Bony decompression vs duraplasty for Chiari I malformation: does the eternal dilemma matter?

PURPOSE: The management of Chiari I malformation (CIM) still raises the problem of the optimal surgical treatment, with special regard to the "eternal dilemma" of the posterior fossa bony decompression alone (PFBD) or with duraplasty (PFBDD). The goal of the present review is to update the results (outcome and complications) of both techniques to better understand the correct indication for each of them. METHODS: A review of the literature has been performed, focusing on the articles and the meta-analyses specifically addressing the problem of PFBD vs PFBDD. Also, the personal authors' experience is briefly discussed. RESULTS: PFBD (usually with C1 laminectomy, often with delamination of the external dural layer) is the most commonly used technique in children, especially if syringomyelia is absent. It ensures a high success rate, with > 80% clinical improvement and about 75% reduction of the syringomyelia, and a very low risk of complications, hospital stay, and costs. A certain risk of recurrence is present (2-12%). PFBDD (with autologous tissues or dural substitutes), on the other hand, is mostly used not only in adults but also in children with large syringomyelia. It is burdened by a higher risk of complications (namely, the CSF-related ones), longer hospital stay, and higher costs; however, it warrants a better clinical improvement (> 85%) and a lower risk of reoperation (2-3.5%). Eight meta-analyses of the literature (three on pediatric series and five in adult series) and one prospective study in children, published in the last decade, largely confirm these findings. CONCLUSION: PFBD and PFBDD are different techniques that are indicated for different types of patients. In children, PFBD has been demonstrated to represent the best choice, although some patients may require a more aggressive treatment. Therefore, the success in the management of CIM, with or without syringomyelia, depends on the correct indication to surgery and on a patient-tailored choice rather than on the surgical technique.

Imaging in craniosynostosis: when and what?

PURPOSE: Currently, the interest on craniosynostosis in the clinical practice is raised by their increased frequency and their genetic implications other than by the still existing search of less invasive surgical techniques. These reasons, together with the problem of legal issues, make the need of a definite diagnosis for a crucial problem, even in single-suture craniosynostosis (SSC). Although the diagnosis of craniosynostosis is primarily the result of physical examination, craniometrics measuring, and observation of the skull deformity, the radiological assessment currently plays an important role in the confirmation of the diagnosis, the surgical planning, and even the postoperative follow-up. On the other hand, in infants, the use of radiation or the need of sedation/anesthesia raises the problem to reduce them to minimum to preserve such a delicate category of patient from their adverse effects. METHODS, RESULTS AND CONCLUSIONS: This review aims at summarizing the state of the art of the role of radiology in craniosynostosis, mainly focusing on indications and techniques, to provide an update not only to pediatric neurosurgeons or maxillofacial surgeons but also to all the other specialists involved in their management, like neonatologists, pediatricians, clinical geneticists, and pediatric neurologists.

Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review.

OBJECT: Desmoplastic infantile gangliogliomas (DIGs) and desmoplastic infantile astrocytomas (DIAs) are tumors typical of the infantile age. A large size, with a mixed solid and cystic component, clinical presentation with progressing signs of increased intracranial pressure, a prominent benign desmoplastic structure at histological examination, and a favorable clinical course in the majority of cases are the prominent features of these tumors. The objective of the present paper was to review the pertinent literature on the topic together with our personal experience, with the aim of an updated review of the subject. RESULTS AND CONCLUSIONS: Only 28 papers are present in the literature devoted to DIGs and DIAs, most of them reporting on single cases or small series, with a total of 107 patients aged from 5 days to 48 months with a slight male prevalence. Most of the reported cases refer to supratentorial and hemispheric locations, a few cases involving the hypothalamic region, the posterior fossa, and the spinal cord. The typical MRI appearance is of large mixed solid and cystic tumors with a spontaneous hyperintense T2 appearance of the solid part which also shows a strong contrast enhancement. Mixed ganglionic and astrocytic cells are identifiable in DIGs, whereas DIAs are typically featured by the exclusive presence of glial cells. In both cases, more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery represents the treatment of choice; however, radical removal has been reported as possible only in around 30 % of the cases: the low age of the patients together with their low weight and the large size of and the hyper-vascularized structure of the tumors represent the main factors limiting surgery. Pure observation is considered as first choice in children undergoing a partial/subtotal tumor resection, chemotherapic regimens being considered in cases of recurrences after a second look surgery. Long-term prognosis is favorable with mortality being related mostly to the rare midline (i.e., hypothalamic) locations, which beyond the functionally relevant site, tend to have an unusually more aggressive histological behavior.

