ReviewChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2020
Surgical management of Chiari malformation type II.
Review in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 2 of them syntheses that pooled it.
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11 citing papers in PubMed, 2 syntheses or guidelines pooled it, 25 citations in OpenAlex.
- Surgical management of Chiari II malformation: a systematic review of literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Pooled it
- Orthopedic management of myelomeningocele with a multidisciplinary approach: a systematic review of the literature.Journal of orthopaedic surgery and research · 2021Pooled it
- Myelomeningocele in Slovenia: An 18-Year National Cohort Study.Diagnostics (Basel, Switzerland) · 2026Article
- Rehabilitation in a child with Chiari II malformation, lumbosacral meningomyelocele, achondroplasia and impaired respiratory regulation - a case report and literature review.Journal of pediatric rehabilitation medicine · 2025Review
- Comprehensive assessment of supratentorial and infratentorial volumes in infants with myelomeningocele with and without Chiari malformation type II.Neuroradiology · 2025Article
- Antenatally diagnosed myelomeningocele with associated chiari ii malformation in the third trimester. A case report.Radiology case reports · 2024Article
- Reflections upon the intrauterine repair of myelomeningocele.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Myelomeningocele: Long-Term Neurosurgical Management.Advances and technical standards in neurosurgery · 2024Review
- Growth patterns and ratios of posterior cranial fossa structures in the Japanese pediatric population: a study utilizing CT scans.Neuroradiology · 2023Article
- Myelomeningocele: the evolution of care over the last 50 years.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Review
- Hundred Pediatric Cases Treated for Chiari Type II Malformation with Hydrocephalus and Myelomeningocele.Asian journal of neurosurgery · 2023Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundChiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients.
methodsWe reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage.
resultsEarly mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence.
conclusionEarly treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial.
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