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Results for “Spinal Dysraphism”

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PubMed1998

Endoscopic treatment for urinary incontinence in children with a congenital neuropathic bladder.

OBJECTIVE: To verify, in a retrospective analysis, the effectiveness of endoscopic treatment (collagen injection) in children with neurogenic bladder and neurogenic urinary incontinence. PATIENTS AND METHODS: From January 1992 to March 1997, 36 endoscopic collagen injections were performed to treat neurogenic urinary incontinence in 23 patients (mean age 10.9 years, range 6-17 at the time of the first procedure) selected on the basis of clinical status and the patient's motivation. Nineteen patients were affected by myelomeningocele and four had an occult spinal dysraphism. All patients underwent a preoperative cystometric urodynamic evaluation (without urethral pressure measurement) which showed detrusor areflexia in 12, normal reflexia in one and hyper-reflexia in 10 patients. Bladder compliance was considered good (> or =20 cmH2O) in 13 patients and low (10-20 cmH2O) in 10, four of whom had detrusor areflexia. Twenty-one children emptied their bladder by intermittent clean catheterization. The mean (range) follow-up was 19.2 (6-54) months. Twenty (group A) and 16 (group B) procedures, performed early and late in the series, were analysed separately to determine any increase in effectiveness with surgical experience. The evaluation criteria were; the increase in the 'dry' interval (between catheterizations) before and after treatment/s (delta dry); the patient's and parent's satisfaction; the number of endoscopic procedures and quantity of injected materials. RESULTS: There was an improvement (dryness of at least 2 h: delta dry of 1 h) in 13 of 23 patients after 1-3 (mean 1.5) procedures; 10 patients had a 0.2 h increase in delta dry after 1-3 injections and none was satisfied. There was no difference in the delta dry (0.9 vs. 0.7 h) between groups A and B, and/or the patient's/parent's satisfaction. Success rates differed with urodynamic patterns; seven of 10 patients showing no improvement had hyper-reflexia and three of the remaining 12 with areflexia had hypocompliant bladders, while nine of the 13 showing improvement had an areflexic detrusor and low-pressure bladders. CONCLUSION: The efficacy of the treatment depends largely on the urodynamic selection of patients (mainly those with detrusor areflexia and good compliance). The outcome may be improved further by increasing the quantity of injected material and with a longer follow-up, performing the procedure again if advisable. A close postoperative clinical (pad test) and personal observation is necessary to evaluate the outcome and thus optimize the strategy of treatment.

Adolescent↗

PubMed2005

Comparative study of complex spina bifida and split cord malformation.

OBJECTIVE: To see the difference in clinical profiles, radiological findings and surgical outcome of the group 1 split cord malformation and meningomyelocele (SCM with MMC) from group 2 (SCM without MMC). METHODS: 46 patients of SCM were selected from a total of 138 cases of spinal dysraphism. They were divided into two groups, based on presence or absence of MMC. Group I (SCM with MMC) n =19 patients and Group II (SCM without MMC) n=27 patients. A detail clinical evaluation and MR screening of whole spine of all cases was performed. All patients underwent surgical detethering of cord. After an average follow-up of 1.7 years, the operative results were clinically assessed and statistical significance was calculated. RESULTS: Male to female ratio was 1:09. Mean age of presentation was 3.6 years. Cutaneous markers like tuft of hair, cutaneous haemangioma, etc, had a higher incidence in group II in comparison to group I (50% vs 10.5%). The incidence of motor deficits was significant in group I in comparison to group II (63% vs 40%). The incidences of sensory loss, trophic ulcers, sphincteric dysfunction and muscle atrophy were relatively more common in group I patients, while neuro-orthopedic deformities such as congenital telepes equinovarus (CTEV), scoliosis and limb shortening were more frequent (67%) in group II children as compared to group I (53%). Type I SCM has higher incidence in group I children. Low lying conus were found in 47% patient of group I, while in group II it was noticed in 69%. The associated cranial anomalies like hydrocephalus, ACM and syrinx, were slightly higher in group I patients. At surgery, dysgenetic nerve roots, neural placode, arachnoid bands and atrophic cord were seen mainly in group I. Postoperative complications like, CSF leak, pseudomeningocele and meningitis were more commonly encountered in group I patients. The patients of group II showed better operative outcome compared to group I cases. CONCLUSION: Incidence of SCM with MMC amount to 41% of total SCM cases. Progressive neurological deficit was higher in this group (SCM with MMC) in comparison to the group harboring SCM without MMC. In view of a significant association of SCM in MMC cases, associated with other craniospinal anomalies, a thorough screening of neuraxis (by MRI) is recommended to treat all treatable anomalies simultaneously for desired outcome.

