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[14 cases of communicating syringomyelia associated with Chiari I malformation in children]
G Lena1, Z Boudawara, L Genitori
1Service de Neurochirurgie Pédiatrique, Hôpital des Enfants de La Timone, Marseille.
Insights
This study on pediatric syringomyelia and Chiari I malformation found that surgical decompression improved neurological symptoms in over half of the children. Early diagnosis and intervention are crucial for managing this complex spinal condition.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Medical Imaging
Context:
- Syringomyelia and Chiari I malformation are complex neurological conditions affecting children.
- This review analyzes 21 years of clinical experience with 14 pediatric cases.
- Understanding initial and admission symptoms is key to diagnosis and management.
Purpose:
- To review the clinical experience with pediatric syringomyelia and Chiari I malformation.
- To evaluate diagnostic methods including myelography, CT scans, and MRI.
- To assess the outcomes of surgical interventions for this condition in children.
Summary:
- Initial symptoms included motor weakness, sensory deficits, scoliosis, and bladder dysfunction.
- Admission signs frequently presented with pyramidal signs, motor deficits, spinal deformities, and syringomyelic syndrome.
- Magnetic Resonance Imaging (MRI) was the primary diagnostic tool in recent cases.
- Surgical decompression of Chiari malformation, with or without cyst drainage and hydrocephalus shunting, was performed in most patients.
- Neurological symptoms improved in 57% of cases following surgical intervention.
Impact:
- Highlights the importance of early diagnosis and appropriate surgical management for pediatric syringomyelia and Chiari I malformation.
- Provides insights into the effectiveness of decompression surgery and shunting procedures.
- Contributes to the understanding of clinical presentations and long-term outcomes in affected children.
Abstract:
The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symptoms were motor weakness (57%), sensory signs (50%), scoliosis (50%) and bladder dysfunction (21.5%), while signs of admission were pyramidal signs (78.5%) with motor deficit (43%), spinal deformities (64.2%), syringomyelic syndrome (36%) and sensory deficit (21.5%). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71.4%) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57% of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversies concerning the treatment and concluded with their attitude concerning this pathology in children.