Pediatric Surgery International

, Volume 27, Issue 12, pp 1283–1287

Percutaneous sclerotherapy of peripheral venous malformations in pediatric patients

Authors

  • Fatih Gulsen
    • Department of Interventional Radiology, Cerrahpasa Faculty of MedicineIstanbul University
    • Department of Interventional Radiology, Cerrahpasa Faculty of MedicineIstanbul University
  • Serdar Solak
    • Department of Interventional Radiology, Cerrahpasa Faculty of MedicineIstanbul University
  • Gokce Gulsen
    • Department of Interventional Radiology, Cerrahpasa Faculty of MedicineIstanbul University
  • Enes Ozluk
    • Department of Interventional Radiology, Cerrahpasa Faculty of MedicineIstanbul University
  • Furuzan Numan
    • Department of Interventional Radiology, Cerrahpasa Faculty of MedicineIstanbul University
Original Article

DOI: 10.1007/s00383-011-2962-9

Cite this article as:
Gulsen, F., Cantasdemir, M., Solak, S. et al. Pediatr Surg Int (2011) 27: 1283. doi:10.1007/s00383-011-2962-9

Abstract

Purpose

To report the efficacy of percutaneous puncture and sclerosis using polidocanol in the treatment of venous malformations (VMs) in pediatric patients.

Patients/methods

Between March 2007 and February 2011, a series of 19 patients with VMs on the upper and lower extremities had undergone a total of 89 sessions of intralesional sclerotherapy using polidocanol. All the procedures were performed in an angiographic suite under general anesthesia in order to maintain sedation for facilitating the procedure. For each injection, approximately 1 ml of 2% polidocanol was injected for each centimeter of the diameter of the lesion, with a maximum of 6 ml. Injection of the sclerosant was guided by real-time sonography and fluoroscopy.

Results

After the treatment, the symptoms completely resolved in four patients (21%). Clinical symptoms, such as bulging and pain were improved in 12 out of 19 patients (63%). They remained unchanged in three patients (16%). During the procedures, no major complications were encountered. The minor complications (65%) encountered were swelling and pain after treatment which were resolved by taking NSAID within a few days.

Conclusion

In pediatric patients, sonographically guided percutaneous puncture and fluoroscopically guided sclerosis using 2% polidocanol is effective, less invasive and safe for the treatment of VMs, with a high success rate and minimal complications.

Keywords

Peripheral venous malformationsSclerotherapyPediatric

Introduction

The International Society for the Study of Vascular Anomalies (ISSVA) has adopted the classification of vascular anomalies described by Mulliken and Glowacki [1]. They originally proposed the biological classification of vascular anomalies that includes two major categories: vascular tumors (hemangioma) and vascular malformations. The modified Hamburg classification presented in 1993 is now the accepted anatomopathological classification of congenital vascular defects [2]. This classification divides these defects into five types according to their predominantly malformed vessels (arterial, venous, lymphatic, arteriovenous shunting and combined/mixed) and their anatomopathological form (truncular or extratruncular).

The pathological features of venous malformations (VMs) are to be composed of dilated, thin walled, sponge-like abnormal channels of variable size and thickness [3]. VMs are the most common symptomatic vascular malformations [4]. VMs are sometimes asymptomatic, but they may become painful when thrombophlebitis or muscular, articular or nerve involvement occur [5]. Other symptoms of vascular malformations include cosmetic disfigurement, limitation of function, and bleeding. In the management of VMs, choosing the appropriate treatment is challenging due to these lesions’ complexity. Although a surgical excision has been the standard treatment for VMs, it may be effective only when the lesion is localized and well-defined. In most of the cases, surgical excision does not produce good results because of its functional and aesthetic sequelae and because of the high recurrence rate [6, 7]. Percutaneous injection of sclerosing agents into the lesion has become the mainstay of treatment [8, 9]. Several vascular sclerosants have been used for percutaneous sclerosis of vascular malformations. Most of these sclerosing agents act by damaging the vascular endothelium and intima and causing subsequent thrombotic occlusion. Two percent polidocanol (hydroxypolyaethoxydodecan; Aethoxysklerol, Kreussler Pharma, Wiesbaden, Germany) has been safely used for the sclerosis of gastroesophageal varices, endoscopic injection of intestinal vascular malformations, cutaneous hemangiomas, and ectatic veins [1014]. We present the results of our experience with sonographic and fluoroscopic guided direct percutaneous puncture and sclerosis using this agent in pediatric patients with various VMs.

