Giovanna Paternoster

On the changing epidemiology of hydrocephalus

PurposeThe purpose of this study is to evaluate the changing epidemiology of paediatric hydrocephalus over the past three decades in a single institution.MethodsAll children treated for newly diagnosed hydrocephalus during the 1985–1990 (group A) and the 2000–2005 periods (group B) were enrolled and classified according to the associated cause of hydrocephalus.ResultsA significant 8.8% decrease of the incidence of hydrocephalus was noticed between the two time periods, resulting from the reduction of hydrocephalus associated to myelomeningocele, aqueduct stenosis (p = 0.04), CNS infection (p = 0.03), cranio-cerebral malformation and head injuries; post-haemorrhagic hydrocephalus remained stable, while the tumour-associated one increased (p < 0.0001). No consistent differences in terms of rate of adjunctive surgery (30.3% versus 23.9%) and number revision procedures (200 versus 104) were recorded.ConclusionsThe present study confirmed data from the literature about the declined incidence of paediatric hydrocephalus, which mainly results from the decrease of congenital malformations. In spite of the recent advances in neuroendoscopy and in the shunting valve design, the impact of hydrocephalus in the paediatric neurosurgical practice remains high.

Sleep disordered breathing in children and adolescents with Chiari malformation type I.

STUDY OBJECTIVES Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents. METHODS Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction. RESULTS Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034). CONCLUSIONS In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB.

Anterior plagiocephaly: epidemiology, clinical findings,diagnosis, and classification. A review

IntroductionAnterior plagiocephaly due to the early unilateral fusion of the coronal suture is associated to a clinical phenotype characterized by the unilateral flattening of the frontal bone and the orbit and by the distortion and maldevelopment of adjacent cranial and facial bones. Homolateral anterior displacement of the ear and contralateral deviation of the nasal pyramid and mandible are other prominent clinical features. The differential diagnosis includes a variety of conditions characterized by facial asymmetry, namely, due to muscular alterations, anomalies of the visual function, congenital anomalies of the cervical spine and craniovertebral junction, abnormal pregnancy, and birth injuries. Anterior plagiocephaly is the direct cause of oculomotor anomalies, with a characteristic strabismus, and of progressive asymmetrical maldevelopment of the face, craniovertebral junction, and cervical spine.MethodsThe medical literature was reviewed in regard to the epidemiology, clinical features, and radiological findings. Different categorization of the affected infants in specific subtypes according to the severity of the condition was analyzed. The aim was to contribute to a practical clinical classification to be utilized for the surgical indication and for predicting the prognosis according to the severity of the condition at presentation.ConclusionsAnterior plagiocephaly represents the most challenging simple suture craniosynostosis. The clinical differential diagnosis with other forms of cranial asymmetry is possible on the grounds of mere clinical findings. A classification system is necessary not only for the establishment of surgical planning but also to predict the late cosmetic and functional outcomes.

Isolated sagittal craniosynostosis: definition, classification, and surgical indications

Sagittal craniosynostosis (SC) remains the most common type of synostosis, accounting for about a half of all forms. It would result from a mesenchymal disorder involving the intramembranous ossification of the sagittal suture and leading to its early fusion. No specific data on the etiologic factors are currently available. The premature ossification of the sagittal suture can result in three main types of SC, according to the different segment prevalently involved: anterior, posterior, and complete SC. The diagnosis is easily obtained by clinical examination. However, a radiological work up (3D CT scan) may be necessary to rule out hidden venous or cranial anomalies possibly associated with most severe cases, or for the surgical planning. The most common indication for surgery is the improvement of the cosmetic appearance of the skull, since a cranial deformation may have a significant psychological impact on affected subjects. To relieve from raised intracranial pressure is a further indication to surgery. Although an increased intracranial pressure can be demonstrated in a minority of affected children at diagnosis, indeed, it can present later (usually after the second/third year of life) with chronic symptoms. The role of surgery in the preservation of cognitive functions in scaphocephalic patients does not seem to be relevant, since minor anomalies of the cerebral development associated with SC would occur independently from the cranial shape. On the other hand, the surgical correction may show a protective effect on some visual skills, like the ability to fix and follow, and the fixation shift.

Arterial spin labeling magnetic resonance imaging: toward noninvasive diagnosis and follow-up of pediatric brain arteriovenous malformations

OBJECT Arterial spin labeling (ASL)-MRI is becoming a routinely used sequence for ischemic strokes, as it quantifies cerebral blood flow (CBF) without the need for contrast injection. As brain arteriovenous malformations (AVMs) are highflow vascular abnormalities, increased CBF can be identified inside the nidus or draining veins. The authors aimed to analyze the relevance of ASL-MRI in the diagnosis and follow-up of children with brain AVM. METHODS The authors performed a retrospective analysis of 21 patients who had undergone digital subtraction angiography (DSA) and pseudo-continuous ASL-MRI for the diagnosis or follow-up of brain AVM after radiosurgery or embolization. They compared the AVM nidus location between ASL-MRI and 3D contrast-enhanced T1 MRI, as well as the CBF values obtained in the nidus (CBFnidus) and the normal cortex (CBFcortex) before and after treatment. RESULTS The ASL-MRI correctly demonstrated the nidus location in all cases. Nidal perfusion (mean CBFnidus 137.7 ml/100 mg/min) was significantly higher than perfusion in the contralateral normal cortex (mean CBFcortex 58.6 ml/100 mg/min; p < 0.0001, Mann-Whitney test). Among 3 patients followed up after embolization, a reduction in both AVM size and CBF values was noted. Among 5 patients followed up after radiosurgery, a reduction in the nidus size was observed, whereas CBFnidus remained higher than CBFcortex. CONCLUSIONS In this study, ASL-MRI revealed nidus location and patency after treatment thanks to its ability to demonstrate focal increased CBF values. Absolute quantification of CBF values could be relevant in the follow-up of pediatric brain AVM after partial treatment, although this must be confirmed in larger prospective trials.

