Lumbar dermoid cyst causing pyomyelia in a child.
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Child, Preschool↗
Biomedical subjects
S Sgouros
Publications and source records attributed to S Sgouros.
The effect of intrathecal baclofen on muscle co-contraction in children with spasticity of cerebral origin.
AIM: Investigation of the effect of intrathecal baclofen administration on the time course of electrical patterns of muscle activation in patients with spasticity due to upper motor neuron syndrome. METHODS: Six children with clinical signs of upper motor neuron syndrome resulting from an acquired cerebral hypoxic injury were tested. Simultaneous multichannel acquisition of surface EMG activity from flexor/extensor muscle groups of the upper and lower limbs was recorded. Investigated muscle group pairs included biceps/triceps brachii, wrist flexors/extensors, rectus/biceps femoris and tibialis anterioris/gastrocnemius. Time-frequency analysis of EMG activity at rest and while eliciting a stretch reflex was performed. The non-linear cross-correlation coefficient and time lag estimation were computed between paired channel groups both for baseline and post-intrathecal baclofen injection conditions for epochs consisting of 2 s prior to and 2 s after voluntary contraction. The effect of baclofen was assessed 3 h following single-bolus intrathecal injections of 25 or 50 microg during the baclofen trial and 6 months after baclofen pump implantation. RESULTS: In the baseline condition, the stretch reflex resulted in a synchronous increase in spectral EMG power in both the agonist and the antagonist muscles. The mean correlation coefficient between agonist and antagonist muscles was 0.948 (SD = 0.034), and the mean time lag was 4.64 ms (SD = 1.84 ms). After intrathecal administration of baclofen, a dramatic decrease in the correlation coefficient between agonist and antagonists (mean value = 0.342) during voluntary contraction was observed. This corresponded to a significant reduction of tone and spasticity in all four limbs, and reduction of the Ashworth score by 2 points on average. CONCLUSION: After intrathecal baclofen administration, we observed a significant decrease in the co-contraction pattern typically associated with upper motor neuron spasticity. This was evident clinically and was quantitatively expressed by the significantly decreased degree of coupling in EMG activity of agonist/antagonist muscles. Although a relatively small sample was investigated in this study, we were able to demonstrate the efficacy of this procedure in restoring selective activation of agonists during voluntary contraction. This is one of the prerequisites of an improvement of motor function in patients with spasticity.
Adolescent↗
The clinical value of electroencephalogram/magnetic resonance imaging co-registration and three-dimensional reconstruction in the surgical treatment of epileptogenic lesions.
With the rapid developments in image processing, new clinical applications of manipulation and three-dimensional (3-D) reconstruction of neuro-imaging are evolving. Combination with other non-invasive techniques aimed at localising electric sources in the brain is of particular interest. These techniques rely on the recording of brain electrical activity and/or the associated magnetic fields from multiple areas on the scalp. Data obtained from an electroencephalogram (EEG) or from magnetoencephalography (MEG) can be fused in 3-D arrangement with anatomical [magnetic resonance imaging/computerised tomography (MRI/CT)] and/or metabolic [positron emission tomography (PET)] data. Such techniques highlight information on the functional correlates of anatomical or space-occupying lesions and their role in the localisation of related symptomatic epilepsy. In the present study we report on methodological issues and preliminary clinical data on spectral EEG/MRI co-registration procedures, offering two examples of children presenting with hemispheric lesions, one frontal tumour and one temporal arterio-venous malformation. The EEG was acquired from 32/64 electrode location. The electrode position and that of four reference points were measured with a dual sensor Polhemus 3D Isotrak digitiser. Sources of EEG activity were determined in 3-D space with the inverse solution method low resolution electromagnetic tomography (LORETA), providing for each frequency component, the topographic distribution of active electrical sources. The positions of the reference points were also measured on MRI, and co-registration of EEG and MRI was achieved using a transformation algorithm. The reconstructed 3-D images of co-registered EEG/MRI clearly demonstrate the relationship between the space-occupying lesion and the epileptic activity. Preliminary results show that in all the patients it was possible to identify with a remarkable accuracy the 3-D topographic relationship between lesion and cortical areas showing localised abnormalities on the EEG. The present method could further enhance the understanding of the effect of resective treatment of structural lesions on brain functioning. The new combined images can be used in combination with image-guided surgery equipment to modify effective surgical resection.
