Cerebral echinococcosis: Case report and literature review.

Echinococcosis is a zoonosis caused by the larval form of the cestode Echinococcus granulosus. The cerebral affectation in the human is uncommon; only in 1-2% cases is observed encephalic involvement. This condition occurs mainly in the pediatric population and it is characterized by the presence of single and unilocular cysts. We report a case of a 29-year-old patient who came to the emergency department with a disabling headache of several weeks of evolution. In the brain imaging study a mass of unknown origin was visualized, finally it turned out to be a bilobed hydatid cyst. Regarding this case, the clinic-epidemiological, diagnostics and treatments aspects of cerebral hydatid disease will be reviewed.

Cerebral echinococcosis: Case report and literature review. / Hidatidosis cerebral: caso clínico y revisión de la literatura.

Echinococcosis is a zoonosis caused by the larval form of the cestode Echinococcus granulosus. The cerebral affectation in the human is uncommon; only in 1-2% cases is observed encephalic involvement. This condition occurs mainly in the pediatric population and it is characterized by the presence of single and unilocular cysts. We report a case of a 29-year-old patient who came to the emergency department with a disabling headache of several weeks of evolution. In the brain imaging study a mass of unknown origin was visualized, finally it turned out to be a bilobed hydatid cyst. Regarding this case, the clinic-epidemiological, diagnostics and treatments aspects of cerebral hydatid disease will be reviewed.

Superior Cerebellar Artery Aneurysms Causing Facial Pain: A Comprehensive Review.

BACKGROUND: Trigeminal neuralgia caused by superior cerebellar artery aneurysms (TGN-SCAAs) is a rare event without previous analysis. OBJECTIVE: To describe the features of TGN-SCAA based on 8 cases (7 from literature +1 illustrative case). METHODS: All cases were thoroughly studied with gathering of their epidemiological, radiological, clinical, therapeutic, and outcome data. RESULTS: The mean age at diagnosis was 61 yr. Gender distribution showed a female predominance (M: F = 2:6). Side distribution had a left dominance (75%). The aneurysms mean size was 15.4 mm (range: 5-27). All 5 proximal SCAAs (SCA-Basilar junction) presented a lateral-posterior projection, while all 3 distal SCAAs (s2 segment) had variable projections but constant direct trigeminal nerve (TN) contact. No hemorrhage occurred. TGN was the clinical onset in all 8 cases. The most frequent pain distribution was V1-2-3 (n = 3), followed by V1-2 (n = 1) and V1 alone (n = 1). Proximal SCAAs caused TGN through direct TN compression (n = 1), third nerve compression (n = 1), cavernous sinus compression (n = 1), or a combination thereof (n = 2). However, all distal SCAAs caused TGN by direct TN compression (n = 3). Two different treatment options were used: clipping (n = 4) and coiling (n = 4). The post-treatment Barrow Neurological Institute score for pain control was I in all cases (100%). The mRS score was 0 in 75% of cases. CONCLUSION: TGN-SCAAs are infrequent lesions, characterized by large size, variable TGN mechanisms depending on their anatomic location, and mostly affecting the first and second trigeminal divisions. Both SCAA clipping and coiling were used equally, providing good neurological and pain relief results.

Surgical Clipping of a Petrosal Tentorial Dural Arteriovenous Fistula (Lawton's Type 5).

Objectives To demonstrate the surgical clipping of a lateral petrosal tentorial dural arteriovenous fistula (DAVF), located in the cerebellopontine angle (CPA), through a retrosigmoid approach. Method A previously healthy 49-year-old man presented a sudden episode of headache, photophobia, and dizziness. Due to the persistence of his symptoms despite proper analgesic treatment, he sought medical attention. The initial computed tomography (CT) scan showed a hyperdense lesion in the left CPA. Magnetic resonance imaging (MRI) demonstrated the vascular nature of the lesion, corresponding to an engorged superior petrosal vein (SPV) and Rosenthal's vein. The preoperative angiography showed a lateral tentorial DAVF (Lawton's type 5), fed by multiple transpetrous branches coming off the external carotid artery, and draining into the SPV. A standard retrosigmoid approach was planned for clipping and exclusion of the DAVF. Results Through a left retrosigmoid craniotomy the DAVF was approached, along with the different neurovascular structures of the CPA. The DAVF originated at the tentorial petrosal junction. The fistulous vein was closely attached to the trigeminal nerve and the anterior inferior cerebellar artery (AICA). The fistulous vein was dissected and clipped close to its base at the lateral tentorium, achieving complete occlusion of the DAVF. The patient fully recovered after surgery with neither relapse of his symptoms nor postoperative complications. Conclusion The retrosigmoid craniotomy is the best surgical approach for lateral tentorial DAVFs, as it provides a direct way to the fistula origin and permits a successful clipping of the draining vein. The link to the video can be found at: https://youtu.be/Fj3uqrTPX5c .