Preoperative neurocognitive evaluation as a predictor of brain tumor grading in pediatric patients with supratentorial hemispheric tumors.

OBJECTIVE: The objective of the present study was to retrospectively evaluate the relationship between tumor grading and a selective evaluation of neurocognitive and behavioral functions in children with supratentorial hemispheric brain tumors. METHODS: Children admitted with a diagnosis of supratentorial hemispheric tumors involving the cerebral hemispheres or the thalamus at the Pediatric Neurosurgery Unit of the Catholic University of Rome between January 2008 and January 2014 were considered for the present study. Exclusion criteria were represented by age less than 2 years, severe neurological deficits, seizures, and a metastatic disease. A selective neurocognitive and behavioral workout was used for children aged less and more than 5 years. RESULTS: Global cognitive functions as well as selective neurocognitive and behavioral profiles were found to be significantly worse in children with low-grade tumors, compared with those affected by higher-grades histotypes. Frontal locations for cortical tumors and thalamic lesions were significantly related with worse results, with a clear contribution of dominant vs. nondominant hemisphere involvement and an age higher than 5 years. CONCLUSIONS: Preoperative global and selective neurocognitive evaluation might contribute to the prediction of the tumor aggressiveness. Due to a longer clinical history, more benign tumors more frequently arrive to the diagnosis with a neurocognitive compromise in spite of an apparently mild presence of neurological symptoms and signs.

Cerebellar mutism.

INTRODUCTION: Cerebellar mutism (CM) is defined as a peculiar form of mutism that may complicate the surgical excision of posterior cranial fossa tumor. The incidence is variable in the literature, occurring in up to one third of cases in some series. Commonly occurring peculiar features of CM are delayed onset following surgery, limited duration, and spontaneous recovery usually associated with dysarthria. METHODS: A review has been performed concerning anatomical substrates and circuits actually considered to be involved in the development of cerebellar mutism, as well as risk factors for its development that have been documented in the literature. Attention has also been given to the long-term prognosis and the possibilities of rehabilitation that can be considered in these children, which has been compared with the authors' institutional experience. RESULTS AND CONCLUSIONS: Tumor infiltration of the brainstem seems to represent the most relevant feature related to the development of CM, along with the histological diagnosis of medulloblastoma. On the other hand, hydrocephalus does not represent an independent risk factor. The higher rate of CM in children seems to be related to the higher incidence in children of tumors with malignant histology and brain stem involvement. Surgical technique does not seem to have a definite role; in particular, the use of a telovelar approach as compared to vermian split to reach the fourth ventricle extension of the tumor has not been demonstrated to prevent the development of cerebellar mutism. Concerning long-term prognosis, around one third of the children who develop cerebellar mutism after surgery have a persistent dysarthria, the remaining ones showing a residual phonological impairment. Long-term dysarthric features tend to be more severe and less prone to recovery in children presenting at diagnosis with associated combined procedural memory and defective neurocognitive functions.

Surgery for posterior quadrantic cortical dysplasia. A review.

BACKGROUND: Posterior quadrant dysplastic lesions represent 3-15 % of multilobar cortical developmental pathologies, 3-5 % of all the indications to surgery for epilepsy resistant to medical treatment. OBJECTIVE AND METHODS: The objective of this study is to review the pertinent literature related to the presurgical clinical, neurophysiological, and neuroradiological evaluation of children affected by posterior quadrant dysplasia in order to discuss the intraoperative management and the different surgical techniques that have been proposed to treat this condition as well as factors related with postsurgical seizure outcome. RESULTS AND CONCLUSIONS: Seizures appear most commonly in infants and rapidly progress to a catastrophic course. They are mostly represented by focal seizures and spasms. Surface interictal video-EEG is characterized by background flattening and paroxysmal discharges prevalent on the affected side but not unusually extending to the controlateral hemisphere. The last occasionally shows an independent irritative activity in spite of the absence of further visible structural abnormalities. Most of the patients have visual field or visual attention deficits at diagnosis. Resective as well as disconnective surgical procedures have been proposed for the management of this condition, none of them having shown clear advantages in terms of seizure outcome and complications. Intraoperative electrocorticography (EcoG) and sensorimotor monitoring have been successfully used to improve the localization of the epileptic focus and reduce surgical complication rates. Undistincted lesion borders, independent controlateral ictal or/and interictal EEG activity, and incomplete resections/disconnections are among the main factors that have resulted to be associated with a worse seizure outcome.

