C Maillot1, T Cloche1, J-C Le Huec2,3. 1. Department Ortho Rachis 2, Pellegrin Hospital, Place Amélie Raba Léon, 33076, Bordeaux, France. 2. Department Ortho Rachis 2, Pellegrin Hospital, Place Amélie Raba Léon, 33076, Bordeaux, France. j-c.lehuec@u-bordeaux2.fr. 3. Orthopaedic and Spinal Department and Surgical Research Laboratory, Bordeaux University Hospital, Bordeaux, France. j-c.lehuec@u-bordeaux2.fr.
Abstract
INTRODUCTION: Gorham-Stout syndrome is an aggressive, non-heritable skeletal disease characterized by osteolysis following minor trauma. The primary involvement of the spine is less common (10 %) and has been described in only about 20 cases; there is no consensus about the best way to treat this condition. PURPOSE OF THE STUDY: To report a case of Gorham-Stout syndrome involving the thoracic spine and to review the literature to suggest a post-operative treatment to prevent osteolysis. CASE REPORT: A thirty-year-old female patient was admitted to the unit in March 2013 for a pathologic T4 fracture. X-rays and CT scan revealed the onset of T4 osteolysis and an increase in thoracic kyphosis (the local kyphosis was up to 100°). We performed surgery by posterior approach, combining posterior fixation with screws and rods from T3 to T9, decompression and vertebral osteotomy of 65°. The immediate outcome of surgery was good and the patient returned home after 24 days. At 6 and 12 months of follow-up, the patient was walking normally with no neurological sequelae. CONCLUSION: We report a case of Gorham-Stout syndrome involving the thoracic spine that was successfully treated by interpedicular osteotomy associated with a 6 month follow-up. We suggest that this strategy can provide good results, because after fusion, the disease process remains stable. Because of the lack of cases reported, surgeons must be careful when using multiple treatments, because these treatments have many side effects. LEVEL OF EVIDENCE: Level IV case report.
INTRODUCTION:Gorham-Stout syndrome is an aggressive, non-heritable skeletal disease characterized by osteolysis following minor trauma. The primary involvement of the spine is less common (10 %) and has been described in only about 20 cases; there is no consensus about the best way to treat this condition. PURPOSE OF THE STUDY: To report a case of Gorham-Stout syndrome involving the thoracic spine and to review the literature to suggest a post-operative treatment to prevent osteolysis. CASE REPORT: A thirty-year-old female patient was admitted to the unit in March 2013 for a pathologic T4 fracture. X-rays and CT scan revealed the onset of T4 osteolysis and an increase in thoracic kyphosis (the local kyphosis was up to 100°). We performed surgery by posterior approach, combining posterior fixation with screws and rods from T3 to T9, decompression and vertebral osteotomy of 65°. The immediate outcome of surgery was good and the patient returned home after 24 days. At 6 and 12 months of follow-up, the patient was walking normally with no neurological sequelae. CONCLUSION: We report a case of Gorham-Stout syndrome involving the thoracic spine that was successfully treated by interpedicular osteotomy associated with a 6 month follow-up. We suggest that this strategy can provide good results, because after fusion, the disease process remains stable. Because of the lack of cases reported, surgeons must be careful when using multiple treatments, because these treatments have many side effects. LEVEL OF EVIDENCE: Level IV case report.
Authors: T G P Grunewald; L Damke; M Maschan; U Petrova; O Surianinova; A Esipenko; D Konovalov; U Behrends; J Schiessl; K Wörtler; S Burdach; I von Luettichau Journal: Ann Oncol Date: 2010-07-06 Impact factor: 32.976