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Purpose: The prevention of Chiari type II malformation (CM) is commonly used as a primary outcome for the evaluation of techniques of fetal myelomeningocele (MMC) surgery in the fetal lamb. The aim of our study was to investigate the frequency of the associated CM in the MMC fetal sheep model and to investigate the contribution of prenatal ultrasound evaluation of CM at the time of prenatal repair. Methods: A MMC-like lesion was surgically created at 75 days of gestation in 21 fetuses performing a L1-L5 laminectomy followed by an excision of the exposed dura and a midline myelotomy. At a 90-day gestation, among the 19 alived fetuses, a conventional repair of the MMC-like lesion was performed in seven, four of whom underwent cerebral ultrasound (US) examination before the repair. Twelve fetuses remained untreated (control group). All fetuses underwent post-mortem examination (PM) at 138 days. Results: At a 90-day gestation, CM was demonstrated by US examination in all four evaluated fetuses. At birth, CM was found in 3/6 control whether CM was absent in all alived fetuses in the prenatal repair group ( n = 4). Conclusions: Creation of a MMC-like lesion with an additional myelotomy does not always lead to hindbrain herniation. Our study suggests that CM should be assessed by ultrasound examination at the time of the prenatal repair to demonstrate the effectiveness of new techniques for the prenatal repair of MMC. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: To compare simple enucleation (SE) and standard partial nephrectomy (SPN) in terms of surgical results in a multicenter dataset (RECORd Project). Materials and methods: patients treated with nephron sparing surgery (NSS) for clinical T1 renal tumors between January 2009 and January 2011 were evaluated. Overall, 198 patients who underwent SE were retrospectively matched to 198 patients who underwent SPN. The SPN and SE groups were compared regarding intraoperative, early post-operative and pathologic outcome variables. Multivariable analysis was applied to analyze predictors of positive surgical margin (PSM) status. Results: SE was associated with similar WIT (18 vs 17.8 min), lower intraoperative blood loss (177 vs 221 cc, p = 0.02) and shorter operative time (121 vs 147 min; p < 0.0001). Surgical approach (laparoscopic vs. open), tumor size and type of indication (elective/relative vs absolute) were associated with WIT >20 min. The incidence of PSM was significantly lower in patients treated with SE (1.4% vs 6.9%; p = 0.02). At multivariable analysis, PSM was related to the surgical technique, with a 4.7-fold increased risk of PSM for SPN compared to SE. The incidence of overall, medical and surgical complications was similar between SE and SPN. Conclusions: Type of NSS technique (SE vs SPN) adopted has a negligible impact on WIT and postoperative morbidity but SE seems protective against PSM occurrence. [Copyright &y& Elsevier]

Different management options are available for the treatment of colloid cysts. Goals of those procedures are to achieve a complete resection avoiding potential long-term recurrence along with CSF pathways restoration with minimal morbidity and mortality. The two main surgical options are endoscopic resection or direct removal by either transfrontal or transcallosal approach. The efficacy of endoscopic technique to achieve gross total colloid cyst excision has been well documented. In the present study, authors describe a series of 29 patients who underwent surgery by a variation of the standard worldwide implemented endoscopic technique. Using a more anterior approach, it is easier to reach the roof of the cyst, its possible adherences with the tela choroidea, plexus, and the internal cerebral veins. The described approach has shown to be safe, quick, and very effective with a total cyst removal rate of 86.2 %. [ABSTRACT FROM AUTHOR]

The article discusses a clinical analysis of a series of patients diagnosed with syndromic craniosyntosis to identify phenotypical characteristics for patients with splicing sites mutations and reports on 2 novel variants, the c.940-10T>A and c.994 1041del. It notes that splicing alterations can modify the probability of activating one splice site preferentially to another leading to a new repartition of FGFR2 and eventually modify the expression of the receptor in the different tissues.

Intracranial aneurysms are exceptional in neonatal patients: There are only 16 cases previously reported. We describe the first case of neonatal posterior inferior cerebellar artery (PICA) aneurysm and review the literature. A 7-day-old girl presented with irritability, anorexia, fever and abnormally enlarging head circumference. Computed tomography (CT) and magnetic resonance (MR) imaging demonstrated intraventricular haemorrhage, secondary hydrocephalus and a pontine cistern haematoma. A PICA aneurysm was suspected on the CT angiogram (CTA) and the diagnosis was confirmed by conventional cerebral angiography. She was successfully treated by surgical clipping of the parent vessel and excision of the aneurysm. Postoperatively, she experienced transient swallowing difficulties and required a ventriculo-peritoneal shunt for hydrocephalus. Histopathological evaluation demonstrated a calcified arterial wall with thrombosis, signs of prior haemorrhage and the absence of the internal elastic lamina. Neonatal intracranial aneurysms are rare. Clinical presentation of subarachnoid haemorrhage in this age group is often non-specific. First-line investigation should start with transfontanelle cranial ultrasound, followed by MR angiography then CTA if necessary. Posterior circulation aneurysms and large or giant aneurysms are more frequent in neonates and children than in adults. Early diagnosis and treatment are important for improved outcome. Surgery is better tolerated than in adults. [ABSTRACT FROM AUTHOR]

Between 1 January 1975 and 31 December 1982, 111 patients with atrioventricular septal defect underwent surgical repair. Of these, 43 had the complete, 11 the intermediate, and 57 the partial form. The postoperative mortality rate was 37%, 9%, and 6% respectively. To determine which factors were independently responsible for the operative risk multivariate analysis of the surgical mortality was applied simultaneously to all three forms of the malformation. The form of defect, although strongly influencing the natural history and clinical presentation, was not by itself an operative risk factor. The risk was related primarily to failure to obtain a well functioning atrioventricular valve, to the presence of left ventricular dominance, to the degree of pulmonary vascular resistance, and, finally, to the technique of reconstructing a two leaflet left atrioventricular valve. The small size of the patient was also a significant incremental risk factor, but if the other factors were not unfavorable good results could be achieved in small infants with mortality rates less than 10%. Multivariate analysis showed that severe postoperative left atrioventricular valve malfunction was related to the technique used to reconstruct a "normal" two leaflet left atrioventricular valve. These findings support the policy of reconstructing the left atrioventricular valve as a three leaflet valve. Nevertheless, the implicit beneficial effect of this technique has not as yet proved to be statistically significant. [ABSTRACT FROM PUBLISHER]