Role of nutrients in modulating microbiota and immunity in COVID-19 disease.

COVID-19 is a novel disease with a broad range of clinical patterns. Several patients show dysbiosis in the intestinal tract, with evidence of reduced beneficial bacteria, such as Bifidobacteria and Lactobacilli. It is well established that human gut microbiota dysbiosis is associated with several clinical conditions, including respiratory tract diseases due to the gut-lung axis. This narrative review discusses the role of nutrients in the relationship between the gut microbiota and the immune response in SARS-CoV-2 infection. In particular, we will focus on the benefits offered by vitamins and micronutrients on different aspects of COVID-19 disease while also discussing which diets seem to provide the most advantages.

Subcutaneous blood patch for iatrogenic suboccipital pseudomeningocele following decompressive suboccipital craniectomy and enlarging duroplasty for the treatment of Chiari I malformation. Technical note.

PURPOSE: Epidural blood patch (EBP) represents one of the best nonsurgical treatment for intracranial hypotension syndrome. Orthostatic headache caused by reduced intracranial cerebrospinal fluid (CSF) pressure, like in "spontaneous" intracranial hypotension or as consequence of lumbar puncture or anesthesiological procedure, can be managed with the injection of autologous blood on the epidural space with a successful rate of 89%, increased to 97% after a second application. MATERIALS AND METHODS: This 9-year-old girl was admitted to our department because of a suboccipital pseudomeningocele. She was previously operated on for a Chiari type I malformation by suboccipital craniectomy, C1 laminectomy and duraplasty. At the admission, she complained for nucal pain. Brain MRI showed a large suboccipital fluid collection that persisted even after the revision of the duraplasty and the placement of an external lumbar drainage. The child underwent a first injection of a mixture of blood (10 ml) and fibrin glue (10 ml) within the subcutaneous space after needle-aspiration of the collection. The same treatment was repeated 3 weeks later. RESULTS: The procedures were well tolerated and no local or systemic complications occurred. The fluid collection was significantly reduced after the first injection and it was completely effaced following the second one. Such a result remained stable after 3 months, as demonstrated by MRI. No recurrence of the pseudomeningocele was detected at the last clinical control (8-month follow-up). CONCLUSION: Subcutaneous blood patch could represent a safe and effective option for the treatment of CSF fistula, especially in case of failure of the traditional management.

Complex craniosynostoses: a review of the prominent clinical features and the related management strategies.

The protocols for clinical evaluation and management of children with complex craniosynostoses are significantly different from those used in single suture forms. The time at which the various anatomical and functional anomalies observed in the affected subjects become clinically relevant varies from patient to patient, consequently requiring a tailored approach. The clinical course is variable and influenced by multiple factors, acting at different steps of the children growth. Intracranial hypertension is a major concern already in the first months of life; active cerebrospinal fluid (CSF) dynamics disorders, venous hypertension, and progressive craniocerebral disproportion are considered the main pathogenetic factors. Cranial vault and skull base sutures synostoses account for the frequently observed increased venous pressure. Skull base abnormalities lead to upper airways obstruction, which, on one side, might create significant upper airways obstructive problems and, on the other, contribute to the increase in the intracranial pressure. Secondary Chiari malformation is common and considered as a progressive disorder, mainly due to progressive craniocerebral disproportion, venous hypertension, and CSF dynamics disorders. Optic nerve and orbit-related eye-globe diseases are also a major concern. Papilledema is mostly related to increased intracranial pressure. The skull base synostotic process is the base of significant abnormalities of the orbital space, ending in the common feature of significant proptosis with the consequent risk of corneal ulcers. Aims of this paper are to analyze the physiopathogenetic mechanisms at the base of the clinical manifestations presented by children with complex craniosynostoses, and the therapeutic options currently available.

