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At least 973 records · Page 54Linked to original sources
PubMed1983

CT appearance of generalized von Recklinghausen neurofibromatosis.

Two cases of von Recklinghausen neurofibromatosis (VRN) with thoracic and abdominal involvement are described. Computed tomography demonstrated the typical lesions of this disease: meningoceles, neurofibromas, and skeletal alterations. In both cases lesion morphology and location were similar and characteristic, in particular in the pelvic region. Computed tomography findings in generalized VRN are sufficiently characteristic to be considered diagnostic for this disease.

Abdominal Neoplasms↗
PubMedDate not supplied

Dural ectasia of the optic nerve sheath in neurofibromatosis type 1: CT and MR features.

OBJECTIVE: Optic nerve glioma is a common finding in patients with neurofibromatosis type 1 (NF-1). Although dural ectasia of the optic nerve is encountered rarely, it has to be considered in the differential diagnosis. MATERIALS AND METHODS: Three patients affected by NF-1 were examined sequentially by CT and MRI. RESULTS: Computed tomography demonstrated nonprogressing enlargement of the anterior optic pathways. Magnetic resonance imaging displayed widening of the perioptic dural sheath without any suggestion of tumor. CONCLUSION: These findings are compatible with meningocele or dural ectasia of the optic nerve sheath. This entity represents a differential diagnosis to optic glioma in patients with NF-1.

Adolescent↗
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↗
PubMed2001

Long-term followup of newborns with myelodysplasia and normal urodynamic findings: Is followup necessary?

PURPOSE: A subset of newborns with myelodysplasia have normal bladder function on urodynamic assessment. We analyzed long-term followup in this population to determine the necessity for subsequent urological surveillance. MATERIALS AND METHODS: We retrospectively analyzed the records of 25 of 204 newborns (12%) with myelodysplasia in whom neurourological evaluation was normal after surgical repair of the spinal defect. Initial assessment included complete urodynamic study, renal ultrasound, urinalysis and urine culture. These patients were reevaluated every 3 months until age 3 years, semiannually until age 6 years and yearly thereafter. The longest followup was 18.6 years. RESULTS: Of the 25 newborns 22 had myelomeningocele and 3 had meningocele. During a mean followup of 9.1 years urodynamics subsequently showed neurourological deterioration in 8 children (32%). No changes in urodynamics were observed in any patient older than 6 years. All children with neurourological deterioration underwent magnetic resonance imaging, which confirmed a tethered spinal cord that was then surgically corrected. After the untethering procedure 2 patients (25%) regained normal voiding function, whereas in 6 (75%) mild or moderate neurogenic bladder dysfunction persisted. CONCLUSIONS: Newborns with myelodysplasia and initially normal urodynamic studies are at risk for neurological deterioration secondary to spinal cord tethering, especially during the first 6 years of life. Close followup of these children is important for the early diagnosis and timely surgical correction of tethered spinal cord, and for the prevention of progressive urinary tract deterioration.

Female↗
PubMed2004

Transnasal endoscopic repair of cerebrospinal fluid rhinorrhea and skull base defect: ten-year experience.

OBJECTIVES/HYPOTHESIS: Many reports have advocated the feasibility of using an endoscope for the treatment of cerebrospinal fluid (CSF) rhinorrhea and skull base defect, and diversified endoscopic techniques and repairing materials have recently been proposed. This study determined the effectiveness of endoscopic repair of CSF leaks and interpreted the indications of the lumbar drain. STUDY DESIGN: Retrospective follow-up. METHODS: A total of 39 patients with CSF rhinorrhea (20 traumatic, 13 iatrogenic, and 6 spontaneous) were treated in one institution over a 10-year period. An underlay procedure was used to make eight (20.5%) repairs by way of a turbinate composite graft (4 patients), turbinate mucosa graft (3 patients), or the Dura Substitute (Preclude) (1 patient). A free turbinate graft with the applied fibrin glue was used to repair the defect by way of an overlay procedure in 23 (59.0%) lesions. Abdominal fat was used to repair the other eight (20.5%) lesions. RESULTS: Defects in the cribriform plate and anterior ethmoid sinus comprised the majority (61.5%) of all lesions. The successful rates for overlay, underlay, and fat obliteration procedures were 91.3% (21/23), 87.5% (7/8), and 100% (8/8), respectively. There were no statistical difference between underlay and overlay techniques (P = .792, Student's t test). Lumbar drainage was performed in 18 of 39 (46.2%) cases. Most patients required nasal packing (89.7%) and prophylactic parenteral antibiotics (97.4%). CSF rhinorrhea was resolved during the first attempt in 36 of 39 (92.3%) patients. All leaks were successfully repaired after a second attempt. No major complications were encountered. CONCLUSION: The endoscopic approach is safe and effective for the treatment of CSF rhinorrhea, even in the cases not successfully treated by previous neurosurgical approaches. Lumbar drain was suggested for defects in the frontal and sphenoid sinus and defects associated with meningocele or encephalocele. Complete exposure of the defect, appropriate selection of a fitting graft, as well as accurate placement and stabilization of the graft are critical to the success of repair.

