Klippel-Trénaunay syndrome, pregnancy and the liver: an unusual interplay
In January 2008, a 29-year-old woman was referred from the Obstetrics Unit because of fever and the development of ascites, 3 days after a Caesarian section (gravida 3) performed because of vaginal-cervical varices. She had had no previous spontaneous vaginal deliveries and bilateral stripping of varicose veins. She was a non-smoker and she abstained from alcohol.\nAt referral, she was normotensive without encephalopathy. Pyrexia (38.8oC), mild tachycardia and moderately distended and tender abdomen were present. There were no cutaneous markers of chronic liver disease and no cardiac, respiratory or neurological abnormalities. However, she had limb asymmetry with the right side (length 95 cm, circumference 35 cm), larger than the left (length 93 cm, circumference 29 cm) (), together with right sided enlargement of the face compared to the left. She had capillary (port-wine) hemangiomas in the right cervical area and right upper limb (). She was diagnosed with the KTS.\nHemoglobin was 8.9 g/dL, white cell count 16500 K/ μL and platelet count 173000 K/μL. The liver and renal function tests were normal apart from an albumin of 2.5 mg/dL. The INR was 1.4. Viral and autoimmune markers were negative. A computed tomography (CT) scan showed hepatomegaly with arteriovenous anastomoses within the liver parenchyma, splenoportal hilar varices, splenomegaly (length 15.5 cm), a moderate amount of ascites and phlebolithiasis in intra-abdominal varicose dilated veins. Smallesophageal varices were seen endoscopically. A brain magnetic resonance imaging (MRI) and echocardiography were unremarkable.\nShe was treated empirically with piperacillin/tazobactam and metronidazole. Subsequently bacterial cultures from the lochia isolated Escherichia coli and Enterococcus faecalis. Piperacillin/tazobactam was continued and linezolid was added according to sensitivities. She responded to these antibiotics. The ascites responded to diuretics. Prophylactic low molecular weight heparin was started and continued for 6 weeks because of persistent tachycardia and raised D-dimers.\nAfter 3 months a percutaneous liver biopsy was performed which showed non-specific features. The ascites had resolved completely and blood biochemistry was normal. A thrombophilia screening was weakly positive for lupus anticoagulant and hyperhomocysteinemia (28.4 μmol/L, normal range 3.7-11). A chest CT was normal whilst CT of skull–cervical region showed muscle enlargement and projection of the right pharyngeal wall.\nReferral for further evaluation was made at 8 months after initial presentation. A transjugular liver biopsy and hepatic venous pressure measurements were performed. Liver histology and hepatic venous pressures were essentially normal. An MRI scan revealed portal vein (PV) thrombosis with cavernoma. There was no evidence of PV aplasia. She was commenced on warfarin for PV thrombosis on a procoagulant background and she has been well for four years since the initial diagnosis.