Management of Pregnancy with Klippel-Trenaunay-Weber Syndrome: A Case Report and Review

Abstract

Background: Klippel-Trenaunay-Weber syndrome is a rare neurocutaneous syndrome with vascular involvement. Given the rarity of the syndrome, its management in pregnancy is based on the outcome of a few case reports and expert opinion.

Case summary: The management of a complicated case with its antepartum, intrapartum, and postpartum concerns has been addressed in this review.

Conclusions: Prenatal consults with anesthesia, general surgery, intervention radiology, and internal medicine should be arranged, prior to delivery in anticipation of all the possible complications. Apart from the pregnancy management, preconceptional counselling including the genetics, prognosis, and contraception has an important role in patient management.

Figure 1

(a) Extremely large port wine stain/hemangioma extending from the right flank to midthigh with superficial excoriations. (b) Toes enlarged to about 5 cm in width and overlapping with each other. (c) Midline laparotomy scar along with multiple other scars from the debridements.

Figure 2

MRI images depicting. (a) Marked hemihypertrophy of the subcutaneous soft tissues on the right side at the level of the lower abdomen. (b) Marked hemihypertrophy of the subcutaneous soft tissues on the right thigh as compared to the left (normal). (c) Numerous venous channels were noted to cross at the potential epidural injection sites from L1 to L5. The thickness from the skin to the thecal space was 9.4 cm.