Surgical treatment of syringomyelia associated with spinal dysraphism

Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). Syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. Foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.     

Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release

OBJECTIVE: To investigate the influence of neurosurgical intervention on the appearance of upper motor neuron (UMN) signs in newborns diagnosed with occult spinal dysraphism and tethered cord (TC) during the first month of life. METHODS: A prospective study (1990 to 1996) of 22 consecutive newborns with occult spinal dysraphism monitored for the appearance of UMN signs. Untethering was performed when neurologic or urodynamic investigation indicated the presence of UMN dysfunction. RESULTS: Of 22 patients, 10 remained free of UMN symptoms during follow-up (mean, 67+/-22 months). Untethering was performed in 12 of 22 patients because of the presence of UMN symptoms. In 7 of these 12 patients, there was a documented asymptomatic period of 13+/-11 months before the onset of UMN symptoms. Untethering at a mean age of 18+/-17 months restored normal neurologic and urinary function in all patients (mean postoperative follow-up, 25+/-16 months). Of the 12 children, 5 presented with UMN signs at birth. In these children, untethering was performed at a mean age of 9+/-5 months. In two of these five patients, UMN symptoms did not resolve after surgery, and ongoing conservative bladder treatment was required (mean follow-up, 37+/-14 months). In none of the 12 operated children did signs of retethering occur. CONCLUSIONS: A significant number (10/22) of children born with occult spinal dysraphism and TC did not develop UMN symptoms during follow-up; neurosurgical correction after the appearance of an UMN sign restored normal neurologic and urinary function in all children; and untethering in children presenting at birth with UMN symptoms resulted in poorer outcome.     

Comparative aspects of occult intrasacral meningocele with conventional X-ray, myelography and CT

A case of occult intrasacral meningocele is reported and the diagnostic reliability of conventional roentgenography, myelography and CT for the management of this rare lesions are evaluated. Probably, CT with the use of an intrathecal contrast agent could yield the most complete information about the precise nature of this cystic congenital dysraphism.     

Radiologic and histopathologic findings in a rare case of complex occult spinal dysraphism with association of a lumbar fibrolipoma, neurenteric cyst and tethered cord syndrome

We present the case of a 60-year-old farmer who reported non-specific back pains for the past 30 years. Increasing paralysis of the left leg led to hospitalization. A complex occult spinal dysraphism comprising a fibrolipoma of the conus medullaris, an neurenteric cyst, and a tethered cord syndrome was diagnosed and operated on. The radiological and histological findings are demonstrated.     

Detection of an occult transclival cerebrospinal fluid fistula by CT and MRI

We describe an unusual occult transclival cerebrospinal fluid (CSF) fistula to the sphenoid sinus demonstrated by MRI. CT was performed because of a posterior cerebral infarct caused by cardiac arrhythmia. Axial sections showed fluid in the sphenoid sinus. High-resolution scans revealed a bony defect 3 mm in diameter of the posterior wall of the sphenoid sinus, and MRI showed a transclival CSF fistula. This occult lesion was confirmed by surgery and duraplasty was successfully performed via an endonasal approach.     

Early coronary sinus reroofing using the left atrial baffle

Surgical correction of unroofed coronary sinus, left superior vena cava, dextrocardia, and situs solitus in a 4-month-old infant consisted of reroofing the coronary sinus by means of a left atrial flap while redirecting the left superior vena cava to the right atrium. Excellent access to the left side of the left atrium was afforded by the associated dextrocardia.     

Latex allergy in patients with spinal dysraphism: the role of multiple surgery

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.     

Occult dysraphism in adulthood. A series of 24 cases

The results of a retrospective study of 24 adult patients with occult dysraphism are described. There were 15 males and 9 females, with an average age of 31.1 years. Specific circumstances precipitated symptomatic onset in 67% of patients. Pain, often referred to the anorectal region, was the most common presenting symptom. Bladder and bowel dysfunction were also common findings. The most common tethering lesions were intradural lipoma and a short thickened filum terminale. Myelography revealed the diagnosis of tethered conus in most cases, but the addition of CT and MRI images provided valuable structural details. The surgical outcome was gratifying in relation to pain and sensory-motor deficits but disappointing in the resolution of sphincter disorders. Our conclusion is that symptoms and/or signs of TCS with onset in adult life are not invariably irreversible.     

