Severe Pain and Edema due to a Widespread Lymphangioma: Disappearance of Symptoms and Reduction of Lesion with Spinal Cord Stimulation

Background The efficacy of epidural spinal cord stimulation on chronic neuropathic pain due to failed back surgery syndrome or nerve root lesions is well reported. There is even literature reporting the effects of spinal cord stimulation in controlling peripheral vascular lesions as in peripheral arteriopathies or diabetic neuropathies and in complex regional pain syndrome type II. This is probably due to an effect of epidural spinal cord stimulation, mainly on the parasympathetic nervous system. Case Description A 14- year-old boy affected, since birth, by a quickly growing widespread lymphangioma at the pelvis and right thigh, underwent repetitive surgical procedures to try to reduce its extension. Recurrence always occurred. Due to a massive swelling of his right lower limb and a wide painful area all over the pelvis and right lower limb, the patient lost his autonomy and was almost bedridden or wheelchaired. On January 2015 the patient underwent the implant of a low dorsal epidural 8-lead magnetic resonance imaging (MRI)-compatible electrode connected to an MRI-compatible stimulator. The patient, 1 month after the implantation, had a complete recover of his autonomy, with a marked shrinkage of his right lower limb and a near disappearance of pain. He resumed the activity of the daily living. One year later, a hip MRI showed partial reduction of the lesion. The patient is still nearly free from pain. Conclusions This is the first case report of severe chronic pain syndrome due to a widespread lymphangioma successfully treated by means of epidural spinal cord stimulation.