Ivemark Syndrome.
نویسندگان
چکیده
Ivemark syndrome or right atrial isomerism is a rare syndrome of asplenia / hyposplenia with malformation of heart and abnormal arrangement of internal organs of chest and abdomen and is classified under heterotaxy disorder. We describe here the case of a 14 year old boy diagnosed with asplenia, dextrocardia with double outlet right ventricle and midline liver.
منابع مشابه
Ivemark syndrome with cor triatriatum, primum ASD, cleft mitrale and pulmonary stenosis
Ivemark syndrome is a rare anomaly with a reported incidence of 1 in 10000 – 20000 live births. It is characterized by agenesis of spleen, a dextropozed, hypoplastic or lobulated spleen or multiple spleens in association with complex cardiac anomalies. These malformations are usually associated with trilobulated or bilobulated lungs and abnormalities of other abdominal organs. Here we report a ...
متن کاملOntogeny of renal dysplasia in Ivemark syndrome: light and immunohistochemical characterization.
Ivemark syndrome is a rare sporadic or autosomal recessive disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis. There have been no data describing the renal changes during embryologic development in this syndrome. In this report, we document the pathological findings of the kidney in three subjects with Ivemark syndrome: 6 months, 21 weeks and 16 weeks, respect...
متن کاملIvemark Syndrome- A Rare Syndrome with Constellation of Anatomical Defects
Ivemark syndrome is classified as a heterotaxy disorder or a laterality disorder. These terms refer to the failure of the internal organs of the chest and abdomen to be arranged in the proper location within the body. It is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleen, malformations of the heart and the abnormal arrangement of the internal organs of the...
متن کاملIvemark syndrome in association with congenital septum transversum defect and pancreatic divisum.
A four-month-old female baby presented with cyanosis and respiratory distress. A provisional diagnosis of congenital posterolateral diaphragmatic hernia was made but on exploration there was a defect in the septum transversum along with features of Ivemark syndrome - asplenia with visceroatrial heterotaxia, malrotation and pancreatic divisum - an association not yet reported in literature. The ...
متن کاملIvemark syndrome-a rare entity with specific anatomical features.
Ivemark syndrome (IS) is a rare embryological disorder which results from failure of development of the left-right asymmetry of organs. It is often associated with cardiac and other organ abnormalities, which are the usual causes of death in early neonatal life. We report a 3 months old girl with IS with dextrocardia, transposition of the great vessels, atrio-ventricular connection, total anoma...
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ورودعنوان ژورنال:
- The Journal of the Association of Physicians of India
دوره 64 5 شماره
صفحات -
تاریخ انتشار 2016