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ORIGINAL RESEARCH |
From the Department of Vascular Surgery and Pathology, Center for Vascular Anomalies, "Istituto Dermopatico dell’Immacolata," I.D.I.-IRCCS, Roma, Italy; and Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas.
Address reprint requests to: Francesco Stillo, MD, Chairman, Department of Vascular Surgery and Pathology, "Istituto Dermopatico dell’Immacolata," I.D.I.-IRCCS, Via dei Monti di Creta, 104, 00167 Roma, Italy; E-mail: f.stillo{at}idi.it.
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ABSTRACT |
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METHODS: Five symptomatic patients were treated. The degree of pain and discomfort was self-assessed by using a horizontal visual analog scale before and after treatment. Preoperative evaluation included Doppler ultrasound scanning in all patients and magnetic resonance imaging (MRI) in one. All patients had direct-injection venography and sclerotherapy during the same session. Ethanol was used in two cases and polidocanol in three. Patients were followed-up by means of Doppler ultrasound scanning and office visits.
RESULTS: All patients experienced marked swelling after the injection, and one developed cutaneous necrosis that healed within 2 weeks. Transient hemoglobinuria was observed in two cases. No early or late major complications occurred. At a mean follow-up of 23 months (range 5–43), all patients experienced complete relief from symptoms and currently have normal vulvar sensation. Four patients had complete ablation of the treated lesion. In one patient the procedure resulted in a significant, albeit incomplete, occlusion of the lesion, and no further treatment was deemed necessary. From a cosmetic standpoint, both patients and physicians considered the results successful.
CONCLUSION: Vulvar venous malformations should be distinguished from vulvar varicosities, hematomas, soft-tissue neoplasms, and other vascular anomalies. Doppler ultrasound, MRI, and direct-injection venography are the most accurate diagnostic modalities. Sclerotherapy can successfully treat this condition. The procedure should be monitored with an imaging modality, preferably direct-injection venography with digital subtraction serial imaging.
Congenital vascular anomalies are classified based on endothelial characteristics that correlate with the clinical features and natural history of these lesions.1 Vascular anomalies can be divided into two major categories: hemangiomas and vascular malformations. The former are soft-tissue tumors characterized by rapid postnatal growth followed by slow, invariable, spontaneous regression during childhood.1 The latter are developmental errors, composed of dysmorphic vessels with normal endothelial turnover, that do not regress.1
Vascular malformations can be further classified based on the predominant channel type and flow characteristics: 1) slow-flow lesions that include capillary, lymphatic, and venous malformations; and 2) fast-flow lesions that include arterial malformations, arteriovenous fìstula, and arteriovenous malformations.1
Venous malformations are the most common, accounting for two thirds of all congenital vascular malformations.2 They can occur in any tissue of the body and are often multifocal.3 Venous malformations involving the external genitalia are rare and can be aesthetically and functionally disabling.3,4
We report our experience in the management of venous malformations of the vulva.
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MATERIALS AND METHODS |
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Symptoms included vulvar dysmenorrhea, swelling, pressure, and discomfort. Two patients had experienced the occurrence of localized thromboses within the malformation that resulted in the development of phleboliths. One patient previously underwent surgical excision of the lesion at another institution.
The degree of pain and discomfort was self-assessed by the patients using a horizontal visual analog scale (0 = no complaints, 1 = slight pain, 2 = moderate pain, and 3 = strong pain), as described previously.5 Differences between the preoperative and postoperative indices were calculated by the nonparametric signed-rank Wilcoxon test for paired observations using the Statistical Package for the Social Sciences 9.0.1, for Windows (SPSS Inc., Chicago, IL). Statistical significance was fixed at P < .05.
In all cases, the venous malformation was located on the vulva and in two cases it also involved the perineum. Two patients had associated venous malformations in other locations.
All patients underwent Doppler ultrasound evaluation before treatment. Magnetic resonance imaging (MRI) was also performed in one patient.
Each patient underwent direct-injection venography and sclerotherapy at one session. Contrast material was injected directly into the venous malformation. The vascular malformation was visualized with digital subtraction serial imaging, at the lowest possible radiation dose, using a C-arm fluoroscope. The size of the lesion in its dilated state and the dynamics of its venous drainage were determined. The sclerosing agent was then injected and contained within the venous malformation by applying proximal manual compression during the procedure and for 10 minutes after. A small amount of contrast medium was added to the sclerosant to visualize its route during the procedure. The volume of sclerosant to be used was estimated from the amount of contrast material required to fill the lesion during venography. After sclerotherapy, contrast material was injected again to confirm the occurrence of intralesional thrombosis.
Ethanol was used in two patients. In these cases the amount of sclerosant injected was 10 mL in one patient and 15 mL in the other. Polidocanol was used in three patients. Its concentration varied from 1% to 3%, and the volume of the solution varied from 5 mL to 15 mL per injection, at the maximum daily dose of 2 mg/kg.