Retrosigmoid Approach for Trapping and Removal of a Distal Dissecting Superior Cerebellar Artery Aneurysm in a Child.

Objectives To demonstrate the feasibility of the retrosigmoid craniotomy for surgical management of vascular lesions located in the cerebellopontine angle (CPA). Method A previously healthy 2-year-old boy presented a sudden episode of torticollis to the left while sleeping. This episode was selflimited but it occurred two more times in a 6-day span. Torticollis worsened in the upright position, caused unsteady gait and refusal to walk from the child. The preoperative magnetic resonance imaging (MRI) showed the presence of a round, heterogenous vascular lesion in the left CPA. The lesion clearly enhanced after contrast administration. The preoperative angiography demonstrated the absence of left anterior inferior cerebellar artery anterior inferior cerebellar artery (AICA), being the left superior cerebellar artery (SCA) the supplier of the left lateral cerebellum. A blurred blush on the distal left SCA was compatible with a fusiform aneurysm. A standard retrosigmoid approach was planned for trapping and removal of the aneurysm. Results Through a left retrosigmoid craniotomy the aneurysm was approached, along with the different neurovascular structures of the CPA. The aneurysm leaned on the VII, VIII nerves complex and the superior petrosal vein, while tightly attached to the lateral cerebellum. Both proximal and distal SCA segments to the aneurysm were dissected, clipped, and divided for a complete trapping. Finally, the aneurysm was completely detached and removed in a whole piece. The patient fully recovered after surgery with no relapse of his symptoms. Conclusion The retrosigmoid craniotomy is a versatile approach that permits wide exposure of all CPA structures and adequate removal of distal aneurysms located in those cerebellar arteries supplying the lateral cerebellum. The link to the video can be found at: https://youtu.be/oEVfy4goFYM .

Arteriovenous Malformations of the Temporalis Muscle: A Comprehensive Review.

Arteriovenous malformations of the temporalis muscle (TM-AVMs) are rare lesions commonly considered as a variant of scalp AVMs. A study was designed in order to analyze TM-AVMs features and to confirm them as a certain entity different from scalp AVMs. Seven TM-AVMs cases (5 from literature plus 2 from our database) were studied. Clinical, epidemiological, radiological, and therapeutic data were analyzed and compared to common scalp AVMs features. All TM-AVMs presented as large soft tumor-like masses on the temporal region (mean diameter: 6 cm). Pain was referred in half of cases, while overlying cutaneous stigmas were described only once. No masticatory malfunction was reported. TM-AVMs tend to appear in young patients (mean: 29 yr), commonly as longstanding and slow-evolving lesions (mean evolution time before diagnosis: 5 yr). All cases displayed a diffuse slow-flow blush on angiogram. All TM-AVMs were completely located within the TM, with no involvement of scalp or bone. The pathological analysis of all cases demonstrated the presence of striate muscular bundles intermingled with fairly mature arterial and venous vessels. No clearly distorted vessel was found within the TM-AVMs. Surgery was performed for all cases with complete removal. Preoperative embolization was done in 4 cases to reduce blood loss during surgery. No clinical/radiological recurrence was reported. Opposite to TM-AVMs, scalp AVMs commonly present as high-flow lesions with no evident tumor mass, mainly located in the subcutaneous tissue. TM-AVMs present clearly distinct features compared to scalp AVMs, for which they should be considered as a certain pathological entity and not as a variant of scalp AVMs.

Posterior communicating artery aneurysms causing facial pain: A comprehensive review.

