A multidisciplinary management approach for patients with Klippel-Trenaunay syndrome and multifetal gestation with successful outcomes

Introduction

Klippel-Trenaunay syndrome (KTS) is a chronic condition that is infrequently encountered during pregnancy. There have been few case reports documenting pregnancies affected by this disease process, and there is a paucity of literature regarding the management of pregnancies affected by the disease [1]. Traditionally, several conditions have been lumped together under the definition KTS, including Parks-Weber syndrome and diffuse capillary malformation with overgrowth (DCMO). As defined by the International Society for the Study of Vascular Anomalies, KTS is a complex congenital disorder characterized by combination of capillary and venous malformation and limb overgrowth, with or without lymphatic malformation [2]. The incidence and prevalence of the disease are unknown, and no gender or ethnic predilections have been identified. The pathophysiology and genetics of the disease are also unknown, and most cases appear to be sporadic [3]. Few familial cases have been reported for which a paradominant inheritance pattern has been proposed [2]. Although there are many complications of KTS, the most significant complications affecting pregnancy are the increased risks of coagulopathy, VTE and bleeding in addition to chronic pain and anatomic anomalies affecting ability to receive neuraxial analgesia. The most common sites for venous malformations are the superficial and deep veins of the lower extremities [4]. Case reports have demonstrated worsening of these venous malformations during pregnancy, particularly of the lower extremities [5]. Although once considered a contraindication to pregnancy, there have been case reports of patients with KTS with successful pregnancy and neonatal outcomes. While there are reports of singleton pregnancies affected by KTS, there is even less literature about multiple gestations affected by KTS. Given the increased blood volume and risk of VTE associated with multiple gestations, we present two cases of pregnancies affected by maternal KTS with the additional risks associated with multiple gestations.

Case presentation 1

The first case was a 21-year-old gravida 2 para 0 with KTS who presented for prenatal care at 7 weeks. She was found to have a dichorionic-diamnionic twin gestation. She underwent genetic counseling. She also used tobacco, although she quit at 22 weeks. The patient was diagnosed with KTS and followed by pediatric hematology throughout childhood. She had previously undergone knee surgery for a right lower extremity vascular malformation in 2008. At baseline her right leg did not extend completely, was slightly larger than her left leg and she walked with a limp. This was managed with compression hose at baseline. She had associated cutaneous findings with significant port-wine staining of her right lower back that extended laterally down her right leg, labia and perineum.

She was referred to hematology who recommended prophylactic low molecular weight heparin (LMWH) and aspirin (ASA) daily with plan to hold 24–48 h prior to delivery with resumption of anticoagulation with LMWH for 6 weeks postpartum with resumption of ASA after discontinuation of LMWH. The patient had self-discontinued LMWH after a bleeding episode. Given bleeding, hematology recommended discontinuation of ASA with continuation of LMWH. She had no additional issues with anticoagulation.

She underwent a magnetic resonance imaging (MRI) of the spine and pelvis without contrast that revealed a vascular anomaly extending from the lower back to the lateral aspect of her right leg and right labia. This reached the level of L4/L5 and radiology recommended L3 entry if neuraxial analgesia was to be performed.

She was referred to anesthesiology for consultation who recommended against neuraxial analgesia with plan for general anesthesia if a primary cesarean section was indicated given MRI findings and plan to deliver in the main OR.

Di-di twins were managed with usual obstetrical management with serial fetal growth ultrasounds and antenatal testing in the 3^rd trimester and a plan for delivery at 38 weeks. Patient presented at 38 weeks + 0 days for scheduled cesarean delivery due to breech/transverse presentation and bilateral tubal ligation as the patient desired sterility. Baby A weighed 2298 g with Apgar scores of 8 and 8. Baby B weighed 2866 g with Apgar scores of 8 and 8. General endotracheal anesthesia was utilized, and she tolerated the procedure well without complication. Her postoperative course was uncomplicated. LMWH was resumed for 6 weeks postpartum.

Case presentation 2

The second case was an 18-year-old primagravida with KTS who presented for prenatal care at 10 weeks. Ultrasound at 8 weeks + 4 days diagnosed mono-di twin gestation. Her prenatal course was also remarkable for significant nausea and vomiting of pregnancy in the 1^st trimester. KTS was diagnosed by her pediatrician in childhood due to port wine staining and lower extremity length discrepancy. She had previously undergone 11 corrective surgical procedures on the left leg and one corrective surgery on the right leg. On initial exam, the patient was noted to have cutaneous findings consistent with KTS including port-wine stains on the groin, perineum and superior aspect of the right lower extremity.

The patient was followed by hematology throughout the pregnancy. A baseline bilateral lower extremity ultrasound was performed due to pain and swelling and showed no evidence of lower extremity deep vein thrombosis (LE DVT). Hematology recommended prophylactic LMWH for the remainder of pregnancy and continuation for 6 weeks postpartum. Repeat imaging postpartum with contrast for better characterization of vascular involvement was also recommended.

