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PubMedDate not supplied

US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases.

Ultrasonography (US) of the spinal cord is performed in newborns with signs of spinal disease (cutaneous lesions of the back, deformities of the spinal column, neurologic disturbances, suspected spinal cord injury due to traumatic birth, and syndromes with associated spinal cord compression). The examination is performed with high-frequency linear- and curved-array transducers in the sagittal and axial planes from the craniocervical junction to the sacrum. Normal variants such as transient dilatation of the central canal and ventriculus terminalis can be demonstrated with US. US allows detection of congenital malformations, such as myelocele or myelomeningocele, spinal lipoma, dorsal dermal sinus, tight filum terminale syndrome, diastematomyelia, terminal myelocystocele, lateral meningocele, caudal regression syndrome, and hydromyelia or syringomyelia. Acquired intraspinal diseases following birth trauma and transient alterations after lumbar puncture can also be detected with US. US can demonstrate the entire spectrum of intraspinal anatomy and pathologic conditions with high geometric resolution. Therefore, US should be considered the initial imaging modality of choice for investigating the spinal cord in newborns.

Birth Injuries↗
PubMedDate not supplied

The sacrum: pathologic spectrum, multimodality imaging, and subspecialty approach.

The sacrum is a structure that is imaged by both general and subspecialty radiologists. A wide variety of disease processes can involve the sacrum either focally or as part of a systemic process. Plain radiographs, although limited in evaluation of the sacrum, should be carefully examined when abnormalities of the sacrum are suspected. Cross-sectional imaging, particularly computed tomography and magnetic resonance (MR) imaging, plays a crucial role in identification, localization, and characterization of sacral lesions. Congenital lesions of the sacrum, including sacral agenesis and meningocele, are optimally imaged with MR. The most common sacral neoplasm is metastatic disease. Primary neoplasms of the sacrum, which include giant cell tumor, chordoma, and teratoma, are infrequent. Infection of the sacrum or sacroiliac joint is most often due to contiguous spread from a suppurative focus. A wide variety of arthritic disorders such as ankylosing spondylitis and osteoarthritis can involve the sacroiliac joints as part of a localized or systemic process. Sacral fractures related to acute trauma or repetitive stress are difficult to diagnose and treat. Knowledge of these abnormalities and familiarity with the imaging of these processes will allow radiologists of all subspecialties to contribute to the diagnosis and management of sacral disorders.

Diagnosis, Differential↗
PubMedDate not supplied

Retrorectal developmental cysts in adults: clinical and radiologic-histopathologic review, differential diagnosis, and treatment.

Developmental cysts are the most common retrorectal cystic lesions in adults, occurring mostly in middle-aged women. They are classified as epidermoid cysts, dermoid cysts, enteric cysts (tailgut cysts and cystic rectal duplication), and neurenteric cysts according to their origin and histopathologic features. Although developmental cysts are often asymptomatic, patients may present with symptoms resulting from local mass effect (eg, constipation, rectal fullness, lower abdominal pain, dysuria), with a palpable retrorectal mass at digital rectal examination, or with a complication. Infection with fistulization, bleeding, and malignant degeneration are the major complications of developmental cysts. A well-defined, unilocular or multilocular, thin-walled cystic lesion is the main imaging feature. Uncommonly, a sacral bone defect and calcifications are associated with developmental cysts. The differential diagnosis includes cystic sacrococcygeal teratoma, anterior sacral meningocele, anal duct or gland cyst, necrotic rectal leiomyosarcoma, extraperitoneal adenomucinosis, cystic lymphangioma, pyogenic abscess, neurogenic cyst, and necrotic sacral chordoma. Complete surgical excision is indicated to establish the diagnosis and avoid complications.

Adult↗
PubMed1984

Traumatic dural tears: CT diagnosis using metrizamide.

Computed tomography of the spine using intrathecal metrizamide was performed on six patients with spinal trauma. Dural tears with contrast material escaping outside the subarachnoid space were documented in five cases. In one case, the thecal laceration had healed and the contrast material was confined in a post-traumatic meningocele. The characteristics of dural tears are demonstrable by metrizamide computed tomography, either in conjunction with myelography or as a separate procedure. Neurological deficits following spinal trauma may be aggravated by dural tears with entrapment of the spinal nerve roots.

