Leg lengthening in spinal dysraphism.

Spinal dysraphism may lead to limb inequality and foot deformities. Fears have been expressed regarding limb lengthening in such patients because of potential sequelae in relation to their peripheral neurologic abnormalities. We performed Ilizarov lengthening of the lower limb on seven community walkers with spinal dysraphism. The procedure was well tolerated with no increase in sepsis, soft-tissue, or bony complications compared with patients undergoing lengthening for reduction anomalies. All the patients mobilised rapidly after frame application and returned to their previous functional level within 3 months of plaster removal.     

Computed tomography in spinal dysraphism.

Computed tomography of the spine has been used to evaluate patients with spinal dysraphism. With this technique, the nature of soft-tissue masses can often be correctly diagnosed and the underlying bony anomalies clarified.     

Multiple subpial lipomas with dumb-bell extradural extension through the intervertebral foramen without spinal dysraphism

BACKGROUND: Intradural subpial lipomas not associated with spinal dysraphism, account for less than 1% of spinal cord tumors. The simultaneous existence of multiple intradural subpial lipomas with dumb-bell extradural extension through the intervertebral foramen in the same patient without any evidence of spinal dysraphism has not been previously reported. CASE DESCRIPTION: A 38-year-old man presented with progressive spastic paraparesis, and weakness of right elbow extension and opposition of the medial three fingers. He also had ascending paraesthesia from the C6 dermatome to the saddle region and loss of joint and position sense of both lower limbs with hesitancy and precipitancy of micturition. There was no spinal tenderness, deformity, neurocutaneous markers, or spinal dysraphism. The total duration of illness was 11 years.The oblique views of the plain radiographs of the cervical spine revealed an enlarged right C7-D1 intervertebral foramen. The T1- and T2-weighted magnetic resonance (MR) images showed two intradural, hyperintense lesions (with extensive loss of signal on fat suppression sequences), one extending from C5 to D2 and the other opposite the C3-4 disc space. The parasagittal and axial images showed the extradural component of the lesion emerging from the right C7-D1 intervertebral foramen.At surgery, a C2 to D2 laminectomy was performed. The lipoma, enclosed in a fine pial membrane, was situated on the right posterolateral aspect of the cord. The right-sided nerve roots from the C6 to D1 levels were completely enmeshed by the lipoma. There was a separate superficial subpial lipoma adherent to the posterior aspect of the cord at the C3-4 level. A distinct area of normal cord was present between the two lesions. A subtotal decompression of the lesions including the component emerging through the right C7-D1 intervertebral foramen and a duraplasty were performed.At follow-up after 18 months, the posterior column impairment, lower limb hypoaesthesia, and right upper limb paraesthesia had improved. However, residual elbow extension and lower limb weakness, mild lower limb spasticity and sphincteric dysfunction persisted. CONCLUSIONS: The multiplicity of intradural subpial lipomas without spinal dysraphism points to a dysembryogenetic basis similar to that seen in patients with spinal dysraphism that results in lipomas, but in which the defect is not severe enough to give rise to coexisting vertebral and soft tissue anomalies. The dumb-bell extradural extension through the intervertebral foramen is extremely rare. The magnetic resonance imaging and surgical principles are discussed.     

Occult spinal dysraphism in patients with anal agenesis

Recent reports have suggested an association between congenital anorectal anomalies and occult spinal dysraphism. Eighty-seven patients with anal agenesis have been treated at this institution over the last 14 years. Two of these patients had spinal cord anomalies recognized at birth (a myelomeningocele and a tethered spinal cord). Two additional patients presented with progressive neurologic deficits in early childhood and were each found to have a tethered spinal cord. To further assess the magnitude of this problem, we have used magnetic resonance imaging (MRI) of the spine to survey prospectively 23 infants with anal agenesis. Twenty-one former patients who were asymptomatic were recalled and also studied. Four of 44 patients (9%) were found to have significant occult spinal dysraphism; each child had undergone neurosurgical operation without morbidity. MRI found each child to have a tethered spinal cord, either as an isolated lesion (2) or in association with a syrinx (1) or lipomyelomeningocele (1). One of these patients had a neurologic deficit detected on careful preoperative evaluation. The other three, two of whom were less than 2 years old, had no detectable deficit. Neither the extent of the anorectal malformation, the absence of associated congenital anomalies, nor the demonstration of normal vertebral anatomy on plain radiographs of the spine precluded the presence of occult spinal dysraphism. Therefore, we recommend that all patients with anorectal anomalies undergo MRI imaging of their spines during initial evaluation to screen for occult spinal dysraphism. In addition, consideration should be given to recalling older patients for MRI evaluation.     

