The outcome and countermeasure of posterior correction surgery for severe kyphoscoliosis secondary to spinal Gorham disease / 中华骨科杂志

ABSTRACT

Objective:To investigate the outcome of posterior correction surgery for severe kyphoscoliosis secondary to spinal Gorham disease, further to explore the countermeasure in such complicated condition.Methods:From January 2005 to December 2019, a total of 12 consecutive patients were diagnosed with spinal Gorham disease. Four patients who had undergone correction surgery were reviewed retrospectively. There were 3 males and 1 female. The median age of surgery was 14.5 years (11.5 years, 27.5 years), with the median of Cobb angle of scoliosis and kyphosis 29° (21.5°, 78.0°) and 94° (78.0°, 103.0°), respectively. After Halo-gravity traction, one-stage posterior correction surgeryand Schwab grade I or II osteotomy, with pedicle screw fixation bridging the diseased vertebrae was performed. Drug therapy of bisphosphonate was recommended after surgery. The Cobb angle of scoliosis and kyphosis, coronal and sagittal balance were measured on the standing upright radiographs of the spine. CT and MRI were used to give precise evaluation of spinal and peripheral soft tissue involvement.Results:After Halo-gravity traction of 3 months (2.5 months, 3.5 months), the median of Cobb angle of scoliosis decreased to 23.5° (15.5°, 77.0°) and kyphosis decreased to 65° (57°, 83.5°) respectively. Two patients underwent facetectomyand 2 received Ponte osteotomy. The median operative time and blood loss were 5.5 h (5.1 h, 5.9 h) and 3 095ml (2 950 ml, 3 320 ml), with the fusion segment of 13.5 (12.5, 14.5) and the fixation density of 47.8% (40.9%, 57.3%). After surgery, the median of Cobb angle of scoliosis and kyphosis decreased to 18° (10.5°, 38.5°) and 59° (42.0°, 78.0°). Compared to the values before traction, the median of correction rates of scoliosis and kyphosis after surgery were 46.7% (33.1%, 59.5%) and 35% (12.3%, 51.1%) respectively. Moreover, the median of coronal balance decreased from 15.5 mm (9.0 mm, 21.0 mm) to 6.5 mm (4.0 mm, 9.0 mm), while the median of sagittal balance decreased from 14 mm (-18.0 mm, 27.5 mm) to 5.5 mm (-5.5 mm, 12.5 mm). During a median of follow-up of 2.8 years (2.0 years, 3.5 years), no complication was detected except one patient whounderwent revision surgery for rod broken.Conclusion:One-stage posterior correction surgery combined with preoperative halo-gravity tractionand postoperative anti-osteoporosis therapydemonstratedto be safe and effective for severe kyphoscoliosis secondary to spinal Gorham disease. More attention should be paid to the failure of internal fixation after surgery.