Adult↗
PubMed1987

Spinal and paraspinal neurofibromatosis: surface coil MR imaging at 1.5 T1.

Twelve patients with neurofibromatosis affecting the spine and paraspinal regions were examined with magnetic resonance (MR) imaging performed using a 1.5-T magnet, surface coils, and spin-echo pulse sequences. In all 11 patients with neurofibromas, these tumors had slightly greater signal intensity than muscle on T1-weighted images and markedly increased signal intensity on T2-weighted images. In seven patients central areas of decreased signal intensity within the neurofibromas were demonstrated on T2-weighted images. In one patient these central areas corresponded pathologically to relatively fibrous components of the tumors surrounded by myxoid matrix. Five patients had extensive plexiform neurofibromatosis, one had lateral meningoceles, one had a neurofibrosarcoma, three had extradural spinal cord compression, and two had cystic intramedullary tumors of the spinal cord. The ability to obtain high-resolution, multiplanar images in a noninvasive fashion makes surface coil MR imaging an ideal technique for evaluating neurofibromatosis involving the spinal cord, spinal canal, and paraspinal soft tissues.

Humans↗
PubMed1989

Fetal and funicular vascular anomalies: identification with prenatal US.

Vascular anomalies of the umbilical cord and fetus were evaluated with ultrasound in 11 fetuses. The anomalies included true knot of the cord (n = 1), umbilical vein varix (n = 1), persistent right umbilical vein (n = 1), and unilateral hypertrophy of the iliac artery in cases of a single umbilical artery (n = 8). One fetus with a single umbilical artery had a meningocele, hydrocephalus, and polyhydramnios; another had mitral atresia. The other fetuses were healthy except for the vascular abnormalities. A previously unreported finding in the identification of a single umbilical artery is described.

Congenital Abnormalities↗
PubMed1991

Fetal cephaloceles: diagnosis with US.

The prenatal sonograms of 15 fetuses with cephaloceles (13 occipital, one ethmoidal, and one frontoparietal) were retrospectively reviewed. The prognosis for fetuses with cephaloceles was poor: Only three of 14 with follow-up were born alive (21%), and all are likely to be handicapped. Karyotypes were abnormal in four of nine fetuses tested (44%). Associated cranial abnormalities observed in various numbers of fetuses included ventriculomegaly, the "lemon" sign, a flat basioccipital, "beaked" tectum, and bone defect. A wide variety of neural and nonneural anomalies were associated with cephaloceles. At least one sonographically observed concomitant neural or nonneural axis abnormality or an abnormal karyotype was present in nine of 15 fetuses (60%). Some associated neural malformations noted pathologically were not observed prenatally. Cranial meningoceles were not accurately distinguished from encephaloceles sonographically, and they also were associated with a poor prognosis. Comparison of findings in fetuses with cephaloceles with those of 15 fetuses with cystic hygromas revealed that the two abnormalities can usually be distinguished by the relationship of the mass with the neck and by other cranial findings present in fetuses with cephaloceles but absent in association with cystic hygromas.

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↗
PubMed2002

Massive pleural effusion as the presenting feature of a subarachnoid-pleural fistula.

A 14-year-old boy presented with a large symptomatic transudative pleural effusion 4 months after spinal surgery for kyphoscoliosis. Computed tomography myelography confirmed a subarachnoid-pleural fistula (SPF) with a pseudo-meningocele communicating with the left pleural space. A review of the literature indicates this to be a rare finding. The possibility of SPF should be entertained in patients who present with a pleural effusion following transthoracic spinal surgeries.

Adolescent↗
PubMedDate not supplied

Tegmental defects and cerebrospinal fluid otorrhea.