Brain CT scan for pediatric minor accidental head injury. An Italian experience and review of literature.

PURPOSE: Every year 300,000 children with accidental head trauma are admitted to Italian emergency departments. Our aims were: (1) to describe patients with minor traumatic brain injury who were admitted to pediatric departments and underwent CT, and (2) to analyze the appropriateness of management according to current guidelines. METHODS: We retrospectively analyzed patients with minor head injury (median age 4.5 years, range 1 month to 16 years) who were admitted to the pediatric department of the Catholic Medical School of Rome, from January 2005 to September 2010, who performed head CT. Univariate analysis was performed using the Fisher's exact test. Multivariate analysis was performed by logistic regression. RESULTS: One hundred and seventy-four patients were enrolled in the study. Fifty-four patients (31%) had pathological CT findings. Eight patients underwent neurosurgical treatments. Vomiting was the only symptom significantly prevalent in the infant group, compared to the children group (10.7% vs. 38.9%, p = 0.007), while loss of consciousness in the children group (50.0% vs. 25.0%, p = 0.040). The relationship between scalp swelling and CT abnormalities was statistically significant in the entire population. The incidence of head abnormalities was significantly higher in children with abnormal CT (92.6% vs. 72.5%). CONCLUSIONS: The best way to manage children with minor head trauma is still matter of debate. Loss of consciousness and scalp swelling are risk factors predicting brain injury that deserve CT control. The radiation risks posed by CT scanning in children must be balanced by the benefits. We believe that even though CT scans may be clinically unnecessary in many cases, the rate of scanning is justified by the even limited number of abnormalities which require neurosurgical treatment.

Survival following treatment for intracranial ependymoma: a review.

The actual definition of survival rates following treatment for intracranial ependymomas is substantially influenced by the strict interaction among different factors. Age, location, and grading, for example, act together, negatively influencing the prognosis of younger children also invariably influenced by the more demanding role of surgery and the still limited use, up to recently, of radiotherapy under 3 years of age. In the same direction, the worse prognosis in most series of infratentorial ependymomas if compared with their supratentorial counterpart should be cautiously considered, midline posterior fossa tumors having completely different implications from those originating or predominantly extending to the cerebellopontine angle, where the extent of surgery has more invariably to compare with patients' quality of life. New radiotherapic regimens and their applications in infancy are promisingly demonstrating an improvement of present prognostic criteria, with the limit of still insufficient information on their long-term secondary effects. Similarly, molecular biology research studies, though still in their preclinical stage, are prompting to change the concept of a substantially chemoresistant tumor helping to stratify these lesions with the final aim of targeted pharmacological therapies. In the present review paper, we investigated singularly the role that the more commonly considered prognostic factors have had in the literature on survival of children affected by intracranial ependymomas, trying to elucidate their cumulative effect on the actual knowledge of this issue.

Brain DSC MR Perfusion in Children: A Clinical Feasibility Study Using Different Technical Standards of Contrast Administration.

BACKGROUND AND PURPOSE: Dynamic susceptibility contrast MR perfusion imaging has limited results in children due to difficulties in reproducing technical standards derived from adults. This prospective, multicenter study aimed to determine DSC feasibility and quality in children using custom administration of a standard dose of gadolinium. MATERIALS AND METHODS: Eighty-three consecutive children with brain tumors underwent DSC perfusion with a standard dose of gadobutrol administered by an automated power injector. The location and size of intravenous catheters and gadobutrol volume and flow rates were reported, and local and/or systemic adverse effects were recorded. DSC was qualitatively evaluated by CBV maps and signal intensity-time curves and quantitatively by the percentage of signal drop and full width at half-maximum, and the data were compared with the standards reported for adults. Quantitative data were grouped by flow rate, and differences among groups were assessed by analysis of covariance and tested for statistical significance with a t test. RESULTS: No local or systemic adverse events were recorded independent of catheter location (63 arm, 14 hand, 6 foot), size (24-18 ga), and flow rates (1-5 mL/s). High-quality CBV maps and signal intensity-time curves were achieved in all patients, and quantitative evaluations were equal or superior to those reported for adults. No significant differences (P ≥ .05) were identified among the higher-flow-rate groups in the quantitative data. CONCLUSIONS: A custom administration of a standard dose of gadobutrol allows safe and high-quality DSC MR perfusion imaging in children.

