SEARCH · PubMed Health

Results for “Spinal Dysraphism”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources

PubMed1991

Early diagnosis and treatment of spinal dysraphism to prevent urinary incontinence.

Spinal dysraphism represents a very rare congenital anomaly of the spinal cord and cauda equina, often associated with cutaneous sacral lesions. This condition results in distorsion of neural tissue, possibly leading to vesical dysfunction and urinary incontinence. A series of 12 children with spinal dysraphism (4 lipomeningoceles with tethered cord, 3 tight filum terminale, 2 dermal sinuses, 2 arachnoid cysts, 1 anterior meningocele) observed during the last 6 years is presented. The patients are divided into two groups: group A includes 5 infants (age range 4 months-2 years) and group B 7 older children (4-12 years). All 7 patients of group B were referred for urinary incontinence as their chief complaint; only 1 had evidence of a skin lesion while 3 had a club foot. In group A, 4 had skin lesions (2 asymptomatic and 2 dermal sinuses referred after several episodes of meningitis) and the 5th child had a club foot. The diagnosis was made by myelo-CT scan in the earlier 4 and by magnetic resonance imaging in the more recent 8 cases. All 12 children were operated. In group B, the patients' urinary incontinence persisted but did not worsen (3 were stable and 4 had slightly improved); in group A, 2 were dry (follow-up greater than 2 years), 1 could not be accurately evaluated because he was less than 2 years of age, and the 2 who suffered from meningitis were incontinent. Early diagnosis and neurosurgical treatment of spinal dysraphism may prevent urinary incontinence. Accurate urodynamic, neurophysiologic, and neuroradiologic evaluation of children with severe voiding anomalies or club foot is recommended even if no lumbosacral cutaneous lesions are present.

Child↗

PubMed2006

Frontal bone agenesis in a patient of spinal dysraphism.

Associated cranial abnormalities with spinal dysraphism are not uncommon. We came across an unusual case of a 1-year-old male child with spinal dysraphism having lumbar meningomyelocele, who also had split cord malformation (hemicord with intervening bony spur) with lipoma of one of the hemicord and filum terminale. The patient also had communicating hydrocephalus without Chiari malformation and also near-total frontal bone agenesis. Single photon emission computed tomography scanning of brain revealed normal perfusion. In the first stage of repair, the patient had postoperative CSF leak for which ventriculo-peritoneal shunt was performed. This constitutes a rare anomaly associated with spinal dysraphism.

Frontal Bone↗

PubMed1997

Surgical treatment of syringomyelia associated with spinal dysraphism.

Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). Syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. Foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.

Child↗

PubMed1985

Delayed diagnosis of voiding dysfunction: occult spinal dysraphism.

The recognition of spinal dysraphism as a potential cause of voiding dysfunction is of the utmost importance if appropriate treatment and followup are to be initiated. We present 2 women with prolonged voiding dysfunction who had spinal dysraphism, intradural lipoma and related urodynamic abnormalities. The importance of initial symptom recognition, physical examination, urodynamic evaluation, appropriate treatment and periodic re-examination to detect progressive neurological impairment is emphasized.

Adult↗

PubMed2002

Multiple subpial lipomas with dumb-bell extradural extension through the intervertebral foramen without spinal dysraphism.