Adolescent↗

PubMed2006

Imaging of congenital anomalies and variations of the caudal spine and back in neonates and small infants.

Spinal dysraphisms are categorized in open dysraphisms with prominent abnormal nervous tissue above the skinlevel and closed dysraphisms with a skin covered malformation. Especially the occult dysraphisms are marked by suspect skin masses and other dermal anomalies. The purpose of this review is to demonstrate the indications and spectrum of spinal sonography in neonates and infants. In comparison typical dysraphisms are demonstrated in sonography and MR Imaging. We demonstrate the value of ultrasound in comparison to MRI and describe a usefull handling of the methods in neonates and infants. The differentiation between the potentially dangerous dimples associated with dermal sinus, which can lead to meningitis and the harmless coccygeal dimple in the cranial gluteal cleft is presented. An inconspicious examination does not need a further imaging, but suspicious results of sonography need an MR imaging dependent of clinical conditions. Neurologically conspicious infants need MR imaging completed by sonography. Great advantages of sonography are the real time examination and the potential to show oscillations of the conus, filum and cauda equina in M-mode-imaging.

Diagnosis, Differential↗

PubMed2000

Prenatal ultrasound evaluation of fetal diastematomyelia: two cases of type I split cord malformation.

Isolated diastematomyelia is a rare form of spinal dysraphism characterized by a sagittal cleft in the spinal cord, conus medullaris and/or filum terminale with splaying of the posterior vertebral elements. This condition is the result of the presence of an osseous or fibrocartilaginous septum producing a complete or incomplete sagittal division of the spinal cord into two hemicords. It may be isolated or associated with other segmental anomalies of the vertebral bodies. Prenatal diagnosis of this anomaly is possible in the early midtrimester by sonography, thus allowing for early surgical intervention and a favorable prognosis. Two cases of fetal diastematomyelia diagnosed by prenatal sonography are presented, each demonstrating the typical sonographic features diagnostic of this condition. The first case, detected at 28 weeks' gestation, presented with disorganization of the bony processes of the vertebral column with a midline echogenic focus. The second fetus, diagnosed at 17 weeks' gestation, had a similar appearance with widening of the posterior elements and the presence of a midline echogenic bony spur. Postnatally, both infants underwent magnetic resonance imaging for a definitive diagnosis. Surgical repair of the defect was performed in the neonatal period in both cases.

Adult↗

PubMed1980

[The value of computer tomography in the diagnosis of congenital spinal malformations particularly diastematomyelia (author's transl)].

The value of computer tomography (CT) in the diagnosis of diastematomyelia (DM) was studied in our own clinical material. Thirty-one patients with congenital spinal anomalies were examined; in 21 a DM could be demonstrated. CT has two major advantages in the radiological investigation of spinal abnormalities, such as spinal dysraphism and DM: 1. Cross sections of the spinal canal can be obtained without overlying opacities and 2. The demonstration of intraspinal soft tissues structures, corresponding to their attenuation characteristics and the relative differences in absorption of the x-rays.

Adolescent↗

PubMedDate not supplied

Prenatal diagnosis of diastematomyelia in a 15-week-old fetus.

OBJECTIVE: A case of prenatal diagnosis of diastematomyelia is presented. METHODS: A case of fetal diastematomyelia, diagnosed by prenatal sonography, demonstrated the typical sonographic features of this condition. In this case it was detected at 15 weeks of gestation, and presented with a midline echogenic focus in the posterior region of the thoracolumbar spine. RESULTS: The pregnancy was terminated by induction of labor. The fetus was female and there was a 1-cm long endurated hyperemic lesion at the back of the fetus. We confirmed the diagnosis of diastematomyelia after termination of pregnancy by plain chest and abdominal X-ray and also MRI scanning. CONCLUSION: Isolated diastematomyelia is a rare form of spinal dysraphism characterized by a sagittal cleft in the spinal cord, conus medullaris and/or filum terminale with splaying of the posterior vertebral elements. Prenatal diagnosis of this anomaly is possible in the early mid-trimester by sonography, thus allowing for early surgical intervention and a favorable prognosis.

