Pain, psychiatric comorbidities, and psychosocial stressors associated with Klippel-Trenaunay syndrome

Background: Klippel-Trenaunay syndrome (KTS) is characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. Patients with KTS have lower scores in general in mental health, physical function, and quality of life than the general population. Objective: To determine the prevalence of pain and psychiatric comorbidity in patients with KTS. Methods: A retrospective review of 410 patients with KTS evaluated during 1976-2012 was conducted to identify the presence of pain, psychiatric comorbidities, and psychosocial stressors. Results: Pain was reported by 260 patients (63.4%) and was associated with any KTS complication (P <.0001) and venous malformations of the lower extremities (P =.0008) and feet (P =.0007). Ninety-five patients had a diagnosed psychiatric condition (23.2%), most commonly 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 associated with having a psychiatric diagnosis. Limitations: This is a retrospective study that relied on physician detection and reporting of variables. Conclusion: Pain and psychiatric conditions, particularly depression and anxiety, are common in patients with KTS. Awareness of the psychosocial impact of KTS and appropriate screening are important.