SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
The practitioner is faced with the challenge of identifying occult presentations of spinal dysraphism and providing follow-up treatment of foot and leg deformities. The initial symptoms of a spinal dysraphic condition may be subtle and involve the lower extremity. An increased understanding of spinal dysraphism will encourage early diagnosis and prompt effective treatment.
The progressive neurologic dysfunction caused by occult spinal dysraphism can be prevented with early clinical recognition, radiographic diagnosis, and neurosurgical treatment. However, detection of occult spinal dysraphism in the infant is difficult because neurologic symptoms often are not apparent until the child becomes ambulatory. Occult spinal dysraphism, however, can be suspected in the asymptomatic neonate when cutaneous stigmata, such as hemangiomas, hairy patches, deep and/or eccentric dimples, or subcutaneous masses are seen over the lumbosacral spine. Because of the serious, often irreversible, sequelae of a delayed diagnosis, spinal sonography of high-risk infants with midline, lumbosacral, cutaneous stigmata should be considered as an effective, noninvasive screening method.
Secondary tethered cord syndrome following initial repair for spinal dysraphism is an important area of interest. In this study, 32 cases with spinal dysraphism in the lumbosacral region were enrolled, in whom radical repair with autologous material had been carried out in the early stage soon after birth. During the follow-up period of up to 19 years 10 months, surgery was considered to be indicated in 2 of the 8 lipomeningocele cases and in 6 of the 24 meningocele and meningomyelocele cases, because of the presence of tethered cord syndrome 4-19 years after the primary operation. In all 8 of these cases, MR imaging demonstrated tethered spinal cord in the form of low conus medullaris. In 6 of the 8 operated cases surgery was followed by improvement of the spinal neurological deterioration. According to our experience, early untethering for secondary tethered cord syndrome is essential. In addition, since the complications of Silastic duraplasty at untethering were all minor and the operative outcome was satisfactory, the use of silicone rubber sheeting as a dural substitute might be recommended to prevent adhesion of the spinal cord.
OBJECTIVE: To investigate whether multiple surgery is responsible for the high prevalence of latex hypersensitivity in patients with spinal dysraphism by comparing the results of a skin-prick test in three groups of patients with different surgical histories. PATIENTS AND METHODS: An in vivo diagnostic kit was used to determine the sensitivity to latex using a skin-prick test in three groups of patients, 23 with spinal dysraphism who had undergone surgery, 20 patients who had undergone non-urological surgery and urological patients who had not undergone surgery (control). There was no history of latex allergy in any individual from the three groups. RESULTS: The skin-test was positive in six patients with spinal dysraphism, in one who had undergone previous non-urological surgery and in none of the control patients. The prevalence of latex hypersensitivity was significantly higher in patients with spinal dysraphism than in the other groups (non-urological surgery P < 0.05 and control P < 0.01). This difference occurred despite the patients in each surgical group having undergone a mean of less than two operations. There was no significant difference in latex sensitivity between control patients and those undergoing non-urological surgery. CONCLUSION: Despite having no history of latex hypersensitivity, about a quarter of patients with spinal dysraphism were sensitive to latex. Repeated surgery alone cannot be responsible for the greater incidence of latex hypersensitivity in these patients. Despite multiple surgery being accepted as playing a major role in the development of latex hypersensitivity, relatively fewer surgical episodes than reported previously seem to be sufficient to elicit latex allergy.
Automated water path ultrasound scanning was used to examine 15 infants and children with suspected spinal dysraphism. The spinal abnormalities demonstrated by ultrasound included meningocele, myelomeningocele, and spinal lipoma. In addition, renal abnormalities (pyelocaliectasis and hydronephrosis) were seen in 5 patients. It is concluded that ultrasound examination of the neural axis should play an important role in evaluation of children with spinal dysraphism.
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.
