3-year-old girl with CLOVES syndrome that especially affected the right lower extremity. There is a leg length discrepancy of about plus 5 cm on the right side due to massive osseous and soft tissue hyperplasia. Typical "ballooned" hyperplasia of the foot with fatty tissue hyperplasia at the dorsum of the foot.
The girl had lost her ability to walk because of pain in the area of the right foot due to repeated thrombophlebitis in the presence of local venous malformation. In addition, lymphorrhea from lymphatic vesicles of the skin in lymphangioma circumscriptum. The preoperative photograph depicts small dark dot-like lymph vesicles on the skin from which lymph is leaking. Complete syndactyly of toes 1 to 4, the little toe is rather hypoplastic.
After interdisciplinary discussion of the case, we recommended to the family amputation of the forefoot in the Chopart joint, as this was the only option for treating the cause of pain, reducing the lymphorrhea and restoring walking function.
Photograph of the whole foot in the operating room before surgery. The left foot is also affected by the syndrome in the form of ballooned hyperplasia, although not as severely. The capillary malformation is also clearly visible as extensive reddening of the skin, not only on the foot but also on the leg.
If performed during early growth age in CLOVES syndrome, pure circumscribed soft tissue resection will most likely lead to even stronger reactivation of tissue overgrowth in the long run. Thus forefoot amputation remained the only option in this rapidly progressive case.
Amputation at the Chopart joint preserves the major weight-bearing portions of the foot (heel and upper ankle) necessary for standing and walking.
A major risk of this therapy was high lymphatic pressure and recurrent lymphorrhea, which could have led to a chronic wound healing problem.
As expected, the patient had persistent lymphatic oozing around the resection edges for several weeks postoperatively. This was treated with customized compression garments.
The girl puts full load on her foot and, in contrast to the situation before surgery, can stand and walk without discomfort.
All images © Kertai