B. J. Cremin

Traumatic Renal Artery Occlusion: 21 Cases

Sixteen cases of unilateral and 5 cases of bilateral traumatic renal artery occlusion caused by avulsion or thrombosis are presented. The injury typically follows automobile-pedestrian accidents to young male subjects. Associated extrarenal injuries are usual but non-pedicle renal injury is infrequent. Suspicion of the unilateral injury depends upon recognition of absence of visible excretion at urography. The clue to bilateral occlusion is anuria. Diagnosis is confirmed by urgent arteriography. Hematuria was absent in 24 per cent and the injury was missed at laparotomy in 29 per cent of the initial explorations. Renal function was salvaged by arterial reconstruction in 2 cases, 12 hours and 5 months after injury respectively. Hypertension developed in 50 per cent of the cases and was generally mild.

Wormian bones in osteogenesis imperfecta and other disorders

When are Wormian bones significant is not an easy question to answer, but its relevance is important in relation to bone dysplasias such as osteogenesis imperfecta. Recognition will differ with age of patient, radiographic objectivity, and personal subjectivity. In order to attempt an answer, the skull radiographs of 81 cases of osteogenesis imperfecta of varying ages were examined for the presence of wormian bones. These were compared against the incidence of Wormian bones in 500 skull radiographs of normal children. Significant Wormian bones as against normal developmental variants were considered to be those more than 10 in number, measuring greater than 6 mm by 4 mm, and arranged in a general mosaic pattern. They were found in all the cases of osteogenesis imperfecta but not in the normal skulls. The occurrence of significant Wormian bones in other bone dysplasias from our material and that of the literature was recorded. Other incidental findings in the skulls of the cases of osteogenesis imperfecta were also appraised.

Skeletal complications of type I gaucher disease: The magnetic resonance features

Abnormalities on magnetic resonance imaging (MRI) are reported in six individuals with various skeletal complications of type I Gaucher disease. The changes were a nonhomogeneous reduction in both T1 and T2 marrow signals with increased T2 signals during avascular episodes. MRI proved an excellent technique for the assessment of bone marrow changes in Type I Gaucher disease and for assessing avascular complications. It was not able to differentiate between pseudo-osteomyelitis and pyogenic osteomyelitis without clinical correlation. The problems studied included the extent of intramedullary Gaucher cell infiltration, avascular necrosis of femoral heads, assessment of bone pain from pseudo-osteomyelitis and the relationship of skeletal disease to splenectomy.

Clinical and Genetic Aspects

The sclerosing bone dysplasias are all uncommon and some are distinctly rare. In order to give a perspective of their relative prevalence, the approximate totals of reported cases in the world literature and their modes of inheritance are given in Table 2–1.

Radiological patterns of pulmonary tuberculosis in the paediatric age group

The radiological patterns of culture-proven pulmonary tuberculosis in 154 children under the age of 14 years were studied. Good quality radiographs were an essential requirement to the study, and in cases where lymphadenopathy was in doubt, tomograms or high kV magnification films were obtained. The radiographical terms used were defined and the results of film review were analysed to show the prevalent patterns. These are summarized at the end of the article. It is felt that awareness of the radiographic patterns in paediatric pulmonary tuberculosis will be of value to those working in communities where tuberculosis is unusual or rare, in immigrant communities, and also for the investigation of children who are inadvertently exposed to the disease.

The radiological spectrum of fibrodysplasia ossificans progressiva.

The radiographic manifestations of nine South African patients with fibrodysplasia ossificans progressiva (FOP) have been analysed in detail. In three of these individuals, a 10--15 year follow-up was available. The condition usually presents before the age of 5 years; in one infant, abnormal calcification was demonstrated at 9 months. The disorder is remorselessly progressive but the hands, diaphragm and viscera are spared. Ossification in ligaments at the site of their attachment to long bones produces a pseudo-exostosis appearance. Tethering of the shafts of long bones and growth at the proximal metaphyses can result in dislocation of shoulder and hip joints. In the hip joint the sloping configuration of the acetabular roof is produced by normal growth at the proximal metaphyseal end of the tethered femur. Calcification in periarticular tissues, together with bony and fibrous ankylosis, contributed to joint fixation. A similar process of growth inhibition leads to disparity in size between the bodies of the cervical vertebrae and their posterior appendages. It is concluded that apart from the developmental abnormality in the great toes, the skeletal changes in FOP are the consequence of soft tissue ossification and that the condition is not a primary bone dysplasia.

Osteogenesis imperfecta congenita.

The clinical and radiographic features of 47 cases of neonatally manifest osteogenesis imperfecta were analyzed. A scoring system was devised to code the degree of skeletal changes. A score of 2.7 and more carried a prospective mortality of 88%. Scores of 2.6 and less were associated with a survival rate of 90%. The prognosis was particularly favourable in a subgroup of patients characterized by marked bowing of the lower extremities, mild involvement of the rest of the skeleton and white sclerae. Neonates with these features tended to have a good long-term prognosis, with few additional fractures and partial or total spontaneous resolution of the limb deformity. The study confirmed the genetic and prognostic heterogeneity of the disorder, which comprises several autosomal dominant and recessive entities.

The radiology of sclerosteosis

Sclerosteosis is an unusual disorder in which increased skeletal density is associated with abnormalities of bony modelling. The condition is progressive and complications arise due to cranial nerve involvement. Death from raised intracranial pressure occurs in early adulthood. In order to define the range and extent of the bony abnormalities, the radiographic features of 18 affected individuals have been analysed. The recognition of characteristic changes permits diagnostic precision.

The spectrum of polycystic disease in children.

Both childhood and adult polycystic disease is seen in our series of eleven children, demonstrating the broad spectrum of both renal and hepatic disease clinically and radiographically. The relationship of radiographic patterns and survival is assessed. Confusion in the definition and classification of these disease entities is discussed.

A review of the ultrasonic appearances of posterior urethral valve and ureteroceles.

Ultrasonographic studies of patients with posterior urethral valve and ectopic ureterocele vary in relation to the severity of obstruction and changes in the upper tracts. A broad review of these conditions, stressing sonography (including use of the perineal approach to the upper urethra) shows the great value of sonography but emphasizes the need to correlate with other imaging modalities (urography, cystourethrography, nuclear imaging).