Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case repor
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CASE REPORT - SPINE - OTHER
Kyphotic deformity of the lumbar spine due to a monostotic fibrous dysplasia of the second lumbar vertebra: a case report and its surgical management Anna Stocsits 1
&
Sara Lener 1 & Pierre Pascal Girod 1 & Anto Abramovič 1 & Claudius Thomé 1 & Sebastian Hartmann 1
Received: 7 July 2020 / Accepted: 10 August 2020 # The Author(s) 2020
Abstract Monostotic fibrous dysplasia (MFD) of the lumbar spine represents an exceedingly rare lesion. A 26-year-old patient presented with a progressive osteolytic lesion of the vertebral body L2 and the diagnosis of MFD. A minimally invasive left-sided eXtreme Lateral Interbody Fusion (XLIF) approach with resection of the vertebral body L2 with placement of a mesh cage was performed. No complications were observed perioperatively and the symptoms rapidly improved. Minimally invasive piecemeal resection with a combined dorsolateral approach showed a favorable clinical and radiological outcome and seems to be a safe and reliable technique for MFD. Keywords Monostotic fibrous dysplasia . Deformity . Lumbar spine . Minimally invasive
Background Monostotic fibrous dysplasia (MFD) of the spine represents a rare disease entity of the lumbar spine [3, 5, 6, 19]. In 1938, fibrous dysplasia (FD) was first characterized as a rare developmental disorder by Lichtenstein et al. [11]. In 1942, Lichtenstein and Jaffe described single (monostotic) or multiple (polyostotic) bone involvement [10]. Nowadays, FD is known as an uncommon mosaic disease, in which normal bone gets replaced by fibro-osseous tissue. This bony remodification leads to a weakened osseous matrix prone to fractures and deformity with corresponding pain and functional impairment [8]. FD represents 5 to 7% of benign bone tumors [4]. The etiology of the disease has been linked to activating mutations of the GNAS gene that encodes the α subunit of stimulatory G protein (Gsα) located at 20q13.213.3 [19]. The monostotic form of the disease is much more common (80%) than the polyostotic form (20%) [4]. Extraosseous involvement of polyostotic fibrous dysplasia (PFD) This article is part of the Topical Collection on Spine - Other * Anna Stocsits [email protected] 1
Department of Neurosurgery, Medical University Innsbruck, Anichstrasse 35, A-6020 Innsbruck, Austria
including the skin (abnormal skin pigmentation) with accompanied precocious puberty, hyperthyroidism, and other extraskeletal abnormalities presenting as the so called McCune– Albright syndrome was described [8]. MFD can affect every part of the skeleton, but is commonly found in jaw bones, ribs, femur, and tibia [14]. Spinal manifestation is rare, but more likely to be observed in the polyostotic form (7 to 24%) than in MFD (1 to 5%) [3, 17]. Especially lumbar spine involvement is extremely rare [1, 3, 6, 9, 12, 15, 19]. We represent the second case of MFD of the second lumbar vertebra and its surgical management.
Case report History and presentation In November 2018, a 26-year-old young man with deep-seated pain in the upper lum
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