Progressive infiltrative destruction of subcutaneous and periosteum soft tissues affect posterior spinal surgery for primary Gorham-Stout disease: a case report

Background : Gorham-Stout disease (GSD) with involvement of the spine is extremely rare, the course of disease is hard to predict. The progressive osteolysis would result in kyphosis, kyphoscoliosis, subluxation or even dislocation of the spine. There is also destructive infiltration of adjacent and periosteum soft tissue, which may potentially raises difficulties to posterior spinal procedure. This adverse affection is hard to evaluate from routine imageological examinations. Case presentation : We surgically managed a 29-year-old female patient with primary GSD which involved lumbar spine and caused regional kyphoscoliotic deformity since 2018. We initially performed posterior short-segmental fixation to correct the regional deformity. Twenty five months after the first spinal surgery, the fixation was failed and the deformity was progressed. During the last spinal revision surgery, we observed diffusive hemangiectasis of periosteum and adjacent soft tissues. Due to the massive hemorrhage, we simplified the procedure by performing unilateral fixation and lateral fusion. The patient was followed for 27 months. Fortunately, owing to the reliable bony fusion and spontaneous arrest of osteolysis, we observed satisfactory clinical results, the deformity remained unchanged. Conclusion : In primary GSD patient with involvement of multiple vertebrae, the affection of infiltrative destruction of periosteum and adjacent soft tissue on posterior spinal surgery is underestimated. Huge hemorrhage would cause big trouble during the procedure, surgeon should be aware of this phenomenon. Scrutinizing the preoperative images may potentially assist the surgeons to predict this phenomenon. Advanced examinations should be considered to avoid embarrassing situation when this affection is unidentifiable.