Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience.

PURPOSE: Atypical teratoid/rhabdoid tumours (ATRTs) are malignant embryonal tumours of childhood that affect the central nervous system (CNS). We aim to determine which factors, including patient age, extent of resection (EOR), presence of distal metastasis and use of adjuvant therapies, affect overall survival in children with atypical teratoid/rhabdoid tumours (ATRTs) treated at this single centre. METHODS: Retrospective cohort review of patients with histological diagnosis of ATRT treated over 21 years (1999-2020) was conducted. Data on demographics, tumour location, presence of metastasis, use of adjuvant therapy, extent of resection (EOR), complications, neurological outcome post-surgery, and overall survival were collected. Kaplan-Meier survival analysis was performed. RESULTS: A total of 45 children (mean age 2 years) underwent 64 operations. 25 patients were <1 year of age. Gross-total resection (GTR) pre-adjuvant therapy was achieved in 15, near-total resection (NTR) in 15, subtotal resection (STR) in 9, and biopsy in 6 children. Most children had good neurological outcomes post-operatively (28/45 with GOS 5). Fourteen patients survived longer than 4 years. Survival analysis showed a significant difference in median survival in favour of GTR and localised disease. There was no significant difference in median survival between patients <1 year vs >1 year of age (p=0.84). CONCLUSION: We find that presence of metastasis was an important factor in poor survival in patients with ATRT. GTR, where possible, may confer significant survival benefit in ATRT. Children aged <1 year appear to have performed as well as those >1 year and therefore should still be considered for radical surgery.

Atlanto-axial rotatory fixation: a serious potential complication of paediatric ENT surgery that requires prompt diagnosis and treatment.

BACKGROUND: Atlanto-axial rotatory fixation is a persistent deformity of the C1-2 vertebral relationship caused by subluxation of the articular surfaces, and can occur after positioning for ENT procedures where the head is rotated - for example to access the ear or posterior triangle of the neck. If promptly recognised, it can usually be managed successfully with conservative methods, without long-lasting sequelae, but delayed or inappropriate management may lead to permanent neck deformity, neurological problems and pain. METHOD: Case review. CASE REPORT: Two children with atlanto-axial rotatory fixation following ENT surgery; one child was referred early and managed successfully, and one had delayed referral resulting in permanent severe positional deformity. CONCLUSION: Atlanto-axial rotatory fixation is easily missed; there are significant clinical and medicolegal implications if it is not promptly recognised. A suggested management algorithm is presented.

Central nervous system infection after Onyx embolisation of arterio-venous malformations in two paediatric patients.

BACKGROUND: Increasingly, Onyx is used for endovascular embolization of aneurysms and arterio-venous malformations. Although reports in the literature on the use of Onyx are favourable, there have been so far no reports on the central nervous system (CNS) infection rate after embolisation with Onyx and no recommendations as to the management of these infections. CASE REPORTS: We present two cases of paediatric patients who acquired CNS infection with Pseudomonas aeruginosa after Onyx embolisation of AVMs and describe their subsequent management. CONCLUSIONS: Presence of established infection after Onyx embolisation should be dealt with by removal of infected material, administration of appropriate antibiotic therapy and supportive treatment.

Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage.

