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PubMed2001

Magnetic resonance imaging appearance of intradural spinal lipoma.

Intradural spinal lipoma not associated with spinal dysraphism is a rare tumor often presenting with nonspecific symptoms and indolent clinical course. Its intradural location and fat component is the key for proper preoperative diagnosis, which could hardly be made by traditional imaging studies including plain film and myelography. Both CT and MRI can reveal the fat component of the tumor, but MRI is superior to CT in demonstrating its relationship with adjacent normal nerve tissue. We report a 32-year-old man who had back pain for years and the symptom progressed rapidly in recent two months. MRI revealed an intradural tumor at T12 level with high signal intensity on both T1- and T2-weighted images. The signal intensity dropped dramatically with fat saturation technique, which confirmed fat as its main component. The patient received surgery and the tumor was proved to be an intraspinal lipoma.

Adult↗
PubMed1990

Leptomyelolipoma: analysis of 20 cases.

Leptomyelolipoma (lumbosacral lipoma) is a common form of spinal dysraphism. The deficits produced include sensory, motor, bowel, and bladder dysfunction, and vary in incidence between the pediatric and adult populations. Twenty patients treated surgically at the Mount Sinai Hospital between 1972 and 1988 are reviewed. Fifty percent were 12 years of age or less and 50% were older than 18 years of age. The surgical approach was designed to accomplish untethering of the conus medullaris, debulking of the lipomatous mass compressing the cord, reconstruction of the dural canal, and reapproximation of the paraspinal muscles and lumbosacral fascia to prevent future trauma. Postoperatively, no patient experienced deterioration of neurological function. Of the symptomatic patients. 67% displayed dramatic improvement or became asymptomatic and 33% experienced stabilization of their deficits. The symptoms most resistant to surgical correction were orthopedic foot deformities and bowel dysfunction, whereas bladder dysfunction, motor weakness, and radiculopathies were most amenable to surgical therapy. Early surgical repair is recommended in these cases to forestall irreversible neurological damage.

Adolescent↗
PubMed2003

Long-term outcome of neurosurgical untethering on neurosegmental motor and ambulation levels.

The aim of this study was to determine the long-term outcome of neurosurgical untethering on neurosegmental motor level and ambulation level in children with tethered spinal cord syndrome. Forty-four children were operated on (17 males, 27 females; mean age at operation 6 years 2 months, SD 5 years). Sixteen patients had myelomeningocele, nine had lipomyelomeningocele, and 19 had other types of spinal dysraphism. Motor level and ambulation level were assessed pre- and three times postsurgery (mean duration of follow-up 7 years 1 month, SD 1 year 8 months). Deterioration of motor level was seen in five of 44 patients, 36 of 44 remained stable, while improvement was seen in three of 44 patients. Deterioration of ambulation level was seen in five of 44 patients, and remained stable in 26 of 44. Thirteen of 44 children were too young to ambulate at time of operation (< 2 years 6 months). Late deterioration of motor or ambulation level was only seen in (lipo) myelomeningocele patients. Deterioration of ambulatory status was strongly associated with obesity and retethering. Revision of the initial tethered cord release was performed in nine of 44 patients, mainly in those with lipomyelomeningocele.

Child↗
PubMed2002

Progression of terminal syrinx in occult spina bifida after untethering.

OBJECT: The association of spinal dysraphism and terminal syringomyelia is a well-known entity, and untethering with or without syrinx drainage is usually the surgery of choice. However, progression of the previously existing syrinx after an untethering procedure is an uncommon course. The objective of this study was to discuss the diagnostic, surgical, and follow-up consequences of such an occurrence. METHODS: Four children with occult spina bifida associated with terminal syrinx at the time of initial presentation are included in this study. After the primary releasing procedure, all developed enlargement of the syrinx. Two of them were operated on for placement of a syringo-subarachnoid shunt, while the other two were followed up conservatively. One of the shunted patients also had a second releasing procedure. CONCLUSION: Progression of the pre-existing terminal syrinx after the primary untethering procedure should be kept in mind even in the absence of overt neurological progression.

