Gorham-Stout disease: A multirod lumbar reconstruction with off-label suppression-remission therapy.

Autor: Krishnan, Ajay, Raj, Aditya, Degulmadi, Devanand, Mayi, Shivanand, Rai, Raviranjan, Bali, Shiv Kumar, Parmar, Vatsal, Amin, Prarthan Chirag, Krishnan, Preety, Dave, Mirant, Dave, Bharat
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Zdroj: Surgical Neurology International; 4/8/2022, Vol. 13, p1-5, 5p
Abstrakt: Background: Gorham-Stout disease (GSD), a fibro-lymphovascular entity in which tissue replaces the bone leading to massive osteolysis and its sequelae, rarely leads to spinal deformity/instability and neurological deficits. Here, we report a 12-year-old female who was diagnosed and treated for GSD. Case Description: A 12-year-old female presented with back pain, and the inability to walk, sit, or stand attributed to three MR/CT documented L2-L4 lumbar vertebral collapses. Closed biopsies were negative. However, an open biopsy diagnosed GSD. She underwent a dorsal-lumbar-to-pelvis fusion (i.e., T5-T12 through L5/S1/S2) using multilevel pedicle screw/rod stabilization and human leukocyte antigens (HLAs) matched allograft (i.e. from her father). Postoperatively, she was treated with "off-label" teriparatide injections, bisphosphonates, and sirolimus. Four years later, while continuing the bisphosphonate therapy, she remained stable. Conclusion: Surgical multirod stabilization from T5 to S2, supplemented with HLA compatible allograft, and multiple medical "off-label" therapies (i.e., teriparatide, sirolimus, and bisphosphonates) led to a good 4-year outcome in a 12-year-old female with GSD. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index