The Klippel–Trenaunay syndrome in pregnancy

Abstract

Case reportA 21 year old primipara was referred to our unit for advice regarding mode of delivery. The woman was 38 weeks pregnant and had an oblique lie. Her pregnancy had been uncomplicated. However, she had a large congenital haemangioma of the left buttock and thigh ( Fig. 1) extending to the lower leg and foot. There was a macular telangiectatic haemangioma that was associated with lateral venous varicosities that had developed over the past few years and had become worse in pregnancy. Both lower limbs were the same length, but the left leg showed hypertrophy of the soft tissues. The index, middle and little fingers of both her hands were unduly short. She had no significant past medical pregnancy.During her pregnancy, the woman developed varicose veins of the vulva, vagina and cervix. An ultrasound scan revealed distended lower uterine and cervical veins. The fetus was normally grown, the placenta was anterior and the liquor volume slightly reduced. We sought the opinion of a dermatologist, who diagnosed the Klippel-Trenaunay syndrome.The woman remained on the antenatal ward until 39 weeks of gestation. In view of the vulval, vaginal and cervical varicosities and a persisting oblique lie, we decided to deliver her by caesarean section. A midline vertical incision in the skin was performed and a classical caesarean section was carried out. A massive plexus of varicosities filled the uterovesical fold, extending over the lower uterine segment. The lower pelvic peritoneum was also involved. A baby girl was born who cried at birth. Her weight was 3.3 kg. Following delivery, an infusion of oxytocin was administered, which caused the uterus to contract well. The varicosities that had been so prominent before delivery shrunk down considerably. The estimated blood loss was 1500 mL and the woman received a transfusion of two units of blood. She made a good postoperative recovery. In view of the increased risk of thromboembolic disease, she was treated with enoxaparin 40 mg daily and elastic stockings for seven days. DiscussionThe Klippel -Trenaunay syndrome is a rare congenital disorder first described in 1900 1 . It consists of a triad of cutaneous port-wine capillary malformations, varicose veins and hemihypertrophy of soft tissues and bone. Fig. 1. Patient's left buttock and thigh showing haemangioma and associated varicosities.

Complications of pregnancy and labour in women with Klippel–Trénaunay syndrome: a nationwide cross‐sectional study

“…The high prevalence of VTE and PPH is in accordance with previously published case reports and case series (Table S1). 7,16,17 Severe complications such as paraplegia and uterine prolapse were reported in case studies, 8,9 but were not found in our study. In addition, our data are in contradiction with current literature on pregnancy outcomes in patients with KTS.…”

En bloc hysterectomy for treatment of symptomatic cervical varix in the early second trimester

“…Most reported cases have resulted in delivery due to hemorrhage during the second or third trimester [3][4][5][6][7][8][9][10][11]. Cervical varicosities diagnosed during pregnancy have been associated with maternal in utero exposure to diethylstilbestrol, Klippel-Trenaunay syndrome, and abnormal placentation [3,[7][8][9][10][11][12][13][14]. Although most cases occur in the setting of placenta previa, there have been cases reported without concurrent abnormal placentation [3].…”

Vaginal delivery in the presence of huge vulvar varicosities: a case report with MRI evaluation

“…For example, there is no indication for caesarean section other than known obstetric indications in cases of the Klippel-Trenaunay syndrome, which is a congenital vascular disorder that often presents vulvar varicosities (4)(5)(6)(7)(8) . However, some reports (2,3,(4)(5)(6) , but not all (7)(8)(9)(10) , have shown that caesarean delivery should be considered in cases of large vulvar varicosities. There is no evidence that either mode of delivery is advantageous in cases of huge vulvar varicosities, and no data are available to quantify the risk of rupture during a vaginal delivery.…”