Left Isomerism - Magnetic Resonance Diagnosis
Abstract Heterotaxy syndrome is a rare congenital condition with multifactorial inheritance in which the internal organs are abnormally arranged in the chest and abdomen. It can be classified into two main subcategories: heterotaxy syndrome with polysplenia (left isomerism) and heterotaxy syndrome with asplenia (right isomerism). Such a distinction between these subcategories offers better means of indicating the genetic alterations responsible for the syndrome. Individuals with this condition have complex congenital abnormalities, affecting various organs such as the heart - interferes in blood transport - and lung - altering the number of pulmonary lobes. We report a case of heterotaxy syndrome with polysplenia without abnormality in the thoracic organs in an adult patient.
| Main Authors: | , , |
|---|---|
| Format: | Digital revista |
| Language: | English |
| Published: |
Sociedade Portuguesa de Radiologia e Medicina Nuclear
2022
|
| Online Access: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S2183-13512022000200039 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| Summary: | Abstract Heterotaxy syndrome is a rare congenital condition with multifactorial inheritance in which the internal organs are abnormally arranged in the chest and abdomen. It can be classified into two main subcategories: heterotaxy syndrome with polysplenia (left isomerism) and heterotaxy syndrome with asplenia (right isomerism). Such a distinction between these subcategories offers better means of indicating the genetic alterations responsible for the syndrome. Individuals with this condition have complex congenital abnormalities, affecting various organs such as the heart - interferes in blood transport - and lung - altering the number of pulmonary lobes. We report a case of heterotaxy syndrome with polysplenia without abnormality in the thoracic organs in an adult patient. |
|---|