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Lipomyelomeningocele is one of the more common forms of occult spinal dysraphism seen in clinical practice. It is now universally accepted that prophylactic surgery is indicated in most cases, but areas of controversy remain. These include the diagnosis and management of retethering, the value of intraoperative physiological monitoring, and indications for surgery in stable adults and when the conus is in the normal position.
Spina bifida, Chiari type II malformation, cerebral aqueduct stenosis and hydrocephalus are the most frequent association anomalies in the congenital malformation of the central nervous system (Warkany et al., 1958). They are potentially treatable and of clinical importance. But the relationship between hydrocephalus and Chiari type II malformation is still a controversial subject. A single oral dose of 240 mg/kg of ethylenethiourea (ETU) was given to Sprague Dawley (SD) rats on the 11th day of gestation. Fetuses were removed in the 20th day of gestation by cesarean sections; high incidence of spinal dysraphism associated with hindbrain crowding was found in these fetuses. They are similar to Arnold-Chiari malformation in humans. We used these experimental models to analyze the relationship between hydrocephalus and Chiari type II malformation. From the present investigation, no hydrocephalus or cerebral aqueduct stenosis was found in the experimental rat fetuses with the Arnold-Chiari malformation. So we do not consider the hydrodynamic theory that Chiari type II malformation was induced by increasing intracranial pressure in hydrocephalus. Hydrocephalus in the Arnold-Chiari malformation may not be the primary disorder but seems to be caused by plugging the foramen magnum in Chiari type II malformation. So the cerebrospinal fluid in the spinal subarachnoid space can not move upward to the cranial subarachnoid space for absorption to venous return. Cerebral aqueduct stenosis may be secondarily compressed by hydrocephalus and not be the primary development anomaly or acquired occlusion due to gliosis. This is in accord with the theory proposed by Russell and Donald (1935).(ABSTRACT TRUNCATED AT 250 WORDS)
Two hundred and fourteen cases of spinal dysraphism were studied to investigate the relationship of sensory level and renal damage. It was found that in 72 cases with a high sensory level of T12 and above, 45 cases (62%) had renal damage which was the cause of death in 12; in 142 cases with a low sensory level of L1 and below, 40 cases (28%) had renal damage which was the cause of death in only 2. A palpable bladder and reflux were associated with renal damage in the cases with a low sensory level but some additional factor appears to operate in those with a high sensory level. Other reasons for the poor prognosis in these cases are discussed. The critical level in the spinal cord at which the prognosis for renal damage sharply changes coincides with the sympathetic outflow to the renal tract (T12-L1). Disruption of the spinal cord at or above the renal outflow may be a determining factor in prognosis.
PURPOSE: The correlation between timing of the primary neurosurgical repair and urological prognosis in infants with open spinal dysraphism remains unknown. We investigated the impact of primary repair timing on neurogenic bladder prognosis in children with myelomeningocele. MATERIALS AND METHODS: We retrospectively reviewed the records of 401 children with myelomeningocele followed at our multidisciplinary clinic between 1996 and 2005. Among these patients 129 were included in the study based on the availability of urological followup data at age 3 years, which was chosen as an ideal point for the standardization of urological findings. Children were assigned to 1 of 2 groups-those who underwent primary neurosurgical repair within 72 hours of delivery (group 1) and those undergoing repair after 72 hours (group 2). We compared the incidence of febrile urinary tract infections, hydronephrosis, vesicoureteral reflux and secondary tethering of the spinal cord at age 3 years, as well as cystometric bladder capacity and detrusor leak point pressure between the 2 groups. RESULTS: Urological followup data at age 3 years revealed significantly increased incidence of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering of the spinal cord in children in group 2 compared to group 1 (chi-square, p < or =0.05 for all comparisons). Urodynamic assessment also showed a significantly lower bladder capacity and a substantially higher detrusor leak point pressure in children who received primary repair at later than 72 hours (t test, p <0.05). A subgroup analysis of group 1 comparing children who underwent repair before and after 24 hours following delivery failed to demonstrate any significant difference in terms of febrile urinary tract infections, vesicoureteral reflux, hydronephrosis and secondary tethering (chi-square, p >0.05 for all comparisons), whereas mean bladder capacity was significantly higher (t test, p <0.05) and mean detrusor leak point pressure was lower (t test, p = 0.08) in children undergoing repair within 24 hours of delivery. CONCLUSIONS: The timing of primary neurosurgical repair has a significant impact on neurogenic bladder prognosis in children with myelomeningocele. Closure of the spinal lesion on the first day of life seems to provide the best chance for favorable lower urinary tract function.