BACKGROUND: Intradural subpial lipomas not associated with spinal dysraphism, account for less than 1% of spinal cord tumors. The simultaneous existence of multiple intradural subpial lipomas with dumb-bell extradural extension through the intervertebral foramen in the same patient without any evidence of spinal dysraphism has not been previously reported. CASE DESCRIPTION: A 38-year-old man presented with progressive spastic paraparesis, and weakness of right elbow extension and opposition of the medial three fingers. He also had ascending paraesthesia from the C6 dermatome to the saddle region and loss of joint and position sense of both lower limbs with hesitancy and precipitancy of micturition. There was no spinal tenderness, deformity, neurocutaneous markers, or spinal dysraphism. The total duration of illness was 11 years. The oblique views of the plain radiographs of the cervical spine revealed an enlarged right C7-D1 intervertebral foramen. The T1- and T2-weighted magnetic resonance (MR) images showed two intradural, hyperintense lesions (with extensive loss of signal on fat suppression sequences), one extending from C5 to D2 and the other opposite the C3-4 disc space. The parasagittal and axial images showed the extradural component of the lesion emerging from the right C7-D1 intervertebral foramen. At surgery, a C2 to D2 laminectomy was performed. The lipoma, enclosed in a fine pial membrane, was situated on the right posterolateral aspect of the cord. The right-sided nerve roots from the C6 to D1 levels were completely enmeshed by the lipoma. There was a separate superficial subpial lipoma adherent to the posterior aspect of the cord at the C3-4 level. A distinct area of normal cord was present between the two lesions. A subtotal decompression of the lesions including the component emerging through the right C7-D1 intervertebral foramen and a duraplasty were performed. At follow-up after 18 months, the posterior column impairment, lower limb hypoaesthesia, and right upper limb paraesthesia had improved. However, residual elbow extension and lower limb weakness, mild lower limb spasticity and sphincteric dysfunction persisted. CONCLUSIONS: The multiplicity of intradural subpial lipomas without spinal dysraphism points to a dysembryogenetic basis similar to that seen in patients with spinal dysraphism that results in lipomas, but in which the defect is not severe enough to give rise to coexisting vertebral and soft tissue anomalies. The dumb-bell extradural extension through the intervertebral foramen is extremely rare. The magnetic resonance imaging and surgical principles are discussed.

Adult↗

PubMed2001

Spinal dysraphism in an elderly patient.

Spinal dysraphisms are diagnosed more frequently at birth or in infancy. We report a spinal malformation compatible with lipomyeloschisis in an elderly patient presenting with symptoms and signs of myelopathy. Magnetic resonance imaging revealed an intraspinal mass continuous with a subcutaneous lipoma. Three-dimensional computed tomography reconstructions better showed the spinal dysraphism; dermal sinus was also evident. Neuroimaging can define the precise diagnosis also in elderly patients presenting with myelopathy and can provide valuable structural details.

Aged↗

PubMed1981

Neuropathic bladder and spinal dysraphism.

The association between spinal dysraphism and a neuropathic bladder is well known, but the diagnosis of the spinal lesion and the associated renal problems is often delayed. Four children referred with orthopaedic problems and in whom the bladder abnormally proved to be the major disability are described. Despite widely differing vertebral involvement, all had lower motor neurone neurological deficits confined to lumbar and upper sacral segments. All had unstable, variably thickened, small bladders, and it is proposed that the bladder abnormality is the result of a partial lesion of lumbosacral innervation, and not of an upper motor neurone lesion.

Child↗

PubMed1977

Computed tomography in spinal dysraphism.

The value of spinal computed tomography in the diagnosis and management of spinal dysraphism is described. Eight patients with diverse etiological agents were evaluated by clinical examination and subsequent computed tomography, without contrast enhancement. The diagnosis and location of the pathology thus ascertained allowed for appropriate surgery without invasive diagnostic studies. The value of this approach in children and in those patients in whom a low conus medullaris is suspected is obvious.

Adult↗

PubMedDate not supplied

Unusual malformations in occult spinal dysraphism.

Two cases of occult spinal dysraphism with different clinical symptoms, signs and congenital pathologies are presented. One had malformations including scoliosis, dermoid tumor, hydromyelia, diastematomyelia, dermal sinus, low conus, vertebrae anomalies and dextrocardia. The occurrence of dextrocardia in association with occult spinal dysraphism was found to be extremely unusual. The second case is presented in relation to the rarity of teratoma with dermal sinus and tethered cord in the lumbar area. Myelography, computed tomography, (CT), Myelo CT and magnetic resonance were used in making a diagnosis.

Abnormalities, Multiple↗

PubMed1981

Lumbosacral skin lesions as markers of occult spinal dysraphism.

Early treatment of occult spinal dysraphism may prevent progressive neurological deficits. However, diagnosis is often delayed until the onset of irreversible neurological damage. A review of data from the literature and patients at Johns Hopkins Hospital suggests that lumbosacral skin abnormalities such as tufts of hair, hemangiomas, lipomas, skin tags, or pigmented nevi should alert the physician to search for occult spinal dysraphism. In the asymptomatic patient with a skin lesion, roentgenography of the lumbosacral spine is a useful screening procedure for identifying treatable underlying problems.

Adolescent↗

PubMed2006

[Diastematomyelia: a form of spinal dysraphism].

