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Results for “Spinal Dysraphism”

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PubMed1997

Assessment of maternal anxiety following neonatal screening and investigation for occult spinal dysraphism.

The objective was to determine maternal anxiety and attitudes associated with a programme of neonatal screening and investigation for occult spinal dysraphism. Questionnaires were completed after diagnostic investigation (time 1) and 6 months later (time 2) by 83 mothers of babies with possible markers of occult spinal dysraphism who were normal on spinal ultrasound. Outcome measures were: attitudes to the programme; maternal adjustment and attitudes to the baby; the State-Trait Anxiety Inventory. Fifty-four other postnatal mothers formed the comparison group for maternal adjustment. No significant differences were found between investigation group (time 1) and comparisons on measures of maternal adjustment. Anxious mothers gave more negative responses to some maternal adjustment items but not to attitudes to the programme. Mean state anxiety at time 1 (33.66) and time 2 (33.69) and mean trait anxiety (36.23) were not higher than in mothers of normal babies. It can be concluded that a programme of investigation for neonatal abnormalities that pays attention to detail need not cause excessive maternal anxiety. However, some mothers remain anxious even after receiving normal results. Anxiety can be assessed during appraisal of a new investigation programme, but refinements are needed.

Adaptation, Psychological↗

PubMed1998

Pre-natal diagnosis of occult spinal dysraphism by ultrasonography and post-natal evaluation by MR scanning.

Eight children born with occult spinal dysraphism were diagnosed in utero by ultrasonography. Post-natally, they were evaluated by MR scans. The ultrasound scans in all 8 fetuses revealed a spina bifida: the spinal cord was long in 5 and in 3 a meningocele was thought to be present, however, in 1, a post-natal MR scan revealed a lipomeningocele instead of a simple meningocele. In 3 fetuses an echogenic area was seen on the ultrasound scan which raised the possibility that an intradural lipoma was present, and was confirmed by post-natal MR scans. In 3 fetuses vertebral body anomalies and an additional ossification centre in a midline bony spur together with widening of the spinal canal were seen in the lower lumbar region. The lesions in all 8 fetuses were skin-covered. None had ventriculomegaly or an Arnold-Chiari malformation. The prognosis for fetuses with spina bifida aperta is well documented in contrast to that for those with spina bifida occulta. The ability to identify a spinal dysraphic lesion pre-natally allows a more accurate assessment to be made of the likely outcome for an individual fetus.

Female↗

PubMed1977

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↗

PubMed2001

Tethered cord syndrome and occult spinal dysraphism.

Tethered cord syndrome is a progressive form of neurological deterioration that results from spinal cord tethering by various dysraphic spinal abnormalities. The syndrome, treatments, outcomes, and current controversies are reviewed.

Humans↗

PubMed2006

A simple method to deter retethering in patients with spinal dysraphism.

OPERATIVE TECHNIQUE: The authors describe a technique that may be of benefit to patients with spinal dysraphism and a tethered spinal cord. Twenty pediatric patients (ten initial operations and ten reoperations) after the detethering of their spinal cord had intradural retention sutures placed with subsequent duraplasty using autologous thoracolumbar fascia. RESULTS: To date, no patient has had signs or symptoms or recurrence of signs or symptoms of a tethered spinal cord. No complications have resulted from this maneuver. The mean follow-up time for this cohort was 8 years. The advantages of this intervention include maintaining a relatively normal position of the spinal cord within the thecal sac, thus decreasing the potential adherence of the dorsally scarred aspect of the dysmorphic cord to an overlying graft whether synthetic or native.

Follow-Up Studies↗

PubMed1994

Skin lesions of the spinal axis and spinal dysraphism. Fifteen cases and a review of the literature.

OBJECTIVE: To catalog the paraspinal skin lesions of early childhood that are associated with occult spinal dysraphism. RESEARCH DESIGN: Retrospective review of a series of patients. SETTING: Tertiary care referral center. PATIENTS: Fifteen patients who had significant paraspinal skin lesions were identified from the personal files of the authors who saw them. RESULTS: The skin lesions included various combinations of hyperpigmentation, hypopigmentation, hypertrichosis, acrochordons, dimples, lipomas, hemangiomas, or teratomas. Not all lesions were evaluated with the same tests, which included plain roentgenography, ultrasonography, myelography, computed tomography, and magnetic resonance imaging. Of the 15 patients, six had spinal anomalies, eight had no apparent spinal dysraphism, and one had insufficient results of the evaluation to assess the spinal column. CONCLUSIONS: Early recognition of paraspinal skin lesions is essential to prevent neurologic damage. Urinary or fecal incontinence, recurrent urinary infections, muscle atrophy, foot deformities, weakness, pain, or decreased sensation in the lower extremities may eventually develop in these patients. Magnetic resonance imaging appears to be the single best screening test for dysraphism.