Congenital midline cutaneous lesions should always alert the clinician to the possibility of spinal dysraphism. These lesions can take many different forms. The physician should be cognizant of such lesions in order to avoid potential neurologic complications. We present a patient with a midline sacral hemangioma associated with a congenital enteric sinus cyst, a previously unreported association.
We report an infant born with lumbosacral agenesis, a subcutaneous lumbosacral mass, and a solitary median maxillary incisor. Operatively, a lipomyelomeningocele was noted to extend from the dorsal hemicord of a split cord malformation. Genetically, the child was found to have a defect on chromosome 7q. To our knowledge, this is the first case report of a patient with the rare lumbosacral agenesis and solitary median maxillary incisor. Furthermore, to our understanding, this is only the fourth reported case of split cord malformation in which the hemicords are oriented in the sagittal plane and one of only a few cases reported in the medical literature of simultaneous occurrence of lumbosacral agenesis and occult spinal dysraphism, that is, split cord malformation and lipomyelomeningocele. Following a review of the medical literature, and considering that our patient did indeed have dysmorphology of chromosome 7q, we hypothesize that this single defect resulted in both cranial and caudal deformation, as exemplified by our patient. We review the medical literature for related case reports and potentially related chromosomal abnormalities and various teratogenic substances.
The syndrome of syringomyelia in children has been extensively described in the literature and is said to most commonly involve the cervical and thoracic spinal cord. We here present two children who had an unusual constellation of signs and symptoms, characterized by bowel and bladder disturbance in one, and in the other with intermittent paroxysmal severe pain and cramping and flexion of the lower extremities accompanied at times by episodes of incontinence. Both were found on magnetic resonance imaging (MRI) to have a syrinx localized to the most distal cord. This was not associated with Arnold-Chiari malformation, trauma, tumors or any other form of spinal dysraphism. Both patients underwent placement of a syringomyelia-peritoneal shunt with complete resolution of symptomatology. Postoperative MRI revealed a complete collapse of the syringomyelia cavity. In those children presenting with bowel or bladder disturbances, associated or not with intermittent pain, flexion attacks, or cramping in the lower extremities, the differential diagnosis of a syringomyelia of the distal cord should be part of the clinical considerations.
The neuropathological picture of four cases of prosencephalic malformations coexisting with spinal dysraphic anomalies inclined us to discuss the nosological position of these abnormalities. The hypoplastic anomalies of prosencephalic commissures combined with well-cleaved (except for basal ganglia) brain hemispheres allow to think of failure within lamina terminalis. The observations support the opinion that described malformations are consequence of abnormal planning of prosencephalic formation. Our description testifies ones more that as a result of prolonged time of injury a complex picture of developmental anomalies is noted.
The clinical course and surgical treatment of 48 children with lipomatous lesions of spinal dysraphism are presented. Preoperative studies consisted of complete neurological examination, IVP, myelography and CT scan if indicated. The complete clinical course and surgical treatment of the children with lipomatous lesions of the lower spine is reviewed. The surgical aim was to free all intra- and extradural adhesions by dural grafting if necessary. The results of surgical treatment are dependent upon the time of treatment (as early as possible), the location and extent of the malformation and the quality of surgical treatment.
The neurogenic bladder represents a vesical dysfunction secondary to a congenital or acquired neurologic lesion, spinal dysraphism being the most common cause in pediatric age. According to the lesion level and severity, neurogenic bladder can be hypertonic hyperreflexic, hypotonic hyporeflexic or of mixed type characterized by different clinical and urodynamic patterns and the potential cause of nephrourologic complications. These types of neurogenic bladder require diversified treatments as anticholinergic drugs, clean intermittent catheterization and antibiotic prophylaxis, separately or in various combinations. Close follow-up of children is necessary to control and prevent the onset of complications with changes in medical therapy or surgical treatment if required.
