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Biomedical subjects

P Casaer

Publications and source records attributed to P Casaer.

At least 19 recordsLinked to original sources

Long-term external drainage for subdural collections in infants.

The treatment of subdural collections in infants remains controversial. In order to evaluate the treatment guidelines that we developed on the basis of our earlier experience, we have reviewed the results obtained in 31 consecutive infants with symptomatic chronic and subacute subdural collections treated with external drainage. Using our guidelines for removal of the drains, there was only a very low rate of permanent shunting (4/31), with a low complication rate and good clinical results. However, the long period of external drainage, and therefore of hospitalization, might make if general application of these guidelines problematic.

Drainage↗

Effects of preterm extrauterine visual experience on the development of the human visual system: a flash VEP study.

To compare the functional maturation of the human visual system between intra- and extrauterine course flash visual evoked potentials (VEPs) in preterm infants (gestational age 24 to 36 weeks). Previously established normal values, with special reference to the two components of the N1 wave, were employed (Tsuneishi 1995). A cross-sectional analysis of 124 infants at 36 weeks postmenstrual age (PMA), showed that there are no differences in the absolute values of VEP peak latencies depending on the postnatal age (PNA). Conversely, the N1 wave form changes with increasing PNA from a wave in which the early peak (N1a) has a higher amplitude than the late peak (N1b) into the reverse situation with a higher amplitude of the N1b as compared to N1a. This observation may correlate with the maturation of the neuronal networks in the visual cortex. In a longitudinal analysis of 50 infants followed for more than 5 sessions of weekly recordings, we found that the individual rapid decrease in the N1a latency, which may reflect the initiation of myelination in the optic radiation, most frequently occurs at around 37 weeks PMA, regardless of PNA. Preterm extrauterine visual experience has little effect on the myelination process in the visual pathway, but has a marked effect on the developmental changes in VEP wave form which reflect the developmental changes of the neuronal networks in the visual cortex.

Cross-Sectional Studies↗

Septo-optic dysplasia in combination with a pigmented skin lesion: a case report with nosological discussion.

In this case report a patient with bilateral optic nerve hypoplasia, schizencephaly and a pigmented skin lesion is described. The diagnosis of de Morsier syndrome or septo-optic dysplasia is put forward on the basis of the diagnosis of optic nerve hypoplasia. The differential diagnosis with Jadassohn's naevus phakomatosis is discussed. The importance of direct ophthalmoscopy of optic nerve abnormalities is stressed, as well as of magnetic resonance imaging, which has become a guideline in the classification of this syndrome.

Brain↗

Treatment of a symptomatic posterior fossa subdural effusion in a child.

We describe the first observation of a child with a posterior fossa subdural effusion with secondary hydrocephalus and tonsillar herniation. We diagnosed this entity in a 14-month-old girl with no history of trauma or coagulation disorder. The patient presented in our emergency department with opisthotonus and raised intracranial pressure resulting from supratentorial hydrocephalus. An emergency ventriculo-peritoneal shunt was placed, which resolved the symptoms only temporarily. Eventually external drainage of the subdural fluid was performed. The collection gradually disappeared, and both the external subdural shunt and the ventriculo-peritoneal shunt were removed. The patient made a complete neurological recovery. We review the physiopathology and treatment of subdural effusions in general, and propose some guidelines for the management of symptomatic effusions occurring in the posterior fossa in particular.

Cranial Fossa, Posterior↗

Results after surgery for lumbosacral lipoma: the significance of early and late worsening.