Materials and methods

Between March 2007 and February 2011, a series of 19 patients (8 boys and 11 girls, age 2–17 years, mean age 12.6 years) with VMs on the upper and lower extremities had undergone a total of 89 sessions of intralesional sclerotherapy using polidocanol in our department (Table 1). This study was approved by the Institutional Review Board at our institution (Ethical Committee of Cerrahpasa Medical Faculty) and all the children/parents provided their informed consent before the procedures. The diagnosis of a VM was made on the basis of clinical history, physical and radiological examination. Before the procedure, all patients underwent magnetic resonance imaging (MRI) to evaluate the extent, distribution, and character of the lesion. At the onset of the procedure, the region of the VM was evaluated with both gray-scale and color Doppler sonography.
Table 1

The patients’ characteristics and results of the treatments

Patient no.

Age

Gender

Location

Diameter of lesion (cm)

Number of session

Evaluation/MRI

Evaluation/clinical symptoms

1

16

M

Right thigh

6

5

Disappearance

Complete response

2

17

F

Right knee

6.5

6

Improve/size reduction

Partial response

3

16

F

Left hand

9

7

No change

No change

4

15

F

Left thigh

5.5

4

Improve/size reduction

Partial response

5

14

F

Right hand

8

5

Improve/size reduction

Partial response

6

17

F

Right ankle

12

9

Improve/size reduction

Partial response

7

8

F

Left forearm

10.5

5

Improve/size reduction

Partial response

8

2

M

Right forearm

9

3

Improve/size reduction

Partial response

9

14

F

Right cruris

7

3

No change

No change

10

10

F

Right foot

3.5

2

Disappearance

Complete response

11

9

M

Left cruris

5.5

3

Improve/size reduction

Partial response

12

13

F

Left shoulder

14

6

No change

No change

13

12

M

Right wrist

7

4

No change

Partial response

14

11

M

Left forearm

8.5

5

Improve/size reduction

Partial response

15

15

M

Right arm

5

4

Disappearance

Complete response

16

8

F

Left cruris

6.5

3

Improve/size reduction

Partial response

17

16

M

Left thigh

11

6

No change

Partial response

18

13

M

Right forearm

7.5

4

Improve/size reduction

Partial response

19

13

F

Right hand

7

5

Improve/size reduction

Complete response

The patients who were scheduled to undergo sclerotherapy were admitted to the hospital 1 day before the treatment. All the procedures were performed in an angiographic suite under general anesthesia in order to maintain sedation for facilitating the procedure. Before each injection, the skin was cleaned with 10% povidone iodine solution and draped using all precautions to ensure aseptic conditions. After a sterile field was gained, sonographically guided direct percutaneous puncture of the lesion was performed with a 20-gauge Teflon intravenous cannula (Fig. 1a). A phlebography with water-soluble nonionic contrast medium is performed to visualize the true extension of the malformation, its hemodynamic characteristics, and its relations with the near venous drainage system, to determine how much of the lesion is accessed, and to calculate how much polidocanol is necessary (case 10 in Table 1 and Fig. 1b, c). For peripheral lesions in the extremities, a blood pressure cuff was placed proximal to the most proximal aspect of the lesion and inflated to a pressure of 60 mmHg just before the injection of polidocanol. This compression was performed to occlude venous return and to prevent rapid outflow of the sclerosing agent into the draining veins.
https://static-content.springer.com/image/art%3A10.1007%2Fs00383-011-2962-9/MediaObjects/383_2011_2962_Fig1_HTML.jpg
Fig. 1

a Ultrasonographic appearance of direct percutaneous puncture (arrow) of VM. Phlebography images showing the VM before first (b) and second (c) treatment sessions