Brain↗
Cerebral abscess associated with a pulmonary arteriovenous malformation in a patient suffering from alpha thalassaemia.
We report the case of a young girl suffering from alpha thalassaemia and presenting with a brain abscess caused by a silent pulmonary arteriovenous malformation. To our knowledge this is the first such case associated with alpha thalassaemia. Diagnostic investigations and treatment options are discussed.
Arteriovenous Malformations↗
Cervical disc prolapse in childhood associated with Klippel-Feil syndrome.
Klippel-Feil syndrome is characterized by a variable degree of congenital fusion of the cervical spine. It can exist in association with other mesodermal deformities affecting several systems. Symptomatic cervical disc prolapse in the context of the syndrome is well documented in young adults. We present a case of a 5-year-old girl with the syndrome, who presented with mild motor developmental delay and cervical cord compression from a prolapsed C3/4 intervertebral disc, seen on MR scan. She also had posterior elements fused from C-2 to C-4. She improved after cervical discectomy. The presence of degenerative disc disease at such a young age and at a level mechanically protected by posterior element fusion indicates a congenital primary defect, rather than mechanical stress fatigue.
Cervical Vertebrae↗
Spontaneous spinal epidural abscess in a neonate. With a review of the literature.
Spinal epidural abscess is uncommon in neonates and infants, and is usually related to previous lumbar puncture or epidural anaesthesia. Diagnosis is often delayed because of the non-specific presentation. We present a 7-week-old girl who developed paraplegia 3 weeks after transient fever and a self-limiting skin rash. MR imaging revealed an epidural contrast-enhancing lesion compressing the spinal cord. At operation, an organised granulated abscess was identified with Staphylococcus aureus the causative organism. Laminectomy and removal of the organised abscess and systemic intravenous antibiotics resulted in complete neurological recovery. The patient did not develop late spinal deformity following the decompressive laminectomy. The rapid onset of paraplegia can often be missed in such a young child but should be promptly investigated, as surgical treatment of cord compression carries an excellent prognosis for neurological recovery. We review the literature on the initial presentation, usual investigations, causative organisms and surgical management of paediatric spinal epidural abscesses.
Epidural Abscess↗
The Influence of hair shave on the infection rate in neurosurgery. A prospective study.
OBJECTIVE: To investigate whether not shaving hair in neurosurgical operations carries an increased infection rate. METHODS: Taking advantage of different practices among neurosurgeons in a single institution, we embarked upon a prospective non-randomised study of 100 consecutive neurosurgical procedures involving 90 paediatric patients aged 7 days to 16.8 years. The patients were split into two groups ('hair shave' and 'no hair shave'). The differences with respect to wound complications, positive microbiology on wound culture swabs and wound infection rates were analysed. Other factors considered were the cleansing solution, prophylactic antibiotic regime, duration of the operation, the surgeon's experience and the patient's age. RESULTS: The only complications observed were 4 incidences of wound dehiscence (2 in the hair shave and 2 in the no hair shave group) and 3 shunt infections (2 in the hair shave and 1 in the no hair shave group). We did not find any significant difference between the two arms for any of the factors assessed. Age was a significant factor in shunt infection, as all shunt infections were seen in patients aged less than 6 months, regardless of whether the hair was shaved or not (p = 0.024, Fisher's exact test). CONCLUSION: This study confirms our clinical experience that no hair shave is a good alternative to the traditional hair shaving approach, allowing patients to enjoy the psychological benefits of undisturbed body image while recovering from major surgery.
Adolescent↗
Congenital middle fossa arachnoid cysts may cause global brain ischaemia: a study with 99Tc-hexamethylpropyleneamineoxime single photon emission computerised tomography scans.
Three children with middle fossa arachnoid cysts, presenting with non-specific symptoms and otherwise well, were investigated before and after surgery with magnetic resonance and 99Tc-hexamethylpropyleneamineoxime single photon emission computerised tomography scans, to assess the effect of the cysts on cerebral blood flow. All patients had evidence of a reduction in cerebral blood flow at presentation, even in the hemisphere contralateral to the middle fossa cyst, implied by perfusion defects seen centrally in the deep white matter of both cerebral hemispheres. After successful surgical excision of the cyst, the perfusion defects disappeared. This was associated with general improvement of pre-existing non-specific symptoms. These findings indicate that middle fossa arachnoid cysts may cause global impairment of brain function by interfering with its blood supply. This does not support the generally held view that such cysts are benign in nature when 'asymptomatic'.
