L. Viart

Syndrome de persistance des canaux de Müller de type féminin : un cas de diagnostic tardif dans le cadre d’une infertilité

We report the case of a 35-year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type (group A). The diagnosis was made in adulthood during an infertility workup. Clinical examination revealed an empty scrotum, a normal penis and bilateral inguinal cystic masses. The spermogram found azoospermia. Imaging using MRI and tomotensidometry found the presence of an uterus, two fallopian tubes and two inguinal positions of polycystic testes. A surgical management was performed for surgical testicular biopsy. Histological examination then found a cystic formation of multi-celled mesothelial origin, with atrophic testis Sertoli cell involution and without sperm. PMDS is a rare form of pseudo-internal hermaphroditism characterized by the presence in a man of the uterus, fallopian tubes and upper vagina with external male genitalia and virilized characters. About 200 cases are reported in the literature. The diagnosis is often made in children intraoperatively during a cure of testicular ectopia. The karyotype is 46xa0XY type. The pathogenesis is related to a deficiency of anti-Müllerian hormone (AMH) or tissue resistance to its action by receptor abnormalities. The regression of the Müllerian duct derivatives can give three types of PMDSxa0: masculine type, feminine type and a transverse type. Surgical treatment is difficult but necessary because of the risk of infertility and ectopic testicular degeneration.

Étude radioanatomique du sinus occipital

INTRODUCTIONnThe aim of this study was to assess the presence of an occipital sinus in both children and adults, and to detail its main associated anatomical characteristics.nnnMETHODSnOne hundred of patients MRI (3D T1 EG) between 0 and 86 years old were studied, in sagittal and axial sections, with the software DxMM. Occipital sinus length, perimeter, and cerebellar falx length measurements were performed with the softwares tools.nnnRESULTSnForty-three percent of patients had an occipital sinus (average perimeter was 3.02 mm, average length was 19.85 mm), and 23.26% of these patients had a cerebellar falx, 30.23% of these patients had one vein or more draining into the occipital sinus. Sixty-two percent of children had an occipital sinus (average perimeter was 2.87 mm, average length was 21.63 mm), and 29.03% of them had a cerebellar falx. Twenty-four percent of adults had an occipital sinus (average perimeter was 3.4mm, average length was 15.28 mm), and 8.33% of them had a cerebellar falx.nnnCONCLUSIONnThis work highlights a link between the age and the occipital sinus existence. The perimeter of this sinus seems to be superior for adults, but its length seems to be superior for children. A cerebellar falx with the occipital sinus was found more frequently for children.