OBJECTIVE: To evaluate the clinical profiles, image findings and surgical outcome of 155 cases of spinal dysraphism. METHODS: 155 patients were studied prospectively (143) or retrospectively (12). The male to female ratio was 1.5:1. Mean age at presentation was 5.7 years. Out of 155 cases of spinal dysraphism, 119 had open spina bifida [meningomyelocele (MMC) in 113 (72%), meningocele in 3 (2%) and myelocystocele in 3 (2%)] and 36 had occult spina bifida [split cord malformation (SCM) without overt MMC sac (pure SCM) in 29 (19%) and midline dermal sinus in 7 (4.5%)]. Lipomeningomyelocele constituted 73 of the 113 cases of MMC (65%). Twenty cases of MMC (18%) had associated SCM (complex spina bifida). The total number of cases with SCM was 49 (32%). Twenty-four children with MMC presented with an operative scar from previous surgery, performed at the periphery. After a detailed clinical evaluation of all patients, craniospinal MRI was the preferable mode of investigation. All patients underwent surgery and were clinically assessed over a mean follow-up period of 3.6 years. RESULTS: MMC sac was the commonest skin manifestation seen in 89/155 cases (57%). Hypertrichosis and previous operative scar were noticed in 24 cases each (15%). Cutaneous hemangioma, skin tag and multiple neurofibroma were the other common superficial skin manifestations. Clinically, 103 patients (66.5%) had variable weakness of the lower limbs, and muscle atrophy was noticed in 56 cases (41%). Graded sensory loss, sphincteric dysfunction, trophic ulcer and backache were present in 89 (57%), 64 (36%), 17 (11%) and 9 cases (6%), respectively. Significant scoliosis in 56 cases (36%) and congenital talipes equinovarus in 51 cases (33%) were the most frequent neuroorthopedic deformities apart from high-arched foot, leg length discrepancy and flat foot. Common image findings were low-lying cord in 101 (65%), neural placode in 76/113 (49%), hydrosyrinx in 42 (27%), hydrocephalus in 71 (46%) and Chiari malformation in 62 cases (45%). The lumbosacral region in 56 cases (38%), followed by the lumbar region in 47 (30%), were the most common sites of occurrence of spina bifida. Of 71 hydrocephalic patients, 39 (55%) needed shunting before definitive surgery and 21 (30%) after the surgery. Eleven patients (15%) did not require shunting at all. CSF leak in 51 patients (33%), pseudomeningocele in 26 (17%), wound infection in 22 (14%) and meningitis in 13 (8%) were the most common postoperative complications. Two patients died in the postoperative period. During an average follow-up of 3.6 years (range 1.5-8 years), motor weakness improved in 47 children (45%) and remained static in 53 (52%), and 3 children showed deterioration in motor power. Sensory dysfunction improved in 43 (48%), remained static in 42 (47%) and deteriorated in 4 patients (4%). Sphincteric function clinically improved in 26 patients (41%) and was static in 38 (59%), and 6 patients (4%) had deterioration. Backache was dramatically relieved in all 9 patients with this complaint (100%). In 13 children with trophic ulcer (76%), it completely healed. CONCLUSION: A significant proportion of patients with open spinal dysraphism harbor an underlying SCM, an entity coined complex spina bifida (in our previous study). The incidence of hydrocephalus in spinal dysraphism is low in India in comparison to the Western world. Some patients with mild hydrocephalus on scan may not require a shunt operation. Surgical intervention should be early after entire neural axis screening by MRI.
Somatosensory evoked potentials (SEP) are not routinely used in spinal dysraphism. In this study 38 patients (29 children and 9 adults) with spinal dysraphism were prospectively studied with the objective of evaluating whether SEPs were a prognostic tool that could be used to predict clinical improvement after repair of a spinal dysraphic lesion. For all patients, preoperative SEP and postoperative SEP (within 1 week of operation) were recorded. Fifteen of these patients also had follow-up postoperative SEP recordings taken within 3 months of operation. A clinical examination was performed at the time of each SEP. Thirty patients had tethered cord, 12 had diastematomyelia and 15 had intra- and/or extradural tumours, which included lipomas and dermoid and epidermoid tumours. Twenty-one children and all adults had abnormal preoperative SEPs. Sixteen children and 4 adults had improved SEPs postoperatively. All these children and 2 of the 4 adults also experienced clinical improvement. Improvement in SEPs preceded clinical improvement in 12/20 patients. We observe that SEPs have a good prognostic value.
