Pain, Psychiatric Comorbidities Common in Patients With Klippel-Trenaunay Syndrome

Klippel Trenaunay
Klippel Trenaunay
Klippel-Trenaunay syndrome is associated with the triad of capillary malformation, venous malformation, and/or lymphatic malformation, and limb overgrowth.

Pain, psychiatric comorbidities, and psychosocial stressors are common in patients with Klippel-Trenaunay syndrome (KTS), with pain affecting nearly two of three patients and a psychiatric condition reported in one of four, according to the results of a retrospective review conducted at the Mayo Clinic in Rochester, Minnesota, and published in the Journal of the American Academy of Dermatology.

The investigators of the current analysis sought to determine the prevalence of psychiatric comorbidities and pain in patients with KTS.

A total of 410 patients were enrolled in the study. Median age at presentation was 19 (range, 11 to 35), and median follow-up time was 22.0 months (range, 0 to 23.2 months). Overall, 46% of the participants were men and 94.6% were white.

Pain was reported by 63.4% (260 of 410) of patients, most commonly in the 18- to 50-year-old age group (80.1% of participants) and least common in patients age <5 (11.9%). Patients with any complications associated with KTS were significantly more likely to experience pain compared with patients without any disease complications (P <.0001). Complications associated with pain-included cellulitis (76.4%, P =.0041); ulcers or skin breakdown (83.5%, P <.0001), capillary malformation complications (74.8%, P =.0064), superficial thrombophlebitis (82.4%, P <.0001), orthopedic complications (66.9%, P =.0429), deep embolic/thrombotic events (85.5%, P <.0001), and gastrointestinal complications (80.3%, P =.0011).

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Patients with vascular malformations of the buttocks (74.0%, P =.0036), lower extremities (66.4%, P =.0008), and feet (80.6%, P =.0007) were significantly more likely to experience pain compared with patients with vascular malformations in other areas. Lymphatic malformations were not significantly associated with an increased risk for pain.

Overall, 23.2% (95 of 410) of patients had a diagnosed psychiatric condition, most often depression (15.1%) and anxiety (5.1%). Pain (P =.0016), superficial thrombosis (P =.0269), deep embolic/thrombotic events (P =.0005), gastrointestinal complications (P =.0085), genitourinary complications (P =.0163), and capillary malformation of the hands (P =.0040) were all significantly associated with having a psychiatric diagnosis.

Limitations of the study included the fact that it was retrospective in design and relied on physician detection and reporting of the variables. The investigators concluded that awareness of the psychosocial impact of KTS and appropriate screenings are critical in this patient population.


Harvey JA, Nguyen H, Anderson KR, et al. Pain, psychiatric comorbidities, and psychosocial stressors associated with Klippel-Trenaunay syndrome [published online June 5, 2018]. J Am Acad Dermatol. doi:10.1016/j.jaad.2018.05.1245