Intracranial Aneurysm in Pediatric Population: A Single-Center Experience.

PURPOSE: Pediatric intracranial aneurysms (PIA) are rarer and more complex when compared to adult aneurysms. In general, the clinical presentation of PIA is due to a mass effect, but the presenting symptoms can be also related to ischemia, subarachnoid hemorrhage (SAH), or in a combination of different symptoms. This paper aimed to report a single-center experience with clinical and angiographic aspects of brain aneurysm in children. METHODS: We retrospectively reviewed our prospectively maintained database for patients with intracranial aneurysms in our institution from July 2015 to February 2021. Among these, all patients under 18 years of age submitted to a diagnostic or therapeutic procedure for an intracranial aneurysm were included. RESULTS: Twelve patients were submitted to diagnostic or therapeutic procedures in our department. Three of them had multiple aneurysms, and in total, 17 intracranial aneurysms were assessed in this study. The most frequent location was in the middle cerebral artery (7 cases/41%). Five out of twelve children (42%) presented SAH due to ruptured aneurysm. Three patients (25%) had symptoms due to the mass effect from large aneurysms, with compression of cranial nerves or brainstem. Aneurysms diameters ranged from 1.5 mm to 34 mm (mean 14.2 mm), with six aneurysms being giant and eight being nonsaccular/fusiform. Twelve aneurysms were submitted to endovascular treatment, with one treatment-related clinical complication and later death. CONCLUSION: PIAs are rare diseases that can arise from a variety of different underlying pathological mechanisms. The management of these conditions requires a detailed understanding of the pathology and a multidisciplinary approach. Despite the availability of new technologies, parent vessel occlusion remains a valid option for aneurysms in the pediatric population.

Spontaneous Bone Flap Resorption Following Cranioplasty Using Autologous Bone.

ABSTRACT: Cranioplasty is a common neurosurgical procedure. The main reasons for performing cranioplasty are, in addition to aesthetic correction and protection of the brain, the reestablishment of the adequate flow of cerebrospinal fluid and the prevention of complications inherent to the perpetuation of bone failure. In our institution the patient's autologous bone remains the best method for performing cranioplasty, despite the existence of other heterologous grafts and bone substitutes. Despite representing for us, the best material for cranioplasty, the use of autologous grafting is subject to complications. In this paper, the authors present the case of a patient who underwent cranioplasty with autologous bone that progressed with spontaneous resorption of the bone flap. The authors herein briefly discuss the case and review the literature on the subject, with an emphasis on the factors that can lead to such an outcome.

Retroclival Pneumocephalus Associated with Bilateral Abducens Palsy in a Child.

Traumatic brain injury (TBI) is less common in children than in adults. Posterior fossa lesions are even more uncommon, but, when present, are usually epidural hematomas. These lesions, even when small, may have a bad outcome because of the possibility of compression of the important structures that the infratentorial compartment contains, such as the brainstem and cranial nerves, and the constriction of the fourth ventricle, causing acute hydrocephalus. Although unusual, posterior fossa lesions are increasingly being diagnosed because of the better quality of and easier access to cranial tomography. In this paper, we report a case of a 12-year-old male patient who had suffered a TBI and presented with several pneumocephali, one of them in the retroclival region, causing a mass effect and then compression of the sixth cranial nerve which is the most susceptible to these injuries. We discuss these traumatic posterior fossa lesions, with an emphasis on retroclival pneumocephalus, not yet described in the literature in association with bilateral abducens palsy. In addition, we discuss associated lesions and the trauma mechanism.

[Davidenkow syndrome. A rare peripheral neuropathy]. / Síndrome de Davidenkow. Una neuropatía periférica rara.