Adipose Tissue↗
PubMed1989

Correlation between magnetic resonance imaging and surgical findings in the tethered spinal cord.

Between October 1982 and August 1987, 20 patients underwent magnetic resonance imaging (MRI) and subsequent surgical release of a tethered spinal cord. The tethering was caused by a thick filum terminale in 6 patients. On MRI scans, the conus medullaris was at L4 in 2 patients, at L2 in 3 patients, and the filum terminale appeared thick in 1 patient. The spinal cord was tethered to an intradural lipoma correctly demonstrated by MRI in 6 patients. Increased epidural fat was misdiagnosed as an intradural lipoma in one patient and a lipomatous stalk was not identified in 2 other patients. Scar tissue resulting from repair of a meningocele had tethered the cord in the remaining 8 patients. On MRI scans, the conus medullaris was located between L3 and S3; in 5 of the patients, scar tissue was apparent on the MRI scan. This correlative study supports the use of MRI as the initial, and possibly the only, imaging modality when a tethered spinal cord is suspected. Improved or more recent MRI techniques will help demonstrate these anomalies better.

Adolescent↗
PubMed1997

Anterolateral lumbar lipomyelomeningocele: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Meningoceles associated with defects of the abdominal wall are exceedingly rare. One such complex case is presented along with a review of the relevant literature. The current pathophysiological theories and surgical management are discussed. CLINICAL PRESENTATION: A case of a patient with an anterolateral lumbar lipomyelomeningocele associated with multiple congenital anomalies, including defects in the abdominal wall and urogenital system, is presented. The lipomyelomeningocele presented as an expanding abdominal mass. INTERVENTION: Ventriculoatrial shunting and two operations to repair the myelomeningocele were performed to control the expanding abdominal mass. CONCLUSION: This report illustrates that the surgical management of complex lipomyelomeningoceles is similar to the more common types. It also demonstrates that in the infant in whom the intra-abdominal cerebrospinal fluid collection is the primary cause of the symptoms, cerebrospinal fluid shunting may be used to delay the definitive repair until the dura strengthens, thus avoiding the complications of complex repairs of dura with low tensile strength.

Abdominal Neoplasms↗
PubMed1978

Traumatic avulsion of the lumbar nerve roots.

Lumbar nerve root avulsion is rare but may follow severe traction injury of the pelvis. Diagnosis is based on neurologic deficit and myelographic demonstration of traumatic meningocele. Nerve root avulsion should be differentiated from peripheral nerve injury because surgical treatment of nerve root avulsion has uniformly proved futile.

Adult↗
PubMed1984

Antenatal diagnosis of Turner's syndrome.

The prenatal identification of a cystic mass in the region of the fetal cervical neck should raise the possibility of a cystic hygroma which is commonly associated with Turner's syndrome. Differential diagnosis should also include benign cystic teratoma, meningocele, encephalocele, and meningoencephalocele. Careful sonographic evaluation of the fetus can help differentiate these possibilities.

Adult↗
PubMedDate not supplied

Congenital cervical scoliosis with unilateral congenital nerve deficit in the upper extremity. Report of two cases.

