A Case of Klippel-Trenaunay Syndrome Complicated by Group A Streptococcemia and Multiple Organ Failure

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder defined as a triad of capillary malformation, venous malformation, and hypertrophy of soft tissue and bones, with or without lymphatic malformation. We report a case of a KTS patient with a hospital course complicated by Group A Streptococcus bacteremia and multiple organ failure. The 39-year-old female with KTS presented to the emergency department with a fever, tachycardia, hypotension, and profuse diarrhea for one week. Blood cultures grew Group A Streptococcus necessitating a multi-antibiotic regimen and intravenous immunoglobulins (IVIG). Secondary to septic shock, the patient's renal function continuously declined requiring eight rounds of hemodialysis. She was electively intubated due to worsening acute hypoxic respiratory failure. Chest X-rays demonstrated consolidation, pneumonitis, pleural embolism, and effusions. The patient also required eight units of packed RBC throughout her hospitalization. An underlying autoimmune etiology was suspected due to multiorgan involvement and abnormal blood smears, which was confirmed by an autoimmune panel. The patient ultimately was stabilized and was optimized for discharge. This case demonstrates the importance of a multidisciplinary approach in managing patients with KTS due to their associated lymphatic abnormalities that predispose them to severe infections.

Keywords: gas sepsis; group a strep sepsis; klippel; klippel-trenaunay syndrome; kts.

Figure 1. Chest CT demonstrating extensive bilateral airspace disease and pleural effusions