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Clinical Analysis and Outcomes of Klippel-Trenaunay Patients Requiring Surgical or Interventional Procedures

Sunday, October 21, 2012: 8:50 AM
Versailles Ballroom (Hilton Riverside)
Rebecca Dawn Fevurly, MD, Surgery, Children's Hospital Boston, Boston, MA, Victor M. Johnson, MA, General Surgery, Children's Hospital Boston, Boston, MA, Cameron C. Trenor III, MD, Department of Hematology and Oncology, Children's Hospital Boston, Boston, MA, Ahmad I. Alomari, MD, Division of Vascular and Interventional Radiology, Children's Hospital Boston and Harvard Medical School, Boston, MA and Steven J. Fishman, MD, FAAP, Department of Surgery, Children's Hospital Boston, Boston, MA

Purpose: Klippel-Trenaunay Syndrome (KTS) consists of combined capillary, lymphatic and venous malformations associated with overgrowth.  Patients suffer from disfigurement, lymphatic drainage, bleeding, and infections.  We analyzed clinical characteristics of patients with KTS who underwent surgical debulking, CO2 laser, or interventional radiology (IR) procedures at our institution.

Methods: Patients were identified via cross-referencing of surgical records with records in our Vascular Anomalies database.  Clinical course was collected from paper records and electronic medical records and compiled on REDCap with IRB approval (IRB-P00001058 and M09-03-0158).  Surveys were sent to the patients and/or parents for follow up.

Results: 96 patients (49 females) with KTS underwent surgical debulking, CO2 cutaneous laser therapy, and/or IR procedures between 1990 and 2011. Clinical review found 40 (42%) patients noted a history of infections.  32 (55%) had gastrointestinal involvement, 30 (71%) with rectal bleeding; 42 (44%) had genitourinary involvement, 15 (36%) with hematuria, and 7 with vaginal bleeding.  84 (91%) patients had lower extremity involvement, 61 (75%) with leg-length discrepancy (p<0.001).  Flexion contracture developed in 15 (25%).  56 (58%) underwent surgical debulking, 16 (24%) had CO2 laser therapy, and 67 (70%) underwent an IR procedure.  Of the latter, 16 (24%) had venous sclerotherapy, 23 (34%) had venous laser embolization, 21 (31%) had sclerotherapy for a lymphatic malformation, and 23 (34%) had skin vesicle sclerotherapy.  Of the 56 who underwent surgical debulking, 25 (44%) had one procedure, 14 (25%) had two, 15 (27%) had 3-5, and 2 (4%) had 6-7 procedures.  15 complications (infection, bleeding, DVT/PE, or wound dehiscence) were documented and were not associated with the number of procedures per patient (p=0.30) or age (p=0.16).  Of the 20 surveys returned for debulking procedures, reasons for undergoing surgery included concern for future complications (19, 95%), cosmetic appearance (16, 80%), lack of mobility (15, 75%), pain (14, 70%), bleeding from skin vesicles (11, 45%), recurrent infections (8, 40%), and lymphatic leakage (6, 30%).  Following recovery, positive outcomes were noted for 16 (80%) for improved mobility, 14 (70%) for pain, 11 (50%) for decreased bleeding from vesicles, and 8 (40%) for decreased lymphatic drainage.  16 (80%) responders reported being extremely or very satisfied with the outcome of the procedure.  The only abnormal baseline coagulation value was D-Dimer level at 8.5 mg/L FEU (n=45, 95% CI 4.6-11.9).  Those with a history of DVT or PE had an average D-Dimer level of 6.8 mg/L FEU (n=15, 95% CI 3.2-10.5).

Conclusion: KTS continues to be difficult and time consuming to treat.  Our results have shown the variety of symptoms associated with KTS and the wide range of techniques employed.  Abnormal baseline coagulation values include D-Dimer level, but it does not appear to correlate with DVT/PE risk.