Rare Diseases Symptoms Automatic Extraction

Bilateral first rib resection and scalenectomy is effective for treatment of thoracic outlet syndrome.

[thoracic outlet syndrome]

Because of the small numbers of thoracic outlet syndrome (TOS) patients treated with bilateral first rib resection and scalenectomy (FRRS), this patient subset has not been well studied. We examined a large cohort of TOS patients who underwent bilateral FRRS to evaluate patient characteristics and outcomes.Patients treated with bilateral FRRS at Johns Hopkins Medical Institutions from 2003 to 2012 were identified by review of a prospectively maintained database. Statistical analysis compared patients with unilateral and bilateral FRRS and bilateral patients with different TOS indications.Fifty-three patients underwent bilateral FRRS with a mean follow-up of 11.4 months. Average time between operations was 17.0 months (range, 5.1-59.8 months). Compared with 408 unilateral FRRS patients, bilateral patients were younger (30 vs 35 years; P = .012), with no significant difference in gender. Among patients with dual-sided FRRS, 25 (47%) had bilateral neurogenic symptoms, 2 (4%) had bilateral arterial symptoms, and 26 (49%) had venous symptoms with the first side due to intermittent compression in 5 (second side: four, intermittent compression; one, neurogenic) and effort thrombosis in 21 (second side: 9, effort thrombosis; 8, intermittent compression; 4, neurogenic). Ten patients had prophylactic FRRS to prevent contralateral venous or arterial thrombosis, and eight had cervical ribs. Compared with neurogenic patients, venous patients were younger (25 vs 35 years; P < .001), with a trend toward more competitive athletes (seven venous vs two neurogenic). Symptomatic restenosis requiring dilation occurred after four FRRS for venous symptoms at a mean of 32.4 months, and rethrombosis occurred after four FRRS at a mean of 4 weeks (one treated with warfarin, three with tissue plasminogen activator), all on the primary side. Overall, 88% of FRRS for symptomatic TOS led to resolved symptoms at last follow-up.Bilateral FRRS is an effective method for treatment of TOS. Venous bilateral patients more often are younger, are competitive athletes, and require close postoperative monitoring for recurrent stenosis and thrombosis.

Diseases presenting "2" symptom

  • 22q11.2 deletion syndrome
  • cushing syndrome
  • epidermolysis bullosa simplex
  • erythropoietic protoporphyria
  • krabbe disease
  • monosomy 21
  • pendred syndrome
  • thoracic outlet syndrome
  • trochlear dysplasia
  • werner syndrome

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