A different and rare type of atrophy with a predominant pattern in scapulo-peroneal distribution was described by Davidenkow in 1939. The syndrome was characterized by some researchers as a variant of Charcot-Marie-Tooth disease, however Davidenkow noticed that clinical and laboratorial manifestations did not corroborate exactly with this hypothesis. We describe a case of a female patient, 39 years-old, clinical picture similar to the syndrome described by Davidenkow, presenting scapulo-peroneal atrophy. Her first symptoms had appeared when she was 24, initially with proximal motor weakness in the upper limbs. This patient did not have family history of myopathy or neuropathy. Several tests were performed to exclude other syndromes that could be included in the differential diagnosis, by testing gene mutation, in addition to the physical examination and electromyography. The large spectrum of neuromuscular diseases makes difficult the diagnosis of Davidenkow's syndrome which always should be considered in the differential diagnosis.

Congenital giant craniopharyngioma.

PURPOSE: Congenital intracranial tumors are extremely rare and the most common is teratoma. Craniopharyngioma is a rare neonatal tumor with only eight cases reported. The management of this tumor in the neonatal period is still controversial, with the best results obtained when radical resection is performed. We present the case of a patient who received the diagnosis of a suprasellar tumor during the prenatal period and reviewed literature regarding the management. METHODS: We report a case of neonatal craniopharyngioma treated surgically. RESULTS: The routine ultrasound at 29 weeks of gestation showed a suprasellar echogenic image measuring 44 mm in diameter with polyhydramnios and macrocephaly. The patient was born at 38 weeks of gestation and underwent a surgical treatment on its 32nd day of life and the excision of almost 80 % of the lesion was achieved. He developed a subdural hygroma and on the 51st day of life, a subduroperitoneal shunt was installed to treat it. This patient died at 8 months of life due to complications of a shunt infection. CONCLUSION: The present case is the ninth diagnosed during the prenatal period and the literature is controversial on the management of this rare tumor. The complete excision of the lesion using the microsurgical technique is the gold standard treatment for these patients; however, there are many factors that limit this approach in neonates. Usually, the resected tumors were smaller than 6 cm. Tumors larger than 8 cm have a worse prognosis, with a short survival time.

Evaluation of konjac noodle as a microsurgery training model: learning curve analysis.

BACKGROUND: classical models of microsurgical anastomosis training are expensive and have ethical implications. Some alternatives join low cost and easiness to store. However, the translation of knowledge acquired by training in these methods into the traditional ones is not clear. This project aims to assess the feasibility of konjac noodles as a reliable microsurgery-training model. METHODS: 10 neurosurgery residents performed an end-to-end anastomosis in a 2-3mm placenta artery. The anastomoses were evaluated quantitatively, recording time; and qualitatively, applying a validated score (Anastomosis Lapse Index - ALI) by three experienced neurosurgeons and verifying the presence of gross leakage through the infusion of fluorescein. Subsequently, they performed 10 non-consecutive sessions of anastomosis training in the konjac noodle. Eventually, a final anastomosis in the placenta model was performed and the same parameters were scored. RESULTS: we observed a 17min reduction in the mean time to perform the anastomosis in the placenta model after the training in the konjac (p<0.05). There was a non-significant 20% reduction in gross leakage, but the training sessions were not able to consistently improve the ALI score. CONCLUSIONS: we demonstrate a reduction in anastomosis performing time in placental arteries after training sessions in the konjac noodle model, which can be regarded as a feasible low-cost method, particularly useful in centers with surgical microscopes only in the operation room.

Cauda equina syndrome caused by spontaneous bleeding in the filum terminale myxopapillary ependymoma: a rare pediatric case.

The majority of the filum terminale ependymomas are of the myxopapillary type, which most commonly present as lumbago or sciatic pain, an insidious clinical condition, at times accompanied by paraparesis, bladder paresis and vesical alterations. We report the case of a 13-year-old patient who presented with acute cauda equina. He underwent total resection of the lesion, which resulted in progressive improvement. The clinical conditions, diagnoses and treatments of the medullary cone and cauda equina myxopapillary ependymomas are also discussed.