Two patients are reviewed, each having an unusual clinical syndrome of progressive congenital cervical scoliosis and unilateral arm paralysis. This appears to be a unilateral developmental defect in both mesenchymal and neural tissue. There was a unilateral spina bifida without myelomeningocele, meningocele, or lipoma. The cervical spine deformity was progressive at a young age, one patient having been fused at age 2 years, the other at age 5 years. At surgery, the dura was intact but unprotected on the concavity of the curve. Bridging bone grafts were utilized to span the defects. The arm paralysis was extensive in both patients, but improvement in function was accomplished by appropriate reconstructive procedures.

Adult↗
PubMed2003

Lumbopedal skin pedicle in an infant with the amniotic band syndrome: a Disorganization-like defect?

A female infant is presented in whom anomalies not commonly seen in amniotic band syndrome (ABS) included non-anatomic lumbopedal union by a skin pedicle, anorectal malformation, meningocele, vertebral segmentation defects, longitudinal limb defects, vestigial feet and skin papillae. The unusual, non-band related malformations in the proposita showed similarity to the mouse mutant Disorganization (Ds). This report, supports the suggestion that Ds-like mutations may cause some cases with apparent ABS associated with "skin pedicles".

Abnormalities, Multiple↗
PubMedDate not supplied

Dural ectasia in the Marfan syndrome: MR and CT findings and criteria.

PURPOSE: To create criteria for detecting dural ectasia on MR or CT images in adult Marfan patients. METHODS: Images were analyzed using a workstation. Parameters that predicted dural ectasia were included in our criteria. RESULTS: Major criteria include: (1) width of dural sac below L5 > width above L4; (2) anterior sacral meningocele. Minor criteria include: (1) L5 nerve root sleeve diameter > 6.5 mm and (2) S1 scalloping > 3.5. Dural ectasia exists if 1 major or 2 minor criteria are present. CONCLUSION: MR and CT diagnose dural ectasia with high specificity and sensitivity. Our criteria accurately diagnose dural ectasia in adult Marfan patients.

Adult↗
PubMed1981

Epidural block during labour in a patient with spina bifida cystica.

A case report is presented of a patient in whom a lumbosacral meningocele had been successfully removed in the neonatal period and who later received a lumbar epidural block for pain relief during labour. Although a high level was chosen for the puncture, deficiency of the dorsal interspinous layer of ligaments complicated the localisation of the epidural space when the 'loss of resistance' technique was applied. Analgesia was achieved more rapidly and with a lower dose of analgesic drug than is normally necessary for satisfactory relief of pain in labour.

Adult↗
PubMed1998

Cell proliferation rate and nuclear morphometry in Roberts syndrome.

Roberts syndrome (RS) is a rare autosomal recessive disorder characterized primarily by symmetric reduction anomalies of all limbs, growth retardation and craniofacial abnormalities. Most RS patients are reported to present a typical abnormality of their constitutive heterochromatin, accompanied by abnormal cytological growth characteristics. We present an extremely severe case of an RS fetus, karyotypically documented, with a clinical presentation including growth deficiency, tetraphocomelia, frontal meningocele, craniofacial abnormalities and penile enlargement with hypospadias. Nuclear morphometrical analysis in tissues of various organs revealed a reduced nuclear size in RS as compared to normal controls, and statistically significant differences in morphometric parameters related to the nuclear shape. Immunohistochemical study of the same organs showed a reduced expression of proliferating cell nuclear antigen in the presented case, thus indicating a decreased cell proliferation rate in RS. Our results reconfirm previously reported findings in cultured fibroblasts of RS cases, thereby reinforcing on a histologic level, the hypothesis that reduced cell proliferation may be involved in the growth retardation and the reduction abnormalities observed in RS.

Abnormalities, Multiple↗
PubMed1990

Associated multiple congenital malformations in domestic animals. Contribution of four cases.

The incidence and significance of the appearance of various associated congenital malformations in a single individual have received poor attention in veterinary medicine. In this article, the malformations exhibited by four animals pertaining to three different animal species are described, and the possible reasons for the association of these anomalies are discussed from the viewpoint of the comparative medicine. All four animals showed two or three malformations constituting unrecognized teratological syndromes, so-called one-of-a-kind syndromes, which have not previously been described, at least in these particular animal species. A pig was affected by a series of craniofacial anomalies consisting in a first arch syndrome together with an occipito-parietal meningocele. Another member of the porcine species showed holoprosencephaly with cyclopia, various defects in the left forelimb and an extensive omphalocele. A dicephalic cat with cervical rachischisis and myeloschisis and a dog exhibiting spina bifida, curled tail and atresia ani are also described.