The human tail and spinal dysraphism.

Recent publications have endeavoured to differentiate between the true, or vestigial tail, and the pseudotail by clinical and pathological examination, and have indicated the benign nature of the true tail. The true tail arises from the most distal remnant of the embryonic tail, contains adipose, connective, muscle, and nerve tissue, and is covered by skin. Pseudotails represent a variety of lesions having in common a lumbosacral protrusion and a superficial resemblance to vestigial tails. A review of the case reports indicates spina bifida to be the most frequent coexisting anomaly with both. A review of occult spinal dysraphism shows it to be associated with cutaneous signs in more than 50% of instances. Three cases of spinal dysraphism with tail-like cutaneous structures are described and their radiological, operative, and pathological findings presented. The classification of each of the appendages into true tail or pseudotail remains obscure. Although the finding of these three tails was the subject of much curiosity, surgical treatment was clearly designed to adequately deal with the associated dysraphic state. The presence of a tail-like appendage in the lumbosacral region should alert the clinician to the possibility of underlying spinal dysraphism. Preoperative assessment must include a complete neurological history and examination as well as computed tomographic or magnetic resonance imaging.     

An unusual presentation of occult spinal dysraphism

Occult spinal dysraphism can lead to irreversible neurological complications, early diagnosis and treatment are necessary. It can be suspected from the presence of any cutaneous abnormality. We report a case with bony spur formation on the top of the 5th lumbar vertebra spinose process covered with skin mimicking a meningocel sac. By the help of this bony spur tethered cord could be diagnosed before any neurological deficit.     

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.     

神经管闭合不全的手术疗效探讨

对手术治疗的４０例神经管闭合不全患者的神经功能状态做临床评价及胫后神经皮层体感诱发电位（ＣＳＥＰ）测量，发现手术前后神经缺陷的临床评分有显著差异（Ｐ＜０．００２）。行胫后神经ＣＳＥＰ检查的２０例患者，１６例手术后双下肢胫后神经ＣＳＥＰＰ４０峰潜伏期明显缩短（Ｐ＜０．０００５）；另４例手术前５根胫后神经ＣＳＥＰ波形消失而术后恢复（Ｐ＜０．０１）。说明手术是一有效的治疗方法，不仅可阻止神经缺陷的发展，而且使神经缺陷得到改善。     

Lumbosacral ectopic nephrogenic rest unassociated with spinal dysraphism

Nephrogenic rests (NRs) are thought to originate from persistent nephrogenic blastema and are considered precursor lesions of Wilms' tumor (WT). These rests usually occur as perilobar and intralobar lesions in the kidney and, rarely, in ectopic sites. We report a midline lumbosacral ectopic NR in a healthy full-term newborn male with no family history of WT or WT-associated syndromes. The NR presented as a soft polypoid mass covered by normal skin. An MRI study revealed no lumbosacral spine abnormalities and no communication with the vertebral canal. The resected mass measured 3 cm and contained fat and had a central 1.2-cm solid nodule. The nodule was composed of blastema, epithelial elements (mature tubules and nephrons), and abundant stroma. No other somatic tissue elements were identified after complete microscopic examination. There are 4 cases of NRs reported in the lumbosacral area associated with spinal dysraphism, and only 2 cases, in addition to our report, unassociated with spinal abnormalities. The pathogenesis of heterotopic immature nephrogenic tissue remains a source of conjecture and speculation. If these lesions are heterotopic rests, their potential for neoplastic progression is probably quite limited, but if a monodermal teratoma, then more scrupulous clinical follow-up is warranted.     

