In patients with BAV, the root phenotype (aortic dilation predominantly at the sinuses, with normal or less dilated ascending tract) may be a marker of more severe aortopathy, warranting closer surveillance and earlier treatment. The more common ascending phenotype proved to be a more stable disease entity, generally with slower progression.

The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

Object In this paper, the authors' goal was to evaluate the role of neuroendoscopy in the treatment of arachnoid cysts of the quadrigeminal cistern. Methods Between March 1995 and February 2008, 14 patients affected by arachnoid cysts of the quadrigeminal cistern were treated endoscopically. The cysts were classified according to their anatomical and radiological appearance. The most frequent form (Type I) extended infratentorially and supratentorially with a dumbbell shape. Type II cysts were confined to the infratentorial space and were associated with the most severe and acute form of hydrocephalus. Type III cysts presented a significant asymmetric expansion toward the temporal fossa. Ten patients underwent an endoscopic procedure as primary treatment and 4 as an alternative to shunt revision. In 6 cases, the first endoscopic procedure was ventriculocystostomy (VC) together with endoscopic third ventriculostomy (ETV). In the other 8 cases, the first endoscopic procedure was VC alone. Results In the 6 cases in which VC was performed with an ETV, the procedure was successful, and the patients did not require further surgery. Of the 8 cases in which the first endoscopic procedure performed was VC without ETV, 7 underwent reoperation. Four of these patients underwent endoscopic procedures (by reopening the obstructed VC and performing ETV or cystocisternostomy) 2, 4, 4, and 5 months later with final success in all cases. Three patients (all of whom were previously treated using ventriculo- or cystoperitoneal shunts) required shunt reimplantation (complete failure). Subdural collection developed in 1 case, which was managed by transient insertion of a subduroperitoneal shunt. Neurological and developmental outcomes were good except for 1 patient who did not show improvement in preoperative developmental delay. No transient or permanent morbidity or mortality was observed. Conclusions The analysis of this series suggests that arachnoid cysts of the quadrigeminal cistern and the associated hydrocephalus can be effectively treated by endoscopy; this approach allows the patient to be shunt independent in more than 78% of the cases. If endoscopy is used as first option, the success rate of endoscopic procedures observed in this series was 90%. Endoscopic third ventriculostomy should be associated with a VC to offer the highest success rate with a single procedure.

When tested on a large population, three previously suggested phenotypic classifications of the BAV aorta proved to categorize patients into significantly different clusters, but only the classification system distinguishing between ascending phenotype and root phenotype showed a potential prognostic value. Phenotypic class of the aorta could be a factor to integrate in future comprehensive models for risk stratification of BAV aortopathy.

Pseudotumor cerebri is an avoidable cause of visual loss, both in adults and children. Few diagnostic measures are usually sufficient to determine the correct diagnosis. Since pseudotumor cerebri is a diagnosis of exclusion, the differential diagnosis work out is of special importance. Modern neuroimaging techniques, especially magnetic resonance imaging and magnetic resonance venography may clarify the role of obstruction to venous outflow in each case. Various therapeutic options are available: medical, surgical, and endovascular procedures may be used to prevent irreversible visual loss. Treatment is usually effective, and most patients will experience complete resolution of symptoms without persistent deficits.

Complication rate of neuro-endoscopic procedures is not negligible even in experienced hands. The majority are minor complications which do not affect the final outcome, but sporadically major events may occur, leading to significant problems in surgical management and, occasionally, to permanent disabilities. Careful selection of patients on pre-operative imaging studies and intensive training of surgeons are mandatory to improve results.

This pilot prospective uncontrolled trial suggests that oral sildenafil is effective in allowing candidacy, safe transplantation and postoperative pulmonary profile normalization in potential recipients initially disqualified because of PH.

OBJECTIVEIn the past, the outcome of surgical treatment for thalamic tumor was poor. These lesions were often considered inoperable. However, contemporary microsurgical techniques, together with improvements in neuroimaging that enable accurate presurgical planning, allow resection to be accomplished in a safer way.METHODSThe medical records, imaging studies, and operative and pathology reports obtained for pediatric patients who were treated for thalamic tumors at the authors’ department were reviewed. Neuronavigation and intraoperative monitoring of motor and somatosensory evoked potentials were used. Preoperative tractography, which helped to identify internal capsule fibers, was very important in selecting the surgical strategy. Postoperatively, an MRI study performed within 24 hours was used to assess the extent of tumor resection as partial (≤ 90%), subtotal (> 90%), or gross total (no residual tumor).RESULTSSince 2002, 27 children with thalamic tumors have been treated at the authors’ department. There were 9 patients with unilateral thalamic tumors, 16 with thalamopeduncular tumors, and 2 with a bilateral tumor. These last 2 patients underwent endoscopic biopsy and implantation of a ventriculoperitoneal shunt. Thirty-nine tumor debulking procedures were performed in the remaining 25 patients. Different surgical approaches were chosen according to tumor location and displacement of the posterior limb of the internal capsule (as studied on axial T2-weighted MRI) and corticospinal tract (as studied on diffusion tensor imaging with tractography, after it became available). In 12 cases, multiple procedures were performed; in 7 cases, these were done as part of a planned multistage resection. In the remaining 5 cases, the second procedure was necessary because of late recurrence or regrowth of residual tumor.At the end of the surgical phase, of 25 patients, 15 (60%) achieved a gross-total resection, 4 (16%) achieved a subtotal resection, and 6 (24%) achieved a partial resection. Eighteen patients harbored low-grade tumors in our series. In this group, the mean follow-up was 45 months (range 4–132 months). At the end of follow-up, 1 patient was dead, 12 patients were alive with no evidence of disease, 4 patients were alive with stable disease, and 1 was lost to follow-up. All patients were independent in their daily lives. The outcome of high-grade tumors in 9 patients was very poor: 2 patients died immediately after surgery, 6 died of progressive disease, and 1 was alive with residual disease at the time of this report.CONCLUSIONSThis institutional review seems to offer further evidence in favor of attempts at radical resection in pediatric patients harboring unilateral thalamic or thalamopeduncular tumors. In low-grade gliomas, radical resection in a single or staged procedure can be curative without complementary treatment. Recurrences or residual regrowth can be safely managed surgically. In high-grade tumors, the role of and opportunity for radical or partial resection remains a matter of debate.