Endoscopic third ventriculostomy: the best option in the treatment of persistent hydrocephalus after posterior cranial fossa tumour removal?

BACKGROUND: Ten to 40% of children operated on for a posterior fossa tumour require a further surgical procedure for the management of a persisting active ventricular dilation. The management of this kind of hydrocephalus is still controversial. OBJECTIVE: To prospectively evaluate the effectiveness of post-operative endoscopic third ventriculostomy (ETV) in the management of persistent active hydrocephalus in a series of children operated on for a posterior cranial fossa tumour. METHODS: The management protocol consisted of: (1) placement of a peri-operative antibiotic impregnated external ventricular catheter (Bactiseal) and tumour removal, (2) post-operative intracranial pressure (ICP) monitoring through the external ventricular drainage, (3) ETV in case of persistent ventricular dilation and persistently abnormal high ICP values and (4) ventriculoperitoneal shunt implantation in case of ETV failure. RESULTS: Thirty on a total of 104 children (28.8%) operated on between January 2001 and February 2007 at our institution needed a further surgical treatment for the persistence of the hydrocephalus after the removal of their posterior cranial fossa tumour. They were sub-divided in two groups according to the early (group 1--21 patients) or later (group 2--nine patients) definition of the persistence of an active ventricular dilation based on clinical, radiological and ICP monitoring data. ETV was successful in 90.0% of the patients in the present series (27/30 patients), without statistically significant differences among the two groups considered. CONCLUSIONS: Post-operative ETV should be considered the best option to treat persistent hydrocephalus after the removal of posterior fossa tumours.

Surgical treatment of moyamoya disease in pediatric age.

BACKGROUND: Moyamoya is a progressive occlusive cerebrovascular disease which affects the distal internal carotid artery and its main branches, and is characterized, from a clinical point of view, by a variety of ischemic manifestations ranging from transient ischemic attacks (TIA) to stroke. Although considered until recently as almost specific of Japanese population, it can also affect individuals of different origin, and there is an increasing interest for this rare disease in Western medical literature. Nonetheless papers dealing with moyamoya disease in pediatric age are still very rare and almost exclusively limited to isolated case reports. These considerations prompted us to review our experience with the diagnosis and treatment of such unusual disease. METHODS: Nine children (seven months to nine years of age) presented with clinical manifestations of occlusive cerebrovascular disease (TIAs or stroke), epilepsy, and/or psychomotor retardation. Neuroradiological investigation, and in particular cerebral angiography, demonstrated the classical pattern of moyamoya disease. Eight of these children underwent cerebral revascularization utilizing the encephalomyosynangiosis (EMS) technique, with a total of 14 operations (six on both sides, two on one side). RESULTS: There was no mortality neither major morbidity related to surgery. One patient with renovascular hypertension, died at home approximately six months after a successful operation, probably from cerebral hemorrhage. In all the cases postoperative angiography revealed a marked improvement in cerebral circulation. However, in spite of the apparently normalized cerebral blood flow, clinical manifestations were affected less significantly. In fact, there was only minimal if any improvement in children operated on with an already established hemiplegia; whereas improvement in motor ability was obtained in those presenting with hemiparesis or TIAs, as well as in epilepsy and psychomotor retardation. CONCLUSIONS: On the grounds of our results we conclude that cerebral revascularization (by means of a simple operation like the EMS) in children with moyamoya disease is worthwhile, provided that the operation be performed before irreversible cerebral damage is established; however, even under these circumstances the operation can at least stop the progression of the disease.

Combined management of intracranial vascular malformations in children.

