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PubMed2005

Blindness following ventriculoperitoneal shunt block in a child with spinal dysraphism: case report.

A six-year-old boy presented with a week history of increasing headache, visual loss and convulsions. He had repair of myelomeningocele early in life and later had insertion of a ventriculoperitoneal shunt for hydrocephalus after the repair. He was treated for meningitis at the referring hospital without improvement. Computed tomography scan of the brain showed ventricular enlargement. The shunt was found to be broken and blocked and was revised. This was followed by rapid improvement but vision was never regained. Shunt malfunction in patients with spinal dysraphism can lead to visual loss but the features may mimic those of meningitis, and delayed referral and treatment. Early shunt revision should prevent this complication.

Blindness↗
PubMed1987

Evaluation of MR in spinal dysraphism with lipoma: comparison with metrizamide computed tomography.

Magnetic resonance (MR) imaging and metrizamide computed tomography (CT) were performed on three patients with spinal dysraphism with lipoma. Sagittal MR images were superior to metrizamide CT reformatted sagittal images in demonstrating anatomic relationships of the spinal cord, filum terminale and lipoma. In the axial plane, CT proved superior to MR. In all cases, the T1 weighted images with exceptional spatial resolution and excellent fat discrimination were more helpful than T2 weighted images. Although both MR and CT are useful in the initial evaluation of these disorders, subsequent evaluation employing only MR is probably adequate. Promising developments in surface coil imaging may eventually replace CT.

Adolescent↗
PubMed2007

SACRAL syndrome: spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localization.

BACKGROUND: Publications concerning perineal infantile hemangiomas are scarce, and comprise no large series. OBJECTIVE: Studying clinical features of hemangiomas of the perineal area, complications and associated malformations. METHODS: Retrospective analysis of all hemangiomas localized in the perineal area, encountered at the Children's Hospital in Bordeaux from 1994. RESULTS: Of 49 perineal hemangiomas (34 girls, 15 boys), 5 patients had accompanying malformation, mainly lipomyelomeningocele with tethered cord. The superficial hemangiomas were more represented in males and presented sooner than the nodular counterpart. The average rate of ulceration was 73%, ulcerations developed earlier in the superficial forms than their nodular counterparts (2 vs. 4 months). CONCLUSION: Superficial perineal hemangiomas are more often complicated by ulceration, and are associated with developmental anomalies. As a counterpart for the PHACE syndrome in facial hemangioma, we propose the acronym SACRAL for perineal hemangiomas: Spinal dysraphism, Anogenital anomalies, Cutaneous anomalies, Renal and urologic anomalies, associated with Angioma of Lumbosacral localization.

Anal Canal↗
PubMed1989

Observations on spinal dysraphism.

An attempt has been made to look into the age at presentation, incidence and clinical presentation of 40 patients with spinal dysraphism. Thirteen cases of myelomeningocele and 11 cases of meningocele constituted the 'aperta' group and there were 16 'occulta' cases. Though among the 'aperta' cases male is to female ratio was 1:1, a marked male preponderance of 2.2:1 was seen among 'occulta' cases. First born child was found to be most frequently (44.50%) affected in the present series. Myelography remained a mainstay in deciding operation in 'occulta' cases. All 'aperta' cases and 11 out of 16 'occulta' cases were treated by operation. Regarding results of treatment, all cases with meningocele were fully cured, cases with myelomeningocele showed no improvement but further deterioration of the neurological status could be arrested. Hydrocephalus was associated in 8 out of 13 cases with myelomeningocele and ventriculoperitoneal (VP) shunt were instituted in all of them. Prognostic significance of presence of hydrocephalus was profoundly grave as was evident from the mortality rate (10%) in this series.

Adolescent↗
PubMed1997

Occult spinal dysraphism: neurogenic voiding dysfunction and long-term urologic follow-up

From 1976 to 1994, we followed 55 children with occult spinal dysraphism (OSD). The average age at diagnosis was 4.5 years (range: 24 days - 21 years). In 13 cases the OSD was associated with anorectal anomalies. Urologic symptoms were present at diagnosis in 24 children (43%), but urinary incontinence affected all patients in the evolution of the OSD. At diagnosis, all children underwent complete neurourologic and urodynamic evaluation. Nine required early neurosurgical correction, before 3 years of age. During follow-up, intermittent clean catheterization was started in all patients. Vesicoureteral reflux was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required bladder augmentation because of upper-tract and renal-function deterioration. Endoscopic treatment for urinary incontinence was performed in 3 children. At long-term follow-up (6 to 18 years), socially acceptable continence was achieved in 78% of the children; renal failure occurred in 8. The long-term results were analyzed in order to compare the evolution of urinary continence and renal function in children with OSD with or without neurosurgery.

Journal Article↗
PubMed1997

Occult spinal dysraphism: neurogenic voiding dysfunction and long-term urologic follow-up.

