LAM- A Post-Op Hypoxia Dilemma




Cota, Donna
Nguyen, Thao


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Background/Abstract: Lymphangioleiomyomatosis (LAM) is a rare multisystem disease that mostly affects young women. It is estimated that approximately 3 to 5 out of every 1 million women are affected. The primary histopathological abnormality is the proliferation of atypical smooth muscle-like cells. The most common presentation of LAM is signs and symptoms of lung disease, however, patients can also present with extrapulmonary manifestations such as renal and lymphatic disease. Lymphangioleiomyomas are lymphatic fluid filled benign tumors found in the pelvic, retroperitoneum, and mediastinum that occur in 16 to 38 percent of patients with LAM. Here we present a case of a young female with no past medical history who presented for elective surgical removal of a pelvic mass. The hypoxia present during the postoperative course led to the diagnosis of LAM. Case Report: A 31 yo Caucasian female with no significant past medical history presented to our hospital for elective surgical removal of a pelvic mass. The operative course was unremarkable. Patient was extubated to nasal cannula after the surgery, but was unable to maintain oxygen saturation on room air. She was found to be hypoxic, requiring at least 2L nasal cannula to maintain saturation levels in the low to mid 90s. Initial chest X-ray was remarkable for bilateral mild interstitial opacities and bibasilar atelectasis. Despite aggressive incentive spirometry use, patient could not overcome the hypoxemia without supplemental oxygen. CT chest was obtained and showed cystic changes diffusely throughout both lungs. The diagnosis of LAM was considered high on the differential at this point. The patient was discharged on supplemental oxygen and advised to follow up in the pulmonology clinic. Two days after discharge, the pathology from the pelvic mass resulted and was consistent with lymphangioleiomyoma. Discussion/Conclusion: This case was unique in the fact that it presented as an extrapulmonary manifestation of LAM that eventually led to the diagnosis of the pulmonary disease. Due to the postoperative nature, atelectasis was initially implicated for the hypoxia in this young female with essentially no prior past medical history, clouding the possibility of other differentials such as LAM. However, in a female with pelvic mass of unknown etiology and pulmonary symptoms, LAM should stay at the top of the differential, despite the rarity of the condition.