Spinal dysraphism is easily recognized in the overt form as a meningocele or myelomeningocele. The closed form or occult spinal dysraphism (OSD) can be overlooked. It occurs predominantly at the lumbosacral level, but OSD at the cervical level, although very rare, also occurs. The value of magnetic resonance imaging investigations in preparation for surgical treatment is emphasized. We discuss the value of various midline posterior skin anomalies as indicators of an underlying developmental defect in the neural axis. Hallmarks for OSD in the inferior third of the back are well known. They can also occur at the cervical level. Among these warning cutaneous midline changes, a vascular stain alone is rarely a clue for OSD whatever the spinal level involved, and specifically in the nuchal area.
An unusual case of dorsal spinal dysraphism in a four year old child is presented. Various abnormalities including diastematomyelia, an extradural arachnoid cyst arising from one of the two dural tubes, and lipomeningomyelocele with a related rib-like bony structure were demonstrated with Iohexol CT and subsequently confirmed at surgery.
We describe two cases of spinal dysraphism where detection of the cutaneous signs - namely a faun-tail naevus and diffuse hypertrichosis - led to early recognition of the occult neurological abnormalities and institution of corrective surgery. The dermatologist may be the first physician to observe these skin changes and an early neurosurgical referral can prevent subsequent neurological complications.
Thirty-seven patients with a wide spectrum of spinal dysraphic lesions were studied by both magnetic resonance imaging (MRI) and myelography complemented by computed tomography (CT). Magnetic resonance imaging proved to be superior for tissue characterization, overall anatomical definition, topographical relationships of the cranio-cervical junction and demonstration of the presence and extent of hydrosyringomyelia. Demonstration by CT myelography of fine neural structures such as the filum terminale and nerve roots remains the superior technique; however, with constantly improving spatial resolution and thinner-slice imaging, MRI will become increasingly competitive in this respect. The osseous component of these lesions was best demonstrated by CT myelography. A high incidence of associated syrinx (8/15 cases) was found in the diastematomyelia group, with an overall incidence of 15 cases in all the dysraphic patients studied. The use of both T1- and T2-weighted sequences is required for tissue characterization. Whilst both imaging modalities proved complementary in the investigation of spinal dysraphism, we suggest that, with its superior tissue characterization and anatomical display of these lesions. MRI should be the primary imaging technique. Avoidance of an invasive procedure and use of ionizing radiation confers additional benefits upon MRI. The relative ease and shorter examination time of MRI makes this technique ideal for pre- and post-operative monitoring and assessment.
PURPOSE: To prospectively evaluate the role of magnetic resonance (MR) urography in the radiologic assessment of patients with spinal dysraphism. MATERIALS AND METHODS: Fourteen patients with spinal dysraphism were referred for MR urography with half-Fourier rapid acquisition with relaxation enhancement (RARE) (repetition time msec/echo time msec = 11.9/95) and RARE (2,800/1,100) sequences on a 1.5-T MR machine. Six patients did not tolerate MR urography owing to claustrophobia (n = 4) or flexion deformities (n = 2), giving a final success rate of 57% (eight patients). Two patients had a single kidney (one after nephrectomy, one with a crossed-fused ectopic kidney). Images were jointly assessed by two radiologists and compared with excretory urographic studies. The signal intensity ratio and contrast-to-noise ratio were also calculated. RESULTS: Visualization of the kidneys, pelvicaliceal system, and ureters was better with half-Fourier RARE than with RARE imaging, whereas visualization of the bladder was comparable with both sequences. The mean signal intensity ratios for half-Fourier RARE and RARE sequences, respectively, were 16.2 +/- 3.65 and 19.2 +/- 3.65 (P =. 58, factorial analysis of variance model), whereas the mean contrast-to-noise ratios were 55.4 +/- 5.16 and 47.8 +/- 5.16 (P =. 34). Cortical scarring was depicted more clearly at MR urography than at excretory urography, whereas a renal calculus seen at excretory urography was not detected at MR urography. CONCLUSION: MR urography was feasible in 57% of patients with spinal dysraphism and is a safe, accurate method of evaluating the upper urinary tract.
