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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

Spinal dysraphism: a comprehensive diagnostic approach.

Twelve patients with the clinical findings of spinal dysraphism form the basis for this report. In eight patients, physical findings, plain x-rays, and unenhanced spinal computed tomography allowed for precise diagnosis and subsequent therapy, without contrast myelography. All patients in the study had intravenous pyelography, and this revealed evidence of renal deterioration in two. The other 10 patients had radiographically normal upper urinary tracts. Urodynamic assessment was performed in seven; three were normal and four were abnormal. The pattern of the abnormal studies (three "flaccid type" with an adequate urethral pressure profile and one with detrusor-sphincter dyssynergia) allowed for appropriate therapy to be employed for bladder emptying and continence. Both spinal computed tomography and urodynamic testing serve as noninvasive studies that can be employed in the follow-up and management of patients with spinal dysraphism.

Adolescent

Cutaneous signs of spinal dysraphism. Report of a patient with a tail-like lipoma and review of 200 cases in the literature.

The term "spinal dysraphism" was coined in 1940 by Dr Lichtenstein to designate incomplete fusion or malformations of structures in the dorsal midline of the back, particularly congenital abnormalities of the vertebral column and spinal cord. Raphes develop on the face and head, brancheal arches, sternum, and spinal column. When dysraphism occurs in these sites, failure of closure of fontanelles, cleft lip and palate, brancheal cysts, and abnormalities of the ribs and spine result. A review of 200 cases of occult spinal dysraphism showed the condition to be more common in female patient and to be associated with cutaneous signs in more than 50% of instances. The age at which neurologic symptoms appeared in recorded cases is from birth to 76 years, the average being three years. A case of spinal dysraphism with a tail-like cutaneous structure is presented. The cutaneous manifestations accompanying spinal dysraphism that may lead to early recognition of this syndrome and early institution of treatment are discussed.

Adult

Spinal dysraphism: genetic relation to neural tube malformations.

The families of 207 index patients treated for spinal dysraphism at The Hospital for Sick Children were studied to discover whether the condition was aetiologically related to the classical neural tube malformation--spina bifida cystica and anencephaly. The index patients had all had a tethered conus medullaris and one or more of a variety of anomalies of the spinal cord, vertebrae, or skin overlying the vertebral column. Of 364 sibs of index patients, 9 had an encephaly and 6 spina bifida cystica, a pro-proportion of 4.12%. This approximates to the proportion of sibs affected by neural tube malformations in the London region when the index patients themselves have spina bifida or anencephaly. It is, therefore, appropriate that the mothers of children with spinal dysraphism should be offered prenatal screening for neural tube malformations.

Adult

Gas myelography in the investigation of occult spinal dysraphism.

In patients presenting with suspected spinal dysraphism of the occult type, particularly children, cisternal gas myelography is coming to be accepted as the radiological investigation of choice. The technique of the examination as performed on 40 such patients is described in detail. There have been no complications. Twenty-five patients showed abnormalities of the cord together with low termination and abnormal fixation of the conus. The visualization was excellent in terms both of reliability and detail. The reasons for the difficulties in demonstrating these lesions using positive contrast and for preferring gas myelography are discussed.

Adolescent

Occult spinal dysraphism in the geriatric patient.

Three patients in their seventies with occult spinal dysraphism are described. All three had tethered spinal cords, intrasacral meningoceles, and severe degenerative lumbar spondylopathy. The mechanism for the late development of symptomatology is related to an acquired lesion, degenerative spinal stenosis.

Aged

[Our experience in the prenatal echographic diagnosis of spinal dysraphism in the fetus].

The investigation consists of 141 cases of prenatal diagnosing structural and chromosomal anomalies of the fetus. Seventeen of the cases, quoted above, concern spinal dysraphism (spina bifida, occipital meningocele, meningoencephalocele). It has been made an analysis of ultrasound signs at this group of defects which turned out the most useful for the definite diagnosis. According to the literary survey and author's experience are formed two main groups of indices at echo-sounding study--direct and indirect. There have been considered the technics of echoscopy in searching the signs of different terms of pregnancy. It has been made a partial comparison of the reliability in the two groups ultrasound signs with this in biochemical diagnosis of spinal dysraphism in the fetus.