Fifteen women with intractable chronic idiopathic constipation dating from adolescence were investigated by anorectal manometry, neurophysiological evaluation of the conus medullaris and external anal sphincter. Comparison was made with 25 asymptomatic female control subjects. Urological disturbances were common amongst the constipated, in five of whom incidental lumbosacral spinal dysraphism was found. No differences in sphincter pressures or the rectosphincteric reflex were demonstrable between the two groups. Rectal defecatory sensation was blunted and the compliance was increased in the constipated group. The latency of the pudendo-anal reflex was significantly prolonged in idiopathic constipation, two women having an absent reflex (greater than 100 ms). Mean motor unit potential duration of the external anal sphincter was not significantly prolonged in the eight constipated women tested. A central neurogenic deficit is postulated in some women with this disorder.
CASE REPORT: We report a 20-year-old male with Russell Silver syndrome and tethered cord syndrome. This patient presented with non-painful scoliosis. MRI demonstrated a conus medullaris at the superior aspect of the L3 vertebral body. Axial images demonstrated no fat within the filum terminale. There was a horizontal sacrum. The patient had no cutaneous stigmata over the lumbosacral spine indicative of occult spinal dysraphism. At operation, the filum terminale was found to be quite large at approximately 5.0 mm in diameter. Grossly, the filum was found to have several large vessels both anterior and posterior to it. Following transection of the filum the distal cord was noted to withdraw cephalad indicating caudal traction. DISCUSSION: We believe this to be the first report of Russell Silver syndrome in conjunction with tethered cord syndrome. Following additional reports, Russell Silver syndrome may be added to the differential diagnoses for tethered cord syndrome.
Magnetic resonance (MR) is a powerful new imaging modality which has recently become competitive with X-ray computed tomography (CT) for imaging of the central nervous system (CNS). The advantages of MR include lack of ionizing radiation and the necessity to use iodinated contrast. MR is able to image directly in the transaxial, coronal, and sagittal planes. With appropriate pulsing sequences, MR is more sensitive than CT in the detection of early disease associated with increased water content. Included in this category are multiple sclerosis, early infarcts, small tumours, and inflammatory lesions. MR is superior to CT in the posterior fossa due to beam hardening artifacts in the latter modality. In the cord, MR is useful in the evaluation of syringomyelia, spinal dysraphism, and intramedullary tumours. Due to its increased sensitivity in the detection of early disease, MR should generally be used as the initial screening procedure in the evaluation of possible intracranial disease. Current limitations of MR include longer single slice imaging times, thicker slices, inability to use contrast agents to define blood/brain barrier breakdown, and the inability to image calcification and cortical bone. Patients on cardiac monitors or on respirators and those with intracranial aneurysm clips and cardiac pacemakers are currently excluded from MR imaging.
The teratogenicity of ethylenethiourea (ETU) was investigated in Sprague-Dawley rats and Landrace pigs. Pregnant rats each received a single intragastric dose of ETU on a given day from day 8 to day 19 of gestation, and pregnant pigs were given a single dose of ETU from day 15 to day 19. Control animals received an ETU-free vehicle. The newborn pigs were all normal, whereas there were high incidences of specific types of congenital malformation of the central nervous and other systems in the rats. A high incidence of lumbosacral myeloschisis associated with hindbrain crowding was observed in rat fetuses exposed to ETU on day 11 of gestation. This abnormality is considered comparable to Chiari type II malformation associated with spinal dysraphism (Arnold-Chiari malformation) in humans. Morphogenetic fetuses were examined by light and scanning electron microscopy and interactive image analysis. At gestational days 12 and 13, the volume of the caudal end of the neural tube from the anterior border of the hindlimb bud to the most caudal portion of the fetus was significantly greater in ETU-exposed than in control rat embryos. Experimental rat embryos also exhibited disturbance in closure of the posterior neuropore, with extrusion of neural tissue through the opening. An apparently unrelated abnormality in ETU-exposed rat fetuses was underdevelopment of the cranium, leading to hindbrain crowding.(ABSTRACT TRUNCATED AT 250 WORDS)
OBJECT: The goal of this study was to determine the importance magnetic resonance (MR) imaging holds as a complementary fetal imaging modality to ultrasonography in deciding postnatal neurosurgical management. METHODS: Between 1999 and 2003, 320 fetal MR imaging studies were performed at a single institution. Twenty-four fetuses were found to have central nervous system abnormalities that could potentially require a neurosurgical intervention. The diagnoses included spinal anomalies (scoliosis, myelomeningocele, and closed spinal dysraphism) and brain anomalies (ventriculomegaly with or without hemorrhage, intracranial cyst, craniosynostosis, and encephalocele). Fourteen of the 24 fetuses underwent surgery based on findings of prenatal MR imaging. In seven cases the pregnancy was terminated, and in three cases conservative follow up continues. CONCLUSIONS: In a variety of brain and spine disorders, prenatal MR imaging can delineate and characterize the abnormality, and thus assist in the diagnosis and in the planning of postnatal surgery and management. This modality provides important multiplanar images and may obviate the need for early postnatal computed tomography or MR imaging. Postnatal management can often be guided by prenatal MR imaging findings.
