A case of intramedullary haematoma associated with congenital alpha2-plasmin inhibitor deficiency.

The association of intramedullary haematoma of the left femoral diaphysis and congenital alpha2-plasmin inhibitor deficiency in a 6-year-old boy is reported. Congenital deficiency of this fibrinolytic system component is very rare and can result in severe bleeding. Clinically, an intramedullary haematoma causes pain but no swelling, and there is no history of trauma. X-rays show a hyperlucent lesion without sclerotic edges in the medulla of the diaphysis of a long bone with thinning of the cortex. MRI is diagnostic with the haematoma generating a specific hyperintense signal on T1-weighted and T2-weighted spin-echo images.

[Postnatal echography and cystography after prenatal diagnosis of minor dilatation of the kidney pelvis. Prospective study of 89 cases]. / Echographie et cystographie postnatales après diagnostic prénatal d'une dilatation mineure du bassinet. Etude prospective sur 89 cas.

We evaluated postnatal management after the prenatal diagnosis of moderate dilatation of the renal pelvis (antero-posterior diameter superior to 1 mm per month of pregnancy and inferior to 15 mm at full term). Eighty-nine neonates who had a moderate dilatation in utero were evaluated by ultrasound and voiding cysto-urethrography. Ultrasound was performed between day 2 and day 7 in 83 cases, after day 7 in 6 cases. Voiding cysto-urethrography was performed during first month in 80 cases, after this period in 9 cases. Sixty-five neonates were shown to have a malformation of the urinary tract: reflux (n = 27), megaureter (n = 20), uretero-pelvic junction obstruction (n = 19), renal duplication (n = 7), posterior urethral valves (n = 2), horseshoe kidney (n = 1), ureteric cyst (n = 1). Reflux which is frequent in neonates with urinary tract infection, is the most frequent cause of moderate dilatation of the fetal renal pelvis. Combination of ultrasound and voiding cysto-urethrography in neonates allows a quick, full diagnosis. It helps rationalize the use of prophylactic antibiotic treatment.