[Prenatal diagnosis and management of two cases of bilateral ureteroceles on simplex ureters]. / Diagnostic prénatal et prise en charge de deux cas d'urétérocèles bilatéraux sur uretères simplex.

Ureterocele is a rare urologic disorder characterized by pseudocystic dilatation of the terminal submucosal ureter. Most cases of ureteroceles are associated with complete ureteral duplicity and ureterohydronephrosis, whereas ureteroceles on simplex ureters are rarer. The authors report two cases of bilateral ureteroceles on simplex ureters diagnosed prenatally at 30 and 32weeks gestation. Fetal ultrasound had revealed bilateral ureterohydronephrosis. The delivery was made at term and renal function was normal at birth. Radiological and isotopic studies of the urinary tract confirmed the diagnosis of bilateral ureteroceles on simplex ureters that were obstructive in one case and not obstructive in the other case. Both cases had urinary antiseptic treatment and neither had urinary infection. Endoscopic puncture with electrocoagulation of ureteroceles was performed at 8 and 14months of age, respectively, with a simple postoperative course. Prenatal diagnosis of ureteroceles is essential to plan early multidisciplinary care to avoid long-term renal consequences.

[Spontaneous rupture of the imperforate hymen in two newborns]. / Rupture spontanée de l'imperforation de l'hymen chez deux nouveau-nés.

Imperforate hymen is the most frequent congenital malformation of the female genital tract and usually remains asymptomatic until puberty. The diagnosis of this malformation is rarely made at birth and surgical treatment is often by hymenotomy. However, spontaneous rupture of the imperforate hymen is possible but is very rare. We report two cases of imperforate hymen revealed by mucocolpos at birth. Ultrasound examination does not objectify hydrocolpos or hydrometrocolpos. Spontaneous rupture of the imperforate hymen was observed respectively in the 4th and 7th day of life with a satisfactory result at 1 month of age. Screening for the imperforate hymen must be systematic at birth to offer early hymenotomy if the spontaneous rupture is not adequate and prevent serious complications of the disease at puberty.

[Neonatal renal vein thrombosis in a heterozygous carrier of both factor V Leiden and the MTHFR gene mutation]. / Thrombose néonatale de la veine rénale et double hétérozygotie pour la mutation du facteur V de Leiden et du gène de la MTHFR.

Renal vein thrombosis (RVT) is a rare but potentially serious neonatal disease. Its epidemiology and its clinical and biological expression are currently well known, but its etiological exploration, like that of venous thromboembolism, is increasingly complex. Perinatal risk factors such as prematurity, dehydration, and birth asphyxia have lost their direct accountability at the expense of their interaction with constitutional disorders of hemostasis. We report a case of RVT in a newborn who was a heterozygous carrier of both factor V Leiden and the methylene tetrahydrofolate reductase (MTHFR) gene mutation. We recall the clinical and epidemiological characteristics. A search for inborn blood coagulation disorders should be systematic in the newborn infant with venous thrombosis because of the risk of recurrence, taking into account perinatal factors and maternal thrombophilia (especially if RVT is established during the prenatal period).

[Spontaneous regression of a giant facial hemangioma]. / Régression spontanée d'un hémangiome géant de la face.

Infantile cutaneous hemangioma is a benign vascular tumor and is very frequent in infants. The evolution is mostly favorable with spontaneous regression in 80% of cases. Clinical diagnosis is easy in its triphasic typical form with a phase of sometimes brutal postnatal growth, a phase of stabilization and a phase of slow secondary regression. We report a case of a giant facial hemangioma diagnosed at birth in full-term female newborn getting the right peri-orbital and fronto-temporal region. Initial evolution was marked by a fast increase of the tumor justifying its stake under prednisone at the age of 3 months but without improvement. At the age of 6 months, the patient was hospitalized for infection of the hemangioma with favorable evolution under oxacilline. Following evolution was marked by the total regression of the hemangioma at the age of 5 years at the price of peri-orbital cutaneous scars. We discuss here the diagnostic, therapeutic and evolutionary aspects of this pathology and review of the literature.

[Management and follow-up of antenatally diagnosed primary megaureters]. / Prise en charge et évolution des méga-uretères primitifs de découverte anténatale.

INTRODUCTION AND OBJECTIVES: To study the diagnostic and outcome aspects of antenatally diagnosed primary megaureters and analyse the results of the postnatal medicochirurgical management. PATIENTS AND METHODS: The authors report a retrospective analysis of 12 patients been born between 1998 and 2009 with primary megaureter antenatally diagnosed between 20 and 32weeks of gestation. A postnatal radiological assessment comprising urinary tract ultrasound, DTPA or MAG3 renal scintigraphy, retrograde cystography, DMSA renal scintigraphy and intravenous urography was performed. All the patients were put under urinary antiseptic treatment and followed since the birth. RESULTS: This study comprised nine boys and three girls, all been born at term with normal trophicity. Mean age of antenatal diagnosis of hydronephrosis was 26weeks of gestation. Mean age of definitive postnatal diagnosis was ten months. Left megaureter was seen in five children, right in two cases and five patients had bilateral megaureter comprising a total of 17 renal units of which eight units were non-obstructed/non-refluxing, three units were refluxing, five units were obstructed and one unit was obstructed and refluxing. Megaureter was associated to controlateral ureteropelvic junction obstruction syndrome in one patient and to ipsilateral ureteral duplicity with a superior polar kidney in another case. Renal function was less than 20% in four renal units. The mean follow-up was four years (range : 10months to 11years). Outcome was marked by urinary infections in seven cases of which four patients had non-obstructed/non-refluxing megaureter. Six patients required surgical correction, two for non-obstructed/non-refluxing megaureter complicated with urinary recurring infections, three for obstructed megaureter with severe renal functional deterioration and one patient for bilateral refluxing megaureter grade V. Two other patients required surgical treatment, one for controlateral ureteropelvic junction obstruction syndrome and the other for ipsilateral ureteral duplicity with destroyed superior polar kidney. Mean age at surgery was 22,6months (range : 6 months to 7 years). CONCLUSION: Half of our patients with antenatal diagnosis of primary megaureter required surgical correction. Urinary recurring infections, renal functional deterioration less than 20% and importance of ureteral dilatation with vesico-ureteric reflux grade V were predictive factors for surgery.