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[Neurosurgical treatment of spinal dysraphism in children]
1Klinika Chirurgii Dzieciecej, Polsko-Amerykańskiego, Wydziału Lekarskiego, Uniwersytetu Jagiellońskiego w Krakowie.
Insights
Tethered cord syndrome encompasses all pediatric dysraphic conditions, detailing their causes, effects, and outcomes. Surgical strategies for myelomeningocele and other anomalies are explored, alongside considerations for non-operative management.
Area of Science:
- Neurology
- Pediatric Surgery
- Developmental Biology
Context:
- Tethered cord syndrome (TCS) is a complex neurological disorder often associated with various forms of spinal dysraphism in children.
- Understanding the etiology, pathophysiology, and clinical outcomes of TCS is crucial for effective management.
- Prevalent pediatric dysraphic conditions require tailored diagnostic and therapeutic approaches.
Purpose:
- To provide a comprehensive overview of tethered cord syndrome and its associated pediatric dysraphic conditions.
- To detail the surgical management of myelomeningocele, the most common defect within TCS.
- To discuss decision-making regarding non-operative treatment and address challenges in surgical interventions for complex cases.
Summary:
- This review covers the spectrum of tethered cord syndrome, including etiology, pathophysiology, and clinical outcomes.
- It elaborates on myelomeningocele management, Arnold-Chiari malformation type II, hydrocephalus, and sacral agenesis.
- Less frequent conditions like sinus dermal, lipomyelocele, diastematomyelia, and retethered cord syndrome are also presented.
Impact:
- Informs clinical decision-making for pediatric neurosurgical interventions related to spinal dysraphism.
- Highlights the importance of early identification and appropriate surgical treatment for specific TCS subtypes.
- Contributes to improved understanding and management strategies for a range of congenital spinal anomalies.
Abstract:
A tethered cord syndrome includes in its meaning all types of dysraphism in children. The syndrome is described in regard to its etiology, pathophysiology and clinical outcome. Next, the most prevalent types of dysraphism in children are presented. A surgical approach in myelocele--the most frequent defect--is discussed in detail. Reasons for taking a difficult decision not to operate are considered. The most important operative problems are also reviewed. It is reminded that myelocele often coexists with Arnold-Chiari malformation type II and hydrocephalus. Problems in selecting an appropriate surgical approach in this syndrome are shortly discussed. A rare syndrome of sacral agenesis, poorly amenable to surgical intervention, is presented. There are also some less frequent syndromes described, including:-sinus dermalis, potentially life-threatening in the case of recurrent meningitis;-lipomyelocele, needing early identification and prophylactic surgical treatment;-diastematomyelia, in half of the cases requiring surgery due to a bony spur;-retethered cord syndrome, an iatrogenic complication of neurosurgical interventions done on the spinal cord.