Iranian Journal of Medical Sciences
Iranian Journal of Medical Sciences

Pulmonary Aplasia with Unusual Associations in a Woman

Document Type : Case Report(s)

Authors

Arshad Altaf Bachh
Sridhar Pulluri
Aadil Beigh
Chippa Raju
Ranganath Deshpande

Department of Pulmonary Medicine, Mamata Medical College and Hospital, Khammam, Andra Pradesh, India

Abstract
Failure of development of the primitive lung bud leads to an extremely rare congenital anomaly with a prevalence of 34 per 10 lac live births termed pulmonary aplasia. In half of such cases, associated congenital malformations of the cardiovascular, skeletal, gastrointestinal, or genitourinary systems are present. The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is defined as the congenital aplasia of the uterus and the upper two thirds of the vagina with normal secondary sexual characteristics, ovaries, and a normal karyotype (46, XX). We report an extremely rare association of right lung aplasia, MRKH syndrome, and right renal agenesis with left pelvic kidney, which to the best of our knowledge is the first such association reported.

Keywords

Lung agenesis
Renal agenesis
Müllerian anomalies
Iranian Journal of Medical Sciences
Volume 39, Issue 2 - Serial Number 2
13
March 2014
Pages 148-151

  • Received 01 March 2014
  • Revised 23 August 2020
  • Accepted 24 December 2018

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