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Management of delayed Sprengel malformation with intracanalar compressive omovertebral bone in a developing country's
Mamadou Salia Diarra1,2, Mohamed Elhassimi Cisse2, Aboubacar Sidiki Ndiaye3
1Neurosurgery, USTTB, Bamako, Mali madoucha@hotmail.com.
Insights
Sprengel deformity, a rare congenital scapular issue, can cause significant problems. Surgical intervention for associated omovertebral bone, as seen in a teen with neurological symptoms, led to good recovery and improved mobility.
Area of Science:
- Orthopedics
- Congenital Abnormalities
- Pediatric Surgery
Background:
- Sprengel deformity is a rare congenital condition affecting scapular ascent during fetal development.
- It can lead to cosmetic concerns and functional limitations, often requiring specialized management.
- This study addresses the infrequent diagnosis and surgical treatment of Sprengel deformity in a specific clinical context.
Observation:
- A 16-year-old male presented with Sprengel deformity.
- Associated findings included an intracanalar compressive omovertebral bone, identified via CT scan.
- The patient exhibited neurological deficits, including spastic paraparesis and gait disturbances.
Findings:
- Surgical resection of the omovertebral bone was performed.
- Post-operative physiotherapy was initiated to aid recovery.
- The patient demonstrated significant neurological improvement, including enhanced walking, balance, and restored autonomy.
Implications:
- Sprengel deformity, though rare, necessitates timely and tailored surgical intervention.
- Early management can mitigate functional deficits and improve patient outcomes.
- This case highlights the importance of addressing associated bony anomalies for neurological recovery.
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.
