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Letter |
University of Michigan Health System Ann Arbor, MI
Mallinckrodt Institute of Radiology Washington University St. Louis,
MO
University of Michigan Health System Ann Arbor, MI
Mallinckrodt Institute of Radiology Washington University St. Louis,
MO
WEB—This is a Web exclusive article.
There is no doubt that the classification system proposed by Mulliken and Glowacki [3] is an excellent means by which to categorize cutaneous vascular lesions. This schema, as originally described, pertained only to cutaneous lesions and failed to account for a variety of vascular lesions (including those affecting visceral organs). The vascular lesion definitions provided in the portion of our article on Hemangiomas, Vascular Malformations, and Arteriovenous Malformations [2] are consistent with those currently described by the International Society for the Study of Vascular Anomalies (ISSVA) [4].
Although many syndromes are associated with vascular lesions, including those mentioned in our article and those listed by Dr. Alomari [1], there are numerous others as well [4]. The syndromes that we did not review were simply beyond the scope of our article because of word and figure limitations. We did not suggest that the syndromes reviewed in our article were the only syndromes associated with vascular lesions, as indicated by the word "spectrum" in the title. In addition, we specifically stated in the "Objective" section of the abstract that our intention was to review the four specific entities listed.
Regarding "Kasabach-Merritt syndrome," we agree with Dr. Alomari that the term may best be reserved for use in the setting of a "Kaposiform" hemangioendothelioma or tufted angioma. Based on a review of the literature, however, the term has been commonly applied, perhaps incorrectly, to any consumptive coagulopathy in the setting of a vascular lesion. Kasabach-Merritt syndrome or, more accurately, "Kasabach-Merritt phenomenon," has been attributed to a variety of other vascular lesions, including angiosarcomas and vascular malformations [5–9].
The vascular lesion in Figure 1 in our article [2] is a proven venous malformation. The patient in that figure had numerous additional cutaneous and gastrointestinal venous malformations that satisfied the clinical diagnosis of blue rubber bleb nevus syndrome (BRBNS). Although it is possible that this patient had both BRBNS and an incidental separate large vascular malformation, we believe it is much more likely that this lesion is related to the patient's underlying syndrome. Figure 2 in our article [2] shows a large lower extremity venous malformation in another patient with clinically diagnosed BRBNS. "Pelvic extension of a lymphatic malformation," as proposed by Dr. Alomari, is impossible because the lesion in Figure 1 of our article did not extend beyond the perirectal space. It should also be noted that phleboliths may be particularly difficult to visualize on MRI, particularly when judging their presence or absence on the basis of a single axial image. A similar large pelvic vascular lesion with involvement of the rectal wall has been described by Atten et al. [10], also in the setting of BRBNS.
The patient in Figure 7 of our article [2] had been clinically diagnosed with Klippel-Trénaunay syndrome (KTS). This diagnosis was not made solely on the basis of imaging. Based on a review of the literature, the extremity hypertrophy observed in KTS may be due to either soft-tissue (muscular or fatty) or osseous overgrowth. On careful review of Figure 7, the pattern of overgrowth observed in this particular patient is predominantly muscular. Fifteen of 54 patients in a large review of KTS MRI findings by Jacob et al. [11] showed ipsilateral enlargement of muscles with respect to the affected extremity. Twenty-eight percent of patients in their study showed muscular as opposed to fatty hypertrophy, which contradicts the assertion by Dr. Alomari [1] that "overgrowth is predominantly fatty and mainly located in the subcutaneous layer, with relative sparing of the musculoskeletal compartment." The purpose of this figure was to clearly show mus cular overgrowth, not to depict the patient's additional vascular abnormalities. We also think that it is impossible to judge the presence or absence of a vascular malformation on the basis of a single axial image.
The large posterior mediastinal vascular lesion shown in Figure 9 in our article [2] was indeed a cavernous hemangioma. Such a mediastinal vascular lesion has been previously described by other authors in the setting of KTS [12]. The exact incidence of such lesions in the setting of KTS, however, is unknown.
The patient in Figure 10 of our article [2] does indeed have the clinical diagnosis of KTS. The MR and angiographic images presented were selected because of their excellent depiction of the patient's venous malformation. We believe that it is impossible to say this patient has or does not have KTS on the basis of the images provided. Appropriate evaluation for fatty overgrowth, as mentioned by Dr. Alomari [1], requires evaluation of more than a single image and should use images with and without fat suppression.
In conclusion, we thank Dr Alomari [1] for these thoughtful comments on our article but must strongly disagree with the assertion that the cases in our article were misdiagnosed. In fact, all patients included in our series were appropriately diagnosed on the basis of the clinical and radiologic criteria discussed in our article. However, we do agree with Dr. Alomari that nosologic confusion remains regarding the classification of vascular lesions, especially visceral lesions.
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