Anaesthetic management of a parturient with Klippel-Trenaunay syndrome

ABSTRACT

Introduction: Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disease which is characterised by capillary, venous and lymphatic malformations. We describe the anaesthetic management of a 36 year old parturient with COVID-19 and KTS, who underwent an elective caesarean section. Very few such cases have been described and the multi-system nature of condition poses various challenges to both the obstetrician and the anaesthetist. Case Report We had a 36-year-old woman who had experienced three previous normal vaginal deliveries and an elective caesarean section (CS) four years previously under general anaesthetic(GA) at 36 weeks gestation. She was told by a vascular surgeon that she was not suitable for regional anaesthesia. There was no recent imaging of her back to rule out arteriovenous(AV) malformations. Her past history included gastric bypass surgery under GA two years ago. She also had depression, varicose veins and three previous deep venous thrombosis andwas on prophylactic lowmolecularweight heparin. She had tested positive for COVID-19 4 days previously, and had mild symptoms of cough and sore throat. After a multi-disciplinary discussion involving an obstetrician, vascular surgeon, haematologist and anaesthetist, a decision was made to proceed with GA despite recent COVID-19, because of the possibility of AV malformations, in agreement with the patient. After securing two wide bore cannulae and adequate preoxygenation, a modified Rapid Sequence Induction was performed, and a tracheal tube was secured. Anaesthesia was maintained with oxygen, nitrous oxide and sevoflurane. After delivery of the baby, oxytocin 5U, followed by an infusion, midazolam, morphine, ondansetron and dexamethasone were administered. Extubation was performed when the patient was fully awake. In recovery, further opioids were given for pain. There were no concerns for the newborn. Estimated blood loss was 200 mL. Discussion: Gestation and its physiology may further exacerbate the manifestations of KTS, with increased obstetric risk. The success in the management of these patients requires the participation of a multidisciplinary team, consisting of obstetrician, anaesthetist, urologist, haematologist and vascular surgeon, with appropriate collaboration among the professionals involved. Periodic imaging and clotting tests are recommended to evaluate the evolution of vascular malformations in the pelvis, uterus and vagina, and identify neuraxial changes, to guide the safest way of delivery and anaesthesia.