Congenital tegmental defects that present as unsuspected cerebrospinal fluid (CSF) otorrhea are diagnostic and therapeutic challenges. We reviewed 5 such patients to determine an optimal strategy for evaluation. Five patients presented with watery otorrhea, 4 of them after ventilation tube placement, and only 1 with rhinorrhea. The preoperative analysis of middle ear effusion for beta(2)-transferrin was positive in 2/4, equivocal in 1/4 and false negative in 1/4. Computerized tomography (CT) revealed nonspecific tegmental defects in all 5 patients. Magnetic resonance imaging (MRI) demonstrated meningoencephalocele in 3/5 and dural irregularity in 1/5. Tegmental defects were confirmed at surgery in all cases, demonstrating meningocele or arachnoid granulations in 2/5 and encephalocele in 2/5 patients. We recommend a combination of beta(2)-transferrin analysis to verify CSF, high resolution CT (axial and coronal planes) to diagnose tegmental defects, and MRI (multiplanar) to evaluate the type of herniation. A combination mastoid and middle fossa approach for definitive repair is suggested.

Aged↗
PubMed2001

Stereotactic neuroendoscopic management of hydrocephalus: a three-year follow-up and analysis of Malaysian children with aqueduct stenosis.

The beneficial effects of stereotactic third ventriculostomy versus ventriculoperitoneal shunt were evaluated in 62 paediatric patients and analysed in relation to age, sex, clinical history, presence of meningomyelocele, magnetic resonance imaging measurements of hydrocephalus and third ventricle floor size. The third ventriculostomy were done on 50 patients using the Richard-Wolf Caemaert Endoscope and the Leksell Stereotactic Frame Model G. These patients were operated using the 4-French Fogarty catheter to open the base of the third ventricle. During the same period of study 12 paediatric patients with aqueduct stenosis who were managed by ventriculoperitoneal shunt were included. Both surgical procedures were compared. Statistically univariate analysis revealed that those patient with an age group of more than six months undergoing ventriculostomy had good outcome. Multivariate analysis revealed that past history of haemorrhage and/or meningitis were predictors of poor outcome. Sex, size of lumbar meningocele at birth, abnormal ventricular anatomy or narrow third ventricular floor size were non predictors of bad outcome in these patients. There was no difference in outcome in both the shunt or ventriculostomy group.

Cerebral Aqueduct↗
PubMed2003

Spinal dysraphism: trends in northern India.

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.

Adolescent↗
PubMed2006

Giant terminal lipomyelocystocele.

Terminal myelocystocele is a rare form of occult spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. A 1-year-old female child presented with a large lumbosacral mass (30 x 20 x 10 cm), flaccid paraplegia and urinary incontinence since birth. Magnetic resonance imaging revealed a low-lying conus (with associated conus lipoma) and a dilated central canal surrounded by a meningocele suggestive of terminal lipomyelocystocele and was operated on successfully. In our experience, this was a giant terminal lipomyelocystocele and such a large lesion has not been reported in the literature before. Terminal myelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.

Diagnosis, Differential↗
PubMed1979

Klippel-Feil syndrome, iniencephalus, anencephalus, hindbrain hernia and mirror movements: overdistention of the neural tube.

Bony anomalies encountered in the 'no neck' form of Klippel-Feil syndrome (KFS) are a wide, short, fused, bifid, retroflexed spinal canal; craniolacunia, cranium bifidum, and acrania. The only symptom may be mirror movement (MM). The CNS anomalies are hindbrain hernia, hydrocephalus, hydromyelia, syringomyelia, meningocele, myelocele, encephalocele, and anencephalus. In severe KFS, i.e. iniencephalus (IN) and in anencephalus (AN), the inion is in contact with the back. In both there is hindbrain hernia and the left thorax may contain the stomach tethered to an anterior spina bifida. KFS results from distortion of somites by an overdistended neural tube. A neural tube that fails to close cannot overdistend.

Adolescent↗
PubMed1983

Diastematomyelia: report of 21 cases surgically treated by a neurosurgical and orthopedic team.

We studied 21 cases of diastematomyelia associated with myelo-meningocele (3 cases) or congenital spine deformities (18 cases). Analysis of bone and neuraxis abnormalities with special reference to the spinal canal, related to operative findings demonstrate in all cases an embarrassed cord by localized compression or global narrowing of the malformed spinal canal (11 cases), bone spur fixation (13 cases) and tethered cord associated lesions (5 cases). Mechanisms of neural damage and prophylactic value of laminectomy before the orthopedic treatment of the curve during the same operation is discussed.