Malignant chondroid syringoma with central nervous system involvement.

Malignant chondroid syringoma is a mixed cutaneous tumor, with epithelial and mesenchymal components, which compromises principally the trunk and extremities. This lesion is quite rare, with few cases related in the literature and no publications demonstrating its involvement of the central nervous system. Histologically, owing to its mixed origin, it represents a lesion that is difficult to recognize, often being confused with basocellular carcinoma. We report the case of a female patient, carrier of malignant chondroid syringoma in the occipital region, with invasion of the central nervous system, who was submitted to surgical excision of the lesion at our service. We also made a brief revision of the literature on the theme.

WEB device for treatment of posterior communicating artery aneurysms.

BACKGROUND: Woven EndoBridge (WEB) device treatment of wide-neck bifurcation aneurysms has proved to be safe and effective, but the use of these devices in sidewall aneurysms has been reported only in a small number of case series. OBJECTIVE: To report our results in a cohort of consecutive patients in whom a WEB device was used as first-line treatment for posterior communicating artery (PComA) aneurysms. METHODS: We conducted a retrospective analysis of a prospectively maintained database of PComA aneurysms treated with a WEB device in our institution from June 1, 2012 to November 15, 2020. Clinical and radiological findings were evaluated at immediate and last follow-up. RESULTS: A total of 219 aneurysms were treated with a WEB device, including 15 PComA aneurysms in 15 patients, 10 of which were ruptured. Aneurysms were wide necked, with a mean aspect ratio of 1.6 (range 0.7-3.0) and a mean neck size of 4.2 mm (range 2.6-7.4 mm). No intraoperative rupture occurred and only one thromboembolic event was noted. Among the group with at least a 3-month digital subtraction angiography (DSA) follow-up, complete and adequate occlusion were obtained in 54% and 72%, respectively (average follow-up 13 months). Re-treatment was needed for two initially ruptured aneurysms. No procedure-related morbidity or mortality was reported. CONCLUSION: This series suggests the high safety profile of WEB devices even when used in off-label indications. Treatment with these devices seems to be a valuable strategy for ruptured wide-neck PComA aneurysms, avoiding the need for antiplatelet medication. However, occlusion rates should be investigated in further larger studies.

Woven EndoBridge device shape modification can be mitigated with an appropriate oversizing strategy: a VasoCT based study.

BACKGROUND: The Woven EndoBridge (WEB) shape modification (WShM) during follow-up may be a potential cause of poor angiographic outcomes. WShM predisposing factors have not yet been determined. Our systematic use of rotational cone beam computed tomography (VasoCT) imaging during follow-up allowed us to perform the first quantitative analysis of the shape of WEBs over time. Our goal was to identify possible strategies to reduce the occurrence of this phenomenon. METHODS: All patients treated in our hospital with a WEB device between October 2015 and January 2019 were included. Using VasoCT acquisitions, systematically performed after implantation and during follow-up, we analyzed WEB morphology. WShM was defined as the percentage reduction in the distance between the two WEB markers. RESULTS: Sixty-three aneurysms treated with a WEB device were finally included in this analysis. At the last follow-up (mean 15.5 months), mean WShM was 48%±24. The mean WShM was significantly higher in the aneurysm recurrence group than in the adequate occlusion group (51±6.5% vs 36±3.4%, difference 15% points (95% CI 0.7 to 30); p<0.05). Conversely, the extent of WShM did not directly correlate with occlusion rates. Indeed, 32% of completely occluded aneurysms presented severe WShM (≥50%). Importantly, the absence of WShM guaranteed complete occlusion in our study (n=12). We demonstrated that oversizing the width of the WEB significantly correlated with WShM reduction during follow-up (r=-0.38, p=0.002). CONCLUSION: WShM can be partly overcome by use of an appropriate width oversizing strategy that could lead to improved angiographic results.

Retroclival epidural hematoma in a child affected by whiplash cervical injury: a typical case of a rare condition.