Dilation of the ventriculus terminalis: sonographic findings

A newborn term male infant was found to have the stigmata of VACTERL syndrome, including cervical and thoracic hemivertebra, partial sacral agenesis, 13 pairs of ribs, high imperforate anus with rectovesical fistula, and tracheoesophageal fistula with proximal esophageal atresia. No open spinal dysraphism or skin dimpling was seen. Ultrasonography of the spinal cord was performed to screen for occult anomalies. The examination revealed a lipoma of the filum terminalis and a low-lying conus medullaris at the L3-L4 level with a large central cystic structure in the terminal cord, which was continuous with the central canal (Fig. 1). This was thought to most likely represent a dilated ventriculus terminalis, and the diagnosis was confirmed on a subsequent MR image (Fig. 2). Fluid in the cyst followed cerebrospinal fluid signal in all sequences. We observed no abnormal signal in the adjacent parenchyma or enhancement associated with the cystic mass. A cranial ultrasonogram did not reveal any intracranial anomalies. The patient demonstrated no neurologic deficits in the lower extremities. Bowel and urinary continence was not assessable in this infant.     

Occult fever in surgical intensive care unit patients is seldom caused by sinusitis

Febrile intensive care unit (ICU) patients were evaluated prospectively for sinusitis. Of 598 admissions, 26 patients with transnasal cannulas, ICU stays over 48 hours, and occult fevers were identified. These 26 underwent physical examinations and sinus computed tomographic (CT) scans. Maxillary centeses and cultures were done in patients with CT abnormalities. Patients with positive scans had nasal tubes removed and received decongestants. Scans were abnormal in 19 (73%). All patients with major CT changes had positive maxillary taps. Most infections were polymicrobial; enteric bacilli were common. Fever resolved with nonoperative care in 18 (95%) patients; in only 1 patient was fever primarily from sinusitis. Sinus CT scans are often abnormal in ICU patients with occult fevers and transnasal cannulas. Pneumatic otoscopy can serve as a screening tool. Most patients respond to nonoperative management. Remote infections are often present. Although radiographic nosocomial ICU sinusitis is common, it is seldom the sole source of fever or the proximate cause of significant morbidity.     

Functional results following the antegrade continence enema procedure

BACKGROUND: Forty children who underwent the antegrade continence enema (ACE) procedure for faecal soiling were studied to determine factors predictive of outcome. METHODS: There were four patient groups: (1) ambulant with spinal dysraphism (n = 13), (2) wheelchair bound with spinal dysraphism (n = 14), (3) ambulant with miscellaneous disorders (n = 11) and (4) wheelchair bound with miscellaneous disorders (n = 2). Effectiveness of the procedure was assessed using technical evaluation and quality-of-life improvement (QOLI) scores (0-5). Objective assessment included colonic transit time (CTT) and anorectal manometry. Median follow-up was 21 (range 5-37) months. RESULTS: Some 28 of 40 children achieved continence. The procedure was reversed in four of 40 children. Of the other 36 children with a functioning ACE stoma, all reported improvement in quality of life (mean QOLI score 3.5). There were no significant differences in technical evaluation score, QOLI score, CTT, manometry findings or continence between ambulant groups and the wheelchair-bound group with miscellaneous disorders. QOLI score, anorectal squeeze pressure and continence were significantly poorer in those who were wheelchair bound with spinal dysraphism. Absent squeeze pressure was associated with poor outcome. CONCLUSION: Wheelchair-bound children with spinal neuropathy have a poorer outcome following the ACE procedure. Although ACE is an effective method of promoting faecal continence, it is essential to determine the aetiology of incontinence and sphincter function before operation.     

Nitrergic innervation and relaxant response of rectal circular smooth muscle

We report a case of an anomalous right coronary artery arising from the morphological left sinus of Valsalva in a patient with Kartagener's syndrome. Literature review has revealed only a small number of cases of anomalous coronary arteries in patients with dextrocardia and none previously reported in Kartagener's syndrome.     