Adolescent↗
PubMed1997

Split cord malformations of the lumbar region. A model for the neurosurgical management of all types of 'occult' spinal dysraphism?

From a group of 84 patients with split cord malformations presenting to our Department between 1976 and 1990, we have selected 47 cases in whom the split cord was confined to the lower dorsal-lumbar region and in whom there were no other dysraphic features such as meningocele, lipoma or dermoid cyst. We have studied these cases of 'pure split cord malformation' in an attempt to decipher its natural history and the effects of surgical procedures designed to untether the spinal cord. We conclude that the 'neuro-orthopaedic syndrome' (which includes lower limb asymmetry, talipes and modest sensory and motor problems) is, in most cases, an inevitable consequence of the abnormal functional anatomy of the split cord and is not due to any mechanical effects of tethering of the spinal cord. Its emergence as a child grows (assuming that it was not obvious at birth) is not influenced by surgery. True neurological deterioration (defined as the loss of a previously established neurological or urological function) is a rarer event. It may occur at any age--including well into adulthood--but in children the average age at presentation is 6.8 years (range 2 years 9 months to 11 years). It is due to the mechanical effects of tethering of the spinal cord and is more likely to be arrested than improved by surgery. 'True' deterioration did not occur in any of our cases who had undergone prophylactic untethering of the spinal cord and we have concluded, therefore, that surgery has a role in the prevention of late neurological problems affecting the lower limbs and bladder. Surgery to untether the spinal cord will not, however, have any affect upon the emergence of the neuro-orthopaedic syndrome.

Adolescent↗
PubMed1991

Early diagnosis and treatment of spinal dysraphism to prevent urinary incontinence.

Spinal dysraphism represents a very rare congenital anomaly of the spinal cord and cauda equina, often associated with cutaneous sacral lesions. This condition results in distorsion of neural tissue, possibly leading to vesical dysfunction and urinary incontinence. A series of 12 children with spinal dysraphism (4 lipomeningoceles with tethered cord, 3 tight filum terminale, 2 dermal sinuses, 2 arachnoid cysts, 1 anterior meningocele) observed during the last 6 years is presented. The patients are divided into two groups: group A includes 5 infants (age range 4 months-2 years) and group B 7 older children (4-12 years). All 7 patients of group B were referred for urinary incontinence as their chief complaint; only 1 had evidence of a skin lesion while 3 had a club foot. In group A, 4 had skin lesions (2 asymptomatic and 2 dermal sinuses referred after several episodes of meningitis) and the 5th child had a club foot. The diagnosis was made by myelo-CT scan in the earlier 4 and by magnetic resonance imaging in the more recent 8 cases. All 12 children were operated. In group B, the patients' urinary incontinence persisted but did not worsen (3 were stable and 4 had slightly improved); in group A, 2 were dry (follow-up greater than 2 years), 1 could not be accurately evaluated because he was less than 2 years of age, and the 2 who suffered from meningitis were incontinent. Early diagnosis and neurosurgical treatment of spinal dysraphism may prevent urinary incontinence. Accurate urodynamic, neurophysiologic, and neuroradiologic evaluation of children with severe voiding anomalies or club foot is recommended even if no lumbosacral cutaneous lesions are present.

Child↗
PubMed1975

Late follow-up in spina bifida cystica.

The further progress of the survivors in 1969, of a group of 150 babies born with spina bifida cystica in the period 1960-66 is described. Children with a meningocele continued to do well. Those with a myelomeningocele are divided into 2 groups. There were 4 late deaths and a considerable degree of handicap in the group of 23 in whom the spinal cord was exposed on the surface as a plaque at birth. There were 2 late deaths and a much lower incidence of handicap in the group of 25 in whom only ectopic nervous tissue was found in the sac at birth. The incidence and treatment of hydrocephalus is described. Changes in the attitude to the treatment of a neonate with a myelomeningocele are discussed.

Follow-Up Studies↗