Biomedical subjects
Publications and source records attributed to S H Bakker-Niezen.
Epidural lipomatosis is most frequently seen in patients on chronic steroid treatment. Only twelve cases of idiopathic spinal epidural lipomatosis have been described. In this report we present an additional case of this condition in a middle-aged male presenting with neurogenic claudication.
A 30-year-old nulliparous woman underwent surgery for a ruptured aneurysm of the left vertebral artery in gestational week 27. The fetal heart rate (FHR) was monitored continuously with an abdominal Doppler transducer. Anesthesia was induced with midazolam, fentanyl, and thiopental and maintained with fentanyl, isoflurane, and nitrous oxide 67% in oxygen. Surgery was performed under moderate hypotension (mean arterial pressure +/- 70 mmHg) and moderate hyperventilation (arterial carbon dioxide pressure +/- 33 mmHg). There was a complete disappearance of FHR variability without decelerations or bradycardia. In the night following surgery, the patient was sedated with large parenteral doses of midazolam and fentanyl. Despite this sedation, some FHR variability reappeared within 40 minutes after discontinuation of the inhalation anesthetics. After discontinuation of parenteral midazolam and fentanyl, normal FHR variability returned within 60 minutes. In week 41 of pregnancy, a healthy girl of 4015 gm was born.
Vesico-intestinal fissure or exstrophy of the cloaca is a rare but serious birth defect of the urogenital tract and distal part of the digestive tract. The most important hallmarks are: bladder exstrophy, fusion between bladder and the exstrophied iliocoecal region, short blind-ending colon and imperforate anus. In addition the lower part of the body shows defects of the abdominal wall, symphysis, upper urinary tract, the internal and external genitals, lumbosacral spine, spinal cord and the lower extremities. It is just after 1960 that corrective surgery started to become successful. Because of the complexity of the anomaly it seems that only management by a team of specialists in a centre for pediatric surgery guarantees optimal treatment. The quality of live of these children can still be improved by application of the newest surgical techniques and excellent cooperation between the different involved specialists. The defects, surgical treatment and results in eight patients with vesico-intestinal fissure, seen at the Universitair Centrum voor Chirurgie bij Kinderen Nijmegen (UCCKN) from 1974 till 1989 inclusive, are presented.
The aim of this investigation is to evaluate the accuracy of the diagnostic parameters of the radiological features, especially in conventional lumbar myelography, since these will be used in the future as a gold standard for new developing diagnostic methods like ultrasonography (US) and magnetic resonance imaging (MRI). A short review of the clinical symptoms and diagnostic radiology of the tethered spinal cord (TSC) is given. The radiological features of the TSC syndrome are compared with the peroperative findings in 30 patients investigated and operated in our hospital. All patients showed lumbar or sacral osseous malformations on the plain X-ray films. This means a high diagnostic sensitivity although the specificity for TSC is low. Conventional myelography had a sensitivity of 0.82 and positive predictive value of 0.94, while the specificity and negative predictive value are respectively 0.96 and 0.85. The positive predictive value of the total diagnostic procedure of plain X-ray films and lumbar myelography for TSC may be estimated at 1.
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The results of surgical treatment of the tethered spinal cord syndrome in twenty-five children in the age range six weeks to nineteen years are reported. After a description of the symptomatology, the importance of neuroradiologic investigations including myelography and CT-scanning as well as urodynamic studies are explained. The operative techniques are described in cases of tight filum terminale, lipoma of the filum terminale, diastematomyelia, lipomyelomeningocele and meningocele. Early operation is essential to avoid the neurological damage which results from awaiting the onset of symptoms. The aim of the operation is to free the conus medullaris and cauda equina before mechanical traction or compression damages function in lumbosacral cord.
The tethered conus is probably a rather unknown entity. Every patient, adult or child, with spinal dysraphia, presenting an unexplained progressive urological, neurological and/or orthopedic disturbance can have a tethered cord. With lumbar myelography using metrizamide it is possible to diagnose the low positioned conus and its tethering causes. A review of the clinical symptoms and diagnostic radiology, illustrated with myelographic evidence, is given.
The Williams elfin facies syndrome (Williams-Beuren syndrome) is a disorder characterized by a typical facies, supravalvular aortic stenosis and mental retardation. Its coexistence with a brain tumor has, so far, never been described. Recently, we treated a patient who was previously diagnosed as having Williams syndrome for a cerebral astrocytoma. In view of the incidence of astrocytomas in children and the occurrence of Williams syndrome, we want to report this coincidence.