Sclerotherapy was performed in an operating room while patients were under general anesthesia. This was necessary with the three pediatric patients to ensure adequate compliance and with the two adult patients treated with ethanol injections because they are extremely painful.
After treatment, antibiotic cream was applied over the lesion and compression applied with cotton gauze. The urinary output was monitored for volume and color. All patients received intra- and postoperative intravenous hydration and short-term, low-dose corticosteroid administration to reduce inflammation (0.05 mg/kg dexamethasone after polidocanol injection and 0.1 mg/kg dexamethasone after ethanol injection). Patients were observed overnight and discharged home 1–2 days after treatment.
Patients were followed-up by means of Doppler ultrasound scanning to assess the presence of residual venous malformation after treatment and to detect recurrence of the lesion. They were also visited and interviewed to evaluate aesthetic results and improvement of symptoms.
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RESULTS |
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At a mean follow-up of 23 months (range 5–43 months), all patients reported complete relief from symptoms and normal vulvar sensation. Visual analog scale scores to assess severity of pain decreased from a preoperative mean of 2.3 to 0 (nonparametric Wilcoxon test for paired observation, two-tailed P = .034). Four patients had complete ablation of the treated lesion, but one of these patients required two sclerotherapeutic sessions with polidocanol due to a recanalization of the lesion that occurred 4 months after the first treatment.
In one patient the procedure resulted in a significant but incomplete occlusion of the lesion. Because her symptoms completely resolved, and the result was considered aesthetically successful, no further treatment was necessary.
From a cosmetic standpoint, both patients and physicians considered the results successful.
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DISCUSSION |
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).6 The diagnosis of venous malformations can be strongly suspected by careful history and physical examination. However, management of these lesions differs depending on their location.7
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).10,12–14
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These venous malformations can occur as isolated lesions or can be associated with other venous malformations located in any part of the body. In this respect, external genitalia are frequently involved by extensive lower limb venous malformations.3
For vulvar venous malformations, Doppler ultrasound, MRI, and direct-injection venography are the most accurate imaging modalities. At gray-scale ultrasound, venous malformations appear as hypoechoic, heterogenous, and compressible lesions. At Doppler analysis, a monophasic, low-velocity venous signal is detected.8 At MRI, venous malformations have a characteristic appearance with an intermediate signal between fat and muscle on the T1-weighted image and a hyperintense signal intensity as compared with fat on the T2-weighted image.15
Doppler ultrasound scanning provides reliable and adequate information regarding the nature and extent of venous malformations of the vulva. Magnetic resonance imaging is a costly and time-consuming procedure and should be reserved for the preoperative assessment of deeper lesions to further define the relationship of the venous malformation to the pelvic organs, or in case of a difficult differential diagnosis. Direct-injection venography provides a precise anatomic assessment of the venous malformation and its tributaries into the normal venous system (Figure 2). It also provides valuable information about the dynamics of the effluent runoff from the lesion. Furthermore, this diagnostic modality can be transformed immediately into a therapeutic procedure by using selective sclerotherapy to ablate the lesion.
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Surgery does not provide precise excision of the lesions or adequate aesthetic results. In addition, it is often complicated by significant bleeding related to the venous malformation-associated coagulopathy.3
Sclerotherapy consists of the direct intralesional injection of a sclerosing agent that causes the destruction of endothelial surfaces with subsequent thrombosis, leading to intimal fibrosis and obliteration of the vessel lumen.16 This procedure has been largely and successfully used for the treatment of symptomatic venous malformations4,16–18 and can be considered the treatment of choice.
A number of different sclerosing agents have been used, but none of them can be recommended for all venous malformations in terms of safety and efficacy. We believe that the choice of the sclerosant should depend on the size and the location of the lesion.
Ethanol is the most effective sclerosing agent and gives the lowest recurrence rate.17,19 However, its injection produces severe pain and can cause extensive tissue necrosis and nerve damage.18 Hence, ethanol sclerotherapy is used for the treatment of larger and deeper malformations, whereas for small and superficial venous malformations or for lesions in close proximity to major nerves other and different sclerosants, such as polidocanol and sodium tetradecyl sulfate, are indicated.4,18 These are detergent sclerosing agents successfully used for the treatment of venous malformations.4,16,18 They are less potent than ethanol but with minimal risks of ulceration and nerve impairment, and they are also associated with a low incidence of adverse effects and allergic reactions.16,18
Sclerotherapy can be performed blindly,4 or with color duplex16 or direct injection venography guidance.17,18 During blind injection, the intraluminal position of the needle tip is confirmed by the aspiration of blood. This procedure has been demonstrated to be safe and effective in small superficial lesions.4 In contrast, for deeper and larger venous malformations, the injection of sclerosants should be monitored with an imaging modality to prevent accidental intra-arterial injection,16 which may result in distal ischemic complications, and extravasation,17 which may lead to tissue necrosis. Compared with color duplex, direct injection venography is invasive and costly, and its routine use may appear cumbersome. On the other hand, direct-injection venography provides information regarding the size of the lesion in its dilated state and the dynamics of its runoff. We believe this information to be particularly valuable for its therapeutic implications. It has been reported that a small amount of sclerosant can effectively ablate a large venous malformation because of the slow intralesional blood flow and the presence of few and narrow tributaries into the normal venous system.20 In fact, this is not always the case. Venous malformations in their dilated state are often larger than expected, have wide venous egress, and their tributaries present undesirable connections with veins that may drain the sclerosant into parenchimatous organs leading to hazardous complications. Hence, for larger and deeper venous malformations we recommend the use of direct-injection venography as it provides a road map to better plan the treatment.
