1. PRETERM DELIVER WITH KLIPPEL TRENAUNAY SYNDROME
Authors: Dr G McLeod, Dr P Gondane
31/03/15
PRETERM DELIVER WITH KLIPPEL TRENAUNAY SYNDROME
Affiliation: Tameside General Hospital, UK
Introduction
KTS
Klippel Trenaunay syndrome (KTS) is a sporadic and rare triad of malformations: capillary malformations (port wine stain); soft tissue and bone hypertrophy; and atypical lateral varicosity. Morbidity arises from these vascular anomalies.
The incidence of KTS in pregnancy is unknown and presumed extremely rare. Pregnancy increases KTS complications through both thromboembolic and haemorrhagic events.
It is thought that maternal and fetal risk is proportional to the severity of KTS(1).
(2). MR image of KTS
(3). Port wine stain
Case Report
27 year old women Gravida 3 Para 2. Two normal vaginal deliveries at term. Had significant blood lost requiring blood transfusion from an episiotomy with her first delivery. The patient had KTS with extensive truncal, pelvic, and vulval varicosities. Vascular surgeons recommended classical caesarean section if needed without interventional radiology. Therapeutic low molecular weight heparin commenced at booking.
A planned 28/40 growth USS demonstrated oligohydraminous. 28+3/40 admitted with vaginal bleeding- steroids were administered. Had recurrent ante-partum haemorrhage until induction offered at 34/40.
The patient proceeded to a preterm normal delivery without episiotomy. Minimal blood loss.
Normal vaginal delivery or caesarean section? The literature has case reports for both. There are complications associated with either mode of delivery.
(4). Vulval varicosities
Discussion
Increased venous pressure, cardiac output, leg oedema, venous stasis, and hyper-coagulable state are normal physiological changes which occur during pregnancy. KTS significantly increases adverse risks in pregnancy.
Careful consideration is needed when deciding mode of delivery. A multidisciplinary approach is required which involves the patient’s point of view. This collaboration should minimise associated morbidity.
References
Atis, Alev·Ozdemir,Gunseli·Tuncer, Gulden·Cetincelik, Umran·Goker, Nimet·Ozsoy, Sibel (2012) Management of a Klippel Trenaunay syndrome in pregnant women with mega-cisterna magna and splenic and vulvar varices at birth: a case report. The journal of obstetrics and gynaecology research Nov;38(11):1331-4
(2) Case courtesy of Dr Javier Salguero, Radiopaedia.org MR scan KTS
(3) Case courtesy of Dr Roberto Schubert, Radiopaedia.org Port wine stain KTS
(4) Reiko Tanaka, Yasuyuki Fujita, Kana Ishibashi Hiase, Yasuo Yumoto, Nobuhiro Hidaka, Kotaro Fukushima, Norio Wake (2011) Successful Management of Pregnancy Complicated by Klippel-Trenaunay Syndrome Using MR Angiography-Based Evaluation Case reports in Obstetrics and Gynaecology Vol 2011 Article ID doi: /2011/72346