Etude des anastomoses entre la veine rénale gauche et les plexus intra-rachidiens

Résumé Des anastomoses entre la veine rénale gauche et les plexus rachidiens existent dans environ 80% des cas (16 cas sur 20); elles sont importantes dans 25% des cas (5 cas). Le siège de leur origine est fixe: le plexus du trou de conjugaison de l'espace L1–L2 reçoit généralement ces voies anastomotiques.Ces anastomoses ont une grande importance, puisqu'elles constituent la seule voie collatérale de sûreté du rein dans 7 cas sur 20. Leur hypertrophie, dans certains cas pathologiques, peut être responsable de troubles neurologiques par compression, réversibles après ligature des anastomoses hypertrophiées.     

Clinical follow-up of the first SF-1 insufficient female patient

ObjectiveSteroidogenic factor 1 (SF-1/NR5A1) plays a crucial role in regulating adrenal development, gonad determination and differentiation, and in the hypothalamic-pituitary control of reproduction and metabolism. In men (46, XY), it is known that mutations in SF-1/NR5A1 gene cause a wide phenotypic spectrum with variable degrees of undervirilization. In recent years, the role of SF-1 in the ovarian function was increasingly discussed and alterations in the gene were related to primary ovarian insufficiency. We describe the follow-up of a 46, XX affected woman with a SF-1 mutation and by comparing our case with the known manifestations reported in the literature, we try to further elucidate the function of SF-1 in the ovary.ResultsDuring infancy, adrenal insufficiency was the only clinical sign of the loss-of-function as ovarian development and function seemed normal. To date, this young woman aged 16.5 years shows normal growth, normal BMI and psychomotor development, has a normal puberty and regular menstruation.ConclusionThis report shows one, to date uniquely described, phenotypic variant of SF-1 mutation in a 46, XX affected person with adrenocortical insufficiency but no ovarian dysfunction nor disturbance of pubertal development. To follow the natural history of SF-1 mutation in a 46, XX individual will further shed light on its role in the ovarian function and thus will help to counsel affected patients in future.     

Management of accessory renal arteries during aneurysm repair in a patient with end-stage renal failure

The presence of accessory renal artery is a frequent anatomic variation that can challenge abdominal aortic aneurysm (AAA) repair. Here, we show an image of an abdominal aortic aneurysm extended to multiple accessory renal arteries in a patient known for an end-stage renal failure. This case raises the questions of the criteria that should be taken in consideration for an optimal management of accessory renal artery during AAA repair.     

L’œdème aigu du poumon à pression négative : illustration à partir d’un cas clinique inhabituel compliquant une paralysie récurrentielle postopératoire

Acute postoperative negative pressure pulmonary edema (NPPE) is a respiratory complication due to upper airway obstruction occurring most commonly in the postoperative period. Pathophysiologically, NPPE is explained by the abrupt generation of highly negative intrathoracic pressure that lead to fluid transudation from the pulmonary capillaries. This observation reports an unusual case of a young patient who has undergone total thyroidectomy for multinodular goiter. The postoperative period was marked by the occurrence of bilateral recurrent laryngeal nerve palsy that was complicated by a NPPE. The outcome was favorable after mechanical ventilation with reversal of NPPE and recovery of the recurrent laryngeal nerve function. This article stresses the importance of prevention of recurrent nerve palsy during thyroid surgery. It also highlights a little known respiratory complication: the NPPE. Understanding the pathophysiology of NPPE, rapid diagnosis and appropriate therapeutic measures could prevent its potential lethal consequences.     

Fibrose systémique néphrogénique

Nephrogenic systemic fibrosis is a recently described entity that occurs in patients with advanced renal failure. Its cause is probably toxic. The patients develop skin thickening, which is usually symmetrical on the limbs and sometimes extend to the trunk. Joint contractures and muscle sclerosis confine the patients to wheelchair. Systemic involvement may occur and includes cardiomyopathy, pulmonary fibrosis and diaphragmatic paralysis. The diagnosis is confirmed by the association of skin fibrosis and a cellular infiltration composed of CD34+ fibrocytes. Prognosis is severe with many deaths, rarely directly related to the disease. An improvement of lesions is possible, especially in case of resolution of the renal insufficiency. Several treatments have been evaluated, but none has shown consistent benefit. The toxic culprit is likely to be the gadolinium ions (Gd⁺⁺⁺), released from some contrast agents used in nuclear magnetic resonance imaging. Evidence of the responsibility of Gd⁺⁺⁺ is based on epidemiologic, biochemical and experimental data. Recommendations have been published for patients with renal insufficiency requiring a nuclear magnetic resonance imaging. If they are followed and efficient, it is likely that nephrogenic systemic sclerosis will disappear.     

Multivessel spontaneous coronary artery dissection with simultaneously three different angiographic patterns

Spontaneous coronary artery dissection (SCAD) is a non-traumatic non-iatrogenic coronary dissection. It's a frequent cause of acute coronary syndrome (ACS) in women without or with few traditional cardiovascular risk factors. We report an unusual case of multivessel SCAD in a middle-aged woman with successful medical management.