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Complex Neurogenic and Venous Thoracic Outlet Syndrome Secondary to Fibrous Dysplasia: A Unique Operative Challenge
Bongseok Jung, Guillermo Escobar
Emory University, Atlanta, GA
INTRODUCTION: Thoracic outlet syndrome (TOS) from a tumor of the first rib is exceedingly rare, and those from fibrous dysplasia (FD) are even more uncommon. Only eight cases of TOS from FD have been reported in the past century, and none of them used a combined supra- and infra-clavicular approach. Due to the thickened, irregular, and sclerotic nature of the bone, along with ossification causing limited surgical exposure, FD can present as a complicated high-risk challenge for the surgeon. We present the first reported case of neurogenic and venous TOS from FD that was treated using a paramedian approach for first rib resection and neurolysis of the brachial plexus.
METHODS: 53-year-old woman with a longstanding history of fibrous dysplasia presented with a 4-year history of progressively worsened right upper extremity weakness, pain, swelling, purple patches on her elbows, and numbness. Any activity using her right arm caused severe lifestyle limiting pain radiating from her neck into her chest, shoulder, and hand. Many physicians deferred interventions over the years due to the high-risk nature of the patient's presentation. CT showed extensive venous collaterals in the right neck, stenosis of the right subclavian vein, and wide thickening of the first rib posteriorly at the junction of the sternum, suggesting concomitant venous and neurogenic TOS.
RESULTS: An anterior first rib resection was planned to decompress the subclavian vein, decompress the spinal plexus of veins to facilitate a potential posterior approach, and address perineural entrapment. After consultation with neurosurgery for a possible posterior approach should the anterior approach prove insufficient, an infraclavicular incision was made. The lateral and medial aspect of the rib was carefully dissected, which extended dramatically further than a normal rib. Multiple irregular bony prominences and hyperossifications were encountered, particularly medially where the rib was unable to be freed up from the inferior approach. A supraclavicular incision was made, exposing the brachial plexus and subclavian artery beneath the scalene fat pad. Careful dissection allowed exposure of the rib from both approaches, which revealed an immensely wide, irregular, and brittle first rib. The rib was mobilized, although areas of hyperostosis limited complete mobilization. The rib was ultimately amputated, leaving only a small, soft inferior crest to avoid pleural injury. Hemostasis was excellent with no evidence of pleural injury. Complete mobility of the brachial plexus was confirmed, and the subclavian vein was decompressed well. The total operation time was 5.5 hours. The patient recovered well with marked improvement in sensorimotor pain and was discharged POD2. Patient continued to demonstrate significant improvement of symptoms 1-month postoperatively.
CONCLUSIONS: TOS secondary to FD is exceedingly rare and can present unique challenges. This was the first reported case of TOS from FD that was operated via a paramedian approach. Although technically demanding, this approach may serve especially valuable in select cases of complex TOS with extensive hyperostosis and neurovascular involvement.
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