J Korean Soc Radiol.  2016 Jan;74(1):26-36. 10.3348/jksr.2016.74.1.26.

Infusion Sclerotherapy of Microcystic Lymphatic Malformation: Clinico-Radiological Mid-Term Results

• Lee J
• Lee SJ
• Chung HY
• Huh S
• Kim HK

• KMID: 2150460
• DOI: http://doi.org/10.3348/jksr.2016.74.1.26

Abstract

PURPOSE
A new sclerotherapy technique by slow mechanical infusion of sclerosant was validated for treatment of microcystic lymphatic malformation (mLM).
MATERIALS AND METHODS
Seventeen consecutive patients with mLM in extremities, cervicofacial area, and trunk were included (21.8 +/- 21.5 years old, male:female = 5:12). All patients diagnosed as mLM were included. A total 4-32 mL 20-38% OK-432 solution was mechanically infused at the rate of 10 mL/hour into the mLM lesions. The treatment effect was estimated clinic-radiologically at the 4-month follow-up. Repeated sclerotherapy followed in the 6th month, if required. The therapeutic effect was evaluated using quantitative ultrasonographic examination including soft tissue thickness, cyst size and number.
RESULTS
In 17 patients, total 31 infusion sclerotherapy sessions were performed and monitored for 425 +/- 266 days. Fifteen patients (88%) showed improvement in all symptoms, signs, and ultrasonographic findings. In all cases, at least one finding presented improvement. The maximal number of cysts per ultrasonographic window and maximal diameter of the largest cyst decreased by 57 +/- 57% and 51 +/- 67%, respectively (p = 0.102, 0.004). The soft tissue thickness decreased by 18 +/- 15% (p < 0.01). No significant complications such as distal lymphedema or skin necrosis occurred.
CONCLUSION
Infusion sclerotherapy is a safe and effective treatment technique for microcystic LM, with improved outcome.

MeSH Terms

Extremities
Follow-Up Studies
Humans
Infusions, Intralesional
Lymphatic Abnormalities
Lymphedema
Necrosis
Picibanil
Sclerotherapy*
Skin
Ultrasonography
Picibanil

Figure

• 

  Fig. 1 A 2-year-old girl (patient number 13 in Tables 1, 2) who presented with soft tissue swelling at left knee area was diagnosed as microcystic lymphatic malformation. A. Initial ultrasonography demonstrates infiltrative lesion containing multiple small cysts in subcutaneous layer of medial aspect of left knee joint. Soft tissue thickness is 16.2 mm. B. Post-sclerotherapy 20-month follow-up ultrasonography shows markedly improved lesion with near disappearance of cysts. Soft tissue thickness was reduced to 11.3 mm. FC = femoral condyle, MCL = medial collateral ligament, TC = tibial condyle

• 

  Fig. 2 A 17-year-old man (patient number 15 in Tables 1, 2) presented a soft tissue mass with vague pain and tenderness in the left temporal area. Also, the patient complained of typical tiny dermal vesicles with intermittent fluid discharge. A poorly-defined non-compressible soft tissue mass was palpated. Clinical impression was agreed as lymphatic malformation. A. The initial ultrasonography reveals an infiltrative soft tissue lesion with multiple non-uniform small cystic and channel-like components (arrows) in subcutaneous fat layer of the left temporal area. B. At the early stage of infusion sclerotherapy, microcystic components are filled by sclerosant containing OK-432 and lipophilic contrast media. The infusion was done by an electromechanical infusion pump at the speed of 10 mL/hr. C. In 2 minutes after infusion start, the sclerosant infiltrates into adjacent microcysts and channels (arrows). D. The second lesion area was punctured and sclerosant was infused in parallel. In 21 minutes after initial infusion start, the lower lesion is fully filled by sclerosant with spill to adjacent normal-looking lymphatic channels (arrowheads). The newer upper lesion shows numerous microcysts interconnected by small channels (arrows). E. In 29 minutes, more microcysts in further distance are filled by sclerosant (arrow). F. In 50 minutes of infusion, no newly filled microcyst is noted and adjacent normal-looking small lymphatic channels are opacified (arrow). In lower lesion, opacification of normal lymphatic vessel is not progressed due to infusion stop (arrowhead). G. Ultrasonography was performed in 1 year after the infusion sclerotherapy. In same scope and plane as the initial image (A), cystic and channel lesions (arrows) show decreased sizes. Also, clinical symptom and sign are improved markedly.

• 

  Fig. 3 Infusion sclerotherapy presented an improvement of symptoms, signs, and image findings in 16, 16, and 16 cases among 17 patients, respectively.

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