Adolescent↗
Neurocutaneous melanosis with leptomeningeal melanoma.
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Biopsy↗
Spinal sagittal malalignment following surgery for primary intramedullary tumours in children.
BACKGROUND/OBJECTIVE: As prior studies analysed predictive factors for various post-laminectomy spinal deformities in mixed spinal regions, age groups or pathologies, their validity and conclusions were unclear. The objective of this study was to determine predictive factors for worsened cervical or thoracic spinal sagittal alignment following laminectomy or laminotomy for primary intramedullary spinal cord tumours in children. METHODS: In this retrospective study, patients treated between 1980 and 1998 were reviewed. Changes in spinal alignment at the last follow-up compared to the pre-operative state were studied. Factors analysed were age, pre-operative spinal alignment, procedure types (laminectomy or laminoplasty), number of laminae operated, surgery of C2 or T1 laminae, histological grade, presence of post-operative neurological deficit and post-operative radiotherapy. RESULTS: There were 27 patients. The mean age was 5.6 years (range 1.3-14.0 years), and the mean duration of follow-up was 3.7 years (range 0.075-9.9 years). In the cervical-cervicothoracic surgical group (n = 12), alignment worsened post-operatively in 3 patients. The number of laminae operated upon had a statistically significant impact on the development of post-operative kyphosis (p = 0.07). In the thoracic-thoracolumbar surgical group (n = 15), alignment worsened in 9 patients. Procedure types were statistically significantly different, with laminectomy associated with an increased risk of post-operative kyphosis (p = 0.01). All 5 patients who had spinal fusion for worsened post-operative alignment were in the thoracic-thoracolumbar group; no patients in the cervical-cervicothoracic group required spinal fusion (p = 0.047). Other predictive factors did not reach statistical significance (p > 0.05). CONCLUSIONS: Worsened spinal sagittal alignment following laminectomy or laminoplasty and the need for spinal fusion is more common in the thoracic-thoracolumbar region than in the cervical-cervicothoracic region. In the cervical-cervicothoracic region, operation on a greater number of laminae tends to increase the risk of worsened alignment. In the thoracic-thoracolumbar region, laminectomy is associated with worsened alignment, while laminoplasty reduces this risk; also, pre-operative kyphotic deformity tends to increase the risk of worsened alignment post-operatively.
Adolescent↗
Acute subdural haematomas following ventouse-assisted delivery.
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Acute Disease↗
Intraventricular haemorrhage due to ruptured posterior inferior cerebellar artery aneurysm in tuberculous meningitis.
A 9-year-old Asian boy with known miliary tuberculosis, tuberculous meningitis and hydrocephalus was successfully treated with chemotherapy and ventriculoperitoneal shunting, but re-presented 7 months later with an intraventricular haemorrhage secondary to a ruptured left posterior inferior cerebellar artery mycotic aneurysm. The aneurysm was successfully treated by craniotomy and clipping. Tuberculous mycotic intracranial aneurysms are rare, but they should be considered in patients with tuberculous meningitis, particularly when there is a rapid neurological deterioration which may represent rupture.
Aneurysm, Infected↗
The treatment of infantile hydrocephalus: "differential-pressure" or "flow-control" valves. A pilot study.
The choice of shunt valve in the treatment of hydrocephalus in children remains controversial. We embarked on a pilot study to determine the differences in outcome between differential-pressure and flow-regulating valves. Prospective data collected on 50 consecutive first-time shunt insertions, performed between June 1993 to June 1996, was analysed. Children with tumour-related hydrocephalus and Dandy-Walker malformations as well as children who had external ventricular drainage prior to definitive shunt insertion were excluded from the study. The defining event was the first complication necessitating surgery, including obstruction, over-drainage and infection. Of the 50 children (31 males), 23 had differential pressure (medium-pressure) and 27 had Delta (performance level 2) valves inserted. The mean age at shunt insertion was 26.4 months. The mean follow-up was 53.8 months. The overall cumulative shunt survival at 5 years was 58.6% for the differential pressure and 58.7% for the Delta valves. The mean shunt life was 37.1 months for the differential pressure group and 34.6 months for the Delta group. This difference was not statistically significant (P=0.72, t-test). Both valves had a similar outcome with respect to obstruction (including proximal, valve, distal). The main differences between the two valves were with respect to the incidence of over-drainage and infection. Amongst the differential pressure valves, there were 4 instances of overdrainage (3 slit-ventricle syndrome, 1 bilateral subdural collection)--all occurring within the first 36 months. The Delta valve group had only one instance of over-drainage (bilateral subdural collection). There were no infections in the differential pressure valve group, whereas 3 of the Delta valve shunts got infected, all within the first month. Whereas both shunt types seemed to have a similar overall survival, there was a relatively higher incidence of over-drainage amongst the differential pressure valves. The Delta valves, on the other hand, had higher rates of infection. Similar studies with larger numbers could suggest whether the choice of shunt type will ultimately have to be a compromise accepting one or the other complication.
