Management of delayed Sprengel malformation with intracanalar compressive omovertebral bone in a developing country's neurosurgery unit

Abstract

Sprengel deformity is a congenital abnormality done to the ascent of the scapular bone (which occurs probably between the 5th and 12th weeks of gestation). It can cause cosmetic or functional problems. The aim of our study was to describe this rare deformity, often neglected in our context, and its surgical management. We report a case of a 16-year-old boy with a Sprengel deformity associated with omovertebral intracanalar compressive bone seen on the CT scan. He reported neurological symptoms such as spastic paraparesis and walk disturbances. The bone was resected surgically and physiotherapy was set up. The outcome was good with noticeable neurological improvement of walk and balance, recovery of autonomy. Sprengel deformity is a rare pathology. Surgery must be done early and be adapted to the case.

Keywords: congenital disorders; head and neck surgery; musculoskeletal syndromes; neurosurgery; spinal cord.

Figure 1

CT scan showing a 90% canalar stenosis (A, B) and a three-dimensional reconstruction showing the left omovertebral bone (arrow) passing through the spina bifida (C).

Figure 2

Postoperative wound (A) and good canal decompression (B, C).