Acetylcholinesterase

Myelography with metrizamide in occult spinal dysraphism.

The initial experiences with metrizamide (Amipaque) in cases of spinal dysraphism are described. The technique, myelographic appearance and adverse reactions are discussed. The results have been promising and it is believed that this contrast medium will provide more accurate diagnostic information. However, further experience is necessary and in particular correlation with surgical findings.

Adolescent

Occult spinal dysraphism: a series of 73 cases.

The subject of occult spinal dysraphism or myelodysplasia is reviewed from standpoints of embryology, clinical manifestations, and treatment, and the management of 73 cases summarized. In general, these concealed lesions arise from developmental variants in the most distal part of the neural tube, a situation which may cause distortion or partial absence of neural tissues and also lead to damage from compression or traction. Lipomyelomeningocele and congenital dermal sinus are two exampled of the many types of such lesions, but some are more complicated and border-line myelomeningocele-like forms occur. Incontinence, deformity or weakness of the feet, impaired gait, and other difficulties may appear late and increase with growth. Surgical treatment is advised to reduce chances of delayed or progressive loss of function.

Adolescent

Computed tomography in spinal dysraphism.

Computed tomography of the spine has been used to evaluate patients with spinal dysraphism. With this technique, the nature of soft-tissue masses can often be correctly diagnosed and the underlying bony anomalies clarified.

Adult

A report of children with spinal dysraphism managed conservatively.

This investigation is a retrospective study of 12 patients with spinal dysraphism. There were 5 males and 7 females in the cohort. Their ages ranged from 4 to 13 years at the time of their last assessment. Initial presentation included one or more of the following--a blemish on the back in the midline (lump, hairy patch, sinus and/or angioma), abnormal neurological signs and symptoms in one or both legs, and in one case dribbling of urine. Investigations performed included plain X-rays of the spine, CT scans alone or in combination with a myelogram and MR scans. Findings included thickened filum terminale, low lying cord, lipoma, syringomyelia, diastematomyelia, spina bifida occulta and sacral agenesis. The children were followed up for between 2 and 10 years. During the period of observation, none developed new symptoms or signs, and there was no progression of existing neurological deficits. This preliminary report suggests that there is no justification for prophylactic surgery in this group of patients.

Adolescent

Computed tomography and spinal dysraphism: clinical and phantom studies.

Two cases illustrating the value of CT in the assessment of spinal dysraphic tissue are presented. In one case, the configuration and origins of two osseous diastematomyelic spurs were shown well; in the second case, the CT recognition of a sacral lipoma led to air myelographic confirmation of the tumor and tethered cord. CT phantom studies indicated that dysraphic tissues, such as fat, cartilage, and fibrous tissue, are better identified and quantitated in the spinal canal when surrounded by air. Varying degrees of image degradation occur with water (simulating CSF) or metrizamide.

Adult

Sacral dysgenesis associated with occult spinal dysraphism causing neurogenic bladder dysfunction.

A case of a neurogenic bladder in a 20-year-old man is described. The patient had difficulty in voiding and incontinence, with a history of several urinary infections. Misdiagnoses included stricture and posterior urethral valves. Early treatment included urethral dilation, a transurethral resection and a left ureteral implantation. Throughout this time the neurological examinations were essentially normal. Final diagnosis was sacral dysgenesis with associated occult spinal dysraphism, after the discovery of a small pock mark over the sacrum and an abnormal pelvic x-ray. Urinary diversion was done.

Adult

Physical and biochemical characteristics of the human dysraphic spinal cord.

The spinal cords were removed from 29 infants between 17 and 46 weeks gestation, all with various forms of spinal dysraphia. The spinal cords were assessed for weight, length, thickness, size and number of cells, and myelination, and then were compared with 86 control spinal cords. The strongest characteristic of the dysraphic cords was a reduced degree of myelination. Cell size and number generally were appropriate for the weight of the cords. There was a tendency for cord weight, length and thickness to be reduced, though in part this was a reflection of poor over-all bodily growth. In a small group of infants these measurements were increased in comparison with the controls.

Body Water