Artériopathies périphériques juvéniles

Peripheral arterial disease (PAD) encompasses disease of all arteries of the body except the coronary arteries. The main etiology whatever the patient's age is atherosclerosis. Different etiologies can induce PAD especially when patients are younger than 50 years old and have no cardiovascular risk factors (smoking, hypertension, diabetes…). PAD that appears before 50 years old can be named juvenile PAD (JPAD) although there is no consensus about the definition. The aim of this work is to present the different etiologies of JPAD according to their hereditary, acquired or mixed origins. The following hereditary causes are addressed: Marfan syndrome, Ehlers-Danlos syndrome, homocystinuria, pseudoxanthoma elasticum, osteogenesis imperfecta “mid-aortic” syndrome. Among the acquired etiologies, inflammatory JPADs without extravascular signs such as atherosclerosis and Buerger's disease, inflammatory JPADs with extravascular signs as Takayasu's disease, Behçet's disease and Cogan's syndrome, JPADs like aortitis, embolic JPADs, iatrogenic JPADs, and mechanical or traumatic JPADs are described. Finally, mixed origins as thrombotic disease and fibromuscular dysplasia are presented. This work will assist clinicians in the diagnosis of JPAD.     

Endométriose péritonéale révélée par une ascite hémorragique : premier cas malgache

The peritoneal endometriosis is defined by the presence of endometrial tissue, in an ectopic way, apart from the uterine cavity. The evocative usual signs of pathology are the secondary dysmenorrhoea, the deep dyspareunia, the chronic pelvic pains and infertility. The form associated with a hemorrhagic ascitis is exceptional with 41 cases published, of which none of them is in Madagascar. Our objective is to report a case of peritoneal endometriosis revealed by a hemorrhagic ascite. It is about a 33 years old woman, from Madagascar, nulliparous and having presented an abundant hemorrhagic ascite associated with chronic pelvic pains characterised by the menstruations. In front of the difficulty of access to the coelioscopy, the complicated diagnosis of peritoneal endometriosis of a hemorrhagic ascite in our patient was retained on lines of evidence epidemiologics, anamnestics, clinicals, morphologicals and biologicals. The evolution marked by the draining of the ascite and the disappearance of the chronic pelvic pains under freinator hormonal treatment represented an additional argument in favour of the diagnosis of peritoneal endometriosis.     

Lymphangiome kystique rétropéritonéal

The cystic lymphangioma is a vascular tumor that arises at the expense of lymphatic vessels. It is a rare and benign dysplasia that occurs mainly in children. Adult forms are rare. Its expression is mainly cervical or axillary, with a few locations in the abdominal cavity. We report the case of a patient who presented with cystic lymphangiomas located in the retroperitoneal region. Various clinical aspects, diagnosis and treatment were studied.     

Corticosurrénalomes: actualités thérapeutiques

Adrenocortical cancer (ACC) is a rare aggressive malignancy with a poor prognosis (5-year survival: 45 %). Their management requires multidisciplinary expertise. Complete resection by an expert surgeon is the only curative treatment. Very few adjuvant treatments are available and their efficacy is not fully proved. Adjuvant mitotane therapy increases the disease-free survival in the majority of patients after surgery but further studies are needed to determine patients in whom this treatment is the more beneficial. Blood concentrations of mitotane between 14 and 20 mg/l are necessary to have a full efficiency but this therapeutic window may cause side effects difficult to control. When aggressive parameters are present, radiotherapy is proposed. In case of residual or unresectable disease, combination of chemotherapy and mitotane is conventionally proposed. FIRM-ACT study establishes that EDP (etoposide, doxorubicine et cisplatine) is the most effective chemotherapy for progressive ACC. The first results of treatment with tyrosine kinase inhibitors, in patients with progressive disease despite one or two lines of chemotherapy are disappointing but this may be partly explained by the interaction with mitotane which reduces the plasma concentrations of sunitinib in particular. Clinical trials are underway to assess the effectiveness of other targeted therapies, including treatments acting on the IGF-1 receptor.