Angiocardiography in congenital subvalvular aortic stenosis: prognosis and operative indications.

Twenty-three patients with congenital subvalvular aortic stenosis are reviewed. The importance of classifying this stenosis into four types based on angiocardiographic findings is stressed, as are the indications for left heart catheterization during the preoperative and postoperative course of the disease. The type of congenital subvalvular aortic stenosis should be an important consideration during the discussion of operative indications in both asymptomatic and symptomatic patients.

Pseudotumoral aspect of posttraumatic digital synovitis.

A case of posttraumatic subacute synovitis with pseudotumor of the digit with bone lesions on plain film is reported. Arteriography showed a pattern of hypervascularity that did not appear malignant and evoked a benign tumor of the synovial. Biopsy helped establish diagnosis of common subacute synovitis. Swelling disappeared spontaneously during subsequent months. Diagnosis of villonodular synovitis is difficllt when proliferation of histiocytes is present.

Computed tomography with geometrical enlargement.

Third generation CT scanners perform examinations of small objects without employing all the detectors. Geometrical enlargement solves this problem by moving the tube-detector assembly according to the size of the object to be scanned; this improves spatial resolution, reducing both the tube current and the skin dose. Such an application for pediatric CT and small objects is of particular interest.

Radiological patterns of truncus arteriosus in plain roentgenography.

15 cases of persistent truncus arteriosus in infants and children were subjected to roentgenographic examination by the authors via plain frontal and profile roentgenography of the thorax. In infants (12 patients) the left midzone section is concave or straight, especially in the lower part. This aspect is typical of this congenital anomaly if it is associated with active pulmonary hypervascularisation. Cases without pulmonary hypervascularisation are very difficult to diagnose safely. In 7 profile roentgenographs 3 retrosternal enlarged areas were seen soon after birth. In children of 2 to 6 years of age, cardiac dilatation with a right-side aortic arch and pulmonary hypervascularisation are very typical. In children over 6 years of age we observed an Eisenmenger's syndrome with a retrosternal enlarged area. According to the authors' experience, a combination of a right-side aortic arch associated with pulmonary hypervascularisation must be considered highly typical.

Radiologic patterns of the hypoplastic left heart syndrome.

The hypoplastic left heart syndrome is a malformative complex which unfortunately can not be treated surgically. This cardiopathy is the result of a serious embryogenic error and takes into consideration the different anatomic varieties described here. Borderline cases are also noted especially isthmic coarctation of the aorta or aortic stenosis in the neonatal period. Though several years ago, the diagnosis of the hypoplastic left heart syndrome was made only on pathology, it is now possible to make this diagnosis using clinical, laboratory and radiologic examinations. However, they are cases that remain difficult to label precisely, especially in minor forms and we feel that these deserve hemodynamic investigations.

Congenital arterio-venous fistulas (4 systemic extra-visceral fistulas).

Arterio-venous fistulas are rare in children but usually congenital. We report four cases which are very unusual because of their origin or their localization. We compiled a classification of congenital fistulas which is easily used and collected 582 cases of congenital arterio-venous fistulas from the literature.

Cardiac catheterization: dosimetry on patients.

Our study was made on 49 patients and we used capsules of lithium fluoride. The average fluoroscopy time was 32 min. and the average cinema film length was 76.5 metres. The relationship between the entry dose, the fluoroscopy time and the length of cinema film allowed us to calculate a fluoroscopy equivalent time (F.E.T) for each patient which was of 51 min. (32 min. + 76/4) as radiation dose of one minute fluoroscopy equal radiation dose of 4 metres of cinema film. The average heart dose was 0.70 X 10(-3) X FET (GY) or 70 mr/min. There was an absence of relation of the exit dosage to the diameter of the patient and a proportionality of the heart dose to the exit dose. Furthermore the maximum eye dose recorded was 200 mrads and the maximum gonadal dose was 350 mrads.

Pulmonary sequestrations of the upper lobe in children: three presentations.