Diastematomyelia is a rare form of occult spinal dysraphism (less than 3 % of cases) that is more frequent in children and mainly affects females. Clinically, there are three groups of manifestations: cutaneous, neurological and orthopedic deformities. We present the case of an 11-year-old girl with a recent, incidental diagnosis of diastematomyelia. Although this entity is unusual, it should be considered together with other forms of spinal dysraphism in children with congenital cutaneous markers of spinal dysraphism, even when these are vague and the patient is asymptomatic. Due to the frequency with which cutaneous manifestations appear, the intervention of a dermatologist is desirable in the diagnosis of this entity. A high index of suspicion is important when evaluating neonates and infants, given that an early diagnosis can alter neurologic prognosis, improving the quality of life of these patients.

Child↗

PubMed2006

[Congenital skin lesions in occult spinal dysraphism--what is typical?].

Occult spinal dysraphism (OSD) includes a group of spinal malformations covered by intact skin. Neurological sequelae include paresis and sensory deficits in the lower limbs, neurogenic bladder and bowel disturbances, pain and neuro-orthopaedic syndrome as well as a high risk for secondary neurological deterioration. In up to 80 % of the cases, OSD is accompanied by lumbosacral skin anomalies. In this article, an overview on the main forms of OSD and characteristic skin lesions is given, which can guide the clinician to early diagnosis and appropriate diagnostic work-up and, if necessary, therapeutic intervention.

Humans↗

PubMed1999

Early clinical signs and symptoms in occult spinal dysraphism: a retrospective case study of 47 patients.

Since occult spinal dysraphism can lead to irreversible neurological complications, early diagnosis and treatment are necessary. We retrospectively studied the presenting clinical signs and symptoms in all 47 cases of occult spinal dysraphism identified in two university hospitals in The Netherlands since 1965. Dermal sinus had been diagnosed in 12, lipomyelomeningocele in nine, and diastematomyelia in eight patients. Thirty-three patients had symptoms due to tethering of the spinal cord, leading to a clinical suspicion of occult spinal dysraphism in only eight cases. Twenty-eight patients had cutaneous back lesions that led to further investigation in eight cases. Nineteen patients had a small backmass leading to further examination in 13 cases. Three patients with dermal sinus presented with meningitis caused by an unusual aetiological agent. This study stresses the importance of identification of neurological dysfunction due to tethered cord syndrome, cutaneous back lesions, a small backmass and meningitis caused by an unusual aetiological agent for the early diagnosis of occult spinal dysraphism.

Adolescent↗

PubMed1998

Occult spinal dysraphism in neonates: assessment of high-risk cutaneous stigmata on sonography.

OBJECTIVE: In this study, we evaluated the incidence of dorsal cutaneous stigmata in a healthy neonate population; we also assessed whether specific types of cutaneous stigmata are associated with underlying spinal dysraphism. SUBJECTS AND METHODS: From July 1993 through December 1996, we prospectively examined term neonates with dorsal cutaneous stigmata. Each neonate underwent spinal sonography and clinical assessment of the cutaneous stigmata. Incidence of dorsal cutaneous stigmata in a healthy neonatal population was determined by dividing the number of neonates with cutaneous stigmata by the total number of neonates examined. RESULTS: The incidence of cutaneous stigmata in the healthy neonate study population was 4.8%. We examined 207 neonates with 216 cutaneous stigmata, the most common of which was the simple midline dimple (74%). None of the neonates with only a simple midline dimple had spinal dysraphism. Of the 207 neonates we examined, 16 had spinal dysraphism. Clinical examination revealed 180 dimples and 36 other types of cutaneous stigmata (e.g., hemangiomas, hairy patches, masses, tails, lesions). Fourteen (39%) of 36 other cutaneous stigmata were positive for spinal dysraphism. Eight (40%) of 20 atypical dimples were positive for spinal dysraphism. Three were large clefts (>5 mm); the remaining five cases were seen in combination with other lesions and were all located more than 2.5 cm from the anus. Six (67%) of the nine neonates with multiple cutaneous stigmata had spinal dysraphism. CONCLUSION: Simple midline dimples are the most commonly encountered dorsal cutaneous stigmata in neonates and indicate low risk for spinal dysraphism. Only atypical dimples are associated with a high risk for spinal dysraphism, particularly those that are large (>5 mm), high on the back (>2.5 cm from the anus), or appear in combination with other lesions. High-risk cutaneous stigmata in neonates include hemangiomas, upraised lesions (i.e., masses, tails, and hairy patches), and multiple cutaneous stigmata.