UNLABELLED: We have reviewed 39 cases of intracranial vascular malformations, observed in the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1980 and 1995 (age 1-15 years). Diagnosis was of parenchimal AVM in 26 cases, in 7 cases of AVM of the Galen region, in 5 cases of dural AVM and in 1 case of venous angioma. RESULTS AND CONCLUSIONS: Haemorrhage was the prevalent clinical onset in patients with parenchimal AVMs (57.7% of cases). Only one patient with a Galen region AVM presented cardiovascular symptoms, while 2/5 children observed for a dural AVM complained a congestive heart failure. Total surgical excision was performed in 17 of the 26 patients with parenchimal AVMs; moreover surgery was combined with preoperative endovascular embolization in 2 cases and with radiosurgery in 1 patient; we think that surgery is still the treatment of choice in patients with parenchimal AVMs. Endovascular treatment and radiosurgery have mainly an adjuvant role; nevertheless we agree that a preoperative endovascular management of parenchimal AVMs reduces the surgical risk, especially in cases of huge AVMs or AVMs localized in eloquent areas of the brain. Radiosurgery may be an alternative choice for complex or deeply sited AVMs; however in most cases, a combination with surgery or endovascular treatment is necessary to reduce the "target" size. AVM embolization was the only treatment in 2 of the 6 cases of dural AVMs; dural AVM embolization was combined with surgery in 1 case. Endovascular AVM exclusion is the treatment of choice in these patients. Surgery may be combined with endovascular treatment in case of a partial persistence of the AVM, or if signs of revascularization are present on control angiography. A conservative management is justified in patients with carotido-cavernous fistulae; in fact a complete spontaneous thrombosis is very frequent in these cases. A high rate of cardiovascular symptoms is reported in patients with Galen region AVMs diagnosed in the 1st year of life, but only 1 of our patients complained of congestive cardiac symptoms. Surgical treatment of Galen region AVMs is associated with a high morbidity and mortality. Actually a transarterial embolization of afferent vessels represents the treatment of choice in these patients; it can be combined with transvenous approach in case of complex AVMs and/or a non complete AVM exclusion.

Surgical options in the treatment of interhemispheric arachnoid cysts.

BACKGROUND: Arachnoid cysts located within the interhemispheric fissure are a matter of discussion as to the surgical indication and the choice of the most appropriate surgical procedure. In fact, in spite of the large dimensions that they can reach, the clinical manifestations may remain subtle and apparently stable over the years. On the other hand, the larger utilization of prenatal ultrasound investigations has increased the number of lesions detected before they can provoke clinical manifestations. As far as surgery is concerned, two main options are available-an extrathecal shunting procedure or a direct approach to the lesion, with excision of its wall. METHODS: In this report, we analyze the results obtained in 25 children harboring an interhemispheric arachnoid cyst, surgically treated in the period 1978 to 1994. Mean age at diagnosis was 18.8 months (10 days to 15 years). RESULTS The main clinical manifestations included macrocrania, mild to moderate signs of increased intracranial pressure, cranial bulging, developmental delay, and neurologic signs. In 14 cases, the diagnosis had been obtained prenatally. The cyst was located in one hemicranium in 16 cases, whereas it was on the midline, with bilateral extension, in the remaining 9 cases; in the latter cases, variable degrees of callosal agenesis were also observed. All 25 children were operated on. In 16 of them, the treatment consisted of a craniotomy with wide excision of the cyst lining and marsupialization into the subarachnoid spaces of the midline (and/or into the ventricular system). In 6 children, the surgical procedure was a cystoperitoneal (CP) shunt. The last 3 children of our series underwent craniotomy and cyst excision after having been treated by means of a ventriculoperitoneal shunt. At follow-up examination, 17 children are normal, and the remaining 8 disclose mild to moderate psychomotor retardation. CONCLUSIONS: Although both craniotomy and CP shunt are associated with good results, the first surgical procedure offers the advantage of avoiding the insertion of a permanent cerebrospinal fluid shunt device and the known complications of this type of procedure.

Treatment of hydromyelia in spina bifida.

