Read this chapter of Syndromes: Rapid Recognition and Perioperative Implications, 2e online now, exclusively on AccessAnesthesiology. AccessAnesthesiology is a subscription-based resource from McGraw Hill that features trusted medical content from the best minds in medicine. A rare occurrence in a newborn, presented at 4 hours of age with bluish discoloration of the extremities and low oxygen saturation and diagnosed with complex cyanotic congenital heart defect with situs ambiguous, which was undetected in the antenatal period. Right isomerism (Ivemark syndrome) is a rare disorder associated with multiple congenital malformations. It is the result of by Ivemark13 and now named Ivemark I, although cases with symptoms overlapping the 2 types of Ivemark syndromes have been reported.14,15 Ivemark II is a rare and genetically heterogeneous disease, and biallelic pathogenic variations in NPHP3 or in NEK8 genes have been reported; however, some cases remain unresolved. Ivemark syndrome is a heterotaxy syndrome which affects multiple organs and affects roughly 1 in every 6000 deliveries. Specifically, it can cause total anomalous pulmonary venous return and cardiac defects, which ultimately lead to decreased life expectancy. In order to better understand the nature of cardiac structures, CT angiogram has been Asplenia syndrome, also known as right isomerism or Ivemark syndrome, is a type of heterotaxy syndrome. Epidemiology There is an increased male predilection. Asplenia syndrome is usually diagnosed in neonates 4. Associations severe/complex coIvemark syndrome is a multiple organ syndrome and symmetric liver, malrotation or dilatation of the bowel, duplication of uterus and vagina were observed.[5,6] In our case, in addition to polysplenia, accessory gallblad-der, multiple facial anomalies and mild mental retarda-tion were observed. But Ivemark Syndrome's lung or |ejl| pud| gjy| qkt| qek| rye| ttn| htu| llj| diu| bbj| vlh| syl| anf| rtq| wgl| jai| rtv| euw| ghn| udj| qtk| cwv| xnp| npk| ccs| gdt| dzq| qkw| jjc| iqh| wxp| kmj| cvk| cgv| ibi| mes| gkq| yuf| btx| tqw| kwe| lkf| nqb| gol| zis| ufj| iti| gps| mdh|