She underwent imaging with MRI of the lumber (L)-spine and pelvis both without contrast. These studies showed no evidence of vascular anomalies or malformations, although arteriovenous malformation (AVM) cannot be totally ruled out without contrast. Given significant port wine stains involving the perineum and increased risk of bleeding within capillary stains as well as risk of severe disseminated intravascular coagulation (DIC), hematology recommended considering a cesarean section for mode of delivery.

She was seen by anesthesia who felt neuraxial analgesia was appropriate given no L-spine disease involvement on MRI, and the patient had previously had an epidural with prior orthopedic surgery without complications.

She was counseled on the risks of multiple gestations and particularly the risk of developing twin to twin transfusion syndrome (TTTS) with mono-di twins. Incidentally, at 18 weeks she was noted to have a short cervix of 1.7 cm and significant funneling. Given decreased mobility due to KTS in the setting of a short cervix a cerclage was recommended to maximize mobility and decrease risk of VTE. An ultrasound-indicated McDonald cerclage was placed at 18 weeks without complication. She underwent standard obstetric management for mono-di twins with ultrasound every 2 weeks monitoring for TTTS, antenatal testing, and plans for delivery at 36–37 weeks or sooner if indicated.

Follow-up ultrasounds remained reassuring with appropriate interval growth and minimal discordance. A decision was made to proceed with cesarean delivery over induction of labor given risk of severe bleeding or DIC from involvement of vascular anomalies across perineum. Cesarean delivery ultimately was scheduled at 36 weeks for breech/vertex presentation.

Prophylactic LMWH was held the evening prior to the procedure. Baby A weighed 2554g with Apgar scores of 9 and 9. Baby B weighed 2270g with Apgar scores of 8 and 9. The cerclage was removed at the time of cesarean section. Her delivery was complicated by a postpartum hemorrhage which was felt to be due to uterine atony. She received pitocin, misoprostol, carboprost tromethamine, methylergonovine and ultimately a Bakri balloon was placed with improvement of uterine tone. Given estimated blood loss of 2 L, she was given 2 units of packed red blood cells and 2 units of fresh frozen plasma intraoperatively. She did well postoperatively and experienced no other complications or coagulopathy. LMWH was resumed for 6 weeks postpartum.

Discussion

Given the associated spectrum of complications, pregnancies complicated by KTS should be considered high risk and therefore care should be overseen by a maternal-fetal medicine specialist. Currently, no guidelines exist for management of pregnancies complicated by KTS. We present these two high risk cases to further demonstrate the importance of multidisciplinary coordination of care.

It is important to perform a thorough history and physical examination to determine the severity and extent of the disorder prior to the onset of pregnancy. Increased VTE risk is important to consider in multiple gestations, especially in patients with KTS already at increased risk. Anticoagulation should be addressed early and patients should be counseled on the importance of compliance. In both cases, patients received prophylactic dose LMWH daily antepartum with resumption of LMWH for 6 weeks postpartum. Low dose ASA should be considered in addition to LMWH depending on comorbidities. In the first case the patient was taking ASA daily, but was discontinued after a bleeding episode. In the second case, VTE prophylaxis was adequate on LMWH alone. Consideration should be given for therapeutic anticoagulation if a patient with KTS had a history of previous VTE.

Given increased bleeding risks associated with KTS, extra precautions should be taken in preparation for postpartum hemorrhage, especially in multiple pregnancies. Although the second case involved a postpartum hemorrhage, it was not felt that this was directly related to KTS or recent anticoagulation, but rather more likely related to atony due to twin gestation.

If not previously done, imaging studies should be performed to evaluate extent of vascular disease and to aid decision making for route of anesthesia as well as mode of delivery. Evaluation of L-spine vascular involvement in both cases was critical in anesthesia’s decisions regarding the route of anesthesia. The first case demonstrates a conservative approach to proceed with general anesthesia given marginal involvement of the lower L-spine. The second case demonstrates that neuraxial anesthesia can be safely administered after ruling out L-spine involvement.

The extent of disease in the pelvis as well as location of port-wine stains are crucial in determining appropriate mode of delivery. The second case demonstrates the importance of thorough physical exam in addition to imaging. Given the significant involvement of perineum with port-wine staining and baseline increased risk of bleeding within capillary stains, hematology recommended against vaginal delivery to avoid trauma to the perineum.

Given the rarity of KTS with unknown incidence and prevalence, the literature regarding the care of these patients is scarce and further investigation in the care of pregnancies affected by KTS is needed. In conclusion, we present two cases of high risk pregnancies affected by KTS and multiple gestations with successful outcomes. Using a multidisciplinary approach with hematology, radiology and anesthesia, we were able to minimize morbidity and optimize patient outcomes.

Teaching points

1. Discussion of Klippel-Trenaunay syndrome and associated obstetrical implications of the disease.

2. Antepartum and postpartum anticoagulation strategies for prevention of VTE in patients with KTS

3. Preoperative planning for patients with Klippel-Trenaunay syndrome