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Hundred Pediatric Cases Treated for Chiari Type II Malformation with Hydrocephalus and Myelomeningocele
Daniel Encarnacion1, Gennady Chmutin1, Bipin Chaurasia2
1Department of Neurosurgery, Morozovskaya Children City Clinic Hospital, Moscow, Russia.
Insights
Chiari malformation type II (CM-II) management is complex, especially in neonates. This study found craniocervical junction decompression effective for CM-II with hydrocephalus and myelomeningocele, with better outcomes in older patients.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Developmental Biology
Background:
- Chiari malformation type II (CM-II) presents management challenges, particularly in neonates with poor prognoses.
- Optimal surgical strategies, including shunting versus craniocervical junction (CVJ) decompression, remain debated.
- This study retrospectively analyzes 100 patients treated for CM-II, hydrocephalus, and myelomeningocele.
Purpose of the Study:
- To evaluate the outcomes of surgical intervention for Chiari malformation type II (CM-II) in patients with hydrocephalus and myelomeningocele.
- To identify factors influencing treatment success and prognosis in this patient cohort.
- To compare the effectiveness of craniocervical junction (CVJ) decompression as an initial surgical approach.
Main Methods:
- Retrospective review of 100 pediatric patients surgically treated for CM-II, hydrocephalus, and myelomeningocele.
- Surgical timing was individualized based on clinical presentation, with urgent surgery for compromised patients and elective for less severe cases.
- All patients initially underwent craniocervical junction (CVJ) decompression.
Main Results:
- The average herniation in CM-II patients was 11.2 mm, but herniation level did not correlate with clinical findings.
- Syringomyelia was present in 60% of patients, with more severe spinal deformity correlating with widespread syringomyelia (p=0.04).
- Younger patients exhibited more frequent cerebellar/bulbar symptoms (p=0.03) and less frequent cephalic syndrome (p=0.005). Satisfactory outcomes were more common in older patients (p=0.02).
Conclusions:
- Craniocervical junction (CVJ) decompression is a key surgical intervention for symptomatic Chiari malformation type II (CM-II) with hydrocephalus and myelomeningocele.
- Age is a significant prognostic factor, with older patients experiencing better outcomes.
- Conservative management is appropriate for asymptomatic CM-II; surgical intervention is indicated for neurological deficits, syringomyelia, hydrocephalus, myelomeningocele, or intractable pain.
Abstract:
Background Chiari malformation type II (CM-II) may not always present as an asymptomatic disorder but prove to be difficult in managing. This is especially true for neonates who show the worst prognosis. There is confounding data over whether shunting or craniocervical junction (CVJ) decompression should be employed. This retrospective analysis summarizes the results of 100 patients diagnosed and treated for CM-II along with hydrocephalus and myelomeningocele. Methods We reviewed all the children who were diagnosed and surgically treated for CM-II at the Moscow Regional Hospital. Surgical timing was decided on the clinical conditions of each patient. Urgent surgery in the more compromised patients (usually infants) and elective surgery for patients with less severe conditions was performed. All patients first underwent CVJ decompression. Results The retrospective review yielded 100 patients operated on for CM-II with concomitant hydrocephalus and myelomeningocele. The average herniation was 11.2 ± 5.1 mm. However, herniation level did not correlate with clinical findings. Concomitant syringomyelia was observed in 60% of patients. More severe spinal deformity was observed in patients with widespread syringomyelia ( p = 0.04). In children of the younger age group, cerebellar symptoms and bulbar disorders were more frequently observed ( p = 0.03), and cephalic syndrome was noted much less frequently ( p = 0.005). The severity of scoliotic deformity correlated with the prevalence of syringomyelia ( p = 0.03). Satisfactory results were significantly more often observed in patients of the older age group ( p = 0.02). Patients with unsatisfactory results at the time of treatment were significantly younger ( p = 0.02). Conclusion If CM-II is asymptomatic, then no specific treatment is prescribed. If the patient develops pain in the occiput and neck, then pain relievers are prescribed. If a patient has neurological disorders or concomitant syringomyelia, hydrocephalus or myelomeningocele, surgical intervention is indicated. The operation is also performed if the pain syndrome cannot be overcome within the framework of conservative therapy.
