[Female hypospadias diagnosed in an adolescent]. / Hypospadias féminin découvert à l'adolescence.

Hypospadias is an exceptional congenital anomaly in females, contrasted with hypospadias in males, which is much more common. In girls, the diagnosis is made only a few months after birth or later. Female hypospadias involves total or partial agenesis of the urethrovaginal septum, resulting in urinary drainage into the genital tract. Rarely isolated, it is usually associated with other urogenital or spinal anomalies. Treatment is based on surgical urethral reconstruction. We report the case of a 13-year-old girl living in a rural area who had no specific medical history. The girl was admitted with anuria, renal failure, and hypertension. After physical examination and investigations, the diagnosis was female hypospadias, with neurogenic bladder due to sacral agenesis. Surgical treatment consisted of urethral meatus apicalization, with meatoplasty for intermittent catheterization via the urethra. Blood pressure and renal function normalized.

[Menorrhagia due to congenital factor V deficiency in an adolescent]. / Ménorragies par déficit congénital en facteur V chez une adolescente.

The frequency of menometrorrhagia in adolescence is estimated at 2-5 %. The functional origin is the most common. Hemostasis abnormalities account for 20 % of cases, and they should be sought whenever there is a personal history of known or suggestive of hemostasis disorders (epistaxis, gingival bleeding, postoperative bleeding, bruising, etc.) or there is a family history of hemostasis disorders. Organic origins are rare, but these must be sought when the characteristics of bleeding point in this direction. The workup aims to investigate the cause and assess the impact of the hemorrhage. It may include an NFS, blood group and rhesus±ß-HCG, hemostasis evaluation, determination of hemostasis factors, and a pelvic ultrasound. Whatever the cause of menometrorrhagia, the therapeutic options are the same and are oriented by the severity of bleeding. The treatment is mainly medical and hormonal. The surgical option should be a choice of last resort. We report a case of a 13-year-old girl admitted in hemorrhagic shock due to abundant metrorrhagia. Her laboratory tests showed an isolated deficiency of factor V with a 2 % rate. Congenital factor V deficiency is a rare autosomal recessive coagulation disorder. It can be diagnosed at any age when a bleeding disorder of varying severity is observed. The diagnosis is based on the hemostasis evaluation with quantitative determination of factor V. Because no FV-specific concentrate is available, fresh frozen plasma remains the mainstay of treatment. Antifibrinolytics can also be beneficial, and platelet transfusions have been successfully used, associated with hormone therapy. These patients may best be managed in cooperation with both a gynecologist and a hematologist.

[Idiopathic chylothorax in an infant: management and progression]. / Chylothorax idiopathique chez un nourrisson. Prise en charge et évolution.

Chylothorax is a rare disease (1-2 % of pleural effusions), with a prevalence between 1/8600 and 1/15,000 births. It is characterized by the presence of chyle in the pleural cavity. Three categories of chylothorax are known: congenital chylothorax, which can be either idiopathic or the result of a malformation, and traumatic chylothorax (mostly postoperative). We report the observation of a 9-month-old infant with idiopathic chylothorax revealed by respiratory symptoms, with pleural effusion and collapse of the ipsilateral lung on chest X-ray and ultrasound examination. Cytology and chemical analysis of the pleural fluid showed an exudative liquid with a chylous aspect, a high concentration of albumin (52 g/dL), triglycerides (11.42 g/L), and a high number of cells (6600 cells/mL), with lymphocyte predominance (96 %). The culture was sterile. Chylothorax is usually revealed by dyspnea, but also by nausea, vomiting, anorexia and/or malnutrition. The diagnosis is suspected when milky white fluid is obtained from thoracocentesis and is confirmed by the presence of a triglyceride level greater than 1.2 mmol/L and more than 1000 cells/mL, with lymphocyte predominance. The treatment of chylothorax can be either conservative or surgical. Conservative treatment (medical) has four goals: ensure pleural emptiness, decrease production of chyle, restore and/or maintain proper nutritional status, and treatment of the cause when identified. Surgical intervention is indicated when conservative management fails and aims to stop a radical and permanent leakage of chyle.