Iatrogenic syringomyelia postcranial surgery in pediatric patients: systematic review and illustrative case

Introduction Iatrogenic syringomyelia has previously been described preoperatively due to space occupying lesions and postoperatively predominantly postspinal CSF drainage. Iatrogenic syringomyelia after cranial surgery is a rare entity. The purpose of this study is to review the current literature for iatrogenic syringomyelia following cranial surgery in children. Material and methods We performed a systematic review in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and meta-analyses) guidelines. Using keywords “de novo,” “acquired,” “iatrogenic,” “postoperative development,” and “syringomyelia,” we searched PubMed and Scopus databases. We paid particular attention to type of surgery, clinical presentation, management, and outcome. We also present a relevant case report, operated on at John Radcliffe Hospital, Oxford. Results The systematic literature review provided 19 cases of iatrogenic syringomyelia postcranial surgery in pediatric patients, in addition to our case report, resulting in a total of 20 cases reported. The patients’ mean age at surgery preceding syringomyelia was 8.1 years (range 0.1–17) with a female sex predilection (2.7:1). The mean time of manifestation of the iatrogenic syringomyelia after surgery was 5.7 years (range 0.1–51), and the median time to presentation was 1.7 years. Nine children had surgery for Chiari I malformation before formation of the syrinx, 8 patients underwent shunt surgery, 2 children had intracranial tumor surgery, and 1 patient had lambdoid and sagittal suturectomy for scaphocephaly before developing a syrinx. Surgery after the manifestation of the iatrogenic syrinx resolved the symptoms in 9 patients; in 8 patients, the symptoms partially resolved; and in 1 patient, the symptoms persisted. After surgery addressing the syrinx, it resolved in 5, decreased in size in 10, and remained unchanged in 2 of the patients. Conclusion Iatrogenic syringomyelia after cranial surgery in pediatric patients occurs mainly after decompressive surgery for Chiari I malformation, shunt surgery, and less frequently following intracranial tumor surgery. Risk factors are postoperative intracranial scarring, shunt malfunction, and mass effect on the foramen magnum. Surgery addressing the iatrogenic syrinx seems to be effective in the majority of the patients in terms of clinical and radiological outcome.