Persistent torticollis revealing Sprengel syndrome: A case report and literature review

Abstract

Sprengel’s deformity is a rare condition, due to the congenital failure of the descent of the scapula. We report the case of an eleven (11) year old patient presenting a permanent flexion attitude of the cervical spine with posterior thoracic swelling in the inner edge of the left scapula and shoulder asymmetry, evolving from early childhood. The limitation of the left shoulder movements and aesthetic discomfort motivated the consultation in the pediatric orthopedics service, which referred her to us for CT scan and MRI imaging exploration. The CT scan noted a seventh thoracic vertebra (C7) posterior arch hypertrophy, of which cortical is in continuity with a costiform bony outgrowth articulating with the superioro-internal angle of the left scapula which is elongated, corresponding to an omovertebral bone. It also noted a vertebral block from C5 to D2 with defective closure of the posterior arches of C2, C5 to C7 and D3 to D5 and a costal bifidity of the transverse process of D1, without anomaly of the spinal cord to the MRI. Springel’s deformity can be suspected on antenatal ultrasound in the presence of asymmetric thickening of the nuchal soft tissues. In postnatal period, a radiological assessment including the standard radiography and especially the CT scan with its multiplanar reconstructions and the possibility that the MRI to analyze the paravertebral soft tissues and the spinal cord, draw up the diagnosis and analysize of the associated anomalies. Keywords: deformity; sprengel; congenital; CT scan; MRI.