Abortion, Induced↗

PubMed2002

Unusual neuroenteric cysts: diagnosis and management.

Six cases of unusual neuroenteric (NE) cysts, occurring in children aged 15 months to 18 years, are reported here. Three of the cysts were extramedullary, while the other three were intramedullary. Two of the extramedullary cysts were located at the lumbosacral region, an unusual site. Only one patient had the cyst located in a ventral relation to the cord in the cervicothoracic region. None of the patients had communication with the mediastinum or intraabdominal viscera. Stigmata of spinal dysraphism were seen in three patients. All the children had variable neurological involvement and it was difficult to differentiate NE cyst from other causes of spinal cord or cauda equina lesion, particularly in the absence of some dysraphic marker. One patient presented with minimal neurological deficits and painful torticollis; this patient was suspected to have an atlantoaxial dislocation. All patients were investigated with MRI, and the only diagnostic feature of an NE cyst was an intradural cyst with an anterior vertebral body anomaly in a single case. It was not possible to radiologically differentiate NE cyst from other intraspinal cysts in the rest of the patients. All patients were operated on by the posterior route; an attempt to excise the cyst nearly in toto was made. Evidence of neural tethering most probably due to cyst contents was seen in three patients. All patients improved after surgery. None showed recurrence of the cyst at follow-up of 3 months to 2 years.

Adolescent↗

PubMed2001

[A case of cervical subpial lipoma].

The authors report the rare case of a 48-year-old woman with a cervical subpial lipoma unassociated with spinal dysraphism. Her symptoms were progressive weakness and numbness in the four extremities and the neurological symptom of mild cervical myelopathy. Plain radiographs of the cervical spine showed diffuse widening of the spinal canal, which was occupied by a large low-density mass revealed by axial CT scan. MR imaging identified the mass as being dorsolateral to the cord in the intradural region. It was hyperintense on both T1- and T2-weighted images. Axial images with fat suppression sequence clearly showed dorsal nerve roots traveling through the lesions, but the interface between the spinal cord and lesion was not well demarcated. A cervical laminectomy between C3-6 revealed a yellowish subpial mass after the dura was opened. The mass was only partially resected and intraoperative SEP monitoring data remained normal including N20 latency. Histological examination found mature adipose tissue covered by thickened pia mater, which was compatible with a diagnosis of lipoma. After surgery, no complications occurred and the patient's myelopathy subsequently improved. Subpial spinal lipomas unassociated with dysraphism are uncommon and they are reported in only 1% of all spinal cord tumors. They are thought to arise from premature dysjunction of the cutaneous ectoderm during neural tube formation. Without any invasive procedure, MR imaging is indispensable to show their longitudinal dimension as well as their infiltrative extension into the spinal cord. The main purpose of surgery is to decompress the lipoma from the adjacent neural structures, because it is benign in nature and there is no cleavage plane identifiable between the lipoma and the spinal cord.

Female↗

PubMed2003

Ultrasonographic screening in infants with isolated spinal strawberry nevi.

OBJECT: The authors performed spinal ultrasonography and/or magnetic resonance (MR) imaging in 20 consecutive newborns with spinal strawberry nevi. METHODS: In 15 patients the strawberry nevi were isolated and in five they were associated with other cutaneous markers of occult spinal dysraphism (OSD). In four of five patients with additional cutaneous markers, but in none of those without, MR imaging and surgical exploration demonstrated OSD. The authors found that strawberry nevi in isolation do not appear to indicate underlying dysraphic states. The sparse clinical literature on this topic, which is reviewed, confirms an association between OSD and strawberry nevi presenting in conjunction with other cutaneous signatures. By contrast, spinal strawberry nevi occurring alone may not indicate the presence of underlying dysraphism. CONCLUSIONS: A prospective study of larger numbers of patients with isolated strawberry nevi, undergoing MR imaging evaluation, is necessary to determine whether neuroimaging screening in these patients is indicated.

Female↗

PubMedDate not supplied

[Spinal sonography of a newborn infant with postpartal paraplegia].

Cranial ultrasonography is a well established diagnostic procedure. In contrast ultrasonography of the spine and the spinal cord is less frequently used. It is indicated in infants with spinal dysraphism and may help to diagnose patients with meningomyelocele, spinal lipoma or cord tethering. We present a newborn with parplectic symptoms as a result of an epidural hematoma, which could be demonstrated exclusively by ultrasonography. We want to stress that spinal ultrasonography is a method of high clinical value.