OBJECT: In this study the authors investigated whether patterns of intracranial venous drainage in children with complex craniosynostosis associated with raised intracranial pressure (ICP) were abnormal and, thus, could support the theory that venous hypertension is a major contributor to raised ICP that can lead to impaired visual function or even blindness in these patients. METHODS: The authors analyzed the anatomy of intracranial venous drainage as demonstrated in the results of 24 angiography studies obtained in 23 patients, all of whom had either a craniosynostosis-related syndrome (18 patients) or a nonsyndromic multisutural synostosis (five patients). Twenty-one patients had experienced raised ICP (in 19 patients diagnosis was based on invasive ICP monitoring and in two patients on clinical grounds alone) 1 to 6 weeks before undergoing angiography. Of the two remaining patients (both with Apert syndrome) whose ICP monitoring was normal immediately before angiography, each had undergone two previous cranial vault expansion procedures. On results of 18 angiography studies a 51 to 99% stenosis or no flow at all could be observed in the sigmoid-jugular sinus complex either bilaterally (11 patients) or unilaterally (seven patients). In 11 of these patients a florid collateral circulation through the stylomastoid emissary venous plexus was also seen. Two angiography studies were performed in one patient with Crouzon syndrome. A comparison of the two studies demonstrated a progression of the abnormal venous anatomy in that case. The authors found no obvious correlation between each patient's baseline ICP and the degree of abnormality of their venous anatomy, as judged on the basis of a venous-phase angiography severity score. CONCLUSIONS: Based on their findings, the authors assert that in children with complex forms of craniosynostosis in whom other factors, such as hydrocephalus, are absent, abnormalities of venous drainage that particularly affect the sigmoid-jugular sinus complex produce a state of venous hypertension that, in turn, is responsible for the majority of cases of raised ICP. The incidence of these changes is unknown, but an analysis of the ages of the children in this study indicated that the period of particular vulnerability to the effects of venous hypertension lasts until the affected child is approximately 6 years old. After that age the collateral venous drainage through the stylomastoid plexus will likely become sufficient to allow ICP to normalize.

The effect of pretreatments on surfactin production from potato process effluent by Bacillus subtilis.

Pretreatments of low-solids potato process effluent were tested for their potential to increase surfactin yield. Pretreatments included heat, removal of starch particulates, and acid hydrolysis. Elimination of contaminating vegetative cells was necessary for surfactin production. After autoclaving, 0.40 g/L of surfactin was produced from the effluent in 72 h, vs 0.24 g/L in the purified potato starch control. However, surfactin yields per carbon consumed were 76% lower from process effluent. Removal of starch particulates had little effect on the culture. Acid hydrolysis decreased growth and surfactant production, except 0.5 wt% acid, which increased the yield by 25% over untreated effluent.

Production of bacterial cellulose from alternate feedstocks.

Production of bacterial cellulose by Acetobacter xylinum ATCC 10821 and 23770 in static cultures was tested from unamended food process effluents. Effluents included low-solids (LS) and high-solids (HS) potato effluents, cheese whey permeate (CW), or sugar beet raffinate (CSB). Strain 23770 produced 10% less cellulose from glucose than did strain 10821 and diverted more glucose to gluconate. Unamended HS, CW, and CSB were unsuitable for cellulose production by either strain, and LS was unsuitable for production by strain 10821. However, strain 23770 produced 17% more cellulose from LS than from glucose, indicating that unamended LS could serve as a feedstock for bacterial cellulose.

Biosurfactants from potato process effluents.

High-solids (HS) and low-solids (LS) potato process effluents were tested as substrates for surfactin production. Tests used effluents diluted 1:10, unamended and amended with trace minerals or corn steep liquor. Heat pretreatment was necessary for surfactin production from effluents due to indigenous bacteria, whose spores remained after autoclaving. Surfactin production from LS surpassed HS in all cases. Surfactin yields from LS were 66% lower than from a pure culture in an optimized potato starch medium. LS could potentially be used without sterilization for surfactin production for low-value applications such as environmental remediation or oil recovery.

The effect of age, visual short-term memory, and performance on a familiar task: a Turkish sample.

Young (M = 25 years) and older (M = 61 years) backgammon players with equal experience reconstructed photographs of structured game positions shown for either 2 or 5 s. Reconstruction occurred either immediately or following a 15-s interpolated delay, during which a second photograph was shown. Reconstruction was completed on a backgammon board with all game pieces available. Recall was more accurate for younger players with a 2-s viewing time, but the age difference disappeared when viewing time was increased to 5 s. Interpolated processing decreased accuracy equally for both age groups. Results were interpreted to mean that although older adults took longer to encode information on this task, memory consolidation for both age groups was equally efficient.

In vitro degradation of natural insoluble lignin in aqueous media by the extracellular peroxidases of Phanerochaete chrysosporium.

