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Peripartum Management of a Patient with Symptomatic Lymphangiomatosis
Abstract Number: RF5AH-343
Abstract Type: Case Report Case Series
ntroduction: Lymphangiomatosis (LYMF) is a rare disease of abnormal lymphatic development with multiorgan involvement. We report a case of a patient with LYMF who presented with worsening disease progression during pregnancy.
Case presentation: The patient is a 29 year old G2P1 with known LYMF who presented at 31 6/7 weeks EGA with worsening dyspnea, hypofibrinogenemia, and thrombocytopenia. She was hypoxemic with SpO2 90% on room air on admission and was placed on BiPAP. Her clinical picture failed to improve after 48 hours and decision was made to proceed with cesarean delivery. On the day of surgery, platelets were 61K and fibrinogen was 134 mg/dL. 1 dose of platelets was transfused preoperatively and spinal anesthesia was performed. The patient was ramped at 30 degrees and placed on continuous BiPAP throughout the case. She received 40mg of furosemide IV with judicious fluid administration intraoperatively. She tolerated the procedure well and delivered a viable female infant. She was transported back to the ICU in stable condition and was weaned from BiPAP on POD #1. Her post-surgical course was complicated by multiple factors including wound dehiscence requiring wound exploration on POD #5, hypofibinogenemia and anemia requiring transfusion of cryoprecipitate and pRBCs, and worsening dyspnea requiring diuresis. She was discharged home on POD #6 but returned on POD #9 with respiratory distress and low grade fever. She was hospitalized for 21 days treating her acute on chronic respiratory failure. CT revealed bilateral pleural and interstitial septal thickening, worse than previous scan. Hypoxia was presumed secondary to disease progression, and she was discharged with supplemental oxygen and BiPAP at night.
Discussion: There are multiple variants of LYMF and a rarer, more aggressive variant, Kaposiform Lymphangiomatosis, (KLA) has been described. KLA demonstrates parenchymal involvement, hematologic abnormalities, and hemorrhagic symptoms. It is a generalized lymphatic anomaly with an abnormal coagulation profile resembling that of DIC. Although our patient has a diagnosis of LYMF, type unspecified, her clinical course does certainly resemble that of KLA. Regardless of specific diagnosis, LYMF can have many anesthetic implications. With no standard treatment, any management plan would be experimental, aimed at reducing symptoms associated with effusions and coagulopathic derangements.