Cerebrospinal Fluid Shunts↗
Ocular manifestations of hindbrain-related syringomyelia and outcome following craniovertebral decompression.
PURPOSE: To analyse and evaluate associated ocular symptoms and signs in hindbrain-related syringomyelia and their response to treatment. METHODS: From a database of 275 patients treated in a single institution for hindbrain hernia and syringomyelia, 39 patients (14%) had ocular symptoms and signs. Only 31 patients were included in this study; the remainder were excluded due to inadequate follow-up information. All patients had confirmed evidence of hindbrain-related syringomyelia with MRI scan or CT myelogram. Treatment included craniovertebral decompression or ventriculo-peritoneal shunting. The mean follow-up was 23 months. RESULTS: In addition to the well-recognised sign of downbeat nystagmus, classically associated with foramen magnum abnormalities, a number of other ophthalmic features were identified. Symptoms included diplopia, oscillopsia, tunnel vision and difficulty in lateral gaze. Signs included nystagmus (downbeat, horizontal, rotatory, and combinations), strabismus, disc pallor, anisocoria, ptosis and field defect. Patients were categorised into two groups depending on whether the ocular features were manifest at first presentation (group 1, n = 14) or developed later in the course of the disease (group 2, n = 17). The delay in diagnosis from first presentation was 5 and 6 years respectively. All patients underwent surgery. Craniovertebral decompression was performed in 13 patients in group 1 and in 15 patients in group 2. Ventriculo-peritoneal shunt was inserted in 1 patient in group 1 and in 3 patients in group 2, for the associated hydrocephalus. Following surgery, 100% of patients in group 1 and 82% of patients in group 2 had complete or partial resolution of their ocular symptoms and signs. CONCLUSIONS: The presence of unexplained ophthalmic features such as nystagmus or oscillopsia should alert one to the potential diagnosis of hindbrain-related syringomyelia. Delay in diagnosis is often associated with poorer outcome. Surgical treatment can offer excellent results for these patients.
Adolescent↗
Spinal teratoma: is there a place for adjuvant treatment? Two cases and a review of the literature.
Spinal teratomas are rare intradural spinal tumours, commonly of benign histological profile. The main treatment remains surgical excision which often is incomplete as the tumour is frequently intimately related to surrounding nerve roots and adjacent spinal cord. The value of adjuvant treatment in the form of radiotherapy has not been clearly identified. We report two patients with spinal teratomas in the lower thoracic and conus region, who were treated with surgical excision and did not receive radiotherapy. One of the tumours recurred at 38 months following surgical excision and required a second operation. We review in addition another 20 patients reported in the literature with such tumours. With a mean follow-up of 25 months a local recurrence rate of 10% was observed, the same in both total and subtotal excision groups. No deaths were reported. Analysis of cumulative survival from surgery to treatment failure demonstrated that recurrence occurs late, verifying a slow biological progression of this tumour type. Although no reliable information of the effect of radiotherapy can be obtained, it is difficult to see how radiotherapy could have an impact on such a slow growing tumour. Initial treatment of choice remains surgical excision. If the teratoma shows any malignant histological features or germ cell elements radiotherapy should be employed after surgical excision, even if apparently totally excised. In the presence of benign histology it would seem appropriate to avoid radiotherapy after initial surgery. In case of tumour recurrence or progression to explore the possibility of further surgery first, before considering radiotherapy which even then may have doubtful efficacy.
Female↗
Spinal lipomas in children.