Posterior communicating aneurysms are commonly associated with oculomotor nerve palsy. However, trigeminal nerve involvement among these aneurysms is a rare event with no previous analysis. In order to describe the main features of posterior communicating aneurysms causing trigeminal neuralgia (TGN-PComAAs) eight cases (six from the literature plus two more illustrative cases) were included in the present series. All cases were thoroughly studied in order to collect their epidemiological, radiological, clinical, therapeutic, pathogenic, and outcome data. The mean age at diagnosis was 57 years. Gender distribution showed a female predominance (M-to-F ratio 3:5), while side distribution showed a slight left dominance (62%). The aneurysms mean size was 24.7mm (range: 7-40mm). In 7 cases (87.5%) the PComAA was reported to project posteriorly, associated with cavernous sinus extension/compression in two of them and posterior fossa extension in three. No case presented with hemorrhage. Facial pain was the clinical onset in all eight cases, which was reported as an atypical TGN (continuous with exacerbating fits) in 87.5%. Trigger points were absent in all cases but one (87.5%). The most frequent anatomical distribution of the pain was V2 alone (n=3), followed by V1-2 (n=2) and V1-2-3 (n=2). Three different mechanisms for trigeminal neuralgia were observed, namely, cavernous sinus compression (n=4), direct trigeminal root compression (n=3), and oculomotor nerve compression (n=1). Three different treatment options were performed: surgery (n=6), trigeminal thermo-rhizotomy (n=1) and medical management (n=1). According to the Barrow Neurological Institute (BNI) score the pain control after main treatment was I (no pain with no medication) in 7 cases (87.5%). The average modified Rankin scale (mRS) score was 1. In sum, TGN-PComAAs are infrequent lesions characterized by large size and posterior-medial-inferior projection. They mainly cause atypical TGN, most commonly involving the first and second trigeminal distributions (V1-V2). Surgical aneurysmal clipping has been the most frequent treatment option for these aneurysms, providing good neurological results in a vast majority and complete trigeminal pain relief in all cases.

Bilateral ethmoidal dural arteriovenous fistula: unexpected surgical diagnosis.

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies.

Management of neonatal hydrocephalus: feasibility of use and safety of two programmable (Sophy and Polaris) valves.

BACKGROUND: Neonates represent a unique group of pediatric patients with special peculiarities. Hydrocephalus valves have not always been designed to meet the requirements of these small children. Few series have addressed the problem of cerebrospinal fluid shunting in newborn babies. OBJECTIVES: We aimed (1) to evaluate the feasibility of the use of two programmable valves (Sophy and Polaris) in hydrocephalic neonates and (2) to ascertain complications and safety issues arising from their use. MATERIALS AND METHODS: We performed a prospective study of 100 consecutive preterm and term babies (<2 months of age) given a programmable valve. Valves' settings were readjusted at different pressure levels as required. Outcomes were obtained from the records of our Outpatient Clinic. RESULTS: The study group was formed by 60 term and 40 preterm infants (average weight 2,440 g, mean age of 25 days). Mean follow-up was 55 months. Only one fifth deaths was shunt-related. In 70 babies, no complications occurred, and hydrocephalus was successfully controlled. Proximal catheter obstruction presented in 20% and infection in 5% of cases. Several external adjustments of the valves apparently avoided several surgical shunt revisions. CONCLUSIONS: (1) Both programmable valves (Sophy and Polaris) can be safely used for treatment of neonatal hydrocephalus, introducing some technical modifications. (2) Both valves are comparable to other shunts with regard to indications, performance, and safety. (3) The possibility of modifying their working pressure seems to constitute their main advantage. Prevention of late overdrainage syndromes with these valves needs a longer follow-up.

Severe constipation: an under-appreciated cause of VP shunt malfunction: a case-based update.