Traumatic posterior fossa epidural hematomas are uncommon lesions. Among these lesions, retroclival epidural hematomas (REDH) are particularly rare conditions that usually occur in the pediatric population due to predisposing anatomical features in this patient group. We describe a typical case of traumatic REDH from the mechanism of trauma to outcome. This 8-year-old girl was involved in a motor vehicle accident leading to whiplash cervical injury and cranial nerve palsy. Any children involved in a severe motor vehicle accident with such a sequence of events should raise suspicion for prompt diagnosis.

Multiple epidural haematomas after meningioma surgery.

Intracranial meningioma removal carries a higher risk of post-operative haemorrhage compared with other intracranial neoplasm surgeries. We report a patient who developed three intracranial haematomas following a frontal meningioma removal.

Periodontoid pseudotumoral lesions.

BACKGROUND: Periodontoid pseudotumoral lesions (PPL) are an uncommon cause of cervical pain and myelopathy. In addition, they may be associated with atlantoaxial instability (AAI). CASE DESCRIPTION: Two patients over 60 years of age presented with neck pain alone. Their MR scans showed expansive lesions involving the odontoid process. One patient with AAI required an occipitocervical arthrodesis, while the other patient without instability was managed with an external orthosis (Philadelphia collar). Both of them experienced full resolution of pain and remained neurologically intact an average 36 months later (range 24-48). CONCLUSION: Here, we discussed the clinical, MR, and non-surgical (without AAI) versus surgical management (with AAI) for different types of PPL.

Acute Disseminated Encephalomyelitis Related to a Cytomegalovirus Infection in an Immunocompetent Patient.

Cytomegalovirus (CMV) infection can cause acute disseminated encephalomyelitis (ADEM). However, it is rare in immunocompetent people. We describe a 17-year-old patient who was brought with flu-like symptoms. After one week, she experienced rapidly progressive weakness in all four extremities, followed by coma. The neurologic examination showed no response to verbal and pain stimuli. A Babinski sign was noted in both lower extremities, along with clonus and hyperreflexia in all four limbs. Brain magnetic resonance imaging (MRI) demonstrated extensive areas of hyperintense signal on fluid-attenuated inversion recovery (FLAIR) sequences in the white matter which was asymmetrically distributed in both hemispheres, as well as in the brainstem and cerebellar peduncles, compatible with acute demyelinating lesions. Cerebrospinal fluid (CSF) showed mild lymphocytic pleocytosis and normal glucose levels. Polymerase chain reaction to herpes simplex virus was negative. Serum immunoglobulin G (IgG) and immunoglobulin M (IgM) were positive for cytomegalovirus. The patient was treated with methylprednisolone pulse therapy for five days. Subsequently, the patient showed neurologic improvement with the recovery of consciousness and muscle strength. In terms of prognosis, in patients with ADEM, the sooner the diagnosis, the better the outcome. The cornerstone of treatment is immunosuppression with steroids. Some patients require intravenous immunoglobulin G (IVIG) or plasmapheresis, and in refractory cases, cyclophosphamide is used.

Slip Clip after successful microsurgery of a blister aneurysm: Should bypass always be the first option?

Blood Blister-like aneurysms are intracranial non-saccular aneurysms with higher rupture risk due to its fragile wall. Diagnosis is performed in the acute phase of a subarachnoid hemorrhage. There are several treatment options based on reconstructive or deconstructive techniques. This paper aims to discuss the limitations of microsurgery clipping for a ruptured blister aneurysm. We report on a case of a female patient presented with a Fisher III subarachnoid hemorrhage. Cerebral angiography revealed an internal carotid artery blister aneurysm. Initially microsurgery clipping was successfully performed. However, after a few days the patient presented new subarachnoid hemorrhage. The new cerebral angiography showed growth of the previously clipped aneurysm, with displacement of the clip from the position adjacent to the artery. High-flow bypass was performed obtaining definitive treatment. This is a definitive approach for blister aneurysms. If microsurgery clipping is chosen, a strict follow-up is required due to the dynamic nature of this lesion and the chance of re-bleeding even after successfully clipping.

