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Archived Comments for: Control of disseminated intravascular coagulation in Klippel-Trenaunay-Weber syndrome using enoxaparin and recombinant activated factor VIIa: a case report

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    Ahmad Alomari, Children’s Hospital Boston

    4 May 2011


    Pradeep Govender, MD, Ahmad I. Alomari, MD, MSc, FSIR

    Division of Interventional Radiology and Vascular Anomalies Center, Children’s Hospital Boston and Harvard Medical School, Boston, MA, USA.

    To the Editor
    We read with interest the case report by Beier et al [1] in which the author presented a child with numerous vascular abnormalities in her extremities. The author described an 11-year-old girl “diffuse hemangioma”, “varicosities”, arteriovenous malformations (AVMs) of involving the soft tissues of the pelvis and the bilateral lower extremities with bilateral lower extremity hypertrophy. The patient carried a diagnosis of “Klippel-Trenaunay-Weber” syndrome on which the authors discussed their experience using recombinant factor VIIa. However, I believe this is an erroneous association as the diagnosis given to this patient is not certain and there are several points of clarification needed in the case report. They include:
    1. The term “Klippel-Trenaunay-Weber” syndrome creates diagnostic confusion as Klippel-Trenaunay syndrome (KTS) and Parkes-Weber syndrome (PWS) are entirely different syndromes. Klippel-Trenaunay syndrome is a slow flow vascular anomaly with three major vascular components – capillary (port wine stain), lymphatic and venous malformation (CLVM) – in an overgrown limb predominantly due to excess subcutaneous fatty tissue. [2] Parkes-Weber syndrome is a high flow vascular anomaly combining an extremity port wine stain and diffuse high flow shunts.
    2. There was mention of “varicosities” in this child; however, no clinical, photographic or imaging documentation was provided by the authors regarding the presence of any venous, capillary or lymphatic malformations to support a diagnosis of Klippel-Trenaunay syndrome or Parkes-Weber syndrome. Venous anomalies in KTS are of the “phlebectasia” type frequently manifested as a prominent marginal venous system (anatomically related to the capillary stain), pelvic phlebectasia, and megacava.
    3. Arteriovenous malformation is not a presentation of Klippel-Trenaunay syndrome. The “varicosities” and bilateral lower extremity hypertrophy may be secondary to the AVM involving the soft tissues of the pelvis. Unfortunately, the author fails to provide any imaging to clarify or pathologic correlation from the vulval capillary hemangioma resection.
    4. “Diffuse hemangiomata” is another imprecise term. Infantile hemangiomata are benign vascular tumors with proliferation and involution phases. The child is of an age where complete involution would have been expected in a hemangioma.
    5. Localized intravascular coagulation (LIC) is less severe consumptive coagulopathy, often seen in patients with large venous malformations. LIC is characterized by elevated D-dimers, hypofibrinogenemia, and normal or occasionally slightly decreased platelet count [3]. LIC is not a common feature in Klippel-Trenaunay syndrome.
    We believe that the case presented represents a patient with a vascular anomaly; however, the author has not presented any supportive evidence to confirm that this patient had KTS. Unfortunately, misdiagnosis in the field of complex vascular anomalies is still common [4]. Careful analysis of the clinical and imaging features and the use of proper terminology are paramount to reach the appropriate diagnosis and treatment in patients with vascular anomalies. Adding the author’s experience to the Klippel-Trenaunay literature without supportive evidence creates confusion in treating these patients.

    1. Beier U, Schmidt ML, Hast H, Keckes S, Valentino LA. Control of disseminated intravascular coagulation in Klippel-Trenaunay-Weber syndrome using enoxaprin and recombinant activated factor VIIa: a case report. J Med Case Reports 2010; 4:92.
    2. Christison-Lagay ER, Fishman SJ. Vascular anomalies. Surg Clin North Am 2006;86:393-425
    3. Mazoyer E, Enjolras O, Laurian C, Houdart E, Drouet L. Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome. Clin Lab Haematol 2002;24:243-51
    4. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial cell characteristics Plast Reconstr Surg. 1982;69:412-22.

    Competing interests

    No conflict of interests or financial benefits to declare