Back↗

PubMed2004

Skin markers of occult spinal dysraphism in children: a review of 54 cases.

OBJECTIVES: To verify the diagnostic value of lumbosacral midline cutaneous lesions in asymptomatic children to detect occult spinal dysraphism (OSD) and to propose a practical approach for clinical investigations with respect to the type of cutaneous lesions observed. DESIGN: Retrospective study of 54 children referred to the Department of Pediatric Dermatology between 1990 and 1999 for congenital midline lumbosacral cutaneous lesions. SETTING: The private or institutional practices of participating dermatologists and pediatricians. MAIN OUTCOME MEASURES: Evaluation of the diagnostic value of midline cutaneous lesions for the detec-tion of OSD. Association of skin examination findings with spinal anomalies detected by magnetic resonance imaging or ultrasound. RESULTS: Occult spinal dysraphism was detected in 3 of 36 patients with an isolated congenital midline lesion and 11 of 18 patients with a combination of 2 or more different skin lesions. CONCLUSIONS: A combination of 2 or more congenital midline skin lesions is the strongest marker of OSD. Careful dermatologic examination is needed to detect suggestive markers and request a spinal magnetic resonance image, which is the most sensitive radiologic approach to detect an OSD.

Adolescent↗

PubMed1992

[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↗

PubMed1977

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↗

PubMed1975

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↗

PubMed1989

Spinal dysraphism with accessory male genital organs (a case report).

Clinico-Radiological and operative observations of a case of spinal dysraphism with accessory male genital organs are presented. Operative observations substantiated the presence of myelomeningocele in association with the spinal dysraphism in a case with normal sized penis, urethra and scrotum on top of a huge swelling over the back.

Genitalia, Male↗

PubMed1999

Extrarenal nephroblastic proliferation in spinal dysraphism. A report of 4 cases.

Four cases of extrarenal nephrogenic proliferation in the sacrococcygeal region with spinal dysraphism are presented. In two of the cases, features of Wilm's or incipient Wilm's tumor were present. The previous literature on sacrococcygeal nephrogenic tissue is reviewed, and the impact of these findings on the histogenesis of extrarenal sacrococcygeal Wilm's tumor is discussed.

Choristoma↗

PubMed1995

The effects of delayed diagnosis and treatment in patients with an occult spinal dysraphism.

From 1987 to 1993, 21 older individuals presented for the first time with signs and symptoms that eventually led to the diagnosis of occult spinal dysraphism. Assessment consisted of a neurological examination, urodynamic studies preoperatively and postoperatively, and spinal cord imaging. Of 21 patients 18 had an abnormal neurological examination, whereas only 15 had an abnormal urodynamic study, as judged by sphincter electromyography. Radiological imaging showed that 9 patients had a tethered cord alone, 4 each had lipomeningocele and lipoma, 2 had a bony spine abnormality and 1 each had thoracic meningocele and diastematomyelia. Of the 21 patients 19 underwent spinal surgery. Postoperatively, the neurological examination improved in 1 case (5%) and remained unchanged in 18 (95%), while urodynamic findings improved in 3 (16%), were unchanged in 11 (68%) and worsened in 5 (26%). Six patients had progressive deterioration and required secondary spinal surgery, which helped only 2 (33%). These observations confirm that older children and adults with occult spinal dysraphism are more likely to present with irreversible urological and neurological findings than younger children, and so it is imperative that a diagnosis be made and treatment be instituted as early as possible.

Adult↗

PubMed1998

Closed spinal dysraphism: a review on diagnosis and treatment in infancy.

This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of closed spinal dysraphism in infancy. Four groups of symptoms are distinguished: (1) cutaneous abnormalities, (2) lower motor neuron dysfunction due to congenital spinal and nerve root abnormalities, (3) upper motor neuron dysfunction due to tethering of the spinal cord, and (4) symptoms caused by associated malformations. Guidelines are proposed concerning timing and type of diagnostic investigations in infancy. This essentially encompasses a neurological assessment, including spinal ultrasonography and nuclear magnetic resonance imaging of the spine and the brain, and a urological assessment, including ultrasonography of kidneys and bladder, cystourethrography and a urodynamic study. As to the tethered cord syndrome it is concluded that first, already in infancy, a progressive neurological dysfunction can be detected; second, surgical untethering should be performed only upon appearance of upper motor neuron signs or upon progression of lower motor neuron signs. Despite its frequently asymptomatic course, the diagnosis of a congenital lumbosacral lipoma, and in the more general sense, of a closed spinal dysraphism, implies a periodic, multidisciplinary follow-up for life.