The acro-osteolyses are a heterogeneous group of disorders characterized by bone resorption. The disorder may occur as familial, idiopathic, or secondary to vascular, inflammatory, or neurologic conditions. Acro-osteolysis is rare in association with spinal dysraphism. It is even rarer for it to be the presenting symptom in spinal dysraphism. We report here four patients in whom the diagnosis of spinal dysraphism was established while investigating for the various causes of acro-osteolysis. All four patients presented with trophic changes and acro-osteolysis. Hyperhidrosis in the affected limb was seen in three patients. One patient had leg pain, the others had no sensory or motor deficits. Magnetic resonance imaging showed spinal dysraphism in all four patients.
The combined investigations of positive contrast myelography and computed tomographic (CT) myelography were performed on 53 consecutive children. Thirty-eight (72%) of these investigations were performed as a routine pre-operative procedure to identify occult spinal dysraphism in patients with childhood scoliosis; the remainder were because of the "orthopaedic syndrome", cervical radiculopathy, back pain and patients with clinical findings to suggest spinal dysraphism. In the 20 patients (38%) with idiopathic scoliosis, there was no case of spinal dysraphism and CT myelography provided no additional information to the myelogram. In the seven patients with spinal dysraphism the plain radiographic abnormalities identified were lumbar vertebral abnormalities (five), thoracic vertebral abnormalities (one), and sacral agenesis (one). Diastematomyelia was found in four patients, a low tethered cord and lipoma in two patients and a large lipoma in one patient. In all of these cases the myelogram indicated the intraspinal abnormalities; however, CT myelography provided more precise anatomical detail. We conclude that CT myelography is not indicated in the initial preoperative assessment of idiopathic scoliosis but should be reserved for patients with congenital or complicated scoliosis where the association with dysraphism is well recognised.
Some skin lesions over the spine are known to be associated with occult spinal dysraphism, but the significance of common skin lesions, such as sacral pits and dimples, is uncertain. In this prospective study, 95 neonates (1.9% of 4989 live births) were referred with possible markers of occult spinal dysraphism. Seven of 94 babies examined had abnormalities demonstrated by spinal ultrasound, compared with 5 of 105 controls. In 2 of 94 and 3 of 105 of these, the conus medullaris was located at L3 but no other abnormalities were found. Of the 75 babies with a sacral dimple or pit alone, none had an abnormality, suggesting that these skin lesions do not indicate a high risk of occult spinal dysraphism.
A 4-year-old child presenting with sudden-onset paraplegia and a sacral tumour in association with spina bifida occulta is reported. There were no stigmata of spinal dysraphism at birth. Imaging studies confirmed a sacral tumour with extradural extension up to T10 and spinal dysraphism. The histological features of the extradural and sacral components of the tumour were consistent with a Wilms tumour. The differential diagnosis included a primary sacral teratoma containing Wilms tumour elements or a primary extrarenal Wilms tumour arising in association with a spinal dysraphism. There was no clinical response to chemotherapy or radiotherapy.
STUDY DESIGN: First published report of an adult complex spinal dysraphism with situs inversus. OBJECTIVES: To describe a previously asymptomatic adult patient of multiple vertebral anomalies with cervical split cord malformation type II, tethering of the spinal cord (cervical and lumbar), and intraspinal arachnoid cyst along with dextrocardia and situs inversus. SUMMARY OF BACKGROUND DATA: Only 5 cases (fetus, 1; neonates, 3; child, 1) of spinal dysraphism with dextrocardia or situs inversus have been reported. All these cases have had associated multiorgan developmental anomalies usually incompatible with survival and requiring multidisciplinary care. METHODS: The case has been described and relevant literature reviewed. RESULTS: The patient was operated for cervical and lumbar levels in the same sitting. A C4-C5 laminectomy was performed, 2 hemicords enclosed in the same dural sac were visualized, dorsal paramedian nerve roots and the tethering arachnoid bands were cut, and the arachnoid cyst wall was partially excised. This was followed by L4-L5 laminectomy and detethering by sectioning of the thickened filum terminale. The patient showed significant neurologic improvement after surgery. CONCLUSIONS: The present case is a rare instance in which there has been an association of adult onset occult spinal dysraphism along with situs inversus totalis. Successful management requires appropriate understanding of embryology, anatomy, and imaging and has implications in neurosurgical and perioperative anesthetic care.