Occult lumbar lateral spinal stenosis in neural foramina subjected to physiologic loading

PURPOSE: To measure the effect of extension, flexion, lateral bending, and axial rotation loads applied to the spine on the anatomic relationship of the spinal nerves in the neural foramen to the ligamentum flavum and the intervertebral disk, anc to determine the effect of disk degeneration on the response to loading. METHODS: Cadaveric lumbar motion segments were examined with CT and MR imaging, loaded with pure moment forces, frozen in situ, reexamined with CT, and sectioned with a cryomicrotome. The morphology of the intervertebral disks was classified on the basis of the appearance of the cryomicrotome sections. The neural foramina were classified as having no evident stenosis, as being stenotic, as having occult stenosis, or as showing resolved stenosis on the basis of the images and sections before and after loading. The stenotic and nonstenotic foramina were stratified by disk level, intervertebral disk classification, and type of loading applied. The effect of spinal level, disk type, and load type on the prevalence of stenosis was studied. RESULTS: On average, extension, flexion, lateral bending, and axial rotation resulted in the ligamentum flavum or intervertebral disk contacting or compressing the spinal nerve in 18% of the neural foramina. Extension loading produced the most cases of nerve root contact, and lateral bending produced the fewest cases. Each of the loading types resulted also in diminished contact between the spinal nerve and the intervertebral disk or ligamentum flavum in some cases. Disk degeneration significantly increased the prevalence of spinal stenosis. All foramina associated with advanced disk degeneration and half of the foramina associated with disks having radial tears of the annulus fibrosus either developed occult stenosis or were stenotic before loading. CONCLUSIONS: The study supports the concept of dynamic spinal stenosis; that is, intermittent stenosis of the neural foramina. Flexion, extension, lateral bending, and axial rotation significantly changed the anatomic relationships of the ligamentum flavum and intervertebral disk to the spinal nerve roots.     

Value of fast gradient echo magnetic resonance angiography as an adjunct to coronary arteriography in detecting and confirming the course of clinically significant coronary artery anomalies

Between January 1988 and February 1995, 133 tethered spinal cord-release procedures in 88 consecutive patients were performed at our institution and were used to determine survivorship data for surgical release of tethered spinal cord. The diagnoses included spinal dysraphism (67), achondroplasia (nine), isolated tethered cord (nine), cerebral palsy (three), and others (seven). Survivorship data were calculated for the initial and first-revision tethered cord release. There was a 50% revision rate by 5 years after initial tethered-cord release and a 57% revision rate by 2 years after a second release. Thirty-six patients were excluded for having <2 years of clinical follow-up, leaving 97 spinal cord releases in 52 patients available for outcome analysis. At a mean follow-up of 4.4 years (range, 2-11.3), 58% of patients required one or more orthopedic procedures after tethered-cord release. The average number of orthopedic procedures per year before release (0.28/year) was found to increase after initial release (0.39/year; p < 0.05). These data demonstrate the frequent need for operative revision after index tethered-cord release, especially in children with spinal dysraphism. In addition, the need for orthopedic procedures after tethered spinal-cord release frequently persists.     

The role of somatosensory evoked potentials in spinal dysraphism--do they have a prognostic significance?

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.     

Pediatric neurosurgical disease

The most common, primary referrals to a pediatric neurosurgeon's office are the evaluation and management of the child with a large head (to rule out hydrocephalus and other space occupying lesions) a mishappen head (to rule out various forms of craniosynostosis), or some form of congenital spinal abnormality (spinal dysraphism). The authors discuss the pathogenesis and clinical features of these disorders, provide a framework for diagnostic evaluation and referral, and discuss the various treatment options available for each.     

Primary non-Hodgkin lymphoma of the sinonasal cavities: correlation of CT evaluation with clinical outcome

PURPOSE: To determine retrospectively the primary site of origin of sinonasal lymphomas with computed tomography (CT) and correlate the CT findings with histologic phenotype and clinical outcome. MATERIALS AND METHODS: In 24 patients with stage I and II non-Hodgkin lymphomas of the sinonasal cavities, the CT appearances and clinical data were reviewed retrospectively. RESULTS: The sites of primary tumor determined at CT were the nasal cavity in 13 patients, the ethmoidal sinus in three patients, and the maxillary sinus in eight patients. B-cell lymphomas were found mainly in the maxillary sinus, while T-cell lymphomas were found in the nasal cavity and ethmoidal sinus (P < .005). The 5-year survival rates in relation to the primary site of the tumor were 64% for the nasal cavity, 50% for the ethmoidal sinus, and 100% for the maxillary sinus (P = .26). CONCLUSION: Patients with B-cell primary lymphoma of the maxillary sinus tended to have a good prognosis in contrast to those with T-cell lymphomas that originated from midline structures. The primary site determined at CT appears to be correlated with the histologic phenotype and clinical outcome.     

