11/25. Neurenteric cyst: case report and a review of the potential dysembryology.We report a 13-year-old female who presented to an outside emergency room following blunt trauma to the head. As part of her evaluation, an unenhanced CT of the face and radiographs of the cervical spine were performed. She was referred to our clinic with "abnormal" imaging. Radiographs revealed dysmorphic cervical spine and ventral clefting of the vertebral bodies from C7-T2. A subsequent MRI was obtained that demonstrated a large spinal cord mass. MRI demonstrated an unenhanced intramedullary mass of the cervicothoracic spinal cord measuring approximately 1.5 cm in greatest axial diameter. Surgical exploration of her intraspinal mass revealed a neurenteric cyst. Spinal neurenteric cysts are categorized in the spectra of occult spinal dysraphism and most likely arise from incomplete separation of the developing notochord and foregut in the embryo. We discuss potential etiologies for the formation of such cysts. The clinician should consider neurenteric cysts in their differential diagnosis of patients with spinal dysraphism and/or intraspinal masses.- - - - - - - - - - ranking = 1keywords = embryo (Clic here for more details about this article) |
12/25. teratoma or enterogenous cyst? The histopathological and clinical dilemma in co-existing occult neural tube dysraphism.BACKGROUND: Better understanding of embryology, histopathology and genetics of dysraphic conditions have lead to an expansion of this concept to entities with a similar microscopic appearance (e.g. enterogenous cysts, colloid cyst of the III-rd ventricle) or rated among neoplasms (e.g. mature teratoma), creating a certain conceptual confusion. Currently the diagnosis of "teratoma" is being substituted by "enterogenous cyst" or "teratomatous cyst". AIM OF paper: Clarification of concepts in this field and presentation of the experience of the Department of neurosurgery of the Children's Memorial health Institute associated therewith. MATERIAL AND METHOD: Since January 1990 through April 2005 we had treated 7 children with the final diagnosis of "an enterogenous cyst". The mean age of the children was 10.5 years and the mean follow-up time was 4.1 years. The study was performed by a retrospective analysis of medical records, imaging studies and histological preparations. RESULTS: the study group included: 3 cases of type I enterogenous cyst, 1 case of type II cyst and 3 cases of type III cyst (in 2 cases a former diagnosis of "mature teratoma" has been revised). Within this follow-up time, a good outcome was obtained in 3 cases, moderate disability - in 3 cases and severe disability - in 1 case. CONCLUSIONS: (1) Enterogenous cyst often co-exists with other dysraphic features; (2) Clinical signs of an enterogenous cyst are non-typical and depend on location of the lesion. The presence of secreting gastric mucosa may lead to chemical myelitis; (3) Severity of the postoperative neurological deficit is due to the developmental nature of the lesion and common vascular supply; (4) Late results of treatment are satisfactory, provided the lesion is excised radically; (5) Enterogenous cyst may be diagnosed in the case of a tumor composed of tissues originating from 1, 2 or 3 embryonic layers coexisting with dysraphic stigmata, congenital vertebral abnormalities or a mediastinal tumor of the same type. Types II and III of enterogenous cyst were historically diagnosed as "adult teratoma".- - - - - - - - - - ranking = 0.4keywords = embryo (Clic here for more details about this article) |
13/25. Primary spinal intramedullary adrenal cortical adenoma associated with spinal dysraphism: case report.OBJECTIVE: The authors report a primary spinal intramedullary adrenal cortical adenoma in a patient with spinal dysraphism presenting with bilateral leg pain and urinary frequency. methods: magnetic resonance imaging, L2 laminectomy with resection of mass, and pathological and immunohistochemical analysis of resected mass revealed the diagnosis. RESULTS: Microscopic and immunohistochemical findings confirmed the diagnosis as a primary intramedullary tumor of adrenal cortical origin. CONCLUSION: The occurrence of a primary adrenal tumor in the spinal cord is rare and difficult to explain based on our understanding of embryology. A review of the relevant literature and discussion of the pathophysiology and clinical implications is provided.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
14/25. The human tail and spinal dysraphism.Recent publications have endeavoured to differentiate between the true, or vestigial tail, and the pseudotail by clinical and pathological examination, and have indicated the benign nature of the true tail. The true tail arises from the most distal remnant of the embryonic tail, contains adipose, connective, muscle, and nerve tissue, and is covered by skin. Pseudotails represent a variety of lesions having in common a lumbosacral protrusion and a superficial resemblance to vestigial tails. A review of the case reports indicates spina bifida to be the most frequent coexisting anomaly with both. A review of occult spinal dysraphism shows it to be associated with cutaneous signs in more than 50% of instances. Three cases of spinal dysraphism with tail-like cutaneous structures are described and their radiological, operative, and pathological findings presented. The classification of each of the appendages into true tail or pseudotail remains obscure. Although the finding of these three tails was the subject of much curiosity, surgical treatment was clearly designed to adequately deal with the associated dysraphic state. The presence of a tail-like appendage in the lumbosacral region should alert the clinician to the possibility of underlying spinal dysraphism. Preoperative assessment must include a complete neurological history and examination as well as computed tomographic or magnetic resonance imaging.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
