Cystic Lymphatic Malformation with Lymphedema Treated by Lymphaticovenular Anastomosis Combined with Ethanol Sclerotherapy

Abstract

The incidence of cystic lymphatic malformation (CL) in an extremity is very rare. CL can be a cause of lymphedema in a lower limb. The most effective treatment for CL is sclerotherapy or excision; however, these treatments have the potential to cause fibrosis and obliteration of ruptured lymphatic vessels, which impairs lymphatic drainage and increases the risk of lymphedema. Lymphaticovenular anastomosis (LVA) combined with sclerotherapy may be a minimally invasive treatment option for CL in a lower limb. In this report, we describe a patient with CL complicated by lymphedema in a lower extremity, who we treated using LVA and ethanol sclerotherapy with satisfactory results. The patient was a 60-year-old man with a CL measuring 16 cm in diameter in the thigh region above the knee with lower limb lymphedema. The percentage of excess volume of the affected lower leg was 24.7%, preoperatively. Combined treatment using LVA with sclerotherapy was performed under general anesthesia. At 2 years after surgery, the reduction of excess volume of affected lower leg between preoperative and postoperative was 85.4%. In our previous report, the same combination therapy was used to treat CL and prevent lymphedema; however, in the present case, it was used to treat CL and lymphedema at one stage. LVA combined with sclerotherapy is a complementary minimally invasive treatment for CL accompanied by lymphedema.

The incidence of cystic lymphatic malformation (CL), also known as hygroma, has been reported to be 75% of cases occurring in the neck, 20% in the axilla, and the remaining 5% in the mediastinum, retroperitoneal region, pelvis, or groin. CL in an extremity is very rare.¹ CL leads to failure of communication and lymph drainage into the venous system² and can be a cause of lymphedema in a lower limb.³ The most effective treatment for CL is sclerotherapy or excision.⁴ However, these treatments have the potential to cause fibrosis and obliteration of ruptured lymphatic vessels,⁵ which impairs lymphatic drainage and increases the risk of lymphedema.³ Lymphaticovenular anastomosis (LVA) has recently garnered attention in the field of microsurgery as a minimally invasive prophylactic treatment for lymphedema.⁶ In a previous report, we described the efficacy of sclerotherapy combined with LVA for a large CL in the axilla that was not complicated by lymphedema.⁷ LVA combined with sclerotherapy may be a minimally invasive treatment option for CL in a lower limb and in the axilla. In this report, we describe a patient with CL complicated by lymphedema in a lower extremity who we treated using LVA and ethanol sclerotherapy with satisfactory results.

CASE REPORT

Patient

The patient was a 60-year-old man with a 10-year history of right knee swelling and pitting edema in both legs, with Stemmer’s sign in right foot and without Stemmer’s sign in left foot. The magnetic resonance imaging (MRI) on T2-weighted images showed a well-described extraarticular multi lobular cystic malformation above the knee region with a maximum diameter of 16 cm, located on the anterior side of the right femoral bone under the muscle layer, without cortical bone destruction and adhesions to surrounding tissues (Fig. 1). Delayed transit of radiolabeled colloid (>50 min)⁸ was seen on the lymphoscintigram for the ipsilateral side. (See figure 1, Supplemental Digital Content 1, which displays the delayed transit of radiolabeled colloid seen on the lymphoscintigram for the ipsilateral side. https://links.lww.com/PRSGO/C465.)

Fig. 1.: Pretreatment. A, Preoperative images for a 60-year-old man with cystic lymphatic malformation in the right thigh area. Four small yellow triangles indicate the area where the cyst existed. The white line shows the plane of MRI view. B, MRI on T2-weighted images showed a well-described extraarticular multi lobular cystic malformation above the knee region with a maximum diameter of 16 cm, located on the anterior side of the right femoral bone under the muscle layer, without cortical bone destruction and adhesions to surrounding tissues.

The patient had no history of causing edema. Based on these findings, a diagnosis of CL with lower limb lymphedema was made. Compression therapy was started immediately after diagnosis, using a class 1 compression garment (JOBST Opaque1, CCL 1, Thigh High, BSN-JOBST Inc., Conover, N.C.) for both legs.

