title: Severe pain related to Klippel-Trénaunay-Weber Syndrome: a case study.
abstract: Klippel-Trénaunay-Weber Syndrome (KTWS) is characterized by a combination of vascular malformations of the skin, abnormalities of the venous and lymphatic systems, and limb enlargement due to bone and soft tissue hypertrophy. The precise etiology is unknown. There is typically no evidence of arteriovenous shunting. Venous malformations are present at birth, are known to progress as the patient grows, and do not regress. Blood flow through these malformations is sluggish, causing ischemia and thrombotic painful events. Nerve blocks are often used to treat pain that is unrelieved by other pharmacologic and nonpharmacologic measures. In this particular case, a stellate ganglion nerve block was the treatment of choice. The stellate ganglion is part of the sympathetic nervous system made up of the inferior cervical and first thoracic ganglia. The nerve block was performed under fluoroscopy by an anesthesiologist board certified in pediatric pain management. The goal of the block was to relieve the patient's pain by calming painful nerve impulses produced by the stellate ganglion and improving blood flow to the area by vasodilating the vessels distal to the site of the block. The goal of this report was to impress upon health care professionals the effectiveness of nerve blocks in improving and sometimes eliminating a patient's pain, as well as the importance of exhausting all options to alleviate/improve pain in our patients. To protect the privacy of this patient and family, the details of the actual case were revised.
authors.affiliation: Children's Medical Center of Dallas, Dallas, Texas 75235, USA. k.dispenza@brhccs.org
authors.forename: Katharyn
authors.initials: K
authors.lastname: Dispenza
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000451:nursing*; D009407:Nerve Block; D010146:Pain / Q000188:drug therapy / Q000209:etiology / Q000451:nursing; D010348:Patient Care Team; D015599:Trauma Severity Indices