Management of Catastrophic Proximal Junctional Failure Following Spinal Deformity Correction in an Adult with Osteogenesis Imperfecta: Case Report and Technical Note.

BACKGROUND

Proximal junctional failure (PJF) is a major and sometimes devastating problem following adult spinal deformity (ASD) correction surgery. Common consensus still lags on guidelines for preventing and managing these complications. Surgical treatment of scoliosis in the presence of osteogenesis imperfecta (OI) in the pediatric population is well described. The complication rates are unusually higher in this special subset of patients owing to poor quality of bone. There is a paucity of literature focusing on surgical techniques, strategies, and problems involved in the management of ASD associated with OI.

CASE DESCRIPTION: We report a 59-year-old female with type 1 OI and adult scoliosis who underwent T10-to-pelvis fusion for ASD according to the principles of adult deformity correction. At a 1-year follow-up, she presented with asymptomatic proximal junctional kyphosis of 45° and 2 weeks later had PJF along with spinal cord injury after a fall. On computed tomography scan, kyphosis was increased to 60° at T9-T10. She underwent decompression and revision deformity correction using quadruple rods, with extension of instrumentation to T2 with soft landing using rib bands. At a 4-year follow-up, she had a good functional outcome after revision surgery.

CONCLUSIONS

This is the first report of successful management of PJF following ASD correction in the presence of OI using this technique. Suboptimal hold of implants due to poor bone quality must be at the focus of any surgical planning for these patients. All possible strategies to prevent PJF must be considered when planning the deformity correction in adults with OI.