Value of CT-angiography in diagnosis of cerebral sinus and venous thromboses

Dural sinus thrombosis is not uncommon. Due to the nonspecific symptomatology, as well as the manifold etiology, clinical diagnosis may be difficult. In these cases imaging procedures are frequently crucial in deciding how to proceed and how to treat. The aim of our study was to evaluate the diagnostic utility of helical CT in the detection of dural sinus thrombosis. In 20 patients with clinically suspected thrombosis CT angiography was performed. In 6 patients dural sinus thrombosis was diagnosed. In order to acquire also arterial vessels, a short delay of about 22 s after the onset of the application of contrast medium was selected. By this method we found an occlusion of the MCA in two patients with clinically suspected sinus thrombosis. In all patients the transverse slices and the multiplanar reconstructions showed filling defects or an "empty delta" sign. With irregular outlines the thrombus could be depicted over the complete course of the sinus. The MIP reconstructions were particularly helpful in the evaluation of the vessel anatomy and the pathological collateral venous drainage. In three patients MR angiograms were available for comparison. The smaller veins, such as the v. vermis inferior, were less clearly depicted than in CT angiography. CT angiography is a fast and reliable method to exclude or verify a sinus thrombosis. It can be performed immediately after non-enhanced CT. According to our present experience CT angiography is sufficient for the diagnosis of a sinus thrombosis.     

Overgrowth and DNA synthesis of neuroepithelium in embryonic stages of induced Long-Evans rat myeloschisis

Overgrowth of the myeloschisis, namely the excessive amount of the neural plate tissue, has been reported in the human myeloschisis. However, it is still debatable how the overgrowth develops and whether the overgrowth is the cause, or the secondary effect of spinal dysraphism. The author induced myeloschisis in the fetuses of Long-Evans rats by the administration of ethylenethiourea (ETU) to pregnant rats on day 10 of gestation. The fetuses were removed 1 hour after the treatment with bromodeoxyuridine (BrdU) to the dams on day 14 and 21. The fetuses were fixed in alcohol and embedded in paraffin. H-E staining and the immunohistologic examination were performed on the staining patterns to anti-neurofilament (NFP), anti-glial fibrillary acidic protein (GFAP) and anti-BrdU antibody by ABC method. On day 14, the lateral portion of everted neural plate showed a loose arrangement of cells and there was rosette formation in the mesoderm. On day 21, cell necrosis was observed at the dorsolateral portion of myeloschisis, although the ventral portion showed almost normal cytoarchitecture and was positive to NFP and GFAP. The cause of myeloschisis in this model is supposed to be the local and direct cytotoxic effect of ETU to neuro-ectodermal junction. On day 14, control animals contained few BrdU-incorporated cells at the basal plate of neural tube. In contrast, everted neural plate showed an active uptake of BrdU diffusely in the subependymal matrix layer cells. Overgrowth was not yet identified. On day 21, overgrowth of myeloschisis was found in spite of a few positive cells to BrdU which was identical to the control animals. These findings seem to suggest that cells in the myeloschisis retain their ability of DNA synthesis for longer periods of development and overgrowth found on day 21 is possibly a secondary effect of spinal dysraphism in this model.     

Sphenoethmoidal mucoceles with intracranial extension--three case reports

Three unusual cases of sphenoethmoidal mucoceles with rare intracranial extension are reported. A 64-year-old female presented with a 7-month history of right visual disturbance. Computed tomography (CT) and magnetic resonance (MR) imaging demonstrated a huge mass in the right middle fossa. She underwent right frontotemporal craniotomy. Postoperatively, her proptosis and cranial nerve dysfunction had improved markedly. A 53-year-old female complained of headache, nausea, and dizziness. CT and MR imaging revealed a cystic mass filling the right sphenoid sinus. The cystic lesion was evacuated through the transnasal approach. She was doing well postoperatively and has been asymptomatic. A 39-year-old male complained of headache, vomiting, and right visual disturbance. CT and MR imaging demonstrated a homogeneous mass occupying the sphenoid sinus. Sphenoidotomy exposed the cyst extending superiorly into the anterior cranial fossa. He recovered from the visual disturbances and has been asymptomatic since. MR imaging provides confirmation of the diagnosis of sphenoethmoidal mucocele and is important for preoperative evaluation.