Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I.

OBJECT

The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation.

METHODS

The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010.

RESULTS

The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and "return to school" time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed.

CONCLUSIONS

The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.