We retrospectively reviewed 32 patients operated on for lipoma of the conus and lipomyeloschisis, the two main anatomical subtypes of congenital lumbosacral lipomas associated with tethered cord syndrome. Surgery was proposed to patients when they were symptomatic, and in most cases for progressive symptoms. The evolution of the different symptoms was studied separately. In most patients, symptoms improved or stabilized after surgery; in some, however, postoperative worsening, at least of some of the symptoms, was seen. This postoperative worsening became apparent either early or late after the operation, and was not associated with surgical trauma or postoperative complications. We suggest it was caused mainly by the natural course of the disease (especially in the case of the orthopedic deformities), and in some cases by retethering. Our series is not large enough to detect statistical significance for the different symptoms or for the anatomical subgroups. Importantly, according to our analysis by the different symptoms, the operation did not seem to protect the patients from later development of new deficits. This can be interpreted as an argument against prophylactic surgery in asymptomatic patients.

Adolescent↗

Neurodevelopmental outcome at 1 year in Zimbabwean neonates with extreme hyperbilirubinaemia.

UNLABELLED: The study concentrates on estimating the magnitude of the effect of a single risk factor, maximum total serum bilirubin (TSB) in excess of 400 micromol/l (23.4 mg/dl), on the neurodevelopmental outcome of 50, singleton, Zimbabwean neonates at 1 year of age. At 1 year corrected age the Bayley Scales of Infant Development (BSID) was administered. Two infants died and five were lost to follow up. TSB was neither associated with birth weight nor with gestational age. Of 43 infants with a TSB > 400 micromol/l (23.4 mg/dl),11(26%) scored abnormal on the BSID at 1 year of age and 5 (12%) infants developed the choreoathetoid type of cerebral palsy. CONCLUSION: Infants with bilirubin levels between 400 and 500 micromol/l (23.4 and 29.2 mg/dl) who scored abnormal or suspect on the Bayley Scales of Infant Development were preterm or had haemolytic disease. All term infants without haemolysis and with bilirubin levels between 400 and 500 micromol/l (23.4 mg/dl-29.2 mg/dl) were normal at 1 year of age.

Analysis of Variance↗

Acquired microcephaly after low Apgar score in Zimbabwe.

Serial head circumference measurements were made on 165 African babies born with a 5 min Apgar score of 5 or less. Measurements were taken at birth and at 4, 9, and 12 months of age. In the majority of infants the onset of microcephaly could be diagnosed as early as 4 months of age. Twenty-five of the 142 infants were microcephalic at 1 year. Neurological development was impaired in 19 of the 25 (76 per cent) microcephalic infants and in 18 of the 117 (15 per cent) normocephalic infants. Fourteen of the 16 (88 per cent) infants with severe quadriplegia developed microcephaly before the age of 4 months. A decreased rate of head growth during the first 4 months of life in African infants born with a low Apgar score correlates closely with the development of microcephaly. Infants with an acquired microcephaly have a high probability of developing neurologic impairment by the age of 1 year. Serial head circumference measurement in low Apgar score babies in developing countries is an easy, simple, and inexpensive method to detect microcephaly.

Analysis of Variance↗

Neonatal neurological examination as a predictor of neuromotor outcome at 4 months in term low-Apgar-score babies in Zimbabwe.

The predictive value of the neonatal neurological examination (NNE) adapted from Prechtl, was investigated in 139 term Zimbabwean infants born with an Apgar score of five or less at 5 min. At 4 months, seven infants had died and 13 were lost to follow-up, leaving 119 infants to undergo the Infant Motor Screen (IMS). Eighty-eight infants were diagnosed as normal, six as suspect and 25 as abnormal at screening. The sensitivity, specificity, positive predictive value and negative predictive value of the NNE were 94%, 55%, 42% and 96%, respectively. Seventeen (14%) infants had developed microcephaly at 4 months and 13 (77%) of them scored abnormal on the IMS. Twenty-three of the 48 (48%) infants who had convulsions within 48 h of birth, were diagnosed as abnormal (P < 0.0001). The NNE proved to be very sensitive in detecting neurodevelopmental abnormalities in the neonatal period and the five abnormal syndromes derived from the NNE were able to correctly identify 94% of the abnormal infants.

Adolescent↗

Intravesical oxybutynin for neurogenic bladder dysfunction: less systemic side effects due to reduced first pass metabolism.