Abnormalities, Multiple↗
PubMed2004

C677T mutation in the MTHFR gene was not found in patients with frontoethmoidal encephalocele in East Java, Indonesia.

BACKGROUND: Frontoethmoidal encephalocele (FEE) is a neural tube defect (NTD) characterized by a congenital bone defect in the anterior cranium and herniation of the intracranial mass through the defect. The C677T mutation in the 5,10-methylenetetrahydrofolate reductase gene (MTHFR) has been reported as a genetic risk factor for spina bifida. However, the role of the MTHFR in the pathogenesis of FEE remains to be clarified. METHODS: A hospital-based survey of FEE patients who were referred to the Department of Neurosurgery and Plastic Surgery, Malang General Hospital, East Java, Indonesia was conducted. Genetic screening of MTHFR substitutions in 13 patients and eight mothers from 11 affected families were performed using a combination of polymerase chain reaction (PCR), denaturing high-performance liquid chromatography (DHPLC), and direct sequencing. RESULTS: In total, 130 patients with FEE among 138 NTD patients (94.2%) were identified. The ratios of cranial encephalomeningocele to spinal meningocele (32 : 1) and of FEE to occipital encephalomeningocele (32 : 1) were higher than those in other populations. Five substitutions were detected in the MTHFR: C121T, C677T, C1060T, A1298C, and G1793A. No significant differences were found in the frequency of each nucleotide substitution between patients or mothers and controls. In addition, none of the subjects in this study were homozygous for T at nucleotide position 677. CONCLUSION: FEE is the most common form of NTD in East Java, Indonesia. Genetic analysis of 11 affected families suggests that the MTHFR gene is not associated with the development of FEE, although the number of FEE families analyzed in this study was very limited.

Adult↗
PubMed1991

Retinochoroidal (optociliary) shunt veins, blindness and optic atrophy: a non-specific sign of chronic optic nerve compression.

Fifteen patients are described in whom the triad of blindness, optic disc swelling followed by optic atrophy, and optociliary shunt veins occurred. The causes of the syndrome included spheno-orbital meningioma, optic nerve glioma, meningocele of the optic nerve, and chronic papilloedema. It is postulated that chronic compression of the intraorbital portion of the optic nerve produces gradual obstruction of the central retinal vein, thus preventing the normal passage of venous blood from the retina through the central retinal vein to the cavernous sinus. Optociliary veins are a pre-existing shunt system that allows retinal venous blood to bypass the central retinal vein and exit from the orbit via the choroidal circulation and its anastomoses.

Adolescent↗
PubMed1976

The reliability of prediction of outcome in spina bifida.

The clinical findings in 85 neonates with spina bifida were given to two neurosurgeons and two paediatricians, who were asked to predict from them the length of survival and quality of survival with regard to intellect, locomotion and continence, without their knowing the actual outcome. All four clinicians correctly predicted the survival of infants with meningocele, closed myelocele and encephalocele. The paediatricians correctly predicted the survival of all infants with open myelocele who actually survived, but also included some who had died. The surgeons correctly predicted the deaths of all those with open myelocele who actually died, but expected a considerable number to die who in fact survived. All four clinicians were similar in their predictions of intellect: they underestimated the outcome in patients with successfully shunted hydrocephalus, they overestimated the intellect in patients who had developed intracranial infection and shunt blockage, and they largely underestimated the outcome in the patients who did not require shunts. They made correct predictions for limb and sphincter function in nearly all the survivors. This investigation underlines the problem of selection for treatment caused by the inability to predict the complications of hydrocephalus and infection. Reasons for the differences between the expectations of the paediatricians and surgeons, and the implications of the results of this study for selection for surgery are discussed. It is suggested that limb paralysis and incontinence ought not to be considered as factors excluding infants from treatment.

Child↗