Neurogenic disorders of bladder emptying in closed spinal dysraphism]

Closed (occult) spinal dysraphism, e.g. lipomyelomeningocele, intraspinal lipoma, diastematomyelia, the tethered spinal cord in its various forms and dysgenesis of the sacrum, is often diagnosed late and only symptoms of neurogenic bladder dysfunction are present. A lipomyelomeningocele mostly causes detrusor and sphincter dysfunction, as was the case in five of six children among our patients. However, improvement of neurological and urological symptoms after the operation can only be achieved in about 40%. Four of eight children with diastematomyelia suffered from neurogenic bladder dysfunction; three have meanwhile undergone surgery with complete recovery in one, no relevant change in the second, and worsening in the third. Originally a specific term, the "tethered spinal cord" when associated with spinal dysraphism has taken on a more general meaning. Nowadays this term is not only used for a short, thickened and tight filum terminale, but comprises any pathology, which prevents the spinal cord from ascending. MRI examination of the craniovertebral junction and spinal cord of patients with treated myelomeningocele often reveals secondary pathologic changes: these may be areas of cord atrophy, hydromyelic cavitation or ventral compression from arachnoid cysts with clinical symptoms mostly after the age of 5 years. In these children a changing urodynamic pattern may therefore be caused by such a pathology and is an indication for a thorough neurological examination including MRI. Of all the dysrhaphic states mentioned above, sacral dysgenesis is the most frequent. The sacral osteological anomaly, as a numerical and as a structural anomaly, also determines the neuro-urological deficit.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Adult presentation of spinal dysraphism and tandem diastematomyelia.

BACKGROUND CONTEXT: Diastematomyelia is a split-cord malformation often accompanied by other cord or column anomalies. PURPOSE: To report on an adult patient with diastematomyelia and discuss the embryological basis and related developmental sequelae of this split-cord malformation. STUDY DESIGN: Case report. METHODS: A summary of the management of a 54-year-old woman with recent clinical symptomatology related to an undiagnosed split-cord malformation is presented with accompanying literature review. RESULTS: A rare adult presentation of diastematomyelia with accompanying intradural extramedullary epidermoid tumor was repaired with resection of the soft-tissue mass and excision of the fibro-osseous septum. CONCLUSION: Initial presentation of diastematomyelia is rarely seen in adults; accompanying pathology includes scoliosis, tethered cord, and intradural tumors. Effective treatment involves identification of the primary pathology.     

Delayed diagnosis of voiding dysfunction: occult spinal dysraphism

The recognition of spinal dysraphism as a potential cause of voiding dysfunction is of the utmost importance if appropriate treatment and followup are to be initiated. We present 2 women with prolonged voiding dysfunction who had spinal dysraphism, intradural lipoma and related urodynamic abnormalities. The importance of initial symptom recognition, physical examination, urodynamic evaluation, appropriate treatment and periodic re-examination to detect progressive neurological impairment is emphasized.     

Occult spinal dysraphism and its association with hip dysplasia in females

We examined the pelvic radiographs of two groups of patients (more than 12 years of age) from six medical centres. Hip dysplasia was considered to be present if Shenton's line was broken and more than one third of the femoral head was revealed to be uncovered in an antero-posterior radiograph of the pelvis. Patients with hip dysplasia due to teratological or neurological causes were excluded. There were 291 patients with treated or untreated hip dysplasia in the dysplastic group. The control group of 415 individuals was collected from consecutive outpatients (with a pre-set standardised female/male ratio) for whom an antero-posterior radiograph of the pelvis had been made in one of two medical centres and which did not disclose any abnormality of the hip joints. The aim of the study was to assess the co-existence of hip dysplasia and occult spinal dysraphism. Radiographs of all patients were examined, and any partial or complete defect of the posterior vertebral arch was recorded. In the dysplastic group, a defect was recorded in 23% (67/291) radiographs and in the control group in 12% (48/415). In both groups, L5 and S1 were the most commonly recorded sites with a defect. In the dysplastic group, a defect was recorded in 56/190 females and in the control group in 30/302 females. In males, there was no significant difference between the recorded findings in the two groups. In females with hip dysplasia, occult spinal dysraphism seems to be fairly common.