BACKGROUND: Cavitation of the spinal cord (hydrosyringomyelia) seems to be common in spina bifida patients, although the overall clinical and radiological incidence has very rarely been analyzed. The real incidence of the condition is, in fact, difficult to assess as patients with this complication may remain relatively asymptomatic. The mechanisms accounting for the occurrence of hydrosyringomyelia in myelodysplasia are still a subject of debate. Similarly, the indications for the surgical treatment are still to be defined and its results to be assessed. METHODS: In this study, 142 patients with spina bifida were screened with magnetic resonance imaging (MRI) to determine the incidence of hydromyelia. Thirty-two patients presented a cavitating lesion on MRIs of which 18 were not a cause of symptoms and 14 were. Clinical characteristics of the lesions and their correlation to the radiological picture were analyzed. All the symptomatic children underwent surgical treatment. Outcomes were evaluated and correlated also with the MRI findings. RESULTS: A correlation between the presence of symptomatology and the extension and/or dilation of the hydromyelia was observed. A significant relationship between the presence of symptoms and adequate control of associated hydrocephalus was also noted. No correlation was observed between the presence of hydromyelia and the level of the myelomeningocele or the degree of ventricular dilation in those cases that presented with hydrocephalus. The mean age at hydromyelia diagnosis for all patients affected was 3.9 years and, in particular, 4 years for those patients who presented with symptoms. A total of 15 operations were performed in the 14 symptomatic patients. Seven direct procedures were carried out to drain the hydromyelia: five hydroperitoneal shunt insertions and two hydrosubarachnoid shunting procedures. Eight indirect treatments were performed to eliminate the filling mechanism of the hydromyelia: five posterior fossa decompressions, two ventriculoperitoneal shunt revisions, and one spinal cord untethering procedure with terminal syringostomy. All patients improved postoperatively, except one who remained clinically stable at follow-up. This patient had undergone a posterior fossa decompression followed by a hydrosubarachnoid shunting procedure. CONCLUSIONS: The incidence of syringohydromyelia is actually higher than that reported in the literature, although not all lesions are symptomatic. The radiological picture may be helpful in planning a more careful follow-up program for patients theoretically at risk for neurologic deterioration. Surgical treatment is possible with good results in the majority of cases and should be carried out as soon as possible before further deterioration occurs. Not all symptoms respond equally to treatment. Generally, improvement of clinical status correlates with a reduction in size of the hydromyelic cavity on MRI.

Intra-axial dermoid/epidermoid tumors of the brainstem in children.

BACKGROUND: Epidermoid and dermoid cysts are rare intracranial space-occupying lesions, which account for about 1% of all intracranial tumors; they are even rarer in the pediatric age group. Among the various locations, that inside the brain stem is quite exceptional (only 12 cases reported to date in the literature). We report two additional cases occurring in children, underlining their clinical characteristics, the difficulties faced in the diagnostic work-up, and the surgical treatment adopted (which consisted of removal of the cyst contents and partial resection of the cyst membrane, because of its adherence to the surrounding nervous and vascular structures). METHODS: We report two cases of intraaxial dermoid/epidermoid cysts observed within the last 5 years. Both patients complained of cervico-nuchal pain, with a remittent/intermittent character in one of them; this patient also exhibited transient 6th and 7th cranial nerve deficit. One lesion was approached through the floor of the fourth ventricle, the second one through the ventrolateral aspect of the brainstem. RESULTS: Subtotal tumor resection was achieved in both cases. Both patients had a smooth postoperative course without permanent neurological deficits. In the first patient residual tumor remains stable on follow-up MRI. In the second one, early tumor regrowth necessitated a second operation, after which the residual tumor has remained stable. CONCLUSIONS: Based on our experience we suggest that a cautious surgical approach ("conservative" resection) to these lesions is the best choice. In fact, attempts at radical removal carry unacceptably high morbidity and mortality rates.

Spontaneous spinal epidural hematoma in toddlers: description of two cases and review of the literature.

The cases of two children under the age of two who presented with spontaneous spinal epidural hematoma (SSEDH) are reported. The patients were operated on soon after the diagnosis had been confirmed by magnetic resonance imaging examination. The present paper highlights the fact that in toddlers the diagnosis of SSEDH based on a mere clinical picture is particularly difficult, and that this pathology presents a good prognosis following the surgical treatment. The pertinent literature is reviewed.

Solid-supported reagents for multi-step organic synthesis: preparation and application.

Since the early days of combinatorial chemistry solid-phase organic synthesis has been the method of choice for the production of large libraries. Solution-phase synthesis is again gaining importance especially for the synthesis of parallel arrays of smaller, focussed libraries containing single compounds with high degrees of purity. In the field of solution-phase library generation, the use of solid-supported reagents, catalysts and scavengers is emerging as a leading strategy, combining the advantages of both solid-phase organic synthesis (e.g. allowing the employment of an excess of reagent without the need for additional purification steps) and solution-phase chemistry (e.g. the ease of monitoring the progress of the reactions by applying LC-MS, TLC or standard NMR techniques). An account of some of the most recent advances in this area of research will be presented.