For each injection, approximately 1 ml of 2% polidocanol was injected for each centimeter of the diameter of the lesion, with a maximum of 6 ml. Injection of the sclerosant was guided by real-time sonography and fluoroscopy, and then the needle was withdrawn. Two minutes after the injection, the blood pressure cuff was gradually deflated. In cases of multiloculated lesions and large, infiltrative lesions, multiple injections were administered during a single session. Repeated sessions of treatment were conducted into large VMs lesions. Following the procedure, patients were observed for 6 h in the recovery room. After the procedure, the lesion was appropriately compressed with gauze and Tegaderm™ Plus for at least 2 days. Due to lack of cooperation of pediatric patients and complaints about the compression procedure, compression period was shorter when compared with the adult patients. All patients took nonsteroidal antiinflammatory drugs (NSAID) orally for 3–4 days after the treatment. As a follow-up examination in addition to the evaluation of clinical symptoms, MRI was used to evaluate the efficacy of the treatment 1 month after the treatment. If either any lesions or clinical symptoms still remained after the treatment, then the treatment was repeated.

The resolution of the presenting symptoms or at least an 80% decrease in the volume of the lesion was accepted as complete response. Persistence of symptoms or less than an 80% decrease in the volume of the lesion was accepted as partial response. Injections were repeated at monthly intervals in patients who did not show a complete response, clinically acceptable resolution of symptoms, or clinically acceptable reduction of the lesion size. The patients were followed up on a monthly basis up to 2 years with clinical findings and control MRI examinations were carried out 2 months after the last session of treatment.

Results

The patients’ characteristics and results of the treatment are summarized in Table 1. All of the VMs were located in extremities. Clinical symptoms of patients were bulging, pain and cutaneous lesions such as erythema, discoloration. In all of the patients, in the gray-scale and color Doppler sonography which was performed before the procedure, there were no deep venous anomalies associated with the venous malformations. Mean diameter of lesions was 7.8 cm (range 3.5–14). With the increment of diameter, the number of required therapeutic sessions is increased as shown in Table 1. 89 sclerotherapy sessions were technically successful, meaning the lesion could be punctured and polidocanol delivered into the malformation. During the procedures, no major complications were encountered. The minor complications (65%) encountered were swelling and pain after treatment which were resolved by taking NSAID within a few days. No severe or life-threatening side effects were observed.

Two months after the procedure, the efficacy of the treatment was evaluated by MRI and clinical examination. Clinical success was defined as complete or partial resolution of clinical signs and symptoms. After the treatment, the symptoms and associated cutaneous lesions completely resolved in four patients (21%). Clinical symptoms were improved in 12 out of 19 patients (63%). They remained unchanged in three patients (16%). In MRI examination, lesions were disappeared in three patients (16%) (Fig. 2). MRI examination revealed size reduction in 11 out of 19 patients (58%). In five patients (26%), no changes has been observed in the lesions’ size. In our study, there was no relation between clinical outcome and site of the lesion. However, there was a negative relationship between the diameter of the lesion and clinical outcome.
https://static-content.springer.com/image/art%3A10.1007%2Fs00383-011-2962-9/MediaObjects/383_2011_2962_Fig2_HTML.jpg
Fig. 2

Pre- and post-treatment MRI images of VM on the dorsum of the right foot in a 10-year-old girl (Case 10 in Table 1). a T2-weighted image shows a high intensity mass on the dorsum of the right foot. b After sclerotherapy. MRI demonstrates almost complete size reduction of the lesion