15/25. Incomplete penoscrotal transposition associated with hemivertebrae.Penoscrotal transposition is a rare anomaly of external genitalia, often associated with a variety of defects usually related to the urinary and the gastrointestinal tracts and the vertebral column. We report such a patient with hemivertebrae at L4-5, who had no other urological anomaly. To our knowledge this anomaly as an isolated urological entity with hemivertebrae has not been reported before. A brief mention is made of the embryology, incidence and various surgical techniques of correction.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
16/25. Tethered cord syndrome: a pediatric case study.Tethered cord syndrome, seen in patients with spinal dysraphism, is a progressive neurological deterioration due to stretching of the spinal cord. spinal dysraphism results from defects during embryonic closure of the neural tube, usually in the lumbosacral region. In tethered cord syndrome an abnormally low conus medullaris is tethered by intradural abnormalities such as a short, thickened filum terminale, fibrous bands, a lipoma or diastematomyelia. This article distinguishes tethered cord syndrome from other forms of occult spinal dysraphism and describes and compares normal anatomy to pathological changes. Clinical symptoms and nursing management of the pediatric patient with tethered cord syndrome will be exemplified by a case study presentation.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
17/25. Pathogenesis of diastematomyelia and spina bifida.Three cases of spina bifida (SB) associated with diastematomyelia (DM) are described and possible differences in pathogenesis are discussed. The frequent simultaneous occurrence of both malformations and the possibility that the cord can be split above, at the level of or below the SB indicate a common pathogenesis for SB and DM. Splitting of the cord symmetrically, asymmetrically, bilateraly or even antero-posteriorly can be explained only by the existence of a destructive phenomenon which is responsible for the bifurcation of the neural tube or its anlage. The existence of such a phenomenon indicates that myelocele can develop due to re-opening of a previously closed neural tube. meningocele may be a manifestation of early embryonic antero-posterior DM in which the anterior tubule develops into an almost normal spinal cord, whereas the posterior tubule provides the wall of the cystic sac of meningocele in which most or all the neural tissue undergoes fibrous replacement.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
18/25. Craniofacial duplication (diprosopus): report of a case with a review of the literature.A case of craniofacial duplication (diprosopus) is presented. Details on this rare form of conjoined twins are described, and the proposed theories of its embryogenesis are discussed with brief review of the pertinent literature.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
19/25. Cloacal exstrophy: 18-year survival of untreated case.A patient is described who was 18 years old and attending high school when initially treated for cloacal exstrophy. The history, embryology and treatment of cloacal exstrophy are discussed, emphasizing that this congenital anomaly is compatible with a useful and happy life.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
20/25. The "filum intermedium" sign: focal in utero spinal cord infarct and extraspinal thecal sac. Case report.This report describes the unique case of a child born with paraplegia and a neurogenic bladder who was found to have a dysplastic, nonossified T-12 vertebral body, midline fusion of the T-12 neural arches, obliteration of the spinal canal at T-12, and an extraspinal thecal sac in the T11-L1 region. Neural tissue was focally absent from T9-12, but neural structures above and below were preserved. Narrowing of the thecal sac on myelograms and sagittal magnetic resonance images signifies in utero focal infarction of the spinal cord after neurulation but before formation of the posterior half of the spinal canal. The infarction resulted in severe focal narrowing of the thecal sac from T10-L1, resembling a premature and duplicated filum terminale; to denote the radiographic appearance of these anomalies, the authors have coined the term "filum intermedium" sign. The extremely unusual radiographic findings in this child illustrate the important interactions between neural tube, neural crest, and somite in the development of the spinal cord and spinal column. Correlation of the radiographic findings with the embryological differentiation and migration of these structures suggests that the spinal anomalies were caused by a focal insult, probably vascular in origin, occurring between the sixth and eighth weeks of gestation. The identification of a focally narrowed thecal sac and spinal cord (the "filum intermedium" sign) localizes the time of the insult to between the first and third month of gestation, and therefore is a useful marker in understanding developmental malformation of the spinal cord.- - - - - - - - - - ranking = 0.2keywords = embryo (Clic here for more details about this article) |
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