Segmental Spinal Myoclonus and Metastatic Cervical Ganglioglioma: An Unusual Association

Segmental spinal myoclonus rarely occurs in association with spinal cord tumor. Only 3 cases have been reported in children so far, mainly concerning astrocytomas of the thoracic spinal cord. We report on a 2-year-old boy suffering from segmental spinal myoclonus involving the upper limbs and harboring a cervical tumor. The clinical and electrophysiological features ruled out a myoclonus of different origin (cortical, subcortical, propriospinal) other than other types of movement disorders. Neuroimaging and histological examinations showed the exceptional presence of a ganglioglioma as the cause of the segmental spinal myoclonus. The clinical and electrophysiological characteristics as well as the possible etiopathogenesis and differential diagnosis are discussed on the basis of the pertinent literature to add some more information about the unusual association between spinal cord tumors and spinal myoclonus.

Mini-invasive surgery for Chiari type I malformation.

Surgical treatment of Chiari I malformation and associated syringomyelia includes several different techniques with various degrees of invasiveness. Most extensive procedures may provide good long-term outcome in a good proportion of cases but are burdened by a quite high risk of postoperative complications. Thirty children operated on by simple bone decompression are retrospectively reviewed to assess the effectiveness of a less invasive technique. The present series comprises 30 children (18 females, 12 males; mean age: 68 months) treated from 1993 to 2005. All patients underwent foramen magnum decompression by means of suboccipital craniectomy and resection of the fibrous band at the level of foramen itself. Twenty-one children also required C1 laminectomy while a dural delamination was performed in 11 cases. The mean current follow-up is 4.3 years (1–12.6 years). Head and/or neck pain was the most frequent preoperative finding (56.7%), followed by upper and lower extremity weakness (20.0%), ataxia (20.0%) and vertigo (27.7%). Syringomyelia was present in 12 patients. A significant improvement of preoperative clinical symptoms and signs was observed in 28 patients (93.3%). Two children required adjunctive surgery. Neuroimaging revealed minor postoperative modifications in most cases regardless of tonsils location, while syringomyelia was reduced in size in 50% of the cases. Complication rate and length of hospital stay were significantly reduced compared with the literature data and our own experience using more invasive techniques. These data, compared with the literature, allow us to conclude that suboccipital craniectomy and Cl laminectomy (possibly integrated by dural delamination) is an effective and safe treatment for symptomatic children with Chiari I malformation and syringomyelia.

Huge cranio-cerebral rhabdomyosarcoma in HIV-positive patient

Rhabdomyosarcomas (RMS) are highly malignant tumors originating from mesenchymal cells. Typically, they affect children, and prognosis is quite poor. In adults, RMS are rare, and cranial locations are exceptional. Here, we report a case of huge cranial RMS in an immunosuppressed adult patient. To our knowledge, this is the first reported case of primitive RMS of the cranial vault in a HIV-infected patient.

The use of a reconstituted collagen foil dura mater substitute in paediatric neurosurgical procedures—Experience in 47 patients

Background. CSF leakage is a common complication of neurosurgical procedures, with defective dural suture thought to be the most frequent cause. We report our experience with a new collagen foil (TissuDura®, Baxter Healthcare SA, Switzerland) utilized as dural substitute in paediatric neurosurgical procedures. Methods. TissuDura® was used in children consecutively operated on at the department of paediatric neurosurgery, Catholic University, Rome, from March 2004 to August 2007. Children underwent surgical procedures in supratentorial, infratentorial and spinal compartments. In supratentorial and spinal procedures, the dural graft was used according to the overlay technique. In the posterior fossa procedures, the underlay technique was used. Results. Forty-seven patients received TissuDura® during surgery. Thirty-one patients underwent surgery for the removal of posterior fossa tumours, nine for supratentorial tumours and seven for spinal dysraphisms. No CSF leakage was observed following the use of TissuDura® in supratentorial procedures. Two post-operative CSF leaks occurred in patients who had undergone spinal surgery. No post-operative hydrocephalus was noted in these two surgery groups. Three cases of CSF leakage occurred in patients who had undergone posterior cranial fossa surgery. All 3 cases had an associated supratentorial ventricular dilation present prior to the removal of the tumour (one case) or occurring after the tumour excision (two cases). No clinically evident adverse reactions directly related to TissuDura® were observed. Conclusions. The main advantages of TissuDura® were its apparent ability to prevent CSF leakage when utilized in a specific subset of patients, and the absence of reactions or postoperative infections.

CSF Otorrhea after Anterior Temporal Craniotomy due to Hyperpneumatization of the Temporal Bone

Pneumatization of the temporal bone is a well-recognized process that can increase the risk of postoperative CSF leakage [1] . As a result of the increasing number of patients undergoing CT scan of the cranium, an increasing number of asymptomatic cases of hyperpneumatization of the temporal bone is diagnosed. For this reason and because of the associated risk of postoperative CSF fistula, we emphasize the need for a preoperative CT scan before a basal temporal craniotomy.