Omodysplasia.

Three cases of a new congenital bone disorder associating facial anomalies (depressed nasal bridge, broad base of the nose, long philtrum) with short humeri. The complex skeletal abnormalities consist of a defect of growth of the distal end of the humerus, a hypoplastic everted condyle, an upper radioulnar diastasis, and a anterolateral dislocation of the head of the radius. The condition is dominantly inherited. Two other cases with the same facial anomalies and osteoarticular abnormalities of the upper limbs are described. These cases also showed a severe micromelic dwarfism due to shortness of the long bones, particularly the femora. The present authors consider that these represent variable expressivity of the same disorder and propose that this condition be called omodysplasia (from the Greek term for humerus).

Early and late results of surgical correction of pulmonary artery sling.

Since 1976, we have operated on 4 children with pulmonary vascular sling. They were 5, 12, 19, and 54 months old. All patients were seen initially with severe stridor. Diagnosis was made by barium swallow in all 4. Each child was operated on through a left thoracotomy; the left pulmonary artery was dissected deep between the trachea and esophagus. Systemic heparinization and microsurgical techniques were used. All patients improved after operation. Radioisotope pulmonary ventilation/perfusion scans were performed 9, 9, 14, and 21 months after operation. Decreased ventilation was noted in the right upper lobe of one scan; the other three ventilation scans were normal. Perfusion scans showed good patency of both pulmonary arteries in all 4 patients. It is concluded that pulmonary vascular slings should be treated surgically as soon as diagnosed. Delayed operation can lead to severe tracheomalacia, as demonstrated in our 54-month-old patient.

The use of stereophotogrammetry to measure acetabular and femoral anteversion.

Stereoradiography with a base shift of the source of illumination was used to produce pairs of radiographs to be measured by stereophotogrammetric techniques. The direction of shift was parallel with the longitudinal axis of the body, so that each radiograph in the stereopair could be used for other clinical purposes. A base shift of 10 centimetres with a distance of 100 centimetres between the focus and the film gave acceptable value of stereoscopic parallax. The radiographs were measured using a Hilger and Watts medical stereometer. This method was checked with test specimens, namely an osteotomised pelvis in which one acetabulum could be rotated and an osteotomised femur in which the whole upper portion could be rotated against the shaft. Measurements made on the acetabulum and its radiographs showed a correlation coefficient of 0.9838 over the range 0 to 30 degrees of anteversion, with a mean error +2.54 degrees and a standard deviation of +/- 1.52 degrees (n = 21). For the femoral neck, over the range from 10 degrees of retroversion to 80 degrees of anteversion, the correlation coefficient was 0.9979, the mean error +2.46 and the standard deviation +/- 1.48 degrees (n = 30).

The diagnosis of airway obstruction in children.

Airway obstruction in children has many causes. Although vascular rings may have early onset of symptoms there was considerable delay in establishing the correct diagnosis. Barium swallow is diagnostic. Other tests, such as angiocardiography, bronchoscopy, bronchography, and lung scan are usually unnecessary. Pulmonary sling is a less common cause of vascular obstruction. Barium swallow showing anterior indentation at the level of the pulmonary hilum is diagnostic. Cysts and tumors are other causes of severe airway obstruction. The diagnostic and operative problems in three patients are discussed as examples. It is concluded that barium swallow is the most important single investigation in the evaluation of airway obstruction.

[Early rehabilitation for neurologic patients]. / Intérêt d'une rééducation précoce pour les patients neurologiques.

Rehabilitation improves the functional prognosis of patients after a neurologic lesion, and tendency is to begin rehabilitation as soon as possible. This review focuses on the interest and the feasibility of very early rehabilitation, initiated from critical care units. It is necessary to precisely assess patients' impairments and disabilities in order to define rehabilitation objectives. Valid and simple tools must support this evaluation. Rehabilitation will be directed to preventing decubitus complications and active rehabilitation. The sooner rehabilitation is started; the better functional prognosis seems to be.

