Cystic spinal dysraphism of the cervical and upper thoracic region.

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.

Cervical spina bifida cystica: MRI differentiation of the subtypes in children.

BACKGROUND: Cervical spinal dysraphism is a rare condition with only 37 cases reported in the literature, of which only seven patients had undergone preoperative magnetic resonance imaging (MRI). OBJECTIVE: By using illustrative cases, we demonstrate the value of MRI in distinguishing the two different subtypes of cervical spinal dysraphism, namely, myelocystocele and meningocele. METHOD: Retrospective review of the MRI scans of six patients with cervical (one high thoracic) posterior cystic swellings, which were diagnosed as cervical dysraphism, was done. RESULTS: Three patients were diagnosed with myelocystocele and three patients with meningocele. A comparison of MRI features between the two entities, as well as the associated anomalies, is discussed. CONCLUSION: MRI is the imaging modality of choice for suspected cervical dysraphism, prior to surgery. It helps distinguish the subtypes, as well as identify the associated anomalies.

Open spina bifida: a complete cohort reviewed 25 years after closure.

This study summarises the long-term results of non-selective treatment of open spina bifida. Every member of a consecutive series of 117 cases was ascertained 22 to 28 years after closure of the back. 56 of the cohort had died. The condition of the 61 survivors ranged from normal to severe disability. Only 33 of the survivors were capable of living independently, 11 required supervision and some help, and 17 needed daily care which was generally provided by the parents. The two main determinants of disability and dependency were the extent of the neurological deficit and IQ. Improvements in the management of patients with open spina bifida have greatly reduced mortality, but they are less likely to influence long-term disability since that is dependent on the severity of the neurological deficit.