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.

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

“…(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 is in contradiction with current literature on pregnancy outcomes in KTS patients.…”

“…The largest case series published in the literature so far consisted of 4 patients. (6) Severe pregnancy complications in this patient category have previously been described in case reports and case series, (7,16,17) but these findings could have been coincidental considering the small sample sizes. This study, enrolling a large proportion of women with KTS in the Netherlands, provides stronger evidence to confirm the high prevalence of these complications.…”

Vulvar Vascular Tumors

“…4 The morbidity experienced includes bleeding, pain, infection, and negative self-body image. 25,37,38 Treatment usually consists of combination surgery and sclerotherapy with aspirin to minimize the pain associated with phlebothrombosis and prophylactic antibiotics to prevent recurrent infections. 25,37,38 Treatment usually consists of combination surgery and sclerotherapy with aspirin to minimize the pain associated with phlebothrombosis and prophylactic antibiotics to prevent recurrent infections.…”

A case of vaginal varix during pregnancy

“…Another case was reported with a fetal death from signifi cant maternal bleeding from the varix rupture, with the bleeding spot seen as a hole in a localized varicosity of a vein [7]. Watermeyer et al [8] reported a 21-year-old primiparous woman with a massive plexus of varicosities fi lling the uterovesical fold that extended over the lower uterine segment and performed a classical Cesarean section with a blood loss of 1,500 mL. This patient experienced spontaneous vaginal bleeding after coughing infrequently.…”

“…It is important to recognize the possibility of associated anatomical or pathological diseases. It may include leg varices, venous malformation of the labia, clitorial area, or vagina with or without arteriovenous malformation on the limbs or trunk (KlippelTrenaunay syndrome) [8]. The Klippel-Trenaunay syndrome consists of a triad of cutaneous port-wine capillary malformations, varicose veins and hemihypertrophy of soft tissue and bone.…”