Pulmonary sequestrations are congenital abnormalities where nonfunctioning lung tissue receives its vascular supply from the systemic circulation (thoracic or abdominal aorta). It is necessary to establish the diagnosis in childhood when the lesions are uncomplicated. The authors present three cases of sequestration of the apex (2 extralobar and 1 atypical) with the main clinical and radiological features. Sequestrations in the upper lobe are rare, and the usual site is the left lower lobe. Plain x-rays show a dense opacity, sometimes air-filled and sometimes with an air-fluid level: angiography is currently the best mean for definitive diagnosis; however, computed tomography will probably be very useful in the future. Differential diagnosis includes tumours of the superior mediastinum (neurogenic tumours, digestive duplication, bronchogenic cysts, pheochromocytoma and hydatid cysts).

Bone lesions secondary to benign phaeochromocytoma. Four cases in childhood.

Metaphyseal sclerotic bone changes associated with benign phaeochromocytoma are very rare in childhood. We report four cases, in each of which the radiographic changes returned to normal after removal of the tumour.

Osteoid osteoma of the rib: a case report.

A case of a 6-year-old boy with a painful dorsal scoliosis secondary to an osteoid osteoma in a rib is described. Surgical excision was performed and the scoliosis decreased. Such cases are very rare.

Malignant change in an intradiploic epidermoid cyst.

We describe a unique case of an intra-diploic epidermoid cyst of the parietal and occipital right bone in a 43 year-old man. The tumor remained quiescent for 12 years and then spontaneously showed malignant changes with intra-cerebral involvement. Fatal outcome occurred one year after surgery, radiation therapy and chemotherapy because of a recurrence. Malignant transformation of the epithelium in epidermoid cyst is very rare. Our case is the first one where this transformation occurred spontaneously without previous surgery.

Low-grade osteosarcoma of metacarpal bone.

A radiograph of the second left metacarpal bone in a 34-year-old man showed irregular lytic areas in the diaphysis that was expanded with perpendicular periosteal reactions. Examination of the first biopsy specimen indicated a fibrous dysplasia. Examination of the second biopsy specimen revealed an unequivocal grade I intramedullary osteosarcoma, desmoid to fibrous dysplasia-like in histological pattern. Three years after an adequate en bloc resection of the metacarpal bone, the man was healthy. The long-term course is usually favorable.

Cone epiphysis of the knee and scurvy.

A rare case of metaphyseal cupping of the distal femur resulting from infantile scury in a child is reported. The radiological and surgical aspects of this case are of particular interest. MRI demonstrated central epiphysiodesis of the right knee, surgery achieved rupture of the epiphyseal plate and resulted in femoral lengthening. The child's limp disappeared as a result.

Cystic degeneration in non-ossifying fibroma.

Two unusual cases of non-ossifying fibromas in boys of 4 years with partial cystic degeneration are presented. The osseous cavities did not respond to corticosteroids.

[How to assess the value of scientific publications?]. / Comment calculer la valeur des publications scientifiques?

The Institute of Scientific Information has edited an index to evaluate the diffusion of scientific articles. It is based on the fact that the more an article is cited as a reference in other articles, the more it is considered to be important, and the higher the coefficient attributed to the journal in which it was originally published. Several methods of evaluation have been proposed, the two main ones being the impact and citation half-life. The impact factor takes into account the average number of times which a journal is mentioned for recent articles published in a given year. The citation half-life gives an insight into the longeivity of the articles of a journal by the number of citations received. This index is an attempt to quantify the notoriety of scientific journals for all scientific medical specialties. Each year, the institute for Scientific Information publishes a classification of the journals of each medical specialty.

[Right aortic arch with retro-esophageal component. 2 cases]. / Arc aortique droit avec segment rétro-oesophagien. A propos de 2 observations.

