Management of Pregnancy with Klippel-Trenaunay-Weber Syndrome: A Case Report and Review

Abstract

Background Klippel-Trenaunay-Weber syndrome is a rare neurocutaneous syndrome with vascular involvement. Given the rarity of the syndrome, its management in pregnancy is based on the outcome of a few case reports and expert opinion. Case Summary The management of a complicated case with its antepartum, intrapartum, and postpartum concerns has been addressed in this review. Conclusions Prenatal consults with anesthesia, general surgery, intervention radiology, and internal medicine should be arranged, prior to delivery in anticipation of all the possible complications. Apart from the pregnancy management, preconceptional counselling including the genetics, prognosis, and contraception has an important role in patient management.

Manejo anestésico para la cesárea en una paciente con síndrome de Klippel-Trenaunay. Reporte de un caso

“…La presencia del síndrome de Klippel-Trenaunay durante el embarazo se clasifica como riesgo obstétrico alto debido a posibles complicaciones tromboembólicas y/o hemorrágicas, particularidad clínica que se asocia con múltiples comorbilidades relevantes y convierte este panorama en un reto para la anestesia obstétrica [3], [4], [8].…”

“…Los casos reportados son escasos y la incidencia de embarazo en pacientes portadoras del SKT no está clara, aproximadamente se reporta 1:27.500 nacimientos [8], por lo cual en la literatura científica la estrategia para la conducción anestésica no ha sido definida completamente [3], [7].…”

Management of postpartum haemorrhage in a patient with Klippel-Trènaunay syndrome

“…The manifestations of KTS are broadly characterised by complex vascular malformations affecting the capillaries, lymphatics and veins in addition to disturbed growth of bone and soft tissue 2. Although KTS has variable expression, it is commonly associated with a triad of cutaneous capillary malformation (port-wine stain), venous varicosities and asymmetric hypertrophy of soft tissue or bone, usually affecting the lower limb 3 4…”

“…The estimated prevalence of KTS is 1 in 30 000–100 000 newborns 2 3. KTS is a result of a mutation affecting a subset of primitive cells that are destined to become blood and lymphatic vessels and bones.…”

“…The physiological changes that occur during pregnancy and labour further exacerbate the venous malformation phenotype in women with KTS leading to obstetric complications that warrant careful monitoring during antepartum, intrapartum and postpartum periods. The main obstetric risks include venous thromboembolism (VTE) and postpartum haemorrhage (PPH) and the severity of these complications is dictated by the integrity of the vascular system and its ability to withstand stress during pregnancy and labour 3…”