The lignin peroxidases (LIP) and manganese peroxidases (MNP) of Phanerochaete chrysosporium catalyze a wide range of lignin depolymerization reactions with lignin models and synthetic lignins in solution. However, their ability to degrade insoluble natural lignin in aqueous media has not been demonstrated. Insoluble isolated poplar lignin similar to natural lignin was treated in vitro in aqueous media for 12 h with LIP, MNP, and both. Treatment with MNP alone slightly increased the solid mass and produced measurable amounts of lignin-derived 2,6-dimethoxyhydroquinone and 2-methoxyhydroquinone but did not appreciably decrease the total lignin content. Treatment with LIP alone did not decrease the mass but produced measurable amounts of lignin-derived p-hydroxybenzoic acid and slightly decreased the lignin content. Finally, treatment with LIP and MNP together decreased the solid mass by 11%, decreased the lignin content by 5%, and released low-concentration compounds with mass spectra containing the typical lignin-derived electron-impact fragments of mass 107, 137, 151, 167, and 181. These results suggest that MNP increases the effectiveness of LIP-mediated lignin degradation.

In vitro degradation of insoluble lignin in aqueous media by lignin peroxidase and manganese peroxidase.

The abilities of lignin peroxidase (LIP) and manganese peroxidase (MNP) from Phanerochaete chrysosporium to degrade an insoluble hardwood lignin in vitro in aqueous media were tested. Neither LIP nor MNP appreciably changed the mass or lignin content, although both produced small amounts of unique solubilized lignin fragments. Treatment with both LIP and MNP, however, decreased the mass by 11%, decreased the lignin content by 5.1% (4.2% as total weight), and solubilized unique lignin-derived molecules. These results suggest that LIP and MNP synergistically degrade high molecular weight insoluble lignin, but singly, neither enzyme is sufficient to effect lignin degradation.

Contributions to the history of psychology: CVIII. On aging and intelligence: history teaches a different lesson.

Early researchers investigating aging have often been accused of forming the foundation for the belief that intelligence peaks in early adulthood and declines steadily thereafter, a concept sometimes known as the classic aging curve. Documentation in this article indicates that at least several early researchers on the subject were actually making very different arguments and that they were well aware of the limitations of their data.

Aetiology of herniation of the hindbrain in craniosynostosis. An investigation incorporating intracranial pressure monitoring and magnetic resonance imaging.

The occurrence and extent of herniation of the hindbrain has been evaluated in a population of children with craniosynostosis by means of magnetic resonance imaging of the craniocervical junction. The role of intracranial pressure (ICP), posterior fossa size and hydrocephalus in the development of this deformity has also been assessed. Magnetic resonance imaging (Siemens Magnetom 1.5T) was reviewed in 27 cases of craniosynostosis in whom there had been no previous cranial vault surgery. The position of the cerebellar tonsils in relation to the plane of the foramen magnum was measured and an index of the size of the posterior fossa relative to the rest of the cranial vault was also calculated for each case. The presence of hydrocephalus (requiring a cerebrospinal fluid diversion procedure) was documented. In 22 of these cases overnight, subdural ICP monitoring using the Camino fibre optic device had also been performed. Herniation of the hindbrain below the plane of the foramen magnum occurred in 10 of 27 cases (37%). The level of ICP showed a significant correlation with the extent of hindbrain herniation (p < 0.001) as did small posterior fossa size (p = 0.0035). Hydrocephalus was present in 4 patients, all of whom had hindbrain herniation. The extent of hindbrain herniation did not correlate with age (p = 0.48). We propose that herniation of the hindbrain in craniosynostosis is a consequence of brain deformation occurring in response to the physical forces imposed by a combination of the anatomical deformity at the skull base and intracranial hypertension rather than a primary malformation of brain development as commonly supposed.

Consequences of cranial vault expansion surgery for craniosynostosis.