Humans↗

PubMed1983

Relationship of fetal hydronephrosis to spinal dysraphism.

Thirty-one fetuses with hydronephrosis and 14 fetuses with spinal dysraphism were sonographically examined. In only one instance was spinal dysraphism associated with hydronephrosis. This single case was identified in a term pregnancy. There was no statistically significant association of urinary tract dilation and open spinal defects in utero. This observation tends to confirm the reports that urinary tract dilatation associated with spinal dysraphism is less frequent when neonates and children are compared. the progressive decrease in this association appears to extend to the fetal period.

Adult↗

PubMed1994

Terminal syringohydromyelia and occult spinal dysraphism.

Terminal syringohydromyelia is a cystic dilatation of the lower third of the spinal cord. The authors describe its incidence and characteristics, its frequent association with occult spinal dysraphism, and its clinical significance and need for surgical treatment. All 143 cases of occult spinal dysraphism treated at the Duke University Medical Center between 1972 and 1992 were reviewed. A terminal syrinx was found in 24 (27%) of the 90 cases that were evaluated by magnetic resonance (MR) imaging. In contrast three (6.2%) of 48 cases evaluated by myelography and postmyelographic computerized tomography had a syrinx documented. The relative radiographic severity of the different syringes was estimated by using measurements of the syrinx and spinal cord on the MR images, classifying the cysts into large and small. Large syringes were frequently symptomatic, commonly presenting with pain, motor and sensory deficits of the lower extremities, scoliosis, and bowel and bladder dysfunction. Terminal syringohydromyelia with occult spinal dysraphic lesions was most often associated with tethered spinal cord from a tight filum terminale in the presence of an anorectal anomaly (67% of cases), meningocele manqué (54%), and diastematomyelia (38%). An infrequent association was seen with other spinal cord anomalies. The results of surgical management of terminal syringohydromyelia were analyzed, highlighting the necessity and effectiveness of shunting the large cysts, especially in the setting of a progressive symptomatology. Of the 11 patients with shunts who underwent MR imaging, 10 showed either complete or significant resolution of the syrinx; all five patients who had presented with pain (mainly back pain) showed complete resolution of the pain after shunting; finally, one-third of patients with shunt placement had significant postoperative improvement in their neurological examination, whereas none worsened. It is stressed that terminal syringohydromyelia is an important pathological entity that should be considered in patients with occult spinal dysraphism, and treated surgically when clinically or radiographically significant.

Adolescent↗

PubMed1995

Rehabilitation of children with spinal dysraphism.

Tremendous advances have occurred over the past several decades in the comprehensive management of spinal dysraphism. Multiple specialists from varied surgical, medical, and allied health professions contribute to the rehabilitation management of children with spinal dysraphism. Rehabilitation goals and treatment strategies have been presented in the contexts of multiple functional outcome domains of critical importance to quality of life. In addition, the child with spinal dysraphism often has unique health care maintenance issues that require careful monitoring within a coordinated, interdisciplinary clinic setting. Prevention of neurologic, musculoskeletal, urologic, and medical complications is necessary for the child with spinal dysraphism to realize his or her maximum functional potential. A comprehensive rehabilitation program provides sufficient training, education, and long-term monitoring to enable the child not only to achieve self-sufficiency, but also to maintain an optimal level of function throughout life.

Activities of Daily Living↗

PubMed2005

Occult spinal dysraphism and Pacinian hamartomas.

CASES: The authors report two patients with occult spinal dysraphism who, following histological analysis, were found to harbor associated Pacinian hamartomas. One patient's hamartoma was found in conjunction with their lipomyelomeningocele and the other was combined with a presacral myxopapillary ependymoma associated with a fatty filum terminale. DISCUSSION AND CONCLUSION: We review the literature and believe this to be only the third report of Pacinian hamartomas in association with occult spinal dysraphism. Although Pacinian hamartomas are seemingly rare in conjunction with occult spinal dysraphism, mesodermal elements are often found in this disease entity.

Hamartoma↗