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PubMed2001

Sonographic diagnosis of diastematomyelia in utero: a case report and literature review.

Fetal diastematomyelia is a rare form of spinal dysraphism that is characterized by a complete or incomplete division of the spinal cord by an osseous or fibrocartilaginous septum. A case of diastematomyelia, which was detected on the routine third trimester detailed ultrasound scan, is presented. The diagnosis was based on the detection of an echogenic focus in the posterior aspect of the spine in association with widening of the interpedicular vertebral space. The case illustrates that diastematomyelia can occur in the absence of overt spina bifida and that prenatal detection will allow timely postnatal investigation and treatment. Prenatal literature is further reviewed to assess the clinical significance of this finding.

Adult↗
PubMed1992

A proposed grading and scoring system for spina bifida: Spina Bifida Neurological Scale (SBNS)

Neurological symptoms present in neonates with spinal dysraphism often progress with growth. A simple, objective scoring system for quantitative analysis of spinal neurological deficits, called the Spina Bifida Neurological Scale (SBNS), is proposed. Scoring is based on (1) motor function, (2) reflexes, and (3) bladder and bowel function. These are each divided into six, four, and five points respectively with respect to the level of spinal function. Motor function and reflexes are bilaterally analyzed, and the maximum SBNS score of 15 points reflects a normal spinal neurological state (grade I). This scoring system was correlated with the clinical condition of 89 patients with spina bifida who were graded from I to V. A total score of less than 5 was associated with a nonambulatory state (grade IV or V) in 84.0% of patients, and a score of 5-9 was associated with an ambulatory state (grade III) in 93.8% of patients. Scores of 10-14 reflected control of bladder and bowel function (grade II) in all patients. The application of a standardized scoring scheme will assist in the evaluation of patients' clinical status and will enable analysis of chronological changes in neurological function.

Adolescent↗
PubMed1995

Immunofluorescent analysis of the spinal cord in dysraphic mice.

Development of the spinal cord was analyzed immunocytochemically at 10-12 days of gestation in normal and dysraphic embryos of the loop-tail mutant mouse, using an anti-neural cell adhesion molecule (NCAM) and the lectin concanavalin A (Con A) as histological markers for evaluating neural cell organization and distribution. The normal and abnormal embryos showed similar patterns of reactivity to anti-NCAM and Con A, even though the neural folds were open and everted in the abnormal embryos, with displacement of the dorsal root ganglia. In the abnormal embryos the floor plate was similar to that in normal embryos, as evidenced by its increased anti-NCAM and Con A labeling relative to that in the rest of the neuroepithelium. Moreover, each lateral end of the everted abnormal neuroepithelium developed an attenuated 'roof plate' that appeared to be structurally similar to the normal roof plate. However, some of these 'roof plates' exhibited prominent clusters of labeled and nonlabeled cells, especially in the 10-day embryos. In addition, whereas normal embryos showed strong luminal labeling of the neuroepithelial cells with Con A, comparable regions in the abnormals were spotty and poorly defined except for the 'roof plate' and floor plate. The results indicate that dorsoventral polarity in the spinal cord, as assessed structurally and histochemically, develops essentially normally in abnormal dysraphic embryos, even though the topographic relationships of the abnormal neural tube are disturbed.

Animals↗
PubMed2004

Adult tethered cord syndrome: relative to spinal cord length and filum thickness.

The adults with tethered cord syndrome (TCS) are divided into two groups: group-1 adult TCS patients with spinal dysraphism; and group-2 patients without dysraphism who develop signs and symptoms in adulthood. A significant number of group-2 patients failed to show elongation of the spinal cord and abnormally thick filum terminale. This paper reports the combinations of the location of the caudal end of the spinal cord and the thickness of the filum terminale in 104 patients with group-2 adult TCS. The caudal end of the spinal cord was found at the L2-3 intervertebral space or above in 37 patients (35.6%) and below L2-3 level in 67 patients (64.4%). The diameter of the filum was <2 mm in 60 patients (57.7%) and > or =2 mm in 44 patients (42.3%). These data clearly indicate that the pre-operative diagnosis of adult TCS must rely primarily on the neurological symptomatology and the specific imaging feature, posterior displacement of the conus and filum that attach the posterior arachnid membrane.

Adolescent↗
PubMed2000

The effect of surgery for split spinal cord malformation on neurologic and urologic function.

The split spinal cord malformation (SSCM) is an occult spinal dysraphism which causes tethering of the spinal cord. We performed a retrospective analysis of 15 patients who had split cord malformations (without associated open neural tube defect) who underwent both pre- and postoperative urodynamic studies (UDS) in order to determine if a significant percentage of these patients, even in the absence of overt urologic symptoms, had evidence of urologic dysfunction. Eleven patients presenting in early childhood and 4 patients presenting later in life are reviewed. Despite the lack of preoperative urologic symptoms in almost all patients, 73% of patients had voiding abnormalities on formal testing. UDS on these patients before and after surgery was a useful adjunct to perioperative management and decision making, helped define the success of surgery, and gave objective information for cases in which retethering was suspected.

