Anaesthetic management for caesarean section in a patient with hereditary haemorrhagic telangiectasia and severe epistaxis during pregnancy

Abstract

A primigravida patient, with a history of hereditary haemorrhagic telangiectasia (HHT) manifesting as nasal angiodysplasia and hepatic arteriovenous malformations (AVM), presented for delivery planning and anaesthetic evaluation at 29 weeks of gestation. She was hospitalised several times during the second and third trimester for serious recurrent epistaxis, leading to severe anaemia. In total, she required the transfusion of 20 units of packed red blood cells during her pregnancy as well as surgical nasal haemostasis under general anaesthesia (GA). The patient was referred to our tertiary centre for delivery. In the context of recurrent severe epistaxis and high cardiac output (due to hepatic AVM) in the third trimester, a multidisciplinary decision was made to plan an elective caesarean section at 35 4/7 weeks combined with nasal packing under GA. This report discusses the implications of HHT, the multidisciplinary planning of the caesarean section, intraoperative anaesthetic management and patient follow-up.

Keywords: Anaesthesia; Ear, nose and throat/otolaryngology; Haematology (incl blood transfusion); Pregnancy.

Figure 1

Postpartum abdominal CT scan (frontal plane) showing a dysmorphic liver with an important hepatomegaly. Liver tissue is heterogeneous due to the progression of numerous hepatic AVM and multiple focal nodular hyperplasia lesions of the liver (some large nodules are indicated by an *). AVM, arteriovenous malformation.

Figure 2

Postpartum abdominal CT scan (transverse plane) with numerous hepatic AVM and multiple focal nodular hyperplasia lesions of the liver (some large nodules are indicated by an *). AVM, arteriovenous malformation.