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).

[Peroperative echography in pediatric neurosurgery. Experience in a series of 40 procedures]. / L'échographie per-opératoire en neurochirurgie pédiatrique. Expérience sur une série de 40 interventions.

Forty neurosurgical operations (supra-tentorial: 27; infra-tentorial: 10; intra-spinal: 3) have been performed under real time ultrasonography control. The supra-tentorial anatomical Landmarks visualized on the screen were the base of the skull, the cranial vault, the falx cerebri and the ventricles. The infra-tentorial landmarks were the petrous bone, the tentorium and the brainstem. The dura-mater, the subarachnoid space and the medulla were easily detected in the spinal canal after laminectomy. Intra operative real time ultra sonography performed on the dura-mater or on the cortex allows the detection of pathological structures. Tumors, whatever their location, are hyperechogenic; cysts, on the contrary, are hypoechogenic. Tumors are therefore precisely delimited by intra operative ultrasonography; thus the amount of normal nervous tissue which must be removed to reach a subcortical or intra medullary tumor can be reduced to a minimum. Moreover, the use of intra operative ultra sonography gives control ever the completeness of tumor removal as it shows any residual tumor intra operatively. Echography also facilitates other operations such as placement of a ventricular catheter, tapping of an abscess or biopsy of a tumor. Large arterial vessels or arterio-venous malformations are easily located because their pulsations are visible on the screen. For all these reasons, real time ultrasonography should always be available in the operating theater. In this series, it has provided essential information in 15 cases out of 40.

Acquired deep venous thrombosis in children.

In this study nine children with an acquired deep venous thrombosis (DVT) are discussed. The condition involved the limbs, pelvis and abdomen and was asymptomatic in five cases. Infection and long-term catheterisation were common predisposing factors. Phlebography was the most common and reliable diagnostic procedure.

Liver involvement in chronic granulomatous disease: the role of ultrasound in diagnosis and treatment.

Ultrasonic features of liver involvement in six children with chronic granulomatous disease (CGD) are reported. Hepatic granulomas appeared as hypoechoic, poorly marginated areas without posterior enhancement. In two cases, the diagnosis of CGD was suggested by this sonographic pattern. Ultrasound follow-up of the granulomas was used to plan therapy: in three cases, the granulomas subsided with antibiotic treatment alone; three patients underwent surgery because of the persistence of the granuloma and/or its modification into a fluid-filled abscess with good sound transmission throughout. Percutaneous biopsy and aspiration under sonographic guidance were performed in the two cases where CGD had not been diagnosed previously; such procedures permitted recognition of the disease based on histologic study, drainage of a defined abscess, and identification of the infecting organism. Healing of the hepatic lesions was documented in all six patients.

The dotted corticomedullary junction: a sonographic indicator of small-vessel disease in hypertensive children.

Six children with hypertension are reported where ultrasound showed a striking increase in the intrarenal vascular echoes. This pattern, the dotted corticomedullary junction, appears to represent a diffuse calcifying process involving the elastic fibers of the wall of small arteries.

Juvenile nephronophthisis: sonographic appearance in children with severe uremia.

The authors reviewed the sonograms in 15 cases of juvenile nephronophthisis. Medullary cysts were demonstrated in 13 children with terminal uremia. The diagnostic value of ultrasound is stressed because in many cases relevant genetic and pathological data are not available.

Pseudocysts of the pancreas in children: which cases require surgery?

The need for surgery in pancreatic pseudocysts in children is directly related to the status of the duct of Wirsung. Clinical deterioration was often the factor leading to surgery. The retrospective study of ten cases of pancreatic pseudocyst pointed to the value of ultrasound as an indicator of pancreatic duct involvement. CT was also of help in one case. ERCP, performed only in one patient before surgery, may be indicated in difficult cases.

Pulmonary vascular nodules: new sign of left lower lobe collapse in children.

Left lower lobe collapse can be suspected in children on a frontal chest film when three or more peripheral left upper lobe vessels are seen end-on along the left heart border. The peripheral vascular changes are mainly due to the backward and downward displacement of the hilus. This "vascular nodule sign" was evident in 25 (70%) of 36 cases of left lower lobe collapse. Its frequency in normals is less than 10% and may be accounted for by lordotic projection. Although not entirely specific, this sign, easily noticeable even on an underexposed film, should raise the possibility of left lower lobe collapse.