Gastrointestinal: Massive intraperitoneal haemorrhage in a young woman with Hereditary Hemorrhagic Telangiectasia

Abstract

A 35 year-old Chinese female patient with Hereditary Hemorrhagic Telengiectasia (HHT) and a prior history of 2 uneventful pregnancies, had undergone an emergent caesarian section at 36 weeks gestation, due to the development of high-output cardiac failure. Post-partum she developed drowsiness and required intensive care support. Radiological imaging of the brain (CT and MRI) excluded meningoencephalitis, intracranial haemorrhage and arteriovenous malformations (AVMs). The patient subsequently developed active pulmonary haemorrhage and gastrointestinal (GI) bleeding. CT imaging of the thorax and abdomen demonstrated multiple AVMs in the lung bases and the liver (Figure 1). Bronchoscopy showed bleeding AVMs in the left lingular branch and an emergent embolisation of the feeding vessel was performed successfully. A duodenal ulcer with a clean base was diagnosed as well (via endoscopy) but further GI bleeding prompted a CT mesenteric angiogram which demonstrated air-fluid levels in the posterior aspect of the large bowel loops indicative of ruptured AVMs (White arrow, Figure 2). Embolisation was not performed due to concerns about bowel infarction. Despite maximal supportive therapy, she deteriorated into disseminated intravascular coagulation. Prior to her demise, she developed a sudden, tense abdominal distension with subsequent hypovolaemic shock and cardio-respiratory arrest. A post-mortem revealed massive haemorrhage in the intra-peritoneal cavity with multiple AVMs in the gastrointestinal tract. Massive intraperitoneal haemorrhage associated with HHT is seen in 25–30% of patients. During pregnancy, the plasma volume expands 30–50% and thus the cardiac output increases and peripheral vascular resistance reduces. This leads to ‘softening’ of the connective tissues in the vascular walls and hence enlargement of AVMs during pregnancy with potential risk of rupture. Although arterial embolisation of pulmonary and hepatic AVMs have been successfully described before, the widespread distribution of AVMs and rapid systemic deterioration in our patient precluded any chance of successful haemostasis. Although rare, women with HHT should be screened for AVMs and monitored closely during pregnancy. Contributed by