ABSTRACT
Patients with osteogenesis imperfecta often present with and are managed for various fractures given the brittle bones associated with this disease. Acetabular fractures are one of the most complicated presentations and management is often strenuous on both the patient and the treating surgeon. There is a lack of evidence on how to approach these patients and not many cases reported in the literature. Open reduction and internal fixation can be successful for these patients given extra care is undergone to protect the patient's increased risk of intra-operative and post-operative complications, and a thorough understanding of the pathophysiology of this disease.
ABSTRACT
Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia (MURCS) association is a rare syndrome. This unique condition consists of Müllerian duct aplasia, cervicothoracic somite dysplasia, and renal aplasia, and skeletal abnormalities manifesting in childhood. We report the case of a 14-year-old girl who presented to the orthopedic clinic with spinal deformity and Sprengel's shoulder complicated by a background of MURCS association. The treatment modalities of scoliosis include posterior spinal fusion and the vertical expandable prosthetic titanium rib. On the other hand, Sprengel's deformity is surgically managed by Woodward's procedure. The management plan for our patient involved correcting scoliosis by the posterior spinal fusion procedure and performing Woodward's procedure to correct Sprengel's deformity simultaneously. Simultaneous scoliosis correction with posterior spinal instrumented fusion and Sprengel's deformity correction with modified Woodward's procedure is a promising surgical technique that can lead to favorable outcomes.
