veins, but full collapsibility was absent. Continuous-wave Doppler studies were similar to those in acute DVT. In order to be able to diagnose new episodes of DVT in patients who have had DVT in the past, a baseline ultrasound study should be carried out approximately 6-12 months after an acute episode. In the absence of such a baseline study, contrast phlebography should be resorted to.

High-resolution real-time ultrasonography in the diagnosis of deep vein thrombosis.

The contribution of high-resolution real-time ultrasonography to the diagnosis of deep vein thrombosis (DVT) was evaluated in a prospective study performed on 96 patients. 45 patients were also examined with contrast venography and 79 with continuous wave Doppler ultrasound. The femoral and popliteal veins were examined for compressibility by pressure with the transducer probe, response to deep inspiration and Valsalva's manoeuvre and presence of an intraluminal mass. The contralateral veins were routinely screened for comparison. The diagnostic sensitivity versus phlebography was 87%, and the specificity 100% for the femoral vein. When including the popliteal vein the specificity was 91%. Real-time ultrasonography together with Doppler ultrasound examination is an effective non-invasive approach for the diagnosis of DVT. Phlebography can be avoided if these two examinations are unequivocally positive or negative for DVT.

Human tails.

Two children and one infant with a "human tail" are presented. The patho-embryology of this medical curiosity is briefly discussed. Treatment is usually unnecessary but resection of part of the coccyx together with the "tail" may become indicated by coccygodynia or for aesthetic reasons.

Lithotripsy and the modern imaging department.

Until recently surgery was required to remove unpassed stones from the urinary tract. Today, percutaneous and extracorporeal methods are replacing surgery, such as chemolytic, nephroscopic, ultrasonic, electrohydraulic and extracorporeal shock wave lithotripsy (ESWL). These procedures require close collaboration between the urologist and the radiologist. It is the radiologist who will localize the stone by X-rays or ultrasound and provide the percutaneous approach and dilatation of the tract, leaving the actual stone extraction to the urologist. Even with ESWL supplementary percutaneous procedures are often necessary, again requiring radiologic-urologic teamwork. The percutaneous procedure can be carried out in the imaging department with existing radiologic equipment. Additional X-ray apparatus for the operating room is not necessary. In the Israeli setting of socialized medicine, the involved physicians are unaffected by financial considerations. Collaboration between radiologists and urologists therefore seems assured.

Evaluation of high viscosity contrast media in canine selective coronary arteriography.

A special high viscosity preparation of water soluble radiopaque contrast media was explored in animals for its suitability in selective coronary angiography. The high viscosity required power injection to accomplish adequate filling during selective coronary arteriography. The anticipated angiographic advantages, such as prolonged visualization and coating of the vascular walls, were marginal. Comparison with conventional preparations of the same contrast agent suggests that the high viscosity itself exerts some protective effect with regard to the immediate side effects on the electrocardiogram and mechanical function of the myocardium. However, the high viscosity preparation induced electrocardiographic signs compatible with myocardial ischemia not usually seen to follow the injection of conventional contrast agents. These were followed by mechanical heart failure or ventricular fibrillation resulting in death of 6 of the 10 experimental animals. It was concluded that high viscosity contrast media preparations are unsuitable for use in clinical selective coronary arteriography as presently practiced.

Image-directed Doppler ultrasound in the diagnosis of lower-limb venous insufficiency.

Ninety-eight legs in 73 patients were evaluated with image-directed Doppler ultrasound for suspected chronic venous insufficiency. Examinations of the entire venous system were performed to determine lumen patency and valvular competence. Reflux during Valsalva maneuver in the femoral and great saphenous vein, and, after release of distal compression in the popliteal vein, indicated valvular incompetence. Twenty limbs had normal veins; Baker's cysts were found in three. Superficial venous insufficiency was found in 25 cases. Deep reflux, either alone or in combination with superficial reflux, was the predominant pathological feature (53 legs). Photoplethysmographic studies of the veins were performed on 24 limbs. Image-directed Doppler ultrasound was more successful in defining the exact site of venous incompetence and in detecting nonvenous pathology. By the combination of real-time imaging of the vein with gated Doppler sonography of flow, image-directed Doppler sonography is becoming the method of choice for the investigation of chronic venous insufficiency.

Thickness of the gallbladder wall in patients with hypoalbuminemia: a sonographic study of patients on peritoneal dialysis.