Spinal cord lipomas are a common cause of cord tethering that can lead to progressive neurological defects. The role of prophylactic surgery for spinal lipomas has recently been questioned. Between 1985 and 1999, 59 children underwent a total of 69 surgical procedures at the Birmingham Children's Hospital in Birmingham, UK. The spinal lipomas were classified into: 18 terminal, 17 transitional, 6 dorsal and 18 filum lipomas - including 12 who had a typical thickened filum terminale. At the first operation, 19 patients (32%) were asymptomatic, and 40 patients (68%) presented with symptoms. Surgical indications in the asymptomatic group included the presence of a dermal sinus tract or syrinx. Prophylactic surgery was undertaken in selected cases. The mean total follow-up for the group since the first surgical procedure was 61.8 months (range: 7.0-203.0 months). In the asymptomatic group, 26% of the patients had late neurological deterioration. Of the 14 patients with asymptomatic conus lipomas, 3 (21%) developed sphincter dysfunction and motor problems at long-term follow-up. In the symptomatic group, 68% improved, 20% remained unchanged, and 12% had late neurological deterioration. None of the 18 patients with symptomatic filum lipoma deteriorated postoperatively. However, 39% had bladder dysfunction, 54% had neuro-orthopaedic deformity, and only 15% returned to overall normal function at latest follow-up. Of the 27 patients with symptomatic conus lipomas, 67% improved, 15% remained stable, and 18% had late neurological deterioration. However, 74% had bladder dysfunction, 67% had neuro-orthopaedic deformity, and 45% had motor problems at long-term follow-up. Spinal lipomas can cause progressive neurological deficits irrespective of spinal untethering surgery. This study demonstrates that filum and conus lipomas have similar clinical presentation, but differ in their outcome following surgery. Filum lipomas are 'benign', for which surgery is safe and effective. Conus lipomas are more difficult to manage. When asymptomatic, our results suggest that prophylactic surgery does provide some protection from future neurological deterioration. When symptomatic, conus lipoma surgery is effective in stopping further deterioration. Improvement in neurological function can occur, but few patients return to normal overall function, and pre-existing sphincter dysfunction is not significantly altered by surgery.
Adolescent↗
Outcome of surgical treatment for subdural fluid collections in infants.
We reviewed retrospectively the management of 47 infants with subdural collections at the Birmingham Children's Hospital. Of those patients who had needle aspiration as their initial treatment 42% required some form of further treatment and 25% were complicated by infection of the subdural collection. Of those patients treated with burr hole evacuation or with observation alone, 78 and 73%, respectively, required no further procedures. However, 17% of those who had burr holes as their initial treatment developed post-operative infected collections. Of those who had subdural-peritoneal shunt as initial or subsequent treatment, none required further procedures to treat the subdural collections. No infections were noted following shunting. There was no statistically significant difference in overall clinical outcome at last follow-up between the different treatment groups. We conclude that needle aspirations should be avoided due to high infection risk, and that observation alone can be sufficient in the absence of intracranial hypertension, as the condition may be self-limiting. In cases requiring surgery, it should be borne in mind that burr hole evacuation has a higher infection risk, whereas subdural shunting has the additional need for removal of the device if the parents and surgeon decide accordingly.
Cerebrospinal Fluid Shunts↗
Skull base growth in childhood.
While studying skull base changes in craniosynostosis, it became apparent that there is a lack of reference studies quantifying the changes of three-dimensional (3D) parameters of the normal skull base throughout childhood. Using advanced 3D visualisation techniques, 34 points of the skull base were identified on MRI scans of 66 normal children, aged 1 month to 15 years. Several distances and angles between the various landmarks were measured in an attempt to quantify the growth of skull fossae with age. Two main growth periods were observed: before and after the first 5 years of life. Most change occurred in the first period. Anatomical regional differences were identified between the two sexes. During the first 5 years of life, the anterior fossa showed rapid growth rate with respect to its anterior projection in males, whereas in the females there was a more concentric growth pattern. The body of the sphenoid bone and the middle fossa showed a rapid growth rate in both sexes which was greater in the females. The posterior fossa showed a concentric pattern of growth in both sexes with a greater growth rate in the females. These findings provide new insight into the pattern of growth of the various parts of the skull base and can be used for comparative study of deformities that affect such growth.
Adolescent↗