多脾综合征代表一种以多个小脾脏为特征的左心房异构疾病。通常与心脏畸形和腹部器官的位置模糊有关。所介绍的病例是一名一个月大的男婴,重约3000克,出生于锡比乌县临床急诊医院,从出生到死亡都住院。在一系列与先天性腹部器官异常相关的复杂心脏畸形的背景下,由于严重的缺氧和败血症,患者遭受了心肺呼吸骤停。身体检查发现常见的心房伴有完全房室管缺损,左心室肥厚,右心室发育不全,动脉干,上腔静脉重复,肺的双联性,腹部器官的位置模糊,右侧胃,一个中线肝脏,胆囊发育不全,多个右侧脾和肠和胰腺完全倒置。组织病理学的结论是,患者的心脏病变与婴儿乳酸性酸中毒一致,以及肺改变,提示先天性肺泡发育不良和与纤维化相容的肝结构改变。
Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The
case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis.