De novo cavernous angiomas associated with developmental venous anomaly: a mini-series and literature review.

BACKGROUND: Despite being previously considered as congenital lesions, recent studies agree to classify cerebral cavernous malformations (CCM) as acquired forms with clear correlations with other pathological affections of the central nervous system (CNS). In addition, a special subgroup, notably known as de novo CCMs (dnCCM), are associated in a significant number of cases with developmental venous anomalies (DVAs) and, in other cases, with Radiotherapy treatments. METHODS: A mini-series of 4 patients with clinical history characterized by developing dnCCM is reported. In three patients, the dnCCM was associated with the presence of an isolated DVA. In one case, no DVA was detected, but the patient underwent brain radiotherapy. In three cases, the dnCCM was clinically symptomatic, and the patients were submitted to a surgical procedure for lesion removal. In one case, the dnCCM was detected during MRI follow-up. RESULTS: Adding a review of the literature, we describe 47 patients who presented dnCCMs. The most common presentation is a sporadic CCM with a DVA, and the onset presentation was bleeding in 4 out of 47 cases (8.5%). Bleeding of dnCCM was observed in 9 out of 47 cases (19%), and the choice treatment was surgical in 24 out of 47 cases (51%). CONCLUSIONS: We present our series with a review of the recent literature and discuss the "de novo" cavernous malformation pathogenesis. A throughout review of recent literature is reported to clarify the predisposing factors that may lead to dnCCM development in patients carrying specific genetic and molecular features. Considering the high risk of bleeding, strict follow-up and aggressive treatment should be evaluated in dnCCM management.

State-of-Art of Standard and Innovative Materials Used in Cranioplasty.

Cranioplasty is the surgical technology employed to repair a traumatic head injury, cerebrovascular disease, oncology resection and congenital anomalies. Actually, different bone substitutes are used, either derived from biological products such as hydroxyapatite and demineralized bone matrix or synthetic ones such as sulfate or phosphate ceramics and polymer-based substitutes. Considering that the choice of the best material for cranioplasty is controversial, linked to the best operation procedure, the intent of this review was to report the outcome of research conducted on materials used for such applications, comparing the most used materials. The most interesting challenge is to preserve the mechanical properties while improving the bioactivity, porosity, biocompatibility, antibacterial properties, lowering thickness and costs. Among polymer materials, polymethylmethacrylate and polyetheretherketone are the most motivating, due to their biocompatibility, rigidity and toughness. Other biomaterials, with ecofriendly attributes, such as polycaprolactone and polylactic acid have been investigated, due to their microstructure that mimic the trabecular bone, encouraging vascularization and cell-cell communications. Taking into consideration that each material must be selected for specific clinical use, the main limitation remains the defects and the lack of vascularization, consequently porous synthetic substitutes could be an interesting way to support a faster and wider vascularization, with the aim to improve patient prognosis.

"Single-step" resection and cranio-orbital reconstruction for spheno-orbital metastasis with custom made implant. A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Brain metastasis involving the skull base is a rare complication of malignant tumors. Besides radiotherapy, surgical treatment is a therapeutical option even though it may apply complex technical procedures that may delay complementary therapies. However, in recent days, the innovation of custom-made implants allows treating selected patients with fewer complications and better results. CASE PRESENTATION: We describe a single case of a complex fronto-orbital skull base metastasis requiring skull bone reconstruction that we treated with a single-step surgery and custom-made implant. Our procedure consists of two steps: in the first one, we perform a "virtual" craniotomy on a 3D phantom model previously built on a high-resolution bone CT scan. In the second step, the actual surgical procedure, the patient undergoes the resection and reconstruction of the cranial defect with an implant of PMMA custom-made cranioplasty. A three-month clinical and radiological follow-up is reported, which documented the extent of resection and good aesthetic results. CLINICAL DISCUSSION: In our case, we performed a skull reconstruction of cranio-orbital region with macroscopic gross-total resection of the tumor. Complementary radiotherapy was obtained after one month. Three-month follow-up showed good esthetic results and progression-free disease. A recent review of the literature is provided to discuss different reconstruction techniques. CONCLUSION: "Single-Step" resection and custom-made reconstruction is a relatively new technique that could be helpful not only for benign tumors, which remains its main application but also in selected cases of malignant tumors when immediate reconstruction and faster complementary treatments are needed.

Synchronized "One-Step" Resection and Cranio-Orbital Reconstruction for Spheno-Orbital Lesions With Custom Made Implant.

BACKGROUND: In the last few years, skull base tumors involving the fronto-orbital region have been approached with complex, invasive, and time-consuming cranial reconstruction techniques. On the other hand, recent custom-made implants allow easier and faster procedures, with excellent aesthetic results. The authors propose an easy surgical-planned protocol with a synchronized "one-step" resection and reconstruction of these complex lesions, with a preformed poly-methyilmethacrylate (PMMA) cranioplasty. METHODS: Our technique consists of a 2-phases procedure. In the first one, the so-called "virtual" phase, the authors get a tailored 3D-PMMA model based on high-resolution (HR) CT-scans of the patient's skull. Afterward, the authors perform the planned craniotomy on the 3D-PMMA model with the assistance of intraoperative navigation system. Therefore, a further CT-scan of the resected model is performed to develop the final implant. In the second phase, the "surgical phase," a single procedure consisting in a cranial resection and custom reconstruction is performed with the assistance of stealth-navigation. The authors describe 6 cases of complex fronto-orbital-sphenoidal benign tumors that required skull reconstruction. All patients were examined 3 months after surgery through cosmetic (facial and eyes symmetry and globe position) and ophthalmology tests (visual field, visual impairment, and diplopia). Postsurgical HR CT-scans of the head and MRI-scans of the brain documented a total resection of the tumor and an optimal accuracy of skull reconstruction. RESULTS: In all cases, the authors obtained a highly accurate skull reconstruction following cranio-orbital tumor resection, with a less aggressive and faster procedure compared to autologous bone graft. Final cosmetic and functional results were excellent, with good results in cases of presurgical exophthalmos and orbital asymmetry. None of the patients developed implant-related complications. CONCLUSIONS: The "one-step" resection and reconstruction of benign tumors involving the spheno-orbital region with neuronavigation assistance is a technique that allows an accurate tumor removal and a cranial-bone reconstruction within a single surgical procedure, with fewer complications and excellent cosmetic and functional results.

Clinical and radiological aspects of cerebellopontine neurinoma presenting with recurrent spontaneous bleedings.

BACKGROUND: Neurinomas are benign, usually encapsulated, tumors growing in peripheral nerve sheath with a high incidence in the cerebellopontine angle. CASE DESCRIPTION: We report a case of vestibular neurinoma (VN) with a "biphasic" pattern of intratumoral hemorrhage presenting with cephalalgia along with progressive ipsilateral mild impairment of both VII and VIII cranial nerves. A thorough preoperative magnetic resonance imaging study better characterized the patchy pattern of the round shaped lesion, resulting in three different intensity signals, due to the peculiar characteristics of the tumoral mass and the recurrent bleedings, respectively. Postoperatively, histological examination confirmed the diagnosis of neurinoma. CONCLUSION: Hemorrhagic VN are rare tumors; from the first case described in 1974 only 43 more have been reported in the literature so far. Noteworthy, "biphasic" bleedings are even rarer. From an accurate review of the literature we collected and thus emphasized the radiological and clinical features of this rare entity. Eventually, we suggest that the early surgical removal of clots and tumor is essential to provide the best chance of neurological improvement.