A 41-year-old multigravid woman with Klippel-Trenaunay-Weber syndrome was seen at 36 weeks’ gestation. This rare congenital vascular disorder is associated with port-wine stains, soft tissue swelling, and vascular malformations.
1.She had a previous vaginal delivery complicated by severe postpartum hemorrhage.
- Atis A.
- Ozdemir G.
- Tuncer G.
- Cetincelik U.
- Goker N.
- Ozsoy S.
Management of a Klippel-Trenaunay syndrome in pregnant women with mega-cisterna magna and splenic and vulvar varices at birth: a case report.
J Obstet Gynaecol Res. 2012; 38: 1331-1334
The presentation was breech, and a detailed ultrasound of the uterus was performed to plan a Caesarean section because of the risk of vascular malformations.
2.The lower uterine segment was thick with multiple venous and arterial vessels (Figure 1). A midline laparotomy with classical uterine incision was performed, followed by tubal ligation. Large vessels were noted on the lower uterine segment at the time of surgery (Figure 2).
- Andreasen K.R.
- Tabor A.
- Weber T.
Klippel-Trenaunay-Weber syndrome in pregnancy and at delivery.
J Obstet Gynaecol. 1999; 19: 78-79
The baby did well, and estimated blood loss was 900 mL. This management averted potentially severe complications associated with this syndrome.
- Sivaprakasam M.J.
- Dolak J.A.
Anesthetic and obstetric considerations in a parturient with Klippel-Trenaunay syndrome.
Can J Anaesth. 2006; 53: 487-491
Consent to publish these images was obtained from the patient.
- Management of a Klippel-Trenaunay syndrome in pregnant women with mega-cisterna magna and splenic and vulvar varices at birth: a case report.J Obstet Gynaecol Res. 2012; 38: 1331-1334
- Klippel-Trenaunay-Weber syndrome in pregnancy and at delivery.J Obstet Gynaecol. 1999; 19: 78-79
- Anesthetic and obstetric considerations in a parturient with Klippel-Trenaunay syndrome.Can J Anaesth. 2006; 53: 487-491
© 2014 Society of Obstetricians and Gynaecologists of Canada. Published by Elsevier Inc. All rights reserved.