Endoscopic third ventriculostomy in an infant with vein of Galen aneurysmal malformation treated by endovascular occlusion: Case report and a review of literature.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAMs) can, through multiple mechanisms, complicate with hydrocephalus (HCP). It is generally agreed that management strategies in this scenario should focus on endovascular embolizations. Treatment options for non-responders, however, have been only scarcely reported upon. CASE PRESENTATION: We present a nine-month-old boy with a mural type VGAM complicated by HCP. Despite endovascular occlusion of the sole feeder, the child exhibited hydrocephalus progression prompting an Endoscopic Third Ventriculostomy (ETV). This procedure restored a cerebrospinal fluid (CSF) circulation otherwise impaired by aqueduct obstruction. Later, a new feeder arose and a second embolization was ultimately needed in order to achieve VGAM regression. Throughout four years of follow up, the child attained all developmental marks. DISCUSSION/CONCLUSION: VGAMs are prone to hydrocephalus development as there is both an underlying venous congestion and a mechanical, obstructive component. Although there is a rationale for addressing both components, the underlying AV shunts and subsequent venous pressure elevations usually determine failure of traditional CSF shunting strategies. It is therefore challenging to manage HCP in patients who failed to improve following endovascular embolizations. For such cases, ETV stands as an elegant minimal invasive alternative with potential to provide a more physiologic drainage route and thus better allow for neurological development.

Minimally invasive endoscopically assisted remodelation (MEAR) of sagittal craniosynostosis: an alternative technique to open and endoscopic procedures with cranial orthosis time span reduction.

INTRODUCTION: Sagittal craniosynostosis represents the most frequent simplex skull suture pathology. There are currently several operative approaches to this defect. Minimally invasive techniques are preferred for young infants. Since July 2017, we have employed endoscopically assisted craniectomies followed by cranial orthosis. Gradually, we have developed our modified technique, the minimally invasive endoscopically assisted remodelation (MEAR). SURGICAL TECHNIQUE: MEAR is a combination of principles gained from classical cranial vault remodeling techniques and minimal invasive approaches. The long and wider lateral osteoectomies performed in the parietal and occipital bones along with loosening of the periosteum and dura adhesions at the lambdoid sutures lead to early correction of parieto-occipital dimensions. RESULTS: Thirty-one consecutive patients with scaphocephaly underwent MEAR. The median preoperative cephalic index of 67 units (P25:63.3, P75:70) was improved to a median postoperative cephalic index of 77 units (P25:75, P75: 81). Sufficient correction was achieved in all patients. Cranial orthosis was needed for a median of 1.5 months (P25:1, P75:2). We had no major surgical complications in this pilot series. CONCLUSIONS: With MEAR, we have achieved good cosmetic results. Duration of cranial orthosis was significantly shortened compared to conventional endoscopic-assisted procedures.

Torcular dural sinus malformations: a grading system proposal.

BACKGROUND: Torcular dural sinus malformations (tDSMs) are congenital complex vascular anomalies often referred as a single unit. Nevertheless, they possess distinct anatomical features, clinical diversity, and markedly different outcomes. OBJECTIVE: On the basis of our institutional experience and analysis of published data, we propose a grading system. METHODS: We have identified 44 papers to which we added our four institutional cases for a total of 126 patients. Eight predictor variables were studied. In order to assess their individual impact on mortality and possible correlations, a logistic regression model was constructed through a stepwise forward process. RESULTS: Overall mortality was 22.1%. Mortality was higher in tDSM patients diagnosed postnatally, 40.7% versus a 15.6% in prenatally found cases (p = 0.007). We divided the patients into four grades. Grade I comprised patients with no feeder evidence and possessed the best outcomes (mortality of 7.55%). Mortality rose for grades II and III defined respectively by scarce and multiple feeders. Brain damage was the defining feature of grade IV. A mortality of 75% could be observed within this grade. Grade IV was further divided into grades IVa (antenatal) and IVb (postnatal cases). Furthermore, our logistic regression model found that brain damage (OR 11.3, p < 0.001, 95% CI 2.97-42.91) and patent feeders (OR 4, p = 0.03, 95% CI 1.15-13.86) were major determinants of poor outcome (area under ROC curve of 81.44%). CONCLUSION: The grading system (tDSM-GS) streamlines classification into four different grades facilitating both diagnosis, clinical decision-making, and proper prognostication.

Torcular dural sinus malformations: a single-center case series and a review of literature.

INTRODUCTION: Torcular dural sinus malformations (tDSMs) are rare vascular pathologies with various anatomoclinical pictures and prognosis. We analyzed our case series and corroborated the complexity of this rare unit by a review of literature. CASE SERIES: From 2003 to 2018, we treated four tDSMs patients. The evolution of three postnatally diagnosed cases of similar angioarchitecture contrasted with a fourth antenatally diagnosed case with significant torcular thrombosis. All patients were examined by computed tomography, magnetic resonance imaging, CT angiography, and MRI angiography. Three patients underwent digital subtraction angiography with embolization of feeders. Unusual pathological images were depicted. CONCLUSIONS: Early diagnosis along with embolization of feeders and lake could improve the outcome for tDSM patients with dural arteriovenous shunts. Ventriculoperitoneal shunt implantation before endovascular treatment led to significant worsening of both clinical presentation and MRI picture. For patients who persist with hydrocephalus despite the endovascular approach, we suggest endoscopic third ventriculostomy as a first-line treatment option. Antenatally diagnosed patients with thrombosed lakes constitute a prognostically better group of patients. Spontaneous thrombosis and remodelation of the lake can, however, still leave neurological sequelae, as observed in our patient.

Sagittal craniosynostosis associated with midline cephalhematoma or vice versa, case report and a review of the literature.

INTRODUCTION: Sagittal craniosynostosis associated with midline cephalhematoma is a rare finding. Despite the controversy regarding its etiopathogenesis, this condition represents a clear indication for surgery. CASE REPORT: We present a case of a 10-week-old boy with an ossified midline vertex cephalhematoma and sagittal craniosynostosis. The child underwent a cephalhematoma excision and minimally invasive non-endoscopic narrow vertex craniectomy, with calvarial vault remodeling followed by 2 weeks use of a cranial orthosis. On 5-month follow-up, mesocephaly was achieved. CONCLUSION: Our case is well documented with native CT, 3D CT, intraoperative pictures, and 3D head scan imaging. We described our minimally invasive non-endoscopic technique that led to a rapid cranial vault remodeling with reduction of cranial orthosis need. A review of literature focused on surgical techniques is included.