Cerebral Hemorrhage↗

PubMed1998

Dilation of the ventriculus terminalis: sonographic findings.

A newborn term male infant was found to have the stigmata of VACTERL syndrome, including cervical and thoracic hemivertebra, partial sacral agenesis, 13 pairs of ribs, high imperforate anus with rectovesical fistula, and tracheoesophageal fistula with proximal esophageal atresia. No open spinal dysraphism or skin dimpling was seen. Ultrasonography of the spinal cord was performed to screen for occult anomalies. The examination revealed a lipoma of the filum terminalis and a low-lying conus medullaris at the L3-L4 level with a large central cystic structure in the terminal cord, which was continuous with the central canal (Fig. 1). This was thought to most likely represent a dilated ventriculus terminalis, and the diagnosis was confirmed on a subsequent MR image (Fig. 2). Fluid in the cyst followed cerebrospinal fluid signal in all sequences. We observed no abnormal signal in the adjacent parenchyma or enhancement associated with the cystic mass. A cranial ultrasonogram did not reveal any intracranial anomalies. The patient demonstrated no neurologic deficits in the lower extremities. Bowel and urinary continence was not assessable in this infant.

Abnormalities, Multiple↗

PubMedDate not supplied

Benign intradural extramedullary masses in children of northern India.

Twenty-five children with benign intradural extramedullary tumors/lesions were retrospectively analyzed to see the frequency of different histotypes in this location and their variation from the Western world. The duration of symptoms, clinical profile and surgical outcome of these cases were evaluated. The age of children ranged from 1.5 to 18 years, with a mean age of 7.5 years. Thirteen amongst them were male. Follow-up ranged from 3 months to 5 years with a mean of 19 months. McCormick functional grading was used retrospectively to find out the functional outcome in these children. Sixty-eight percent of these cases presented with functional grade IV or V. The mean duration from the appearance of the first symptoms to admission to our hospital in these children was 13 months. Improvement in the functional grade following surgical excision was noted amongst all, except one, who presented with schwannoma, and did not improve from grade III even after a 14-month follow-up. Reoperation/two-staged operation was required in 3 children. The following conclusions were drawn from this study: (1) approximately half (48%) of intradural extramedullary benign tumors are nerve sheath tumors, i.e. neurofibromas, neurilemomas and schwannomas. Giant neurofibromas with giant extraforaminal extension may rarely manifest with a large extraspinal mass; (2) meningioma is an infrequent tumor in this location; (3) developmental cystic dysraphic lesions (neurenteric cyst, arachnoid cysts), unassociated with the stigmata of spinal dysraphism, are significantly more frequent (28%) than reported in the literature; (4) the possibility of infective lesions (granuloma, abscess) causing compressive features cannot be denied even in intradural extramedullary tumors; (5) long-standing lesions, with a long segment involvement, also have a good outcome following a good microsurgical excision, and (6) more than half of the children (68%) present with a severe neurological compromise, either because of ignorance and poor education or due to the scanty diagnostic facilities available at the peripheral hospitals of the developing milieu.

Adolescent↗

PubMed1987

The evolution of surgical treatment of spinal deformity in myelomeningocele.

Although modern anterior and posterior fusion techniques can provide excellent correction of dysraphic spinal deformities, the surgery can be formidable. Newer surgical techniques utilizing segmental spinal fixation of implants has afforded both a greater degree of three-dimensional correction of spinal deformity and more rigid control of the fusion mass. However, due to the inherent loss of posterior elements and the associated high level of paralysis, the use of bivalved total contact spinal orthoses are essential to achieving a high fusion rate with a minimum number of complications.

Child↗

PubMed1984

[Suspected herniated disk--differential diagnostic case studies by lumbar CT].

The most common reason a patient is referred for spinal CT examination is to exclude a ruptured intervertebral disc. Besides nerve root entrapment due to herniated disc, a number of unusual or unexpected conditions have been encountered in the course of CT lumbar spine studies. These include spondylolisthesis, spinal dysraphism, Paget's disease, and inflammatory, neoplastic, or metastatic lesions. The application of spinal (small-circle) target imaging includes the risk to overlook soft tissue lesions that extend beyond the reconstruction circle. Therefore, complete (large-circle) circumferential abdominal scanning is recommended in case of a suspected extraspinal cause of sciatica.

Adolescent↗

PubMed1992

[Clinical manifestations of spinal lesions in dysraphic malformations of the caudal neuropore in children].

