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[Neuroradiologic diagnosis of diastematomyelia].

A report is given on 5 patients with diastematomyelia, in one case the fissure formation of the marrow being without a provable septum. In the frequently very complex pathological-anatomical conditions of the spinal dysraphic malformation, CT can be a considerable help. In combination with metrizamide myelography, details of the marrow parts and their envelopes can be demonstrated. The preoperative planning of the marrow separation becomes more exact. Other causes of a deep spinal marrow level (tethered cord) such as teratomas can also be recognized by CT.

Diagnosis, Differential↗

Terminal myelocystocele.

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. This condition is often associated with OEIS complex i.e. opmphalocele, exstrophy of the bladder, imperforate anus and spinal abnormality. We studied 4 cases of terminal myelocystocele. They revealed no preoperative neurological deficit. None of these had associated OEIS complex. One of the cyst was unique due to presence of copious amount of pus in its cavity. All four cases underwent successful repair and surgery remained uneventful in all of them. No child showed neurological deterioration during the follow-up.

Female↗

In vitro expression of neural tube pathology in the vl mutant mouse.

The pathogenesis of lumbosacral spinal dysraphism was analyzed in vitro in early homozygous embryos of the vl (vacuolated lens) mutant mouse, and comparisons were made between the lag in closure of the posterior neuropore at the beginning of culture and the degree of severity in the spinal defect at the end of culture for each embryo. In the majority of abnormal (vl/vl) embryos, the neural tube closed completely in culture, although mild defects were observed in the spinal roofplate comparable to those that occur in vivo. Although some abnormal embryos did exhibit small openings in the caudal neural tube at the end of the culture period, in none of the cultured embryos was the extent and severity of the defect as great as that observed in some abnormal embryos of comparable age obtained in vivo. Moreover, the degree of delay in closure of the posterior neuropore at the outset of culture did not necessarily correlate with the severity of the defect obtained at the end of culture. Thus, the expression of the neural tube defect in this mutant appears to be modulated and attenuated by biochemical and/or mechanical factors which may be peculiar to the culture milieu.

Animals↗

Split spinal cord malformation.

The split spinal cord is a rare congenital malformation. We report the rare finding of a split cord malformation in a young girl. Further evaluation of this anomaly revealed a Type I split cord malformation (midline bony septation), with no other concomitant pathological entities. Various hypotheses have been made regarding the embryology of this unusual form of spinal dysraphism, and these are reviewed along with the common clinical manifestations of this intriguing pathological entity.

Child↗

Lumbosacral lipomas: in utero diagnosis and prognosis.

INTRODUCTION: For the purposes of prenatal counselling and prognostication, it is essential to keep in mind the key diagnostic criteria allowing recognition of the diverse forms of spinal dysraphism. DIAGNOSIS: It is most crucial not to confuse lipomas and myelomeningoceles, the former carrying a usually favourable prognosis, while the latter a high risk of serious neurological deficits. In our experience, these two conditions can be clearly distinguished on prenatal ultrasound and by fetal biology. Lipomas are skin-covered, which explains the normality of the amniotic fluid and probably that of the intracranial content (no Chiari, no hydrocephalus). These features are distinct from those observed in the case of myelomeningoceles. The distinction between a lipoma and a meningocele is more difficult. The prenatal ultrasound presentation of many lipomas actually resembles that of a meningocele. In a few cases, the diagnosis of lipoma can, however, be suspected by the presence of an abnormally thick skin covering and intradural echoic structures that evoke a low-lying cord. CONCLUSIONS: However, the consequences of confusing a lipoma and most of the other types of occult dysraphism are minimal. Most of these lesions carry a favourable outcome.

Female↗

Sonographic evaluation of caudal spine anomalies in children.

Retrospective analysis of B-mode spinal sonography in 15 pediatric patients with spinal dysraphism and caudal anomalies documents a high degree of accuracy in identifying the presence, site, size, and configuration of meningoceles; and slightly less accuracy in identifying extension of neural tissue into the meningocele and presence of concurrent lipoma. B-mode sonography is capable of displaying spinal anatomy clearly enough to permit accurate retrospective interpretation of pathologic changes. When radiologists learn to interpret sonographic images well enough to achieve equal accuracy in prospective diagnosis, then B-mode spinal sonography will supplant computed tomography and myelography as the preferred screening procedure in the initial evaluation of children with selected anomalies of the caudal spine.