Cerebrospinal Fluid Shunts↗
PubMedDate not supplied

Prenatal diagnosis of pentalogy of Cantrell in three cases, two with craniorachischisis.

In this report, we describe 3 cases of pentalogy of Cantrell diagnosed prenatally with sonography. All 3 fetuses had an omphalocele and ectopia cordis; 2 of them also had craniorachischisis, in which the open cranial defect (anencephaly or exencephaly) is continuous with the completely open spine (spinal dysraphism). The association of the pentalogy of Cantrell with exencephaly is rare, and to our knowledge, the association of this syndrome with craniorachischisis has not been reported previously. In addition, 2 of our cases had clubfoot, and 1 of them also had clubhand, neither of which has been reported previously in association with the pentalogy of Cantrell. Finally, polyhydramnios, which occurred in 2 of our cases, has been reported to occur in association with that syndrome in only 1 previous case. We believe that sonographers should be aware that these anomalies may occur simultaneously with the classic anomalies of the pentalogy of Cantrell.

Abdominal Wall↗
PubMed2005

Prenatal ultrasound diagnosis of cloacal exstrophy associated with myelocystocele complex by the 'elephant trunk-like' image and review of the literature.

A case of cloacal exstrophy (CE) was detected by ultrasound as early as 22 weeks of gestation in association with myelocystocele complex, an unusual form of occult spinal dysraphism often associated with such a disease. The ultrasonographic diagnosis was made through the detection of a wavy cord-like segment of soft tissue protruding from the anterior abdominal wall, just below the umbilical cord insertion, strongly resembling the trunk of an elephant. Our article enforces the suggestion that the ultrasound elephant trunk-like image should be added to the existing major criteria for making prenatal diagnosis of CE.

Abnormalities, Multiple↗
PubMed2003

Association between prenatal sonographic findings and post-natal outcomes in 30 cases of isolated spina bifida aperta.

This study presents 30 cases of fetal isolated spina bifida aperta (SBA) to identify prenatal ultrasound findings that could predict the prognosis. Comparisons between surviving patients who had normal (group 1) and abnormal (group 2) post-natal neurological outcomes were made for three different prenatal signs, that is, site of vertebral lesion, presence and degree of ventriculomegaly and presence of talipes. The site of the lesion was the most significant outcome predictor, as high spinal dysraphisms were observed in 2 patients (2/7-28.6%) in group 1 and in 15 patients (15/19-79.0%) in group 2 (p = 0.03). The presence of fetal ventriculomegaly was associated with impaired post-natal neurological development, as it occurred in 4 patients (4/7-57.1%) in group 1 and in 18 patients (18/19-94.7%) in group 2 (p = 0.04). The presence of talipes did not significantly differ between the two groups. Patients with abnormal intellectual outcome (8/26-30.8%) had significantly greater (p = 0.018) lateral ventricle/hemisphere ratios (mean = 0.74, standard deviation = 0.13) than those with normal intellectual development (mean = 0.54, standard deviation = 0.18). Mean post-natal follow-up was at 23 months (standard deviation = 15 months).

Abnormalities, Multiple↗
PubMed1982

Computed tomography of sacral and presacral lesions.

Forty-two patients with various sacral and presacral lesions were examined by computed tomography (CT). CT was sensitive in detecting intraosseous neoplasms and presacral soft tissue masses and in seven cases identified lesions not visible on plain films. The total extent of each lesion was readily determined using CT. However, CT is diagnostically nonspecific and differentiation between primary and secondary tumors of the sacrum in generally not possible from the CT appearance alone. CT is also of considerable use in the evaluation of several non-neoplastic conditions of the sacrum, including spinal dysraphism, anterior and internal meningocele and trauma.

Adolescent↗
PubMed1985

Congenital bone anomalies associated with lipomas.

