title: Clinical presentation and surgical outcomes of intramedullary neurenteric cysts.
abstract: OBJECTIVE
  Intramedullary neurenteric cysts (NECs) are exceedingly rare lesions and have been previously reported in case reports. The aim of this study was to determine the clinical manifestations, radiological features, and long-term prognosis of patients with such lesions.
  
  
  METHODS
  The authors retrospectively reviewed the records of 13 patients with an intramedullary NEC. Each patient underwent MRI, laminotomy, and microsurgery. The accurate diagnosis was based on imaging and pathology findings. Each patient's follow-up status was determined through individual office visits and a structured telephone interview.
  
  
  RESULTS
  The series included 7 male and 6 female patients. Progressive or intermittent motor deficit was the main symptom associated with or without pain or sensory disturbance. Five cysts were located in the cervical cord, 1 in the cervicothoracic cord, 3 in the thoracic cord, and 4 in the conus medullaris. Concurrent malformations included scoliosis (3 cases), fusion of rib (1 case), enlarged spinal canal (1 case), tethered spinal cord (1 case), and ectocardia (1 case). Gross-total resection of the cyst was achieved in 8 cases, and subtotal resection (STR) was achieved in 5 cases. All patients were followed up, with a mean duration of 66.5 months. Cyst recurrence was observed in 4 cases after STR. In 2 cases the patients underwent reoperation; the other 2 patients remained clinically stable and did not undergo reoperation. At the last evaluation, neurological function was improved in 11 patients and remained stable in 2 patients.
  
  
  CONCLUSIONS
  Intramedullary NECs should be considered in the differential diagnosis of a middle-aged patient with intermittent neurological symptoms and concurrent malformations. Early surgery is advocated to prevent permanent neurological deficits. When gross-total resection cannot be achieved, maximally safe removal under the protection of intraoperative neuromonitoring is advised. Because of the high risk of cyst recurrence, routine follow-up MRI is needed. If a residual cyst shows obvious regrowth and results in neurological deficits, timely reoperation with a goal of STR should be performed.
authors.affiliation: Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital; and.
authors.forename: Tao
authors.initials: T
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital; and.
authors.forename: Liang
authors.initials: L
authors.lastname: Wu
authors.affiliation: Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
authors.forename: Jingyi
authors.initials: J
authors.lastname: Fang
authors.affiliation: Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital; and.
authors.forename: Chenlong
authors.initials: C
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital; and.
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Deng
authors.affiliation: Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital; and.
authors.forename: Yulun
authors.initials: Y
authors.lastname: Xu
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007796:Laminectomy; D007902:Length of Stay / Q000706:statistics & numerical data; D008279:Magnetic Resonance Imaging; D008297:Male; D008866:Microsurgery; D008875:Middle Aged; D009436:Neural Tube Defects / Q000175:diagnosis* / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: CDVT = cystic dilation of the ventriculus terminalis; EMA = epithelial membrane antigen; GFAP = glial fibrillary acidic protein; GTR = gross-total resection; IDEM = intradural extramedullary; NEC = neurenteric cyst; STR = subtotal resection; T1WI = T1-weighted imaging; T2WI = T2-weighted imaging; congenital; intramedullary cyst; long-term outcome; neurenteric cyst; surgical resection