From 1976 to 1994, we followed 55 children with occult spinal dysraphism (OSD). The average age at diagnosis was 4.5 years (range: 24 days - 21 years). In 13 cases the OSD was associated with anorectal anomalies. Urologic symptoms were present at diagnosis in 24 children (43%), but urinary incontinence affected all patients in the evolution of the OSD. At diagnosis, all children underwent complete neurourologic and urodynamic evaluation. Nine required early neurosurgical correction, before 3 years of age. During follow-up, intermittent clean catheterization was started in all patients. Vesicoureteral reflux was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required bladder augmentation because of upper-tract and renal-function deterioration. Endoscopic treatment for urinary incontinence was performed in 3 children. At long-term follow-up (6 to 18 years), socially acceptable continence was achieved in 78% of the children; renal failure occurred in 8. The long-term results were analyzed in order to compare the evolution of urinary continence and renal function in children with OSD with or without neurosurgery.

Adolescent↗
PubMed2003

Multidisciplinary evaluation of occult spinal dysraphism in 47 children.

OBJECTIVE: To present the clinical (urologic, orthopedic and neurologic) and urodynamic findings of 47 children suffering from occult spinal dysraphism, together with the long-term follow-up results obtained with various treatment modalities. MATERIAL AND METHODS: Between 1997 and 2000 a total of 47 children (27 girls, 20 boys; male:female ratio 1.3) referred to the Urology and/or Pediatrics Departments with symptoms and signs of closed spina bifida were enrolled in the study program. All patients underwent routine assessment of the urinary tract, including detailed anamnesis, physical examination and radiologic evaluation (X-ray, renal bladder ultrasonography and sacral MRI). In addition to video-urodynamic evaluation of the lower urinary tract, all patients were also evaluated by the Orthopedic Department with respect to possible lower extremity deformities. RESULTS: The age range of the children was 2 months to 16 years (mean 6.9 years). At first referral, 23 children were found to have normal urinary and fecal continence after toilet training; among the other presenting symptoms and signs, 34% of patients demonstrated recurrent urinary tract infections and 38.2% had abnormal findings on urinary tract investigations. Evaluation of urodynamic parameters before and after conservative treatment demonstrated an increase in age-related bladder capacity in 34 patients and detrussor instability had been cured in 23/30 patients (p < 0.05). Overall, bladder capacity was found to be normal in 40 children following conservative management (p < 0.05). The conservative approach proved to be effective in 40 children (85.1%), and intravesical instillation therapy with oxybutynine hydrochloride was successful in one of the remaining seven children (14.2%). Bladder augmentation was performed in six children (12.7%) in whom conservative measures were ineffective. CONCLUSIONS: In the light of our findings and the literature data it is obvious that a multidisciplinary approach together with early urologic evaluation to determine the extent of neurologic involvement of the lower urinary tract is essential to ensure a successful treatment outcome and to prevent the occurrence of serious functional and structural complications. Clinical, radiologic and video-urodynamic assessments should be performed to define the neuro-urologic pathophysiology and to provide management guidelines and a baseline for future comparison.

Adolescent↗
PubMed1996

Occult spinal dysraphism--the common combination of lesions and the clinical manifestations in 50 patients.

Fifty patients (26 males, 24 females) aged from birth to 51 years with occult spinal dysraphism were identified. All had Magnetic Resonance (MR) scans carried out. The MR scans were examined to determine the vertebral level of the conus and to see if one or more of the following were present; lipoma, syrinx, dermoid, diastematomyelia, and meningocele. In 43 patients the conus lay below the level of L3, 23 had a lipoma, 23 a meningocele, 20 an open central canal in the spinal cord or a syrinx, 15 a diastematomyelia and 4 a dermoid. The commonest combination of lesions was a long cord, a syrinx or an open central canal in the spinal cord and a lipoma. The diastematomyelias were always associated with a long cord and had the highest incidence of vertebral body anomalies (60%). The most frequent recorded signs were deformities of the feet, short legs, wasting of the calf muscles, weakness of the legs, back pain and bladder dysfunction. Combinations of these occurred with all of the lesions although some had none. Whilst deterioration was seen in about half of the patients, acute deterioration was uncommon and was associated most frequently with a dermoid or an expanded syrinx.

Abnormalities, Multiple↗
PubMed1986

Occult spinal dysraphism. Case report and review of the literature.

This case report and review of the literature is presented to create a greater diagnostic awareness of occult spinal dysraphism. Early recognition is based upon an understanding of this congenital anomaly and its variable presentations. These most commonly include abnormal gait, various cutaneous manifestations, particularly subcutaneous lipomata, and less frequently urological complaints. Surgical intervention, to arrest disease progression, is the primary mode of treatment, and functional improvement is variable. Long-term prognosis is dependent upon severity of neurologic deficits prior to surgery and the type of lesion found intraoperatively. Familial occurrence has been reported and genetic counseling may be an important preventive measure. Recent radiologic investigations have been concerned with the use of ultrasonography in screening infants at risk.

Child↗
PubMed1996

[Closed spinal dysraphism. Apropos of 3 cases].

