Fibrous Dysplasia

uveřejněno: 08. 11. 2010

In casuistry of 56-year-old patient with multicentic fibrous dysplasia of 5th and 6th rib the authors demonstrate healing procedures conducted by thoracic surgeon and neurosurgeon. A tumour compressed the right lung and caused breathlessness by creating pleural effusions. A part of the tumour grew into the spinal canal and compressed the spinal cord. The patient had been examined at various departments; a surgical solution was suggested after a spastic paraparesis of lower limbs emerged. In the acute phase during laminectomy the spinal cord compression was released. In the second phase the tumour was extirpated from thoracic wall, with resection of affected parts of ribs and thoracic vertebrae. In the third phase the spine was stabilized with thoracic wall plastic surgery. The tumour of size 60×110×80 mm was 1200 g in weight. A large resection area was a source of difficult-to-stop bleeding; after application of local haemostat Traumastem TAF the blood loss significantly reduced. The result entitles us to recommend a surgical treatment also in case of enormous, benignant tumours in cooperation with other surgical fields

Fibrous Dysplasia of Ribs and Spine – Multiple Fields Solution

P. Habal, K. Kaltofen+ Charles University in Prague, Cardiac Surgery Clinic, +Neurosurgical Clinic of University hospital and University hospital in Hradec Králové (study supported by the Research Assignement MZO 00179906)

Summary In casuistry of 56-year-old patient with multicentic fibrous dysplasia of 5th and 6th rib the authors demonstrate healing procedures conducted by thoracic surgeon and neurosurgeon. A tumour compressed the right lung and caused breathlessness by creating pleural effusions. A part of the tumour grew into the spinal canal and compressed the spinal cord. The patient had been examined at various departments; a surgical solution was suggested after a spastic paraparesis of lower limbs emerged. In the acute phase during laminectomy the spinal cord compression was released. In the second phase the tumour was extirpated from thoracic wall, with resection of affected parts of ribs and thoracic vertebrae. In the third phase the spine was stabilized with thoracic wall plastic surgery. The tumour of size 60×110×80 mm was 1200 g in weight. A large resection area was a source of difficult-to-stop bleeding; after application of local haemostat Traumastem TAF the blood loss significantly reduced. The result entitles us to recommend a surgical treatment also in case of enormous, benignant tumours in cooperation with other surgical fields.

Key words: thoracic wall tumours – benignant fibrous dysplasia – Traumastem TAF

Introduction In comparison with other benignant tumours, mainly long bones tumours, primary benignant tumours of thoracic skeleton occur rarely. They represent only about 7–8% of all benignant bone tumours (1). One of them is a benignant fibrous dysplasia, emerging as abnormality during a development and differentiation of mesenchymal osteoblasts (2) Most often this abnormality is localised in skeleton long bones (3). The surgical treatment is mostly indicated in younger age as various deformities or pathologic fractures of long bones develop during the organism grow (4) The benignant fibrous dysplasia with localisation into the thoracic skeleton bones occurs most often in the ribs, but represents only around 4% of all primary benignant thoracic tumours (5).

Casuistry 56-years- old man working as tinsmith until disability pension, has been complaining about back-ache for minimally 15 years. He assigned the progression of pain to post-traumatic complications. 5 years ago he fell and suffered ribs multiple fracture with pneumothorax emergence. But during last three years, the pain and progression of palpable resistance under the shoulder blade angle on the right were joined by relapsing effusions, for which the patient was repeatedly punctured. The gradually increasing resistance under the shoulder blade angle on the right he assigned to poorly healed rib fractures. The patient was hospitalised at surgical department where he underwent repeated diagnostic surgical performances. Neither histological finding was definite at the beginning; the primary diagnosis tended to sarcoma. Only histology, evaluated at a different workplace, diagnosed the fibrous dysplasia. During the last year the patient had difficulties with resting dyspnoea by progression of massive relapsing effusions with a complete compression of the whole right lung. (Pic.1)

Therefore the patient was repeatedly hospitalised at pulmonary ward, where a bronchoscopy was performed. Due to the spinal compression the patient developed hypotonia and hypotrophy of both lower limbs musculature, for which he became immobile and consequently was pensioned for disability. The progression of the finding known from CT examination, where the canalicular stenosis on level of ninth (Th9) and tenth (Th10) thoracic vertebrae with a flattening thoracic part of the spinal cord to 8 mm was described, caused the lower limbs spastic paraparesis of central type with development of incomplete spinal cord lesion syndrome. The patient was urgently operated and by back approach the spinal cord decompression from laminectomy Th9 and Th10 with partial excision of significantly bleeding tumour was performed. (Pic.2)

