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Surgical experience in 130 pediatric patients with Chiari I malformations
R Shane Tubbs1, Matthew J McGirt, W Jerry Oakes
1Department of Cell Biology, University of Alabama at Birmingham, Birmingham, Alabama, USA. richard.tubbs@ccc.uab.edu
Insights
Posterior fossa decompression surgery effectively treated most pediatric Chiari I malformation symptoms, with 83% experiencing relief. While complications were rare (2.3%), some patients required repeat operations for persistent issues.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Congenital Malformations
Background:
- Chiari I malformation is a complex hindbrain anomaly affecting pediatric patients.
- Symptomatic presentation often includes headache, neck pain, and scoliosis.
- Associated conditions like hydrocephalus and neurofibromatosis Type 1 are common.
Purpose of the Study:
- To report long-term outcomes of surgical intervention for pediatric Chiari I malformation.
- To evaluate the efficacy of posterior fossa decompression in this population.
Main Methods:
- A surgical series of 130 pediatric patients with Chiari I malformation.
- Posterior fossa decompression with duraplasty was performed.
- Follow-up ranged from 3 months to 15 years.
Main Results:
- 83% of patients experienced relief from preoperative symptoms.
- Headache/neck pain and scoliosis improved in most, but not all, cases.
- Complications occurred in 2.3%, with rare instances requiring further intervention or revision surgery.
Conclusions:
- Posterior fossa decompression is a safe and effective treatment for pediatric Chiari I malformation.
- The study represents one of the largest surgical series for this condition in children.
- Surgical intervention can significantly improve quality of life for affected children.
Object:
The aim of this study was to present the long-term findings of a surgical series of pediatric patients with Chiari I malformations.
Methods:
One hundred thirty symptomatic pediatric patients with Chiari I malformations underwent posterior fossa decompression. The age in this group of patients ranged from 2 months to 20 years (mean 11 years). The length of the hospital stay extended from 2 to 7 days (mean 2.7 days), and follow up was from 3 months to 15 years (mean 4.2 years). Patients most often presented with headache/neck pain (38%) and scoliosis (18%). Examples of associated diagnoses included neurofibromatosis Type 1 (5.5%), hydrocephalus (11%), idiopathic growth hormone deficiency (5.5%), and Klippel-Feil anomaly (5%). Syringes were present in 58% of patients. Seventeen percent of patients had caudal displacement of the brainstem and fourth ventricle. Postoperative relief of preoperative pathologies was experienced in 83% of patients. Of the most common presenting symptoms--headache/neck pain and scoliosis--12 and 17%, respectively, were not alleviated postoperatively. Complications occurred in 2.3% of this group and included the development of acute hydrocephalus postoperatively and severe life-threatening signs of brainstem compression that necessitated a transoral odontoidectomy. Nine patients have had to undergo repeated operations for continued symptoms or persistent large syringes. During surgery 10 patients (7.7%) were found to have arachnoid veils occluding the fourth ventricular outlet, and nine of these had syringomyelia. In our experience almost all syringes will stabilize or improve with posterior fossa decompression and duraplasty.
Conclusions:
The authors believe this to be the largest reported series of pediatric patients who have undergone posterior fossa decompression for Chiari I malformations.