title: Cystic spinal dysraphism of the cervical and upper thoracic region.
abstract: BACKGROUND
  Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles.
  
  
  METHODS
  A retrospective study of 18 patients was conducted.
  
  
  RESULTS
  In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles.
  
  
  CONCLUSIONS
  Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.
authors.affiliation: Section of Pediatric Neurosurgery, Department of Pediatric Surgery, Fernandes Figueira Institute, Oswaldo Cruz Foundation MS-Fiocruz, Av. Rui Barbosa, 716, Rio de Janeiro, Brazil. fsalomao@ism.com.br
authors.forename: J Francisco
authors.initials: JF
authors.lastname: Salomão
authors.forename: Sérgio
authors.initials: S
authors.lastname: Cavalheiro
authors.forename: Hamilton
authors.initials: H
authors.lastname: Matushita
authors.forename: René D
authors.initials: RD
authors.lastname: Leibinger
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Bellas
authors.forename: Elide
authors.initials: E
authors.lastname: Vanazzi
authors.forename: Luiz A M
authors.initials: LA
authors.lastname: de Souza
authors.forename: Andréa G
authors.initials: AG
authors.lastname: Nardi
mesh_terms: D000015:Abnormalities, Multiple; D002574:Cervical Vertebrae / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008591:Meningomyelocele / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D016137:Spina Bifida Cystica / Q000145:classification / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D013904:Thoracic Vertebrae / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal