Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment.
Spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment.
Department of Pediatric Neurosurgery, Erasmus Medical Center, Sophia Children Hospital, Rotterdam, The Netherlands.
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MP
Arts
T H R
TH
de Jong
D000328:Adult; D001139:Arnold-Chiari Malformation / Q000175:diagnosis / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006849:Hydrocephalus / Q000175:diagnosis / Q000601:surgery; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008588:Meningocele / Q000175:diagnosis* / Q000601:surgery*; D008591:Meningomyelocele / Q000175:diagnosis* / Q000601:surgery*; D009436:Neural Tube Defects / Q000175:diagnosis* / Q000601:surgery*; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis / Q000601:surgery; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012086:Reoperation; D016137:Spina Bifida Cystica / Q000175:diagnosis* / Q000601:surgery*; D013116:Spinal Cord / Q000473:pathology; D013595:Syringomyelia / Q000175:diagnosis / Q000601:surgery; D013904:Thoracic Vertebrae / Q000002:abnormalities* / Q000601:surgery*; D016216:Ultrasonography, Prenatal; D017287:Ventriculoperitoneal Shunt