Circumference perimeters were taken at different levels, before and after surgery with a tape measure. The superior border of the patella and the 10 cm above the superior border of the patella were taken for thigh region. The inferior border of the patella, the 10 cm below the inferior border of the patella, and the ankle were taken for lower leg region. The volumes of thigh or lower leg were calculated separately according to the truncated cone formula to assess lymphedema improvement after surgical treatment without the effect of volume reduction by cyst treatment.⁹

The percentage of excess volume (PEV) was calculated as follows:

PEV = [(volume of affected limb − volume of unaffected contralateral limb)/ volume of unaffected contralateral limb] × 100.

PEV of lower leg and thigh were 24.7% and 19.2%, preoperatively. ICG lymphography revealed a linear pattern along the great saphenous vein. (See figure 2, Supplemental Digital Content 2, which displays the ICG lymphography that revealed a linear pattern along the great saphenous vein. https://links.lww.com/PRSGO/C466.)

Surgical Technique

Combined treatment with LVA and ethanol sclerotherapy was performed under general anesthesia. Ethanol sclerotherapy was performed first after confirming the CL cavity with echography on the spot. All the fluid in the cavity was initially evacuated via a small skin incision. The cyst was then catheterized using a round, 15-Fr, 5-mm diameter Blake silicone drain, (Johnson & Johnson K.K., Tokyo, Japan). Next, absolute ethanol was instilled to fill the cavity and left in situ for 20 minutes. All the fluid was then discharged, and saline was instilled up the cavity. Next, all the fluid was discharged again. Ethanol sclerotherapy was performed using 20 mL of absolute ethanol each time. The procedure was repeated twice, after which the catheter was left in place for drainage.

Soon after ethanol sclerotherapy, 2- to 3-cm skin incisions were made vertically on the lines that marked the linear pattern, and the collecting lymphatics and subcutaneous veins were dissected and identified under a microscope. The veins and lymphatic vessels were anastomosed in an end-to-end manner using 11-0 or 12-0 Nylon suture under a microscope. Four LVAs were established in the right leg and two in the left leg.

Results

The silicone drain was left in place until the daily drainage volume had decreased to less than 50 mL, and removed on postoperative day 7. Compression therapy was resumed immediately after surgery using a cotton bandage. A class 1 compression garment, the same as used preoperation, was resumed after discharge. Compression therapy was stopped 6 months after surgery.

The reduction of excess volume (REV) between preoperative and postoperative was calculated as follows:

REV = ([preoperative PEV - postoperative PEV]/ preoperative PEV) × 100. At 2 years after surgery, REV of affected lower leg and thigh were 85.4% and 44.3% (Fig. 2A). The CL has resolved with no recurrence during follow-up (Fig. 2B).

Fig. 2.: Posttreatment. A, Lymphedema was improved with decreasing volume of lower limbs. The white line shows the plane of MRI view. B, MRI on T2-weighted images showing that the cystic lymphatic malformation resolved with no recurrence during 2 years of follow-up.

DISCUSSION

The REV in the lower leg was higher than the thigh by nearly twice the difference, although including the volume reduction of cyst of in thigh. The result shows that LVA prevents the aggravation of the lymphedema of the lower leg. LVA was also assumed to contribute to decreasing the volume of inflowing into CL, and resulted in no recurrence during the observation period.

There has been a suggestion of a relationship between CL and lymphedema,¹⁰ and CL in a lower limb could cause lymphedema.³ Although the connection between cyst and lymphatics was not detected with ICG, lymphoscintigraphy, or under microscope during operation, our patient had CL in the thigh that was accompanied by lymphedema. Sclerotherapy is an effective option for CL. However, sclerosants cause fibrosis and obliteration of lymphatic vessels, impairing lymphatic drainage.⁵ There was some concern in this case that sclerotherapy alone would worsen the lymphedema. Therefore, a combination of ethanol sclerotherapy and LVAs was performed, and LVAs were kept apart from the area where the sclerotherapy seemed to affect the lymphatics and veins.

In our previous report,⁷ the same combination therapy was used to treat CL and prevent lymphedema; however, in the present case, it was used to treat CL and lymphedema at one stage. LVA combined with sclerotherapy is a complementary minimally invasive treatment for CL accompanied by lymphedema.The excision modality combined with LVA is considered as another option for CL with lymphedema. While the sclerotherapy modality has the advantage of less invasiveness, the surgical modality has advantages of curability and visibility during operation. Curability would be preferred in a reoccurring case, and visibility is preferred in a case where CL is adjacent to important tissues (eg, main nerves), so as to avoid damaging them with sclerotherapy.

CONCLUSION

LVA combined with sclerotherapy is a relatively noninvasive treatment for CL accompanied by lymphedema in the lower limb, as previously established for the axilla.