A Case of Pregnancy in Patient with Pulmonary Lymphangioleiomyomatosis.

Lee, Dong Yun; Park, Sang Shin; Cho, Hyun Il; Lee, Dong Ock; Park, Chan Wook; Sohn, Yoo Kyung; Hwang, Kyu Lee; Shim, Soon Sup; Jeon, Hye Won; Park, Joong Shin; Jun, Jong Kwan; Bae, Kwang Bum; Yoon, Bo Hyun; Syn, Hee Chul

Case Report

Korean Journal of Obstetrics & Gynecology 2004;47(11):2229-2235.
Published online November 1, 2004.

A Case of Pregnancy in Patient with Pulmonary Lymphangioleiomyomatosis.

Dong Yun Lee, Sang Shin Park, Hyun Il Cho, Dong Ock Lee, Chan Wook Park, Yoo Kyung Sohn, Kyu Lee Hwang, Soon Sup Shim, Hye Won Jeon, Joong Shin Park, Jong Kwan Jun, Kwang Bum Bae, Bo Hyun Yoon, Hee Chul Syn

¹Department of Obstetrics and Gynecology, College of Medicine, Seoul National University, Seoul, Korea.
²Department of Obstetrics and Gynecology, Seoul Municipal Boramae Hospital, Seoul, Korea.

Abstract

Pulmonary lymphangioleiomyomatosis is a rare disorder of unknown cause and characterised by hamartomatous proliferation of smooth muscle occurring in women of reproductive age exclusively. It causes dyspnea, recurrent pneumothorax, chylothorax, hemoptysis and respiratory failure eventually. Chest radiographs show diffuse interstitial infiltrates and cysts of uniform size, and pulmonary function tests often show airflow limitation with increase in residual volume. Hormonal factors are thought to play a role because it generally affects premenopausal women, but there is no definite treatment yet. We present an unusual case of pulmonary lymphangioleiomyomatosis during normal pregnancy with a review of literature.

Key Words: Pulmonary lymphangioleiomyomatosis, Pregnancy