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Monostotic fibrous dysplasia of the thoracic spine RUBEN A. MAENZA (MD), JORGE HOKAMA (MD) and HECTOR R. MALVAREZ (MD) Pediatric Spine Department and Scoliosis Center. Italian Hospital of Buenos Aires. Correspondence: Pediatric Spine Department and Scoliosis Center. Italian Hospital of Buenos Aires. Potosi 4215. Zip code 1199. Buenos Aires. Argentina. Telefax 54 11 4 981 0991. E-mail: [email protected] Abstract Monostotic Fibrous Dysplasia of the thoracic spine is a rare condition. The present case is a 17-years old female patient with involvement of the 11th thoracic vertebrae and believed to be the sixth published case of Monostotic Fibrous Dysplasia of the thoracic spine. Sicot Case-Reports: June 2002 Page 1

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Monostotic fibrous dysplasia of the thoracic spine

RUBEN A. MAENZA (MD), JORGE HOKAMA (MD) and HECTOR R. MALVAREZ(MD)

Pediatric Spine Department and Scoliosis Center. Italian Hospital of Buenos Aires.

Correspondence:Pediatric Spine Department and Scoliosis Center. Italian Hospital of Buenos Aires.Potosi 4215. Zip code 1199. Buenos Aires. Argentina. Telefax 54 11 4 981 0991.

E-mail: [email protected]

AbstractMonostotic Fibrous Dysplasia of the thoracic spine is a rare condition. The presentcase is a 17-years old female patient with involvement of the 11th thoracic vertebraeand believed to be the sixth published case of Monostotic Fibrous Dysplasia of thethoracic spine.

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IntroductionFibrous Dysplasia is a benign process, probably of malformative nature,characterized by the presence of whirling connective tissue with immature laminarbone. It was first described by Lichtenstein in 1938. Later on Lichtenstein and Jaffe,published two different types of Fibrous Dysplasia: monostotic and polyostotic. Thepolyostotic form have been reported to affect the spine frequently whereasmonostotic involvement is rare.

Case-ReportThis 17-years old white female patient, had a history of 3 months of back pain. Herphysical examination revealed only pain in her back at the thoracic area extending tothe thoracolumbar region. The cervical and lumbar spine were clinically intact with anormal range of motion. The neurological status was normal. X-Ray showed a lyticlesion at T 11 without associated spinal deformity. The Tc 99 MDP bone scanshowed an uptake at T 11 level. A Computed Axial Tomography demonstrated anexpanded and lytic lesion with involvement of the vertebral body and posteriorelements specially the left pedicle of the 11th thoracic vertebra (Figure 1) MagneticResonance Imaging (MRI) showed involvement of the spinal canal and expansion ofthe tumor to the T10 foramen with displacement of the spinal cord to the right (Figure2) (Figure 3) A needle biopsy was done twice, obtaining an abundant bleedingmaterial thought to be an Aneurismal Bone Cyst. The first operation was done by theposterior approach in which tumoral resection, spinal canal release andinstrumentation with fusion from T7 to L2 were done. Histopathological examinationof the obtained specimen reached the final diagnosis of a cystic type of FibrousDysplasia (Figure 4) The second operation was done one week later by the anteriorapproach with resection of the vertebral body of T 11 and spinal stabilization andfusion with autologous rib strut grafts. The patient was discharged from the hospital14 days after her first operation with a TLSO brace. She is in now the twelfthpostoperative month, neurologically intact, free of symptoms and doing a normalactive life, without any complication up to the present report.

DiscussionThere are only few reports of monostotic Fibrous Dysplasia with vertebralinvolvement in the literature. Oba et al. published the fifth case of monostotic FD atthe thoracic spine (T10) in a 48 years old woman [5]. Troop and Herring reported acase of monostotic Fibrous Dysplasia in the lumbar spine with involvement of thevertebral body and the posterior elements [7], and Chow et al described anotherpatient with involvement of the transverse process of L4 [2]. Avimadje et al haspublished a case of a 61 years old woman with compromise at L2 [1]. Only 21 casesof the monostotic type affecting the spine have been described. Guille and Bowenpublished 3 cases of polyostotic Fibrous Dysplasia with scoliosis [3]. Two of themunderwent to "in situ" posterior arthrodesis with a good spinal stability and solidfusion at final follow up. However, Janus [4] reported a patient with thoracolumbarscoliosis who developed a pseudoarthrosis after a 6 years follow up. The presentpatient might represent the sixth case published in the literature of the monostotictype of Fibrous Dysplasia of the thoracic spine.

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Legends

Figure 1: A CAT scan showing the involvement of the vertebral body and posterior

elements of T11 with an expanded and lytic lesion which mimics an Aneurysmal

Bone Cyst.

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Figure 2: Weighted T 1 images demonstrating the T 11 lesions and the expansion of

them up to T 10 foramen.

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Figure 3: Weighted T 1 images demonstrating the T 11 lesions and the expansion of

them up to T 10 foramen.

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Figure 4: The histological pattern of Fibrous Dysplasia showing the presence of a

spindle shaped cells proliferation with regular nuclei and verticillate disposition with

calcificated and osteoid trabeculas, fibrohyaline tissue and giants cells areas

surrounding little cysts, reaching to the final diagnosis of a cystic type of Fibrous

Dysplasia.

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References1. Avimadje AM, Goupille P, Zerkak D, Begnard G, Brunais-Besse J, Valat JP (2000)Monostotic fibrous dysplasia of the lumbar spine. Joint Bone Spine 67: 65-70.

2. Chow LT, Griffith J, Chow WH, Kumpta SM (2000) Monostotic fibrous dysplasia ofthe spine: report of a case involving the lumbar transverse process and review of theliterature. Acta Orthop Trauma Surg 120: 460-4.

3. Guille JT, Bowen JR (1995) Scoliosis and fibrous dysplasia of the spine. Spine 20:248-51.

4. Janus GJ, Engelbert RH, Prujis JE (1998) Instrumentation for correction andfixation for scoliosis in fibrous dysplasia of the thoracolumar spine. Eur Spine J 7:260-2.

5. Oba M, Nakagami W, Maeda M, Kobayashi K (1998) Symptomatic monostoticfibrous dysplasia of the thoracic spine. Spine 23: 741-3.

6. Troop JK, Herring JA (1988) Monostotic fibrous dysplasia of the lumbar spine:case report and review of the literature. J Pediatr Orthop 8: 599-601.

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