Right-sided aortic arch with a retro-oesophageal segment is much rarer than right-sided aortic arch without a retro-oesophageal segment. As opposed to the latter situation which is always associated with congenital heart disease, it is usually an isolated finding. The left subclavian artery arises from a posterior diverticulum and may be stenosed at its origin, giving rise to a systolic murmur as in one of the reported cases, or to a subclavian steal syndrome. The ligamentum arteriosum completes the aortic ring and may give rise to respiratory problems at varying ages and of variable severity, sometimes attributed to asthma. In one of the cases followed up over 15 years chronic respiratory problems and late tracheal lesions were observed. Although the possibility of a double aortic arch may be raised, early surgical treatment is desirable.

[Contribution of MRI to the diagnosis of double aortic arch]. / Apport de l'IRM au diagnostic de double arc aortique.

The authors present a case of double aortic arch explored by MRI and discuss the advantages and disadvantages of MRI as compared to other noninvasive imaging methods, such as ultrasounds and digital angiography, in congenital pathology of the aorta. Because it provides good quality images, can explore the aorta in all three dimensions, has optimal vascular contrast without contrast injection and carries no risk, MRI is indispensable in all cases where ultrasonography finds its limitations. Besides, the subjective factor inherent in the operator is very small, and MRI is the best available method to study the relations between the aorta and the surrounding organs; this is particularly important in case of double aortic arch owing to compression of the neighbouring organs with, notably, stridor. However, the vascular map is less accurate than with angiography, and the cost, the problem of access to the machines and the long time required for acquisitions limit the use of MRI.

[Atypical pulmonary stenosis in certain polymalformative syndromes. Prevalence of associated hypertrophique myocardiopathy]. / Les sténoses pulmonaires atypiques au cours de certains syndromes polymalformatifs. Fréquence de la myocardiopathie hypertrophique associée

Certain pulmonary stenoses differ markedly from the usual types of isolated valvular stenosis in their clinical features (maximal murmur situated lower down), their radiological signs (absence of a prominent median arc), the electrocardiograph (atypical electrical axis), the haemodynamic findings (usually a poor gradient), and especially the angiocardiography (valvular dysplasia, lack of a post-stenotic dilatation). They are encountered especially in the multiple malformations, as shown by this series of 23 cases comprising the syndrome of Noonan (9), of Gorlin or 'leopard' (7), of Watson or 'café-au-lait spots' (3), or those defying classification, but always having a disorder of facial structure and mental deficiency, sometimes with deafness (4). The frequent finding of a gradually progressive hypertrophic cardiomyopathy is a feature of these syndromes, and usually accounts for the abnormal ECG findings. It leads to a discussion of the pathogenesis, and of the place of these syndromes in a wider context, alongside the phacomatoses and the hereditary forms of neuromuscular degeneration.

[Radiological anatomy of the arteries of the foot]. / Radio-anatomie des artères du pied.

Two hundred arteriographies of the feet performed after injuries were studied. The patients had no arterial disease. Various patterns of the vascularisation of the feet are described. The authors emphasize the frequent variations of distribution which are sometimes different from the usual pattern but are however not pathological and can be explained by hemodynamic disturbances. The authors also describe some anatomical criteria which are useful to identify the different arteries of the feet on the arteriographies with different positions of the feet. These criteria will be very useful with digital angiography.

[Hemimelic epiphyseal dysplasia. Apropos of a case]. / Dysplasie épiphysaire hémimélique. A propos d'un cas.

Dysplasia epiphysealis hemimelica (tarsomegaly, Trevor's disease) is a rare osteochondrodystrophy producing cartilaginous epiphysial hypertrophy of hemimelic type involving mainly the lower limb. Lesions are generally medialy located (medial condyle of the femur, medial malleolus, talus, tarsal scaphoid and first metatarsal bone) and clinical signs are not specific; therefore radiological diagnosis is very important. The disease gives rise to a massive ossification of the hypertrophic cartilaginous areas and fast degenerative changes (arthrosis) of the involved joints. Surgery is often unsuccessful.