Disorders in the development of the spine and the spinal cord in the caudal neuroporus region significantly contribute to the morbidity and the lasting invalidity of children. Clinical investigations of the malformations manifest through different forms of body abnormalities, ranging from asymptomatic forms of "spina bifida occultae" to the severe forms of open "meningomyelocele". The nature and the level often cannot be detected before the development of the structural changes in the course of growth and development of the child, which can hardly be treated later. At the Department of Child Surgery in Novi Sad 12 infants were surgically treated in the last 5 years, while 30 children were surgically and conservatively treated after the detection of the clinical and subclinical manifestations of dysraphism. The indications for the surgical treatment were open meningomyelocele as well as those subclinical forms indicating the development of the tethered corn syndrome, hydrocephalus and tumefactions in the lumbosacral region. Moreover the study points to the significance of certain clinical features and investigations in the diagnosis of spinal dysraphism.

Cauda Equina↗

PubMedDate not supplied

[Own experience in surgical treatment of intramedullary spinal cord lipomas].

The aim of this report is to present own experiences in surgical treatment of extremely rare occurring intramedullary spinal cord lipomas without spinal dysraphism. Our experience with such tumours based on 2 cases out of 15 intramedullary spinal cord tumours that were treated at our department from 1984 to 1997. The tumours were located at C4-C7 and C6-Th6 spinal cord region. The patients were 16-year-old woman and 57-year-old woman. Both patients presented with long history of progressive spinal cord damage with rapid deterioration after mild injury. The diagnosis was based on myelography in one case and on MRI in the second one. In first case the tumour was totally removed and we achieved neurological improvement. In the second case the tumour was subtotally removed and after 12 months on MR examination we found relapse of the tumour without neurological deterioration. We found two types of the tumours. The first one was well separated from the normal spinal cord and easy to remove. In the second it closely adhered to the spinal cord without possibility of total excision. The surgical treatment of intramedullary spinal cord lipomas is very difficult and dangerous but only in this way we can protect the patients from total paralysis and to improve their comfort of the life.

Adolescent↗

PubMed1992

[Cervical meningocele and meningomyelocystocele. Apropos of 4 cases].

Published cases of cervical dysraphic lesions are rare. Their estimated proportion is about 5% of the entire group of spinal dysraphism. Four cases are reported, representing 2 types of lesion: 2 meningoceles with a simple fibrous band fixing the posterior aspect of the cord to the skin and 2 meningomyelocystoceles with other associated anomalies (hydromyelia, hydrocephalus, Chiari II, Peter's ocular anomaly). The clinical presentation is that of a soft posterior cervical mass without marked neurological impairement. The surgical treatment is simple, consisting in the resection of the lesion, intradural exploration to untighten the cord if fixed by a fibrous band, and drainage of the hydrocephalus. Neurological outcome is generally good immediately but may need sometimes secondary surgical exploration.

Abnormalities, Multiple↗

PubMed1980

Early development of the brain and spinal cord in dysraphic mice: a transmission electron microscopic study.

The hindbrain and spinal cord were studied by transmission electron microscopy with and without lanthanum nitrate treatment in nine-day embryos of the loop-tail (Lp) mutant mouse. Homozygous (Lp/Lp) individuals exhibit dysraphism from the hindbrain caudally throughout the embryo; in +/+ and Lp/+ individuals, the brains and spinal cords are normal. In contrast, the ventricular cells in the abnormal hindbrain and spinal cord showed increased amounts of intercellular space in an area intermediate between the luminal border and basal zone, and a flattening which occurs variably in their luminal surfaces. A most striking difference occurred in the frequency of gap junctional vesicles, circular structures bounded by a double membrane and containing ribosome-like material. A quantitative analysis of the distribution of these organelles revealed that they are more numerous in the dysraphic hindbrain and lumbosacral spinal cord of the abnormal animals than in comparable regions of the normal; however, in the cervicothoracic spinal cord, the frequency of these vesicles is similar. In specimens treated with lanthanum, the tracer freely penetrated the luminal junctional complexes in both normal and abnormal animals, but was not present between the membranes of the gap junctional vesicles. The developmental significance of gap junctional vesicles frequently located in juxtaluminal regions of mitotic cells, but also found deeply in dividing cells, is not known; they may relate to cell-to-cell attachment and/or communication. In any event, further study of them may prove valuable in understanding normal and abnormal development of the neural tube.

Animals↗