Authors: Zara Arain, Mansi Tiwari, Ali Nakhosteen, Ade Adeyemo, Abha Govind, Reeta Subba, Suchana Dhital, Beena Subba.
Objective: This case study aims to review the management of pregnancy in a patient with Klippel Trenaunay syndrome (KTS), a rare condition characterised by venous malformations, affecting an estimated 1 in 30,000- 100,000 live births (1). We focused on tailoring management to adverse outcomes specifically associated with this condition; including venous thromboembolism and postpartum haemorrhage.
Design: We followed the case of a 35 year old primip who had been identified as needing specialist input for KTS at 35 weeks pregnant. Obstetric management was based largely upon current literature as there are no specific guidelines for the management of this condition in pregnancy.
Methods: Recent literature was reviewed at the point of referral to antenatal clinic. Primary risks were identified including venous thromboembolism, post-partum haemorrhage, complicated administration of loco- regional anaesthesia and difficult delivery by caesarean section. In view of these risks, the patient was initiated on low molecular weight heparin, an MRI spine and pelvis was organised and a plan was made for induction at 38 weeks. Blood was crossmatched and readily available at delivery. The patient was reviewed by an anaesthetist and discussed in a multidisciplinary meeting prior to induction.
Results: The patient was induced at 38 weeks and had a forceps delivery, with 800ml of blood loss. She unfortunately sustained a 3c tear which was repaired.
Conclusion: Klippel-Trenaunay Syndrome is associated with significant morbidity at all stages of pregnancy and therefore requires careful multidisciplinary obstetric management. Specific anticipation of venous thromboembolism and post partum haemorrhage is needed. A guideline for management of KTS in pregnancy would be of great benefit.
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