Discussion

Clinically VMs are present at birth, but they may not be apparent always and they tend to grow steadily in accordance with the growth of the child, especially during puberty and pregnancy. VMs can occur anywhere in the body but are most frequently seen in the head and neck (40%), extremities (40%), and trunk (20%). Diagnosis of VMs depends on the clinical history and physical examination. In case of a suspicion that deep structures may be involved, especially in extensive lesions, complementary imaging examinations should be performed. The best imaging modality is magnetic resonance imaging. On T2-weighted or inversion-recovery sequences, VMs consist of hyperintense channels or areas containing septation. A pathognomonic feature of VMs is intralesional phleboliths formed because of stagnating blood flow and seen as low signal foci on T2-weighted sequences. T1-weighted postcontrast imaging demonstrates homogeneous or heterogeneous enhancement, and dynamic contrast-enhanced MRI imaging has increased the specificity of VMs diagnosis [15]. On ultrasonographic imaging, VMs can appear as hypoechoic/heterogeneous lesions with anechoic structures visible in <50% of cases. In addition the Doppler flow is generally monophasic low velocity flow, and in some cases flow is only discernible with compression and release of the lesion [16]. In the anatomic characterization of VMs, contrast venography is a helpful tool that can be performed for the treatment planning to confirm the patency of a normal deep venous system, especially in the extremity, and to fully assess the extent of the VM and draining venous channels. Venography is performed once the lesion is accessed at the time of the procedure to determine the volume of contrast needed to fill the malformation before efflux into draining veins. This allows the operator to determine the amount of sclerosant that can be safely injected [16].

Most of VMs are asymptomatic in the early ages. However, in case of accompanying symptoms or cosmetic defects, the lesion should be treated. A surgical excision is thought to be best option for the treatment of VMs. However, this approach is often associated with cosmetic disfigurement and frequently results in incomplete removal of these lesions owing to their infiltrative nature, inaccessibility, or proximity to vital neurovascular structures. Conventional surgical excision is indicated only for very localized, but symptomatic lesions and complete extirpation of more complex VMs is impossible [17]. Therefore, the indications for surgical treatment should be limited. Because of its many benefits, such as less invasiveness and the ability to repeat as often as necessary, sclerotherapy, which can induce a regression of VMs, is now becoming the first choice of treatment for VMs on the body surface [3].

Most commonly used sclerosing agents are polidocanol, ethanolamine oleate and ethanol [15, 18]. Of these, ethanol is thought to be the most effective agent and it achieves a low recurrence rate [18]. But, the major disadvantage of this agent is that severe complications can occur such as pulmonary hypertension due to direct ethanol induced vasospasm of the pulmonary microvasculature, or skin necrosis [15, 17]. Because of these side effects, other sclerosing agents may become valuable options. Ethanolamine oleate induces thrombogenesis as a result of chemical damage to the vascular wall [15, 18]. Polidocanol is a mixture of 5% ethyl alcohol and 95% hydroxypolyethoxydodecane the detergent action of which induces a rapid overhydration of endothelial cells, leading to vascular injury and subsequent fibrosis around the vascular spaces into which it is injected. In addition, because polidocanol has an anesthetic effect, injection of this agent is almost painless.

To our knowledge, most data in the literature are obtained from a few case series that addresses the outcome of percutaneous sclerotherapy of VMs among the pediatric population. In one of these Uehara et al. [18] reported that in 19 treatment sessions of 8 pediatric patients, MRI examination revealed reduction in size of lesions in 11 sessions (58%), and no change in 5 sessions (26%). In the clinical assessment of same patients, they reported significant clinical improvement in 14 sessions (74%). Rimon et al. [19] reported a success rate of 76% (16/21) in their article that includes both adult and pediatric patients. In the literature, reported success rates in percutaneous sclerotherapy of VMs are 74–91% [17, 19]. In our pediatric series, we achieved partial or complete symptomatic relief in 16 of 19 patients (84%) which is similar to the success rates reported in the literature.

Traditionally, absolute ethanol has been the agent of choice for sclerotherapy. It is the most effective sclerosant available, but it also results in the most serious complications, including tissue necrosis, peripheral nerve injury, pulmonary embolism and, unpredictably, pulmonary vasospasm, cardiac arrhythmias, and electromechanical disassociation [20]. During the procedures and follow-up periods, the most common minor complication encountered was swelling and pain which is also similar to the complications of polidocanol reported in the literature. We did not encounter a major complication or recurrence in the follow-up period. Even though our follow-up period is relatively short and VMs can recur months to years after successful sclerotherapy, our results confirm that polidocanol is an effective sclerosant that is associated with minimal pain and complications.

Conclusion

In pediatric patients, sonographically guided percutaneous puncture and fluoroscopically guided sclerosis using 2% polidocanol is effective, less invasive and safe for the treatment of VMs, with a high success rate and minimal complications.

Copyright information

© Springer-Verlag 2011