After the patient recovered in 10 days, we resected parts of 7th – 9th ribs from posterolateral thoracotomy, affected by tumour masses and we excised the tumour in several portions. We also resected an affected capitulum of relevant ribs and we released Th9 and Th10 vertebrae bodies from tumorous masses. We also extirpated Tumorous masses from the dorsal mediastinum area. This stage of operation was complicated by the expected blood loss around 2000 ml. On assumption of further blood loss in postoperative period from large resection area of thoracic wall and thoracic spine area, we withdrew from stabilization operation of spine and plastic surgery of thoracic wall at the same time. In this phase of operation the tumour in three portions of total weight 1750g was extirpated, the biggest portion of size 110 mm x 80 mm x 60 mm and 1200g in weight. With very good effect we managed to influence blood losses by applying a local haemostat Traumastem TAF reticulum on the large wound area of thoracic vertebrae and pulmonary parenchyma. (Pic.4)

The final histological result describes a well-surrounded proliferation consisted of fascicular cells with spindle-cell stroma without atypicality or mitotic activity. The section contains also cavities without own epithelium creating incipient bone cysts. It is a benignant form of tumour; the finding’s rarity is its multicentric positioning in ribs skeleton and vertebrae bodies. After the large operation the patient well recovered and after 10 days we approach the third phase, when a corporectomy of TH9 and Th10 thoracic vertebrae bodies with removing of intervertebral discs Th8/9 and Th10/11 were performed. Three Kirschner wires were inserted into space of extracted bodies and the place after corporectomy was filled by palacost. The segment Th 8–11 was anterolaterally fixed with TSLP splint. The performance was finished with plastic surgery of thoracic wall using a muscular lobe, consisted partly from fascicles musculus serratus anterior a musculus latissimus dorsi without inserted artificial material. The patient rehabilitated well in three-point corset. Ha was able to walk with forearm crutches and on 14th postoperative days he was transferred to a rehabilitation centre.

Discussion By sources, the fibrous dysplasia of ribs can transform into cystic degeneration during growth and may even lead to a forming of aneurysm cysts (6), which in our case were histologically proved. The histological diagnosis might be difficult mainly when examination biopsy samples the dysplasia can be morphologically similar to a sarcoma (7). This also could have been the reason for the original misdiagnosis. In most cases this disease is benignant; malignant transformation is less than 1% of all known cases (8). Within a long-standing growth and monstrous size the compression of surrounding structures is very risky. For example, there are described sporadic cases of the thoracic outlet syndrome occurrence with affection of the first rib (9)

A radical surgical treatment has certain difficulties connected mainly with expected big blood loss. The reason why we chose this procedure was that the first operation performance was in the acute phase with lower limbs paraparesis in progress. The second, the most risky phase went under the expected blood loss as the radical extirpation of a tumour. Since the postoperative biopsy confirmed a benignant character of the disease, we could afford to postpone the third, stabilization and thoracoplastic performance. It is possible to object that blood losses could have been recuperated by using the cell-saver, but despite the process benignity, we do not use recuperated blood in similar cases. With very good effect we used a local haemostat Traumastem TAF in form of reticulum (Pic.3). We routinely use this material in thoracic surgery, where massive blood loss from a large wound area connected with capillary bleeding are expected. Thanks to its ability to accelerate biochemical processes this material significantly helps with a healing process. It is absorbed within three days depending on level of secretion from surrounding tissues. It is eliminated from the organism within 21 days without any side effect and does not call any allergic reaction.

Conclusion The rarity of this disease is evidenced by certain groups of patients, for example from the register of more than 2000 cases of orthopaedy and pathology department at St. James´s University Hospital Leeds, where only 32 cases localised was into the bones of thoracic skeleton, most often in ribs area. Neither localisation into thoracic vertebrae bodies nor the multicentric positioning in the thoracic skeleton bodies has been described (10). Also a clinical manifestation immediately after an urgent neurosurgical performance was rare; the spastic paraparesis of lower limbs caused by this disease has not been mentioned through available sources. We assume that a surgical treatment of the disease before side effects develop and the tumour is small should be preferred to surgical intervention performed just for the purpose of diagnosing. Our correct procedure is based not only on experiences and the good result of this case, but is also supported with sporadic literary data of rare cases treatment (11). An involvement of various surgical fields’ specialists in causal treatment is an indisputable advantage. Also new haemostatic materials participate in successful results of operation. Thanks to its properties and incomparable economic levels, Traumastem TAF surpasses materials used in the past.

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Gallery

Pic. n. 1 CT of fibrous dysplasia of 8th and 9th thoracic vertebra
Pic. n. 2aSpinal cord decompression from laminectomy
Pic. n. 2b
Pic. n. 3 - The scar after spine decompression
Pic. n. 4 - The tumor and the thoracic wall extirpation
Pic. n. 5
Pic. n. 6a
Pic. n. 6b - Th8 - Th11 segments stabilisation by TSCP splint
TPic. n. 7 - Traumastem TAF application
Pic. n. 8 - Patient 3 months after operation
Pic. n. 9
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