Remodelling the cranial vault in an attempt to increase the intracranial volume and thus control intracranial hypertension, whilst at the same time improving the patient's appearance, has been the mainstay of surgery for syndromic craniosynostosis. We report a case of craniosynostosis in whom cranial vault expansion was followed by the development of hind-brain herniation and hydrocephalus. This prompted a review of our other cases of craniosynostosis who had been evaluated by magnetic resonance imaging following surgery in order to assess the frequency of hind-brain herniation and hydrocephalus in these children. Magnetic resonance imaging had been performed in the postoperative evaluation of 34 cases of craniosynostosis who had undergone procedures intended to increase the intracranial volume. The position of the cerebellar tonsils and the presence or otherwise of hydrocephalus was recorded for all cases. The effectiveness of surgery in treating raised intracranial pressure (ICP) was evaluated by means of postoperative ICP monitoring and had been performed in 22 cases. Herniation of the hind-brain below the level of the foramen magnum was observed in 18 cases (53%). Hydrocephalus, requiring the insertion of a ventriculoperitoneal shunt, was present in 14 cases (41%) and had developed after the cranial vault procedure in 9. The mean sleeping ICP measured postoperatively was normal (<10 mm Hg) in 5, borderline (10-15) in 7, and raised (>15 mm Hg) in 10 cases. Cranial vault expansion in complex craniosynostosis may fail to address the underlying aetiology of intracranial hypertension. Furthermore, both hydrocephalus and hind-brain herniation may develop following such surgery. Neither the increase in intracranial volume afforded by cranial vault expansion nor the shunting of hydrocephalus precludes the persistence of abnormal ICP. These findings are discussed in the light of possible mechanisms, in addition to cephalocranial disproportion responsible for intracranial hypertension in complex craniosynostosis. The implications for the surgical management of complex craniosynostosis are reviewed.

Sellar tuberculoma: report of two cases.

Hypophyseal tuberculomas are exceptionally rare. We report two patients with sellar tuberculoma but with no evidence of concurrent extrasellar disease. Although the lesion is often mistaken for adenoma, there are characteristic radiological features: intense enhancement on contrast CT and thickening of the pituitary stalk on MRI in 86% of cases. Accurate diagnosis is important because pituitary tuberculoma is curable.

Shift from growth to nutrient-limited maintenance kinetics during biofilter acclimation.

During long-term operation of a biofilter, the mandatory absence of net cell growth forces the cells into maintenance metabolism, which is of relatively low rate compared to substrate consumption during the active growth of the acclimation phase. A model based on this shift in metabolism can explain the postacclimation decrease in activity sometimes reported for biofilters. The cessation of growth can be caused by nutrient depletion in the bed. Postacclimation nutrient addition increases activity primarily by allowing a return to the high substrate consumption rate of active growth, and only secondarily helps raise bed activity because of the ultimately higher amount of biomass in the bed. Simulations incorporating the acclimation period and the role of maintenance metabolism predict about 4 logarithms of growth during acclimation of a hexane biofilter, which was confirmed experimentally. Changes in a biofilter's biomass during the acclimation phase can be estimated from substrate conversion data using two approximate methods. The first follows the cumulative amount of substrate converted and uses the estimated yield of cells from substrate during active growth to estimate the total biomass created. The second method follows a rate constant for conversion of substrate in the bed.

The beaten copper cranium: a correlation between intracranial pressure, cranial radiographs, and computed tomographic scans in children with craniosynostosis.