Child↗

Introduction of a urodynamic score to detect pre- and postoperative neurological deficits in children with a primary tethered cord.

OBJECT: An increasing number of asymptomatic children are diagnosed with occult spinal dysraphism, raising the question of their optimal management. Urodynamic study (UDS) is the most reliable method of detecting neuro-urological abnormalities in these children. The rate of postoperative retethering ranges from 10 to 20% and is not always immediately clinically significant. The aim of this prospective study was to develop a reliable method that could be used in the preoperative assessment and postoperative follow-up of children with a tethered cord syndrome (TCS). METHODS: From 1989 to 1997, 15 children underwent spinal cord untethering for TCS. Preoperatively, patients were assessed with MRI and UDS. Postoperative UDS were repeated at 6- to 12-month intervals. Four UDS parameters were identified, graded, and added to obtain a UDS score. A group of 38 children without dysraphic condition was used as control and allowed the calculation of a normal score. CONCLUSIONS: There was a statistically significant difference in the preoperative UDS scores between the control group and the study group ( p<0.001). Postoperatively, there was a statistically significant improvement ( p<0.001) in UDS scores. UDS score is a reliable tool for identifying and quantifying neuro-urological disorders in patients with TCS. Postoperatively, this score was useful in the early diagnosis of spinal cord retethering.

Adolescent↗

Importance of early radiologic diagnosis of congenital anomalies of the spine.

Manifest or occult spinal dysraphism in the absence of neurological symptoms is likely to remain undetected. Therefore, accurate and early diagnosis of such underlying anomalies is of prime importance for early surgical intervention in order to avoid late irreparable damage. During a period of just over 1 year, 17 such cases of spinal congenital anomalies were diagnosed by myelography with metrizamide and computed tomography-myelography. There were 16 cases of tethered cord, six with myelomeningocele, two with diastematomyelia, two with a split spinal cord, three with a lipoma, and the remaining three with vertebral anomalies. Scoliosis was present in 60%, weakness of both legs in 45%, and asymmetry of the feet in 25%. Some of these patients were initially brought to the orthopedic department for corrective surgery before undergoing a complete neurological evaluation; thus the radiologist was acting as the link between patient and neurosurgeon. Clinical experience has shown that surgical treatment can be successful and can thereby obviate further progression of neurological symptoms.

Adolescent↗

[Spinal dysraphia and disordered ascension of the spinal cord in adults].

Spinal dysraphism and tethered cord syndrome which frequently present with clinical symptoms during infancy, childhood and adolescence are rarely encountered in adults. In this retrospective study covering a period of 15 years were report of 12 patients aged from 22 to 71 years with delayed symptoms from the tethered spinal cord. The clinical signs and symptoms which consisted of progressing neurological, urological and orthopedic malfunctions as well as the aspects of current neuroradiological diagnosis including myelography, CT-scan and MRI are presented. The possible pathophysiological mechanism responsible for the delayed onset of the sequelae of the tethered cord are discussed. It is concluded that neurosurgical therapy should be performed in cases of progressing clinical deterioration and the results of surgery in our patient group are presented.

Adult↗

The use of Gore-Tex membrane for adhesion prevention in tethered spinal cord surgery: technical case reports.

OBJECTIVE: The incidence of retethering caused by postoperative adhesions at the repair site after initial tethered spinal cord surgery is not uncommon. To assess the effectiveness of a Gore-Tex membrane in preventing these adhesions, only clinical radiological and experimental animal evaluation has been reported. In this report, we describe two cases in which Gore-Tex membrane was implanted at the initial untethering surgery and in which we were able to confirm the real effectiveness of the Gore-Tex membrane during a second operation. METHODS: In the first patient, Gore-Tex membrane was used for dural repair in the untethering surgery of the split spinal cord malformation. Because of the suspicion of a thickened filum terminale, repeated surgery was indicated 10 months after the initial procedure. In the second patient, Gore-Tex membrane was implanted during the initial untethering surgery for a lipomyeloschisis and a dermal sinus. Because of a persistent fistula of the dermal sinus, a second operation was necessary 1 year after the first operation. RESULTS: During the repeated surgery, a thorough inspection of the implanted Gore-Tex membrane revealed no adhesions between the Gore-Tex membrane and the intradural content in both cases. CONCLUSION: We support the use of Gore-Tex membrane in the prevention of postoperative dural adhesions in the repair of spinal dysraphism.