PURPOSE: To unravel why intravesical oxybutynin is more effective and causes significantly fewer systemic side effects than oral oxybutynin in the treatment of neurogenic bladder dysfunction, we tested the hypothesis that the absorption and metabolism of oxybutynin are changed after intravesical instillation. MATERIALS AND METHODS: A high-performance liquid chromatography assay was developed for both oxybutynin and its active metabolite, N-desethyl-oxybutynin. Plasma concentrations were quantified after intravesical (n = 11) and oral (n = 5) administration of oxybutynin in children under steady-state conditions. Pharmacokinetic parameters were calculated. RESULTS: Oral administration of oxybutynin (0.2 mg./kg./dose) resulted in peak plasma concentrations for N-desethyl-oxybutynin which were 7.4 +/- 1.3 times higher than corresponding values for oxybutynin (n = 5). Also the AUC (area under the plasma concentration time curve) values were higher for N-desethyl-oxybutynin compared with those of oxybutynin, the ratio being 10.8 +/- 1.0 (n = 5). Intravesical instillation (0.2 mg./kg./dose), on the other hand, resulted in reduced metabolite generation and peak plasma concentrations for N-desethyl-oxybutynin which were in the same range as those for oxybutynin, the ratio being 1.2 +/- 0.1 (n = 11). The ratio for the AUC values for N-desethyl-oxybutynin and oxybutynin was 2.1 +/- 0.2 (n = 11). CONCLUSIONS: The significantly lower AUC ratio of the N-desethyl metabolite over the mother compound, due to a reduced first pass metabolism, may explain the clinically relevant reduction of side effects that characterizes intravesical compared with oral oxybutynin therapy.

Administration, Intravesical↗

Closed spinal dysraphism: a review on diagnosis and treatment in infancy.

This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of closed spinal dysraphism in infancy. Four groups of symptoms are distinguished: (1) cutaneous abnormalities, (2) lower motor neuron dysfunction due to congenital spinal and nerve root abnormalities, (3) upper motor neuron dysfunction due to tethering of the spinal cord, and (4) symptoms caused by associated malformations. Guidelines are proposed concerning timing and type of diagnostic investigations in infancy. This essentially encompasses a neurological assessment, including spinal ultrasonography and nuclear magnetic resonance imaging of the spine and the brain, and a urological assessment, including ultrasonography of kidneys and bladder, cystourethrography and a urodynamic study. As to the tethered cord syndrome it is concluded that first, already in infancy, a progressive neurological dysfunction can be detected; second, surgical untethering should be performed only upon appearance of upper motor neuron signs or upon progression of lower motor neuron signs. Despite its frequently asymptomatic course, the diagnosis of a congenital lumbosacral lipoma, and in the more general sense, of a closed spinal dysraphism, implies a periodic, multidisciplinary follow-up for life.

Humans↗

Neurological status in severely jaundiced Zimbabwean neonates.

Neurological status was studied in 50 jaundiced infants with a total serum bilirubin of > 400 mumol/l (23.4 mg/dl). Infants were assessed in the neonatal period with the Neonatal Neurological Examination and 4 months of age with the Infant Motor Screen. Twenty-six (52 per cent) infants were premature. Analysis of variance did not show a significant difference between gestational age, birth weight, and maximum total serum bilirubin or between gestational age, birth weight, and neurological optimality score. Based on the presence of abnormal neurological syndromes the infants were classified as normal (n = 27), suspect (n = 11), or abnormal (n = 12). Serum bilirubin levels were higher (p < 0.0001) and the neonatal neurological examination scores lower (p < 0.0001) in the seven (14 per cent) infants who received an exchange transfusion. In the transfused group four out of seven infants and in the non-transfused group seven out of 43 infants were classified as abnormal (p < 0.03). The Neonatal Neurological Examination was shown to be sensitive in detecting neurodevelopmental abnormalities in the neonatal period, with a sensitivity of 83 per cent, specificity of 88 per cent, positive predictive value of 62 per cent, and negative predictive value of 96 per cent.