Humans↗

PubMed2004

Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism.

OBJECT: Historically, cutaneous stigmata representative of occult spinal dysraphism (OSD) have included lumbar hemangiomas. Frequently, this skin change is found in conjunction with other cutaneous alterations such as dermal sinus tracts and subcutaneous lipomas. Debate has recently surrounded the question of whether these skin changes in isolation might indicate underlying spinal disease. The authors reviewed their experience in their most recent 120 cases in which OSD was diagnosed. METHODS: The authors retrospectively reviewed records obtained in 120 patients with OSD. They found that many of the patients reviewed harbored only a flat capillary hemangioma as an indicator of OSD. In 21 patients (17.5%) with only midline lumbosacral flat capillary hemangiomas, underlying OSD was present. No single variety of OSD had a higher incidence of association with this single cutaneous stigma. CONCLUSIONS: Based on their experience, the authors recommend magnetic resonance (MR) imaging evaluation in cases involving this skin lesion in isolation to discern the potential for surgically significant spinal cord anomalies. Prospective studies are now needed to examine MR images obtained in all children with this lesion in the midline lumbosacral spine and assess for OSD.

Child↗

PubMed1991

The mermaid malformation: cloacal exstrophy and occult spinal dysraphism.

Five infants with cloacal exstrophy underwent neurological evaluation and radiographic examination of the caudal spine shortly after birth. Each was found to have occult spinal dysraphism. Four had terminal myelocystoceles, and one had a lipomyelomeningocele. Pathological anatomy was confirmed during surgery for the release of the tethered spinal cords. The striking association between cloacal exstrophy and occult spinal dysraphism suggests a common developmental defect in the caudal pole of the embryo. A hypothesis is offered to explain this association. Terminal myelocystocele and lipomyelomeningocele appear to be part of a continuum of lesions associated with skin-covered spina bifida.

Abnormalities, Multiple↗

PubMedDate not supplied

[Recurrent meningitis as a manifestation of spinal dysraphism in a young adult].

INTRODUCTION: Dysraphias are a varied set of anomalies affecting neuroectodermic tissue caused by the alteration of the neural tube during embryogenesis. Neuroradiological classification is broad and clinical manifestations are numerous: they affect the skin, osteomuscular tissue and the vascular, urological and nervous systems. We describe the case of a young adult with infrequent spinal dysraphism, which appeared as recurrent meningitis, and we stress the importance of studies using neuroimaging to define dysraphias. CASE REPORT: Male aged 23 with a history of urinary sphincter dysfunction, spina bifida and meningitis in infancy. He was admitted for treatment for recurrent meningitis and neuroradiological exploration revealed a hidden spinal dysraphism associated with spina bifida, sacrococcygeal dermal sinus with a fistular duct and communication with the dural sac, epidural lipoma and anchored spinal cord. The patient was submitted to decompression laminectomy, the abovementioned sinus was cut and the lipoma was removed. CONCLUSIONS: When faced with a case of recurrent meningitis, one of the etiopathogenic mechanisms to be taken into account is the presence of different malformations that can act as a point of entry to the nervous system not only in the cranial area but also, as in our case, in the spinal region. Late diagnosis of the cases is infrequent, but the presence of spina bifida with skin and urological manifestations point to possible malformations of the neural tube. Early surgical treatment must be performed in order to prevent neurological complications, which are potentially serious and give rise to high morbidity and mortality rates.

Adult↗

PubMed1988

Anorectal malformations: evaluation of associated spinal dysraphic syndromes.

The early recognition and treatment of correctable lesions of the terminal spinal cord in patients with anorectal malformations may preserve important neurologic function. Tethered cord and intraspinal masses are detectable with the use of high-resolution ultrasonography in the neonate. Fourteen infants and children with anorectal malformations and associated spinal dysraphism have been identified in our institution over the past 7 years. Six patients had cloacal exstrophy, and eight had imperforate anus (four high and four low lesions). The spinal lesions caused symptoms in only seven children; progressive neurologic deficit in five, and urinary incontinence or retention in two others. Five asymptomatic patients with cutaneous abnormalities on the back were studied and two were discovered during scoliosis evaluation. Imaging techniques included high-resolution ultrasonography, computed tomography with and without metrizamide myelography, and magnetic resonance imaging. Spinal sonography was highly accurate in the neonatal period. The application of ultrasonography can be of great advantage in early screening of patients with anorectal malformations and, in some cases, may eliminate the need for invasive imaging techniques.

Anus, Imperforate↗