Nonoperative management of enlarging syringomyelia in clinically stable patients after decompression of Chiari malformation type I

Abstract

OBJECTIVE The authors aimed to describe the natural history and optimal management of persistent syringomyelia after suboccipital craniectomy for Chiari malformation type I (CM-I). METHODS A cohort of all patients who presented to a tertiary pediatric hospital with newly diagnosed CM-I between 2009 and 2017 was identified. Patients with persistent or worsened syringomyelia were identified on the basis of a retrospective review of medical records and imaging studies. The management of these patients and their clinical courses were then described. RESULTS A total of 153 children with CM-I and syringomyelia were evaluated between 2009 and 2017. Of these, 115 (68.8%) patients underwent surgical intervention: 40 patients underwent posterior fossa decompression (PFD) alone, 43 underwent PFD with duraplasty, and 32 underwent PFD with duraplasty and fourth ventricle stent placement. Eleven (7.19%) patients had increased syringomyelia on subsequent postoperative imaging. Three of these patients underwent revision surgery because of worsening scoliosis or pain, 2 of whom were lost to follow-up, and 4 were managed nonoperatively with close surveillance and serial MRI evaluations. The syringes decreased in size in 3 patients and resolved completely in 1 patient. CONCLUSIONS Persistent or worsened syringomyelia after CM-I decompression is uncommon. In the absence of symptoms, nonoperative management with close observation is safe for patients with persistent syrinx.