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VOLUME 10 , ISSUE 3 ( September-December, 2021 ) > List of Articles


Unilateral Pulmonary Agenesis with Mayer-Rokitansky-Kuster-Hauser Syndrome: A Rare Association

Amiya Pandey, Manoj Pandey, Hemant Kumar, Natasha

Keywords : Contrast-enhanced computed tomography thorax and abdomen, Mayer-Rokitansky-Kuster-Hauser syndrome, unilateral pulmonary agenesis

Citation Information : Pandey A, Pandey M, Kumar H, Natasha. Unilateral Pulmonary Agenesis with Mayer-Rokitansky-Kuster-Hauser Syndrome: A Rare Association. Indian J Respir Care 2021; 10 (3):355-357.

DOI: 10.4103/ijrc.ijrc_59_21

License: CC BY-NC-SA 4.0

Published Online: 06-12-2022

Copyright Statement:  Copyright © 2021; Indian Journal of Respiratory Care.


Unilateral pulmonary agenesis is a rare congenital condition characterized by the absence of one or both lungs. Most of the cases have unilateral presentation as bilateral agenesis does not support life. Simultaneous Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome also has very rare presentation. It is characterized by congenital aplasia of the uterus and the vagina with normal female karyotype (46XX). A 30-year-old female, presenting with dyspnea from last 6 months and amenorrhea for last 15 years was further evaluated with gynecological examination, contrast-enhanced computed tomography thorax with the abdomen and karyotyping. She was eventually diagnosed as a case of Type I pulmonary agenesis with Type I MRKH syndrome. K

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