Child, Preschool↗

Rare combination of spinal lesions and spina bifida occulta: case report.

An infant presented with spina bifida occulta and a rare combination of spinal lesions, consisting of fibrous band with skin tag, lipoma, congenital dermal sinus and arachnoid cyst. The literature concerning the arachnoid cyst in occult spinal dysraphism is reviewed and the embryogenesis of these lesions is discussed.

Arachnoid↗

Diastematomyelia of the cervical spinal cord with tethering in an adult.

A case of cervical diastematomyelia associated with spinal cord tethering in an adult is presented. The differences between cervicodorsal and lumbar region spinal dysraphic states are emphasized with a review of the embryological factors involved. Possible causes and subsequent treatment are outlined.

Adult↗

Segmental spinal dysgenesis.

Segmental spinal dysgenesis is characterized by focal agenesis or dysgenesis of the lumbar or thoracolumbar spine, with focal abnormality of the underlying spinal cord and nerve roots. Children are symptomatic at birth with lower limb deformities and neurological deficits that may be segmental. Myelography and computed tomography disclose hypoplastic or absent vertebrae and atrophic or absent neural elements adjacent to the bony deformity; the spinal column distal to the abnormality may be partially bifid, but is otherwise normal. Spinal ultrasonography was a helpful diagnostic adjunct in one patient. Surgery may be helpful in decompressing partially functioning spinal cord or nerve roots, but may exaggerate the tendency toward spinal instability. The embryology of this abnormality is not clear, but two children had other anomalies suggesting a spinal dysraphic syndrome, and its cause is probably related to a segmental maldevelopment of the neural tube.

Female↗

Myelography in spinal disorders--experience of 1400 cases.

OBJECTIVE: To evaluate the diagnostic importance of myelography in spinal disorders, in correlation with clinical presentation of the patients. METHODS: Patients selected for myelography had presented with history of various spinal disorders such as low backache, neurogenic claudication, paraparesis or paraplegia, quadriparesis or quadriplegia, trauma to spinal region and infective spondylitis. Patients excluded were those with history of allergies to iodinated contrast agents, seizures coagulopathy and pregnant women. Contrast agent was water soluble non ionic agent--lohexol. Spinal Needles used were of 20,22 and 25 gauge. X-Ray machine with tiltable table was used for myelography. RESULTS: There were 1400 patients of whom 935 were males and 465 females with male to female ratio of 2.1. Age range was 8 to 65 years. Spinal disorders diagnosed on myelography were lumbar disc prolapse 866 (60%) cases, lumbar canal stenosis 113 (8%), thoracic disc protrusions 15 (1%), infective spondylitis 53 (4%) cases, spinal tumors 36 (2.5%), spinal dysraphism 28 (2%) and traumatic spine in 85 (6%) cases. Free flow of contrast agent with no block was found in 149 (10.64%) cases. These were subjected for MRI scan which revealed significant pathological lesion of surgical importance in 23 cases (1.64%) only. CONCLUSION: Myelography is the least expensive valuable diagnostic test in spinal disorders specially in lumbar disc prolapses and lumbar canal stenosis.

Adolescent↗

Silastic duraplasty in pediatric patients.

The role of adhesions in the retethering of the neural elements after surgical treatment of spinal dysraphism has produced refinements in the technique of the closure of the neural tube. Silastic, because of its relative inert property, has been used for duraplasty, but a few reports of late complications of hemorrhage or fibrotic capsule formation have caused concern over its use. We report 33 patients with the following diagnoses--eight spinal lipomyelomeningoceles, three myelomeningoceles, six symptomatic Chiari II malformations, seven tethered cords, six spinal cord tumors, two diastematomyelia, and one cerebellar astrocytoma--in whom Silastic dural grafts were used to prevent the adherence of neural structures to the overlying tissues. Our surgical technique is presented here. The patients have been observed for up to 6 years. Only one became infected, was treated with antibiotics without graft removal, and has remained without sequelae for over 3 years. One had an incidental pseudomeningocele that was noted on follow-up magnetic resonance imaging scan that was not clinically apparent. There have been no hemorrhages, leakage of cerebrospinal fluid, nor other complications from using nonreinforced Silastic sheeting. In one patient, Dacron-reinforced Silastic was used and, upon reexploration, a marked fibroblastic response was noted. A review of the literature suggests that fibrotic complications are related to this Dacron-reinforced Silastic. The hemorrhagic complications that were reported occurred in instances where Silastic grafts were large and no technical modifications were made preventively. We conclude that the use of nonreinforced Silastic dural grafts, with appropriate technical modifications in surgical technique, is safe and may prevent retethering of neural tissues in a variety of neurosurgical lesions.