CASE REPORT: Closed spinal midline developmental defect was observed in three children, two girls aged 7 and 10 years and one boy aged 9 years. There were no similar cases in the families. The first signs appeared at approximately 6 months, 9 years and at birth. In the first two cases, developmental defects of the toes were followed a few months or a few years later by chronic ulcerations of the plantar surface of the foot. On physical examination there was a greater toe, amputation of the toes, perforating plantar ulceration, prolonged cauda equin and neurological disorders. Radiology revealed defective closure of the dorsal and/or lumbar vertebrae and lysis of the third phalanxes and magnetic resonance imaging showed diastematomyelia. The third child was born with a pilous nevus and a prolonged cauda equin. A soft subcutaneous tumor developed 3 years later over the lumbar area, increasing in size becoming quite voluminous and painful. In this case, there was no amputation of the toes or perforating plantar ulceration or neurological disorders. Radiography showed hemisacralization of L5 and spina bifida of S1. Magnetic resonance imaging showed a normal spinal cord with a tumor independent of the neural canal. Biopsy exeresis led to the diagnosis of neurofibroma. DISCUSSION: Closed spinal midline development defect occurs more readily in girls than in boys. Multiple and complex abnormalities are observed: diastematomyelia, diplomyelia, dermal fistulae, neuroenteral cysts, dysembryoplasic tumors, abnormal position of the spinal cord, and usually vertebral malformations. There are three types of clinical signs involving skin, bone and neurological manifestations. Radiological explorations should be completed with myelography and a computed tomography as well as magnetic resonance imaging to determine the extent of the malformations and possible surgical indications.

Child↗
PubMed2003

Occult spinal dysraphism: evidence-based diagnosis and treatment.

This article reviews the scientific evidence behind the diagnostic tools available for the appropriate workup and management of patients with occult spinal dysraphism (OSD). The diagnostic tools include the use of detailed history and physical examination, plain films, ultrasound, MR imaging, and neurophysiologic tests. In addition, the article discusses the epidemiology of the most common causes of OSD in children, which will allow physicians caring for children to develop a pretest probability of disease and make a more educated decision as to when additional diagnostic testing is required.

Evidence-Based Medicine↗
PubMedDate not supplied

Occult spinal dysraphism.

When spina bifida is associated with skin abnormalities such as dimples, sinus tracts hypertrichosis, or capillary hemangiomas, there is a high incidence of an occult intraspinal lesion such as epidermoid and dermoid tumours, lipomas, diastematomyelia, dural bands and tethered spinal cord. The present study consists of 50 patients with occult spinal dysraphism treated with the diagnosis of lipomeningomyelocoele (20), lumbosacral lipoma (15) and diastematomyelia (15). The clinical presentation varied from lipoma in the lumbosacral region, dermal sinus, cutaneous hemangioma and hypertrichosis. The age range varied from 2 months to 7 years with an average of 2 years. 40% patients had neurological deficit at the time of presentation which varied from lower limb weakness to bladder & bowel involvement. All patients underwent laminectomy of the lumbar and lumbo-sacral spine with excision of intraspinal lipoma, excision of bony or cartilaginous spur in diastematomyelia and detethering of the conus medullaris & cauda equina. No patient developed late neurological deficit.

Child↗
PubMed1998

Radiologic and histopathologic findings in a rare case of complex occult spinal dysraphism with association of a lumbar fibrolipoma, neurenteric cyst and tethered cord syndrome.

We present the case of a 60-year-old farmer who reported non-specific back pains for the past 30 years. Increasing paralysis of the left leg led to hospitalization. A complex occult spinal dysraphism comprising a fibrolipoma of the conus medullaris, an neurenteric cyst, and a tethered cord syndrome was diagnosed and operated on. The radiological and histological findings are demonstrated.

Follow-Up Studies↗
PubMed1994

Cutaneous stigmata of occult spinal dysraphism.

We describe a 4-year-old Hispanic boy with a dermal pit and an overlying macular vascular malformation in the lumbosacral area. Magnetic resonance imaging of the region revealed an intraspinal lesion at L1-L2. A fibrous tract was excised. A benign lipoma intrinsic to the roots of the cauda equina was noted at surgery. The cutaneous stigmata of occult spinal dysraphisms are reviewed.

Child, Preschool↗
PubMed1988

Occult spinal dysraphism in established congenital dislocation of the hip.

Of a consecutive series of 117 one-year-old infants with 130 established dislocations of the hip, 11% failed to respond to primary surgical treatment. Genetic and iatrogenic factors accounted for half the failures. There were no obvious causes in the remainder, though a few had the superficial stigmata of spinal dysraphism, and by two years of age, most of the group had developed a lateral rotation posture of the affected leg associated with a relatively smaller foot on that side. Radiologically, the femoral head had drifted and rotated laterally out of the surgically deepened acetabulum, causing persistent subluxation. Although there was no clinical evidence of sensory or motor denervation, sensory spinal evoked potential tests revealed the presence of neurological deficits in the majority of patients in this group.

Foot↗