OBJECTIVE: The beaten copper appearance of the cranium, as well as other cranial radiographic and computed tomographic findings in children with craniosynostosis, is often interpreted by clinicians as evidence of elevated intracranial pressure (ICP). However, a correlation between radiological findings and ICP measurements has not been previously demonstrated, and their usefulness in detecting elevated ICP has not been defined. METHODS: To address those issues, 123 children with craniosynostosis who had cranial radiographs and ICP monitoring were studied. To assess the specificity of certain radiological findings to patients with craniosynostosis, cranial radiographs of patients with craniosynostosis were compared to those of age- and sex-matched controls. In patients with craniosynostosis, findings on cranial radiographs were compared to computed tomographic scans of the brain. Radiographic findings were then correlated with ICP measurements obtained while the patient was sleeping, which was measured using a Camino fiberoptic ICP monitor (Camino Laboratories, San Diego, CA). All radiographs were independently analyzed by two radiologists who were blinded to clinical and ICP data. RESULTS: A diffuse beaten copper pattern, erosion of the dorsum sellar, and suture diastasis were seen more commonly in patients with craniosynostosis than in controls (P < 0.05), but the presence of the beaten copper pattern was no more common in children with craniosynostosis. ICP was greater when a diffuse beaten copper pattern, dorsum sellar erosion, suture diastasis, or narrowing of basal cisterns was present (P < 0.05). CONCLUSION: Although this study demonstrates that some cranial radiographic and computed tomographic findings do correlate with elevated ICP, the sensitivity of radiological methods for detecting elevated ICP is universally low and they are not recommended to screen for elevated ICP in children with craniosynostosis.

Congenital cervical spinal fusion: a study in Apert syndrome.

The occurrence and pattern of cervical spinal fusions have been assessed in 59 cases of Apert syndrome (acrocephalosyndactyly type 1). Radiological evidence of vertebral fusion either in progress or completed was observed in 37 (63%) of the cases. Fusion was limited to a single vertebral level in 18 cases and multiple levels, involving either contiguous or skipped levels in the remaining 19+ C3-4 and C5-6 were the levels most commonly involved. This distribution of fusions is different from other instances of congenital spinal fusion including those associated with other varieties of craniosynostosis. There was a significant association between age at the time of radiograph and the presence of spinal fusions (p < 0.001, Wilcoxon 2-sample test). Analysis of sequential radiographs in 17 patients revealed evidence of progressive fusion in 10. Small size of the vertebral body and reduced intervertebral disc space were indicators of subsequent bony fusion. The fusions seen in Apert syndrome thus appear to be progressive, occurring at the site of subtle congenital vertebral anomalies and may not be apparent as a congenital feature. The implications for the aetiology of so-called "congenital' spinal fusions in Apert syndrome and other situations are discussed.

Lessons from a case of kleeblattschädel. Case report.

The authors describe the clinical, radiological, and postmortem findings of a case of cloverleaf skull syndrome. The presence of hindbrain herniation, abnormal cervical segmentation, and atlantoaxial subluxation illustrate the anatomical complexity of the skull base and the craniocervical junction that may coexist in this condition. Unavoidable division of occipital emissary veins during elevation of the skin flap at the time of vault remodeling surgery led to an acute and, ultimately, fatal rise in intracranial pressure. Postmortem examination and review of magnetic resonance imaging revealed an anomalous pattern of venous drainage of the intracranial structures that appeared to have developed in response to venous obstruction, secondary to intraosseous venous sinuses and stenosis of the jugular foramina. The relationship between venous hypertension, hindbrain herniation, and hydrocephalus in this situation is reviewed, and the implications for evaluation and management of this vexing disorder are discussed.

Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management.

In the management of craniosynostosis subdural intracranial pressure (ICP) monitoring has proved a useful and safe means of identifying those children with raised ICP who are at risk from its long-term sequelae and who would benefit from early surgical intervention. Overnight subdural ICP recordings have been obtained in 136 unoperated cases of craniosynostosis. Fifteen patients were studied both before and after cranial vault remodelling procedures. ICP was raised (> 15 mmHg) in 35%, borderline (10-15 mmHg) in 37% and normal (< 10 mmHg) in 27% of cases. Raised ICP was present in 28/53 of the syndromic craniofacial dysostosis cases and in 20/83 non-syndromic craniosynostosis cases investigated (P < 0.001). Raised mean ICP and periodic plateaux of sustained ICP during sleep were particularly associated with the syndromic cases. Of the 15 patients studied following cranial vault surgery, 9 showed a reduction in ICP, 3 were unchanged and 3 had higher ICP postoperatively. The results of ICP monitoring can contribute significantly to formulating a rational and staged surgical management plan incorporating the need to normalise ICP and correct the frequently severe functional and cosmetic consequences of these disorders.