Child↗

The nature of congenital posterior cervical or cervicothoracic midline cutaneous mass lesions. Report of eight cases.

Between 4% and 8% of cases of spina bifida cystica occur in a cervical or cervicothoracic location. Despite a large body of literature concerning spinal dysraphism, there has been little written specifically about patients afflicted with this disorder in a cervical location. Eight children who presented at birth with posterior cervical or cervicothoracic lumps, all of which represented a dysraphic state, are discussed. Two types of abnormalities were noted. Three patients had hydromyelia with an associated myelocystocele herniating posteriorly into a meningocele sac. In these three patients there was an associated Chiari II malformation and hydrocephalus. The other five children had a meningocele in which a band of tissue extended from the posterior aspect of the spinal cord through a defect in the bone and fascia to the posterior part of the meningocele sac itself. No patient had a lesion that could be described as a meningomyelocele. The investigation and surgical management of these conditions are discussed and the need for intradural exploration to untether the spinal cord in the cervical region is stressed.

Female↗

Anterior endoscopic treatment of a huge anterior sacral meningocele: technical case report.

OBJECTIVE AND IMPORTANCE: Anterior endoscopic treatment of a huge anterior sacral meningocele, a rare example of spinal dysraphism, is described. CLINICAL PRESENTATION: A 43-year-old woman presented with severe headache and fever, increasing abdominal pain, and a several-year history of right lower-extremity radicular pain. She exhibited meningeal irritation signs and Currarino's triad. Lumbosacral myelograms and magnetic resonance imaging scans revealed a huge anterior sacral cyst connected with the spinal subarachnoid space. INTERVENTION: A straight rigid endoscope was introduced into the meningocele through a small abdominal incision. The fistula between the meningocele and the spinal subarachnoid space was obliterated with multiple fat grafts harvested from the abdomen. CONCLUSION: An anterior endoscopic procedure is an alternative for the treatment of a huge anterior sacral meningocele.

Adult↗

Lumbar teratoma presenting intradural and extramedullary extension in a neonate.

BACKGROUND CONTEXT: The association of teratomas and spinal malformations such as spina bifida, partial sacral agenesis, hemivertebrae, and diastematomyelia has been described in the literature. Reported cases, however, are mainly presacral or sacrococcygeal with an extremely rare presentation of intradural extension. PURPOSE: A case of lumbar teratoma with an intradural extension and extramedullary component and the clinical outcome following surgical treatment are reported. STUDY DESIGN/SETTING: To our knowledge, among the reported teratoma cases with an intradural extension and extramedullary component, our case has a distinguishing feature regarding the involvement of the lumbar spine. It is also the first case, showing no neurological deficit during the postoperative period. METHODS: A full-term, female infant presented with a 30 x 30 x 10 mm lumbar mass covered with normal skin. The mass contained an irregular, bone-like, hard and mobile material accompanying cystic components. Magnetic resonance imaging revealed a total closure defect of the first and second lumbar laminae and a subcutaneous mass with intradural extension. The lesion was found to penetrate the dural sac through an extended exposure from T12 to L3. There was no firm attachment of the intradural, extramedullary component of the lesion. Total removal of the tumor was achieved. RESULTS: The patient was discharged on day 7 without any neurological deficit or sign of hydrocephalus. The pathological examination showed a benign teratoma containing mature cartilage, muscle, adipose tissue, and glandular tissue. Follow-up at 2 years showed no recurrence or neurological deficit and a normal sphincter tone. Urodynamic evaluation was within normal limits. CONCLUSION: Accompanying a spinal dysraphic state, the mature teratoma in our case may support the idea of a tumor actually arising from a dysraphism and growing outward to produce the mass.

Biopsy, Needle↗