BACKGROUND: Increased intra-abdominal pressure has been reported to result in raised intracranial pressure in a variety of conditions such as obesity and pregnancy, and it also constitutes an infrequent cause of ventriculoperitoneal (VP) shunt malfunction. Patients with neurological deficits, as those with myelomeningocele or cerebral palsy, are prone to developing a neurogenic bowel and to suffer chronic constipation. Although previously recognized, VP shunt failure attributed to constipation has only recently been described. We briefly review the etiopathogenesis, diagnosis and management of severe constipation leading to VP shunt malfunction. Our aim is to draw the attention of pediatric neurosurgeons towards severe constipation as a possible cause of VP shunt failure thus avoiding unnecessary surgical valve revisions, to which children with hydrocephalus are often submitted to. ILLUSTRATIVE CASES: We report two children that developed transient VP shunt failure because of intense constipation that caused exacerbation of previously shunted hydrocephalus. One of the patients constitutes the first description of this complication associated with an anteriorly placed anus and the other with intestinal paresis after ileostomy. Conservative treatment aimed at alleviating the increased intra-abdominal pressure resulted in complete resolution of the children's manifestations of VP shunt failure, without having to resort to surgical revision of the VP shunt.

Cauda equina-penetrating injury in a child. Case report.

The authors report the case of a 10-year-old boy who sustained an injury to the cauda equina as a result of the accidental penetration of a wooden pencil into the spinal canal. After neuroimaging evaluation to exclude visceral and vascular lesions, the foreign body was removed and the wound was repaired. This is the first report of a cauda equina injury caused by a pencil.

Shunt-related craniocerebral disproportion: treatment with cranial vault expanding procedures.

Two patients with intracranial arachnoid cysts, one with myelomeningocele-hydrocephalus and the other with a subdural fluid collection, were given a cerebrospinal (CSF) extracranial shunt. All four patients developed features of CSF overdrainage following shunting and were treated by cranial vault expanding procedures. Before undergoing decompressive craniotomy, the patients were treated by a variety of procedures, including changing of obstructed ventricular catheters (n=4), insertion or upgrading of programmable valves (n=3), and foramen magnum decompression (n=1). Clinical manifestations of these four patients were attributed to craniocerebral disproportion caused by chronic and progressive skull changes due to dampening of the CSF pulse pressure, which is necessary for maintaining normal cranial growth. On the basis of our previous experience with expanding craniotomies in cases of minimal forms of craniosynostosis, we treated these patients with bilateral parietal craniotomies, with satisfactory results. In conclusion, biparietal decompressive craniotomy constitutes a useful and safe procedure for relieving the clinical manifestations of some CSF overdrainage syndromes, especially in cases with slit-ventricle syndrome and craniocerebral disproportion that prove to be refractory to simpler management procedures.

Hydrocephalus in intraspinal dermoids and dermal sinuses: the spectrum of an uncommon association in children.

BACKGROUND: Hydrocephalus associated with intraspinal tumors constitutes a well-documented occurrence. The accepted mechanisms for this association seem to be well established. On the contrary, hydrocephalus in the context of intraspinal dermoids has been rarely recognized and its pathogenetic mechanism appears to be different. PATIENTS AND METHODS: The authors report four pediatric cases of spinal dermoid tumors and dermal sinuses that developed hydrocephalus during the evolution of these congenital lesions of ectodermal origin. DISCUSSION: In two children, the mechanism leading to the development of hydrocephalus consisted of leptomeningeal inflammation due to bacterial meningitis or to spillage of dermoid cyst contents in the cerebrospinal fluid spaces. We hypothesize that ventricular dilatation in the other two might be the result of chemical meningitis occurring during intrauterine life. Two cases developed ventriculomegaly prior to the diagnosis of their spinal cord disease. CONCLUSIONS: The unusual evolution of these cases suggests that neuroimaging studies that include the spine should be performed in cases of childhood "unexplained hydrocephalus".

Rhabdoid meningioma: a new subtype of malignant meningioma also apt to occur in children.

CASE REPORT: The case of a 14-year-old girl who presented with a 2-week history of raised intracranial pressure is reported. A left frontal extra-axial tumor was totally removed, whose histopathologic diagnosis was rhabdoid meningioma (RM). DISCUSSION: Rhabdoid meningiomas constitute a special malignant phenotype of meningioma that has been recently included in the WHO classification of tumors of the nervous system. Usually, RMs affect middle-aged and elderly individuals. We report the fourth case of a RM occurring in a child to illustrate that the diagnosis of this tumor subtype, given its prognostic implications, must also be considered in pediatric patients.