Though effective, sclerotherapy of venous malformations has been reported to be followed by frequent recanalization of the lesions.16,18 In these cases, the treatment can be safely repeated and, if necessary, followed by surgical resection.3,18
Marked swelling is the most common local complication of sclerotherapy, and this is due to a combination of intralesional thrombus and edema.18 The swelling can be reduced by short-term, low-dose corticosteroid administration.
Blistering and necrosis of the skin overlying the injected venous lakes may occur and should be managed conservatively with local wound care.17 These complications are a consequence of reflux of the sclerosant from superficial venous channels into the skin capillaries.20 When this phenomenon occurs, the skin begins to blanch, and in these cases, the procedure should be immediately discontinued.
Rare systemic complications, such as allergic reactions, cardiac arrest, pulmonary embolism, neurologic deficit, or deep vein thrombosis have also been reported.18,19
Hemoglobinuria can occur, secondary to intralesional hemolysis,16,17 and therefore patients should be aggressively hydrated during and after the procedure to prevent renal impairment.4
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Footnotes |
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Received January 2, 2001. Received in revised form June 27, 2001. Accepted July 3, 2001.
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REFERENCES |
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2. Eifert S, Villavicencio JL, Kao TC, Taute BM, Rich NM. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc Surg 2000;31:462–71.[Medline]
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4. Marrocco-Trischitta MM, Nicodemi EM, Stillo F. Sclerotherapy for venous malformations of the glans penis. Urology 2001;57:310–3.[Medline]
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6. Mulliken JB. Cutaneous vascular anomalies. Semin Vasc Surg 1993;6:204–18.[Medline]
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9. Paltiel HJ, Burrows PE, Kozakewich HPW, Zurakowski D, Mulliken JB. Soft-tissue vascular anomalies: Utility of US for diagnosis. Radiology 2000;214:747–54.
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11. Ninia JG, Goldberg TL. Treatment of vulvar varicosities by injection-compression sclerotherapy and a pelvic supporter. Obstet Gynecol 1996;87:786–8.[Abstract]
12. Veltman LL, Ostergard DR. Thrombosis of vulvar varicosities during pregnancy. Obstet Gynecol 1972;39:55–6.
13. Travers JP, Brookes CE, Evans J, Baker DM, Kent C, Makin GS, et al. Assessment of wall structure and composition of varicose veins with reference to collagen, elastin and smooth muscle content. Eur J Vasc Endovasc Surg 1996;11:230–7.[Medline]
14. Jones GT, Solomon C, Moaveni A, van Rij AM, Thomson IA, Galvin I. Venous morphology predicts class of chronic venous insufficiency. Eur J Vasc Endovasc Surg 1999;18: 349–54.[Medline]
15. Dobson MJ, Hartley RW, Ashleigh R, Watson Y, Hawnaur JM. MR angiography and MR imaging of symptomatic vascular malformations. Clin Radiol 1997;52: 595–602.[Medline]
16. Yamaki T, Nozaki M, Sasaki K. Color duplex-guided sclerotherapy for the treatment of venous malformations. Dermatol Surg 2000;26:323–7.[Medline]
17. Shireman PK, McCarthy WJ, Yao JST, Vogelzang RL. Treatment of venous malformations by direct injection with ethanol. J Vasc Surg 1997;26:838–44.[Medline]
18. Berenguer B, Burrows PE, Zurakowski D, Mulliken JB. Sclerotherapy of craniofacial venous malformations: Complications and results. Plast Reconstr Surg 1999;104:1–11.[Medline]
19. Yakes WF, Haas DK, Parker SH, Gibson MD, Hopper KD, Mulligan JS, et al. Symptomatic vascular malformations: Ethanol embolotherapy. Radiology 1989;170: 1059–66.
20. de Lorimier AA. Sclerotherapy for venous malformations. J Pediatr Surg 1995;30:188–94.[Medline]
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