Adolescent↗
PubMed1994

Treatment and prevention of tethered and retethered spinal cord using a Gore-Tex surgical membrane.

The incidence of the tethered cord syndrome after repair of spinal dysraphism is not insignificant. A retethered spinal cord may also develop after an untethering operation. In order to treat and/or prevent the tethered and retethered spinal cord, the authors developed and successfully used a new method in 12 cases. After complete release and reconstruction of the spinal cord, a Gore-Tex surgical membrane was placed over the cord and fixed to the lateral dural surface with stay sutures. During a postoperative follow-up period ranging from 23 months to 7 years, no further neurological deterioration was observed in the 12 patients and magnetic resonance imaging studies showed no adhesion of the spinal cord to the operative site. It is concluded that this simple new method is effective for the treatment and prevention of tethering and/or retethering of the spinal cord, although a longer follow-up study is required.

Female↗
PubMed2005

Terminal myelocystoceles: a series of 17 cases.

OBJECT: A terminal myelocystocele is a rare form of spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. In their study of 17 cases, the authors attempt to develop treatment guidelines for patients suffering from this condition. METHODS: Seventeen patients (age range 2 months-5 years) underwent surgery during a 7-year period. Surprisingly, nine of 17 patients had no neurological deficits. Among these nine asymptomatic cases, three patients had a dermal sinus and two had a skin dimple, whereas one had congenital talipes equinovarus deformity (CTEV). Among the eight patients with neurological deficits, seven had lower-limb weakness (two had complete paraplegia), and one patient had urinary incontinence and constipation. Four patients each had a dermal sinus and CTEV; interestingly, one infant had an accessory phallus. Radiologically, eight patients had pure myelocystoceles, and a diagnosis of lipomyelocystocele was made in nine instances on the basis of magnetic resonance images. All patients underwent surgery. The meningocele sacs were excised, and the bands tethering the myelocystocele sac to the meningocele sacs were incised, with as little damage to the roots as possible. Two patients experienced postoperative CSF leakage (one required reexploration), and two others had subcutaneous CSF collection. No patient had deterioration of the neurological condition after surgery. CONCLUSIONS: The authors present the largest study of patients with terminal myelocystocele to date and emphasize that the possibility of this condition must be kept in mind when addressing all congenital lumbosacral masses. The authors assert that myelocystoceles and lipomyelocystoceles should be kept in the same category and recommend early surgery in all cases.

Child, Preschool↗
PubMed2002

Spinal subdural epidermoids - a separate entity: report of 3 cases.

Intradural extramedullary epidermoid cysts are rare tumors especially those not associated with spinal dysraphism. We report 3 cases of spinal intradural extramedullary epidermoid cysts. In all the cases, the lesion was situated at dorsal level. The clinical features, MRI characteristics and surgical treatment of such rare intradural extramedullary benign tumors are discussed and relevant literature is reviewed.

Adult↗
PubMedDate not supplied

[Ultrasonography of the spine in infancy].

QUESTION: Based on a standardized technic and reliable attachment of the presented structures the suitability of spinal sonography as a screening-method in newborns and infants should be estimated. METHODS: Enabled by the comparison of sonographic transverse and sagittal plains with identical anatomic sections of an infant corpse the anatomic structures of an healthy spine were associated, which was verified in the clinical use later on. RESULTS: Exemplary photographs of a collective of 50 newborns and infants reproduce the presentation of the spine found in the preparation of the corpse. The technical practicability of such an examination in clinical use was confirmed herein and it became clear that the application of this method is limited from the 6.-8. months of living on because of the increasing ossification which involves a reduction of the image quality. The deviations from the spine presentation in normal relations were shown by some ultrasound examples of malformed spines (spinal dysraphism, hemivertebrae). CONCLUSION: Reproducible attachment of the structures obviously seen with ultrasound and the clear demarcation of deformations as well as the simply practicable examination make evident the suitability of spinal sonography in newborns and infants as a noninvasive screening for the exclusion of spine malformations.

Female↗
PubMed2006

Caudal block complication in a patient with trisomy 13.

In this report we describe a complication of a caudal block in a 4-year-old child with trisomy 13. The patient's history was remarkable for microcephaly, developmental delay, seizures, apnea, and prolonged emergence times. Induction of anesthesia and tracheal intubation were uneventful. A caudal block was aborted after positive aspiration of cerebrospinal fluid. A radiogram suggestive of spinal dysraphism, found on subsequent review, was confirmed by a magnetic resonance imaging scan consistent with tethered cord and dural ectasia. Congenital abnormalities associated with trisomy 13 and cutaneous signs suggestive of spinal abnormalities are reviewed. Avoidance of neuraxial regional anesthesia in these patients is recommended.

Abnormalities, Multiple↗
PubMed2000

Terminal Myelocystocele:an unusual presentation.

Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin-covered lumbosacral spina bifida. An arachnoid-lined meningocele, continuous with the spinal subarachnoid space, is traversed by the hydromyelic cord. Clinically, this presents with a skin-covered lumbosacral mass, but often no neurological deficit is present. We present a case of terminal myelocystocele in a child born without deficit and without an obvious back mass. Diagnosis was delayed until sphincter disturbance and lower limb inequalities developed. We discuss the presentation, imaging and operative findings in this case.

Child, Preschool↗
PubMed1996

MR imaging of the pediatric spine.

An overview of MR imaging of the pediatric spine is presented with a systematic approach to the analysis of spinal dysraphic disorders. An introduction to the most commonly used MR imaging techniques, with an emphasis on the newer pulse sequence of cine MR flow of the cerebrospinal fluid and embryogenesis of the spine and spinal cord, provides the orientation for diagnosis of this common pediatric disorder.

Child↗
PubMed2003

Prenatal diagnosis of diastematomyelia.

INTRODUCTION: Diastematomyelia, also termed split cord malformation, is a form of occult spinal dysraphism characterized by a cleft in the spinal cord. Prenatal diagnosis of this anomaly is possible by ultrasonography (US), and fetal MRI can be used to diagnose the type of diastematomyelia precisely. Diastematomyelia can be isolated or associated with other dysraphisms, segmental anomalies of the vertebral bodies, or visceral malformations (horseshoe or ectopic kidney, utero-ovarian malformation, and anorectal malformation). We present three cases of fetal diastematomyelia investigated using a multimodal prenatal work-up (US, MRI, 3D-CT). CASES: The first case, detected at 20 weeks' gestation, had a lumbar meningocele. At 30 weeks' gestation, direct US visualization revealed the division of the spinal cord into two hemicords. This patient illustrates an isolated type II diastematomyelia with a favorable prognosis. The second case, detected at 22 weeks' gestation, presented with disorganization of bony process of the vertebral column with a midline echogenic bony spur, asymmetrical hemicords, and a foot malposition. Fetal MRI at 26 weeks' gestation and CT/3D reconstructed at 32 weeks' gestation confirmed a type I diastematomyelia with orthopedic malposition. The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis. Prenatal work-up (US, MRI) disclosed other visceral malformations (pelvic kidney), which led to the assumption of a complex polymalformative syndrome. The pregnancy was terminated. Fetopathologic examination disclosed even more visceral malformations (anal atresia and unicorn uterus).

Adult↗
PubMed2002

Spinal ultrasound in infants.

This paper discusses the indications for spinal ultrasound, including its advantages and disadvantages compared with spinal MRI. The features and ultrasound findings both in normal infants and in those with spinal dysraphism are reviewed.

Humans↗
PubMed1994

[Lumbosacral lipomas with spinal bifida].

Spinal dysraphism is usually accompanied with lumbosacral lipoma. The neurological deficits such as lower extremities sensorimotor disturbances, bowel and bladder dysfunction are produced by tethering, compression of lipoma and direct transmission of external force on the spinal cord during growth period. 25 patients treated surgically at our hospital between May, 1985, and January, 1993, were reviewed. Surgical approach was designed for debulking the lipomatous mass, untethering and decompressing the cord, repairing the dural defect and paraspinal muscle cleft. 2-59 months (mean 37.5 mons) after operation the 2 patients whose neurological system were normal before operation remained intact. In other 23 patients, bowel function recovered in 75%, active urination restored and improved in 66.2%. Motor dysfunction and paresthesia of the lower extremities alleviated in 78.3%. A case of mild uremia returned to normal and another moderate case improved. None was deteriorated by the operative procedure except one case in which a posterior nerve root was injured.

Adolescent↗
PubMedDate not supplied

[A rare variant of neuroenteric cyst: split notochord syndrome].

OBJECTIVE: We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism. DESCRIPTION: We treated a 2 month-old boy presenting with an extensive lumbosacral deformity, hydrocephalus and apparent enteric segments in the dorsal midline, accompanied by an enteric fistula and imperforated anus. The malformation was diagnosed as split notochord syndrome. The baby died as a result of sepsis before surgical treatment could be attempted. COMMENTS: Split notochord syndrome is the rarest form of neuroenteric cyst described until this moment (< 25 cases in the literature). It is frequently associated with anorectal malformation, intestinal fistulae and hydrocephalus. Prognosis is not necessarily poor and survival is possible if digestive malformations, hydrocephalus and the dysraphism itself are treated simultaneously.

Abnormalities, Multiple↗
PubMed2005

Split notochord syndrome variant: prenatal findings and neonatal management.

Spilt notochord syndrome is an extremely rare form of spinal dysraphism characterized by a complete cleft of the spine and a persistent communication between endoderm and ectoderm. A variant of split notochord syndrome was diagnosed in a 25-week-old fetus showing a prolapsed congenital colostomy and a spinal cystic lesion. The final diagnosis included protruding colon segment, imperforate anus with a rectourethral fistula and lipomyelomeningocele. The ultrasound features of the condition and the post-natal management are discussed. The neonate was successfully treated with a posterior sagittal anorectoplasty, while the lipomyelomeningocele was resected at a later stage.

Abnormalities, Multiple↗