Analysis of Variance↗

Intravesical application of a stable oxybutynin solution improves therapeutic compliance and acceptance in children with neurogenic bladder dysfunction.

PURPOSE: To improve patient compliance with and acceptance of intravesical oxybutynin therapy for neurogenic bladder dysfunction we developed a stable oxybutynin solution that eliminates the complicated crushing procedure. MATERIALS AND METHODS: From January 1995 to January 1997 we prospectively evaluated 15 children with a mean age of 6.1 years with persistent detrusor hyperactivity or significant side effects on oral oxybutynin therapy who received intravesically 0.2 mg./kg. (maximum 5 mg.) of a stable oxybutynin solution (5 mg./5 ml., pH 5.85) twice daily. RESULTS: The oxybutynin solution remained stable up to 24 months. In 13 of the 15 children therapeutic compliance was excellent. Detrusor hyperactivity decreased and systemic side effects were absent or minimal. After 4 and 24 months mean cystometric bladder capacity plus or minus standard error of mean increased from 114+/-15.2 to 161+/-26.6 and 214+/-21.7 ml. (p <0.01), mean ratio of cystometric-to-expected bladder capacity increased from 0.88+/-0.12 to 1.18+/-0.14 and 1.24+/-0.16 (p <0.01), and end filling bladder pressure decreased from 57.0+/-7.1 to 25.6+/-4.4 and 30.8+/-4.4 cm. water (p <0.01), respectively. CONCLUSIONS: Intravesical instillation of a specially prepared oxybutynin solution is safe and reliable in children with persistent detrusor hyperactivity or side effects on oral oxybutynin therapy. Eliminating the complex crushing preparation of the solution by the child or parent has made this therapy easy to use and acceptable in the long term.

Administration, Intravesical↗

Neuropathological findings in Moebius syndrome.

Pathological findings in two patients with Moebius syndrome and lethal fetal akinesia sequence are described. In both patients a congenital brain stem malformation with neuronal loss in the cranial nerve nuclei and tegmental microcalcifications was observed. In one patient, the association with splenogonadal fusion was observed, whilst in the second patient, the association with tetraperomelia was present. As the association of peromelia and splenogonadal fusion is a well-known association, the different combination of splenogonadal fusion, peromelia and Moebius syndrome due to congenital brain stem anomalies with necrosis might be the result of a disruptive phenomenon during a prolonged vulnerable critical period in the 5th and 6th week of embryonic life. The finding of olivary dysplasia in one case, reminiscent of olivary dysplasia in Zellweger syndrome and in Miller Dieker syndrome, might suggest a primary malformation underlying Moebius syndrome due to brain stem defects.

Abnormalities, Multiple↗

Epidermoid tumours associated with lumbar punctures performed in early neonatal life.

Three children (ages 5, 7, and 12 years) with epidermoid tumours in the spinal canal, all of whom had a lumbar puncture during the early neonatal period, are reported. A considerable delay occurred from the first symptoms until the diagnosis was made. MRI of the lumbar spine was the method of choice in the diagnostic work-up. All three cases were successfully surgically treated. The link between lumbar punctures and epidermoid tumours and the possible risk factors involved are explored. Because of the variable clinical presentation, the possibility of the existence of these tumours should be considered in the differential diagnosis.

Age Factors↗

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.

Child, Preschool↗

MR of cerebellar cortical dysplasia.

MR imaging findings are described in four patients with cerebellar cortical dysplasia. Typically, cerebellar disorganized folia were seen as an irregular bumpy gray-white matter interface. In addition, cystlike cortical abnormalities were observed in two patients and associated supratentorial developmental abnormalities were seen in three patients. To our knowledge, cerebellar cortical dysplasia without supratentorial abnormalities, as seen in one patient, has not been reported before. We suggest that cerebellar cortical dysplasia represents a spectrum of abnormalities ranging from mild to extensive in severity.

Adolescent↗