Observational study of patients with occipital condyle fracture at a brazilian referral trauma center.

OBJECTIVE: to evaluate the clinical-epidemiological characteristics, treatment, and evolution of patients with occipital condyle fracture (OCF) at one of the largest referral trauma centers in Latin America. METHODS: this was a retrospective observational study of OCF identified from trauma cases admitted between December 2011 and December 2019 by the neurosurgery team at a Type 3 trauma center. RESULTS: a total of twenty-eight occipital condyle fractures were identified in twenty-six patients. The incidence was less than 0.2% per year and more common in male patients (4:1 ratio) involved in traffic accidents. The mean age was 42.08 years. Anderson and Montesano type II and Tuli type 1 were the most frequent (67.9% and 89.3%, respectively) and no case presented C0-C1-C2 instability. All patients were treated with a cervical collar for 3 to 6 months. About 65% of the patients exhibited good progression (Glasgow Outcome Scale equal to 4), and the severity of traumatic brain injury was the main determinant for negative outcomes. CONCLUSION: the findings of this study are in accordance with available literature data. The use of external stabilization with a cervical collar is reinforced for the treatment of stable lesions, even when these are bilateral. Assessment of the patients' follow-up results in the studied sample may contribute with useful information for the treatment of occipital condyle fractures.

Extending the stroke treatment window beyond DAWN in patients with very slow progressor type collaterals: How far can we go?

Five trials published in 2015 showed the benefit of endovascular thrombectomy (ET) in patients with stroke and large vessel occlusion, extending the treatment window has become an obsession of all physicians. In 2018, the DAWN and DEFUSE-3 trials showed that, with careful selection of patients, the procedure could be carried out up to 24 hours after symptom onset with good outcomes. In addition, there have been cases where the DAWN criteria were met, and treatment occurred >24 hours after symptom onset. We present the case of a 68-year-old female whose groin puncture occurred 52 hours after the time last known well (TLKW), after neurological worsening of the initial situation, with a large mismatch ratio observed on magnetic resonance imaging, achieving TICI (the Thrombolysis in Cerebral Infarction scale) grade 3 recanalization. Five days after the procedure, the patient was discharged with NIHSS (National Institutes of Health Stroke Scale) score of 3. Some types of collateral circulation (slow progressors and "turtle" progressors, our term for very slow progressors) can extend the treatment window beyond 24 hours of the TLKW but can lead to a hyperperfusion-like syndrome immediately after the ET. Further studies are needed to evaluate the reproducibility of this hypothetical syndrome.

Changes in management strategies after spontaneous migration of a retained intraorbital metallic foreign body.

PURPOSE: Because of the controversial biologic tolerance and management, retained intraorbital metallic foreign body (RIMFb) poses a formidable challenge to surgeons. Besides location of the foreign body, indications for surgical management include neurologic injury, mechanical restriction of the eye movement, and development of local infection or draining fistula. The authors describe an unusual case of spontaneous migration of a RIMFb. METHODS: A 26-year-old man had a gunshot injury on the left orbit. The patient was initially managed conservatively because of the posterior position of the bullet fragment. Thereafter, because of the clinical impairments and anterior migration of projectile, surgical treatment was considered. RESULTS: Spontaneous anterior migration has led to mechanical disturbances and inflammatory complications that comprise explicit surgical indications for removal. The patient underwent surgery with complete relief of symptoms. We suppose that extrinsic ocular muscles might play a role in shifting large RIMFb over time, leading to change in the management strategies. CONCLUSIONS: Spontaneous migration of RIMFb is a rare clinical situation that can lead to pain, local deformity, as well as changes in the management strategies of the affected patients even in the late phase of follow-up.