Posterior Fossa Tumours in the First Year of Life: A Two-Centre Retrospective Study.

Posterior fossa tumours (PFTs) in infants are very rare, and information on these tumours is scarce in the literature. This retrospective study reports their pathological characteristics and describes surgical aspects and treatment outcomes. A two-centre cohort of infants with PFTs treated from 2007 to 2018 was retrospectively reviewed. Patient characteristics, clinical, and treatment data were reviewed. Survival curves for progression-free survival (PFS) and overall survival (OS) were generated. Thirty-three infants were retrieved. There were 11 low grade and 22 high-grade tumours. The most common presenting symptom was intracranial hypertension. Fifteen children out of thirty-three progressed. Five-year PFS was significantly lower in children with high-grade tumours (38.3%) than those with low-grade tumours (69.3%), p = 0.030. High-grade pathology was the only predictor of progression (HR 3.7, 95% CI 1.1-13.31), p = 0.045. Fourteen children with high-grade tumours died, with a 5-year OS of 55.25%. PFTs in children below one year of age still represent a unique challenge. Infants with high-grade tumours display the worst outcomes and the lowest survival, indicating that more effective strategies are needed.

Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case.

Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision. Authors of this report describe a 4-year-old boy from a developing country who was referred to their hospital with an ulcerated bulging lesion in the midline/right parietooccipital region, extending to the right laterocervical and parotid regions, resulting in significant craniofacial deformation. Magnetic resonance imaging of the brain revealed a highly enhancing tumor with intracranial and extracranial development extending mainly at the level of the right parietooccipital region, with lytic and hypertrophic alterations of the skull. The patient was managed with neoadjuvant and adjuvant chemotherapy and radically resective surgery on metastatic lymph nodes and the primary tumor of the skull. Scheduled radiotherapy was not performed, according to the parents' wishes. The patient returned to his native country where the lesion recurred, and he ultimately died approximately 10 months after the end of the treatment. The literature indicates that tumor removal alone has been the treatment of choice in most isolated cases, but in cases of highly advanced tumor with involvement of the skull and cervical lymph nodes, it is preferable to proceed with preoperative chemotherapy with the aim of reducing the tumor volume, allowing better technical conditions for complete surgical removal, and decreasing the risk of local recurrence or metastasis.

Initial experience with endoscopic ultrasonic aspirator in purely neuroendoscopic removal of intraventricular tumors.

OBJECTIVE Neuroendoscopic removal of intraventricular tumors is difficult and time consuming because of the lack of an effective decompression system that can be used through the working channel of the endoscope. The authors report on the utilization of an endoscopic ultrasonic aspirator in the resection of intraventricular tumors. METHODS Twelve pediatric patients (10 male, 2 female), ages 1-15 years old, underwent surgery via a purely endoscopic approach using a Gaab rigid endoscope and endoscopic ultrasonic aspirator. Two patients presented with intraventricular metastases from high-grade tumors (medulloblastoma, atypical teratoid rhabdoid tumor), 2 with subependymal giant cell astrocytomas (associated with tuberous sclerosis), 2 with low-grade intraparaventricular tumors, 4 with suprasellar tumors (2 craniopharyngiomas and 2 optic pathway gliomas), and 2 with pineal tumors (1 immature teratoma, 1 pineal anlage tumor). Hydrocephalus was present in 5 cases. In all patients, the endoscopic trajectory and ventricular access were guided by electromagnetic neuronavigation. Nine patients underwent surgery via a precoronal bur hole while supine. In 2 cases, surgery was performed through a frontal bur hole at the level of the hairline. One patient underwent surgery via a posterior parietal approach to the trigone while in a lateral position. The endoscopic technique consisted of visualization of the tumor, ventricular washing to dilate the ventricles and to control bleeding, obtaining a tumor specimen with biopsy forceps, and ultrasonic aspiration of the tumor. Bleeding was controlled with irrigation, monopolar coagulation, and a thulium laser. RESULTS In 7 cases, the resection was total or near total (more than 90% of lesion removed). In 5 cases, the resection was partial. Histological evaluation of the collected material (withdrawn using biopsy forceps and aspirated with an ultrasonic aspirator) was diagnostic in all cases. The duration of surgery ranged from 30 to 120 minutes. One case was complicated by subdural hygroma requiring a subduro-peritoneal shunt implant. CONCLUSIONS In this preliminary series, endoscopic ultrasonic aspiration proved to be a safe and reliable method for achieving extensive decompression or complete removal in the management of intra- and/or paraventricular lesions in pediatric patients.

Childhood acute basilar artery thrombosis successfully treated with mechanical thrombectomy using stent retrievers: case report and review of the literature.