Cervical meningocele and myelomeningocele are rare spinal dysraphic lesions. Unlike lumbosacral dysraphic lesions, there is often no neurological deficit in infants with cervical lesions, thus the subtle features of cervical cord tethering may be overlooked on imaging. We report a case of cervical meningocele in an 8-month-old girl. The tethering band, confirmed intra-operatively, was not evident on imaging. Untethering of the cord was performed together with resection of the sac and repair of the dura. Tethered cord should be suspected in the presence of cervical meningocele and intact neurology. It should be carefully looked for using high-resolution magnetic resonance imaging or computed tomography. Treatment aims to prevent future neurological deterioration, and should include careful intradural exploration with untethering of the cord.
To determine the value of ultrasound scanning of the craniocervical junction in neonates via a posterior approach, we examined 50 infants with normal posterior fossae, 10 with congenital abnormalities, and eight with intracranial hemorrhage. Good evaluation of the cisterna magna, medulla, tonsils, vermis, cervical cord, and central canal was possible in most cases. In nine patients with spinal dysraphism, all displayed a Chiari II malformation; of these, a kink at the medullary cord junction was seen in six, and a cerebellar peg was noted in four. In one case, the Chiari malformation was confirmed by myelography, and all nine patients had some of the intracranial features of Chiari II malformation seen via the standard anterior fontanelle approach. The 10th patient in this group had a quadrigeminal plate cyst and gross hydrocephalus. In another four infants, diffuse subarachnoid blood in the cisterna magna was seen after recent intraventricular hemorrhage. A further two of four patients with posthemorrhagic hydrocephalus had localized clots. Direct scanning at the craniocervical junction was easily performed and allowed good evaluation of this area in normal infants and in patients with Chiari II malformation. This technique also allowed visualization of subarachnoid blood and clots obstructing the outlet of the fourth ventricle.
This is a case of an adolescent with kyphoscoliosis due to congenital partially segmented vertebrae T12, L1, L2 who was treated operatively by a back-front-back, one stage operation. A 16 year old patient neurologically intact with a rounded gibbous in the lower thoracic region and a mild scoliotic element had no other congenital anomaly. His kyphotic deformity was 85 degrees measured with the Cobb method. Preoperatively, a CT and MRI scan of the spine was performed and a three-level anterolateral failure of segmentation in the thoracic spine was diagnosed without spinal dysraphism. The operation lasted 8 hours and the Moss-Miami anterior and posterior fixation systems were used for fusion from T10 to T4. It included initially posterior approach for transpendicular screw insertion, wedge resection of the posterior elemens followed by anterior approach (thoracotomy), osteotomy of the defected vertebrae, anterior correction and fusion T11 to L1 and final correction with rod placement posteriorly. The wake-up test was performed twice. The follow-up was 3 years. The postoperative correction of the kyphosis was 45% (42 degrees) and there was practically no loss of correction in the last follow-up. No complications were observed. Detailed preoperative assessment of the patients with congenital deformities is essential in order to establish the correct diagnosis and choose the proper treatment. Substantial kyphotic deformities require combined antero-posterior procedures.