Congenital bone anomalies are occasionally located adjacent to deep lipomas. These bone dysplasias may take the form of localized overgrowth or osseous malformation. Two patients with iliac anomalies and spinal dysraphism and one with a hypertrophied rib and vertebra associated with lipomas are described. As both the lipomas and osseous anomalies tend to lie in the same sensory nerve distribution, there may be a common embryonic neural dysfunction affecting formation and subsequent growth of these structures.

Bone Neoplasms↗
PubMed1990

MR phase imaging and cerebrospinal fluid flow in the head and spine.

Motion of the cerebrospinal fluid (CSF) in and around the brain and spinal cord was examined in healthy subjects and in a number of patients with abnormalities of the CSF circulation. The pulsatile motion of the CSF was determined by spin echo phase (velocity) imaging, sometimes in combination with gradient echo phase contrast cine. Differences in flow patterns across CSF spaces were observed: flow reversal in the cerebellomedullary cistern and lumbar area relative to cervical CSF, and in the posterior versus the anterior subarachnoid space in the spinal canal. Flow communication was demonstrated in known communicating cysts or cavities. Differences in flow were also noted across spinal narrowing or block, and across the walls of a variety of cystic lesions in the brain and spinal cord. MR phase imaging of CSF flow provides pathophysiological information of potential clinical importance for the assessment of diseases affecting the CSF circulation.

Brain↗
PubMed2000

Spinal lipomatous malformations.

Spinal lipomatous malformations (SLM) include all the closed neural tube defects (NTD) with excessive lipomatous tissue in the spinal cord or filum terminale. We evaluated 65 cases of SLM seen & operated at our department in the last 7 years. Of these only 9 were asymptomatic and 8 were below 2 years of age. There were more males than females. In addition to subcutaneous lipoma many patients also had hypertrichiosis and dermal sinus as cutaneous makes. Twenty patients had foot deformity and 5 had unilateral limb shortening. Sixty-two patients had MRI and 3 had CT myelogram for evaluation. These revealed 7 patients with Chiari malformations, 10 with focal syrinx and 2 patients underwent VP shunt for hydrocephalus. Sacral agenesis was seen in 5 patients. Clinical features were similar to other cases of spinal dysraphism except that sensory loss and trophic ulcer were more frequent. Intradural lipoma and tethering was seen in 18 cases whereas intramedullary lipoma & conus lipoma was seen in 40 & 7 cases respectively. Additional tethering lesion was seen in 1/3 cases and was treated simultaneously. Preoperative deficits improved in 28 cases and stabilized in 33 cases. Three patients developed fresh deficits after surgery. We observed 8 CSF leaks and 4 wound infections in postoperative period. It is very clear from our data that a patient has about 95% chances that his neurological status may improve or stabilize following surgery and the risk of developing fresh deficits is about 5%. We, therefore, suggest that all patients of SLM should be treated with aggressive surgical management for best results.

Adolescent↗
PubMed1999

[Urological diagnosis in children with myelomeningocele].

Neurogenic bladder dysfunction in childhood is mostly caused by spinal dysraphism. The urologic diagnostic procedure tries to evaluate the underlying form of neurogenic bladder, dysfunction the associated disturbances at the upper tract and the development of changes by body growth. Therapeutic main aim is the conservation and protection of the kidney function and, secondly, the development of social dryness and a form of bladder emptying, that is related to the individual situation and associated disabilities. We report here about the diagnostic procedure and result of a group of 190 patients, that have been seen in our department since birth and for more than ten years continuously. Developmental changes of neurogenic bladder dysfunction has been found in more than 25% of the children. This figure shows the need of life-long close urodynamic follow-up.

Child↗
PubMed2006

[Pitfalls and special features of modern imaging diagnostics of the upper cervical spine].

Recent technical developments led to a change in the significance of the different imaging modalities in the whole spectrum of neuroradiology and thus also in the field of imaging of the cervical spine. The main part in diagnostic imaging of the upper cervical spine is taken by high-resolution multislice CT and MRI. Conventional X-rays serve as an initial screening technique if required.

Angiography↗