Thickening of the gallbladder wall in patients with ascites has been attributed not to the presence of ascites but to the associated hypoalbuminemia. The contribution of hypoalbuminemia was examined by sonographically measuring the thickness of the gallbladder wall in 12 patients with chronic renal failure who were on peritoneal dialysis. All patients had hypoalbuminemia, with serum albumin levels of 2.2-3.3 g/dl (normal range, 3.5-5.0 g/dl). In all patients, the thickness of the gallbladder wall was within the normal range of less than 3 mm. In the patients studied, hypoalbuminemia was not a cause of thickening of the gallbladder wall even when peritoneal fluid was present.

Progressive diaphyseal dysplasia (Camurati-Engelmann): radiographic follow-up and CT findings.

Sixteen patients with progressive diaphyseal dysplasia (PDD) and aged six months to 76 years were examined. Fourteen cases were hereditary, two were not. The progression of the radiologic manifestations in 13 patients who were followed up from 1 to 32 years and the computed tomography (CT) scans from five patients were obtained. The progression of PDD was slow and unpredictable, from minimal endosteal thickening of the mid-diaphyses in one pair of long bones to severe sclerosis of long bones, skull, and vertebrae. The severity of the osseous changes was not age dependent. A six-stage system was used to grade the severity of involvement and progression of PDD. CT scans demonstrated muscle mass that was preserved and showed the distribution of the osteosclerotic process, which was irregular and inhomogeneous. CT scanning was advantageous over plain radiography in this respect. Endosteal involvement was more extensive than periosteal thickening. CT scans also showed a distinct pattern of vertebral sclerosis that was confined to the posterior areas of the vertebral body and arches. In light of the paucity of characteristic clinical signs of PDD, the recognition of the radiologic features is mandatory for the diagnosis of this disease.

False uroradiologic pathology in children with urinary tract infection and fecal impaction.

Intravenous pyelography (IVP) in 5 children with urinary tract infection detected various uroradiologic abnormalities. Despite the lack of history of constipation, a common denominator to all 5 patients was the finding of fecal impaction in the rectosigmoid. In the first 3 patients, repeated complete IVP series done several weeks later and after preparatory bowel enema were normal. In the last 2 patients, the radiologic investigation was interrupted when the first exposure disclosed uroradiologic abnormalities and fecal impaction. Following immediate bowel enema, the studies were completed on the same settings to be found normal. It is concluded that in the face of fecal impaction, pathologic uroradiologic findings should be interpreted with great caution. Moreover, in such cases, intraprocedure cleansing enema is feasible and recommended.

High-resolution real-time ultrasonography. Diagnosis and follow-up of jugular and subclavian vein thrombosis.

Thrombosis of the internal jugular and subclavian veins was demonstrated by high-resolution real-time ultrasonography in eight patients. The sonographic findings were an intraluminal mass of low or mild amplitude echoes, loss of respiratory rhythmicity and venous pulsation, absence of vein response to respiratory maneuvers (Valsalva and sniff test) and incompressibility of the thrombotic jugular vein. Follow-up examinations after anticoagulant and local streptokinase therapy allowed monitoring of the treatment. High-resolution ultrasonography is a useful noninvasive technique for the diagnosis and follow-up of therapy in thrombosis of the jugular and subclavian veins.

The use of ultrasound scanning in the management of developmental disorders of the hip.

Ultrasound was used, together with clinical examination, to help in the diagnosis and to evaluate the treatment of 48 babies with congenital disorders of the hip. The femoral head, and its relation to the acetabulum, can be demonstrated before it becomes visible on radiographs. Ionising radiation is avoided. Ultrasound scanning can be used to visualise the repositioning of the femoral head. It is also possible to examine babies on an abduction frame.

Ultrasonographic detection of plastic materials in various organs of the human body.

The ultrasonographic appearance of different plastic catheters, tubes, and prostheses used in medical practice were tested in vivo and in vitro. According to the direction of the sound beam, the different plastic catheters and tubes appeared as parallel echogenic lines if scanned longitudinally, or as echogenic dots if scanned transversely. The exact position of the tip of the catheter and its relationship to neighboring organs can be established by ultrasonography. Repositioning of tubes, if necessary, can be done under ultrasonographic guidance. The flexibility and mobility of modern ultrasound scanners allows one to examine the sick patient with minimal disturbance, and to evaluate the location and effect of the various plastic catheters and tubes inserted into the body for medical reasons.