Surgical management of vascular ring.

Between 1968 and 1980, 51 children had an operation for various forms of vascular ring. Additional cardiac malformations were present in five patients, and six had noncardiac congenital anomalies. Although symptoms started within the first month of life in 39 infants, only 16 came to operation under three months of age, and a delay of more than six months occurred in 15. Stridor, often life-threatening, and recurrent infections were the most common symptoms, but dysphagia was also important. The reasons for delay in diagnosis are discussed. Barium swallow provided the diagnosis in 44 patients and suggested it in a further four patients. Innominate artery compression of the trachea was not diagnosed by barium swallow. Operations of various types were performed. Accuracy in diagnosis is important because not all patients could be treated through the classic left thoracotomy. Severe tracheomalacia was responsible for the only two deaths in the series; one of these had tracheostomy performed in the referring hospital, the second child also had Fallot's tetralogy. The large majority of the patients have done well, 76% being asymptomatic at follow-up. Minimal to moderate stridor persists among the remainder to the present time.

Pulmonary artery sling in identical twins--report of two cases.

Pulmonary artery sling was successfully treated by division and reimplantation of the left pulmonary artery in identical twins. Long-term patency of the reimplanted pulmonary artery in both twins as well as in 4 other operated children was confirmed by lung perfusion scan. Early operative treatment of pulmonary artery sling is advocated.

Renal hypoplasia and dysplasia: a radiological and pathological correlation.

Forty-nine cases of congenitally small (hypoplastic) or maldifferentiated (dysplastic) kidneys have been reviewed. From a detailed consideration of the radiological and pathological data in these patients a classification of renal hypoplasia and dysplasia has been derived which is based not only on the abnormalities in the kidney, but also on the associated anomalies seen in the ureters and in the lower urinary tract.

Unusual gastrointestinal features in a child with pernicious anemia.

Pernicious anemia in childhood is a rare disease. The radiologic demonstration of gastric mucosal atrophy led directly to this diagnosis in an 11-year-old girl. In addition, there was deformity of the pyloric antrum and superficial ulceration in the stomach and duodenum. Endoscopy and biopsy confirmed these findings.

A method for calculating acetabular anteversion in children.

A method of calculating the degree of anteversion of the acetabular fossa is described. A posterior-anterior projection of the prone pelvis with a long focus film and focus object distance is used. The distance between the anterior and posterior rims of the fossa as projected on the films is measured and then divided by a distance equivalent to the diameter: this yields the sin of the anteversion angle. The angle is rapidly calculated with a pocket calculator.

Urographic changes in acute papillary necrosis in the rat.

Intravenous high-dosage urography was performed in rats which had renal papillary necrosis induced with ethyleneimine or renal tubular necrosis produced with mercuric chloride. In both groups, nephrograms were abnormally persistent. In animals treated with ethyleneimine dense selective opacification of the necrotic renal pyramid occurred. It is suggested that this selective opacification may be a valuable radiological sign of recent renal papillary necrosis.

Cryptic mediastinal masses causing airways obstruction.

In 3 infants, severe airways obstruction was caused by mediastinal lesions which were not evident on the antero-posterior chest radiograph. Their presence was demonstrated by barium swallow examination. Each infant had thoracotomy carried out urgently. Duplication cysts (without associated cervicodorsal vertebral anomalies) were present in 2 patients and neuroblastoma in the third.

The relationship between chest radiographic scores and respiratory function tests in children with cystic fibrosis.

Chest radiographic scores and respiratory function on 80 sets of results from 50 patients with Cystic Fibrosis were analyzed. Chest radiographic scores were assessed independently using the method of Chrispin and Norman. Respiratory function tests were found to correlate well with the chest radiographic score, the best correlation being with the forced expiratory volume in 0.75 sec. to forced vital capacity ratio F.E.V. 0.75/ F.V.C. (r = -0.674 n = 80 p less than 0.001).