INTRODUCTION: Acute basilar artery occlusion (ABAO) is an infrequent but potentially fatal cause of strokes in both adults and children, and it is usually due to vertebral artery dissection (VAD). VAD has been found to be usually a consequence of traumatic vertebral artery injury. ABAO usually presents with symptoms of acute ischemic stroke (AIS) of the posterior circulation or transient ischemic attack (TIA). It may lead to death or long-term disability if not promptly recanalized. Basilar artery recanalization in children can be achieved safely and with excellent clinical outcome using endovascular thrombectomy with the new generation self-expanding and retrievable stents. CLINICAL PRESENTATION: We report the case of a 23-month old baby that came to the emergency room of our hospital for progressive impairment of consciousness associated with widespread stiffness and plaintive cry, appeared after accidental fall from stroller. An emergency brain CT scan was obtained showing multiple infarction lesions in the brainstem and left cerebellum suggestive of acute stroke in posterior circulation territories. An MR scan with angiography and diffusion-weighted sequences confirmed the multiple infarction lesions and demonstrated poor representation of the flow signal at the V3 segment of the left vertebral artery and absent representation of the flow signal at the distal segment of the basilar artery suggestive of acute thrombotic occlusion. The patient was immediately referred to interventional neuroradiology unit, and digital subtraction angiography showed complete basilar artery occlusion and left vertebral artery dissection at extracranial V2-V3 segment. The patient underwent intra-arterial thrombectomy using stent retrievers and occlusion of the V2-V3 segment of the left vertebral artery. The patient survived and long-term outcome was excellent. CONCLUSIONS: To our knowledge, only nine cases of ABAO in children treated with intra-arterial thrombectomy have been previously reported in the literature. In only three cases, the Solitaire stent was applied. Our case is the first case of basilar artery occlusion treated with Solitaire stent, in a child under 24 months. The reports that are available so far indicate that basilar artery recanalization in children can be achieved safely and with excellent clinical outcome using endovascular thrombectomy with the new generation self-expanding and retrievable stents.

Intracranial aneurysms and optic glioma--an unusual combination: a case report.

BACKGROUND: The coexistence of glial high grade tumors (glioblastoma, anaplastic astrocytoma) and cerebral aneurysms is common but the association with optic glioma is rare. The treatment of these associated lesions is problematic. CASE PRESENTATION: A 36-year-old white woman presented to our institution with recurrent attacks of headache. Her preoperative radiological studies revealed a lesion of her left optic nerve with extension to the optic chiasma, an aneurysmatic dilatation of the left carotid bifurcation, a second aneurysm on the left of her middle cerebral artery, and a third one on her right anterior cerebral artery in the A2 tract. A left pterional approach was used to remove the tumor and clip the three aneurysmatic dilatations in a single stage. CONCLUSIONS: We report this unusual case of optic glioma associated to multiples aneurysms and review the pertinent literature. An adequate knowledge of this association is mandatory to plan the correct approach to avoid complications.

Posterior fossa tumors in infants and neonates.

INTRODUCTION: Management of posterior fossa tumors in infants and neonates is challenging. The characteristics of the young babies make surgery very difficult, sometimes precluding a safe complete removal. METHODS: A review of the literature was undertaken to examine the incidence, histology, surgical aspects, and prognosis of posterior fossa tumors in the first year of life. Therapeutical strategies of the most frequent tumor types are also discussed in detail. RESULTS: Histology is dominated by tumors with aggressive behavior, such as medulloblastomas, atypical teratoid/rhabdoid tumors, and anaplastic ependymomas. The most important surgical considerations in small children are the small circulating blood volume; the poor thermoregulation; and incomplete maturation of the brain, of the skull, and of the soft tissue. Treatment toxicity is inversely related to the age of the patients. Radiation therapy is usually considered as contraindicated in young children, with few exceptions. Proton therapy is a promising tool, but access to this kind of treatment is still limited. The therapeutic limitations of irradiation render resection of this tumor and adjuvant chemotherapy often the only therapeutic strategy in many cases. CONCLUSIONS: The overall prognosis remains dismal because of the prevalent aggressive histologies, the surgical challenges, and the limitations of adjuvant treatment. Nevertheless, the impressive improvements in anesthesiology and surgical techniques allow, in the vast majority of the cases, complete removal of the lesions with minor sequelae in high-volume referral pediatric centers.

Grisel Syndrome Following Adenoidectomy: Surgical Management in a Case with Delayed Diagnosis.

BACKGROUND: Grisel syndrome is a nontraumatic rotatory subluxation of the atlantoaxial joint, following nasopharyngeal inflammation or ear, nose, and throat (ENT) procedures. The syndrome should be suspected in cases of persistent neck pain and stiffness, especially after ENT surgical procedures. The primary treatment of early detected Grisel syndrome is conservative. If conservative treatment fails to achieve a stable reduction or it is followed by neurologic symptoms, arthrodesis of the first and second cervical vertebrae is indicated. We report the case of a 9-year-old boy who developed Grisel syndrome after adenoidectomy and was treated with C1-C3 internal fixation and fusion. CASE DESCRIPTION: A 9-year-old boy was referred to our hospital with a 3-month history of painful torticollis, which appeared 4 days after adenoidectomy. The patient underwent a neuroimaging study that documented the presence of atlantoaxial rotatory subluxation. The patient underwent C1-C3 internal fixation and fusion, using lateral masses and laminar and pars interarticularis screws. On the third postoperative day he was mobilized with a rigid collar. Postoperative computed tomography scans showed the resolution of rotational deformity and a solid fusion. CONCLUSION: Early treatment of Grisel syndrome is of utmost importance to avoid neurologic complications and surgical intervention. In a patient with torticollis following ENT procedures, Grisel syndrome should be always suspected. In case of failure of conservative treatment or in case of delayed diagnosis, rigid C1-C2 or C1-C2-C3 fixation is a straightforward and valid surgical technique, even in children, because it provides immediate spinal stability in all planes at the atlantoaxial complex, avoiding the need for prolonged rigid external bracing.