Vermian

INTRODUCTION AND IMPORTANCE: Rosette forming Glioneuronal tumours (RGNT) are rare WHO grade I tumours. They have been recognised as a sole entity in the WHO classification since 2007. They are typically described as having a favourable prognosis. Since their description as a distinct entity, there have been only four reports of malignant or anaplastic transformation of RGNT. We report a case of recurrent RGNT with new anaplastic histopathological features. CASE PRESENTATION: We present the case of a 48-year-old female who presented with a vermian region RGNT...

BACKGROUND: Hydrocephalus frequently occurs with midline posterior fossa cystic collections. The classification of this heterogeneous group of developmental anomalies, including Dandy-Walker malformation, persisting Blake's pouch, retrocerebellar arachnoid cysts, and mega cisterna magna, is subject of debate. The absence of diagnostic criteria is confusing regarding the ideal management of PFCC-related hydrocephalus. OBJECTIVE: To decipher the surgical strategy for the treatment of children with PFCC-related hydrocephalus through a retrospective analysis of the surgical outcome driven by their clinical and radiological presentation...

OBJECTIVE: Cerebellar mutism syndrome (CMS) is a well-known complication after posterior fossa tumor surgery in pediatric patients. We evaluated the incidence of CMS in our institute and analyzed its association with multiple risk factors, such as tumor entity, surgical approach, and hydrocephalus. METHODS: All pediatric patients who had undergone intra-axial tumor resection in the posterior fossa between January 2010 and March 2021 were included in the retrospective analysis...

Fetal MRI is an important tool for the prenatal diagnosis of brain malformations and is often requested after second-trimester ultrasonography reveals a possible abnormality. Despite the immature state of the fetal brain at this early stage, early suggestive signs of the presence of brain malformations can be recognized. To differentiate between the normal dynamics of the growing brain and the developing pathological conditions can be challenging and requires extensive knowledge of normal central nervous system developmental stages and their neuroradiological counterparts at those different stages...

OBJECTIVE: Central nervous system tumors are the most common solid neoplasm in children, 60%-70% occurring in the posterior fossa. Surgery is the mainstay of treatment but surgery in the pediatric population is associated with a high risk of perioperative complications. We aimed at analyzing the perioperative complications after posterior fossa surgery in a pediatric population and identifying the associated risk factors. METHODS: Retrospective study of all pediatric patients undergoing surgery for resection of a posterior fossa tumor between 1999 and 2019, at the University Hospital of Lausanne...

Chiari III malformation (CM-III) is the rarest anomaly among CMs1 . Treatment of choice is surgical repair2 , although poor outcome and postoperative mortality has been reported3 . Surgical timing is still debated4,5 . We present the case of a male infant with a prenatal diagnosis of encephalocele. Presentation was characterized by hemodynamic instability, horizontal nystagmus, and left shoulder dystocia due to caesarean section, with a 64x49x76 mm soft, fluctuant, and translucent suboccipital-cervical sac...

OBJECTIVE: The diagnosis of posterior fossa abnormalities (PFA) in the intrauterine period; and pregnancy outcomes are still controversial. PFA is generally referred to as maternal-fetal medicine specialists. The primary purpose of PFA diagnosis is to screen for other accompanying abnormalities, provide prognostic information to families, and discuss the termination option. MATERIAL AND METHODS: This retrospective study was conducted with patients diagnosed with PFA between January 2013 and September 2020 in the tertiary Perinatology Clinic...

OBJECTIVES: Pial arteriovenous fistulas (pAVFs) are direct connections between the pial artery and vein without an intervening nidus. We report a rare case of craniocervical junction (CCJ) pAVF causing medullary and spinal cord edema resulting from surgical removal of the varix with remnant shunt after coil embolization. CASE DESCRIPTION: A 16-year-old man presented with subarachnoid hemorrhage. Digital subtraction angiography revealed a CCJ pAVF with multiple fistulas at the 2 varices (varix A and varix B), which was fed by the bilateral lateral spinal arteries and anterior spinal artery (ASA), and drained into the median posterior vermian vein with varix (varix C) and anterior spinal vein (ASV)...

AIM: To compare the size of the corpus callosum (CC) and cerebellum on magnetic resonance imaging (MRI) brain scans conducted at term equivalent age (TEA) in extremely preterm infants who received systemic postnatal corticosteroids (PCS) to extremely preterm infants who did not receive systemic PCS and determine the dose-dependent effects on these outcomes. METHODS: Single-centre retrospective cohort study including extremely preterm infants (born < 26 weeks' gestation) who had MRI brain scans at TEA...

BACKGROUNDS: Dandy-Walker syndrome (DWS) is a group of brain malformations which occasionally accompanied by psychotic symptoms. The co-occurrence of DWS and epilepsy in children is quite rare. CASE DESCRIPTION: We reported a 14-year-old male who presented with a 8-month history of inconsistent upper limb tremor and accidental seizure. The MRI showed the typical alterations of DWS: cystic dilatation of the fourth ventricle, vermian hypoplasia, enlarged posterior fossa...

AIM: To find out the relative incidence and outcome of posterior fossa abnormality (PFA) in terms of survival at birth until 2 years of age. METHODS: We conducted a prospective study; all fetuses diagnosed with posterior fossa abnormality were followed-up. The outcome was observed with respect to survival, the presence of associated anomalies, the existence of developmental delay after a telephonic interview. RESULTS: Out of 2703 children with congenital anomalies, 921 (34...

BACKGROUND: Prenatal diagnoses of cystic malformations of the posterior fossa mainly encompass arachnoid cysts, Blake's pouch cysts and Dandy-Walker syndrome. To date, vermian cysts have not been reported prenatally. OBJECTIVES: To report a series of fetuses with a vermian cyst. MATERIALS AND METHODS: This was a single-center retrospective study conducted from 2012 to 2021. We included all fetuses presenting with a vermian cyst and excluded all other types of posterior fossa cyst...

"Pediatric acute-onset neuropsychiatric syndrome", or PANS, is a rare syndrome characterized by an acute onset of obsessive-compulsive disorder (OCD), and/or severely restricted food intake accompanied by a variety of neuropsychiatric symptoms. To our knowledge, this is the first case report of twin adolescents with COVID-19-associated PANS. Dizygotic twin sisters in late teens, with abrupt and acute onset of severely restrictive food intake, weight loss, OCD, anxiety with intermittent auditory and visual hallucinations, depression, attention deficit, and sleep disturbances, simultaneously accompanied by milder neurologic symptoms such as hand tremor, tinnitus, dizziness, headache, and weakness of proximal muscles, were applied to child and adolescent psychiatry clinic...

Cerebellar arteriovenous malformations (AVMs) are associated with higher risk of rupture compared with cerebral AVMs.1 Microsurgical resection of a ruptured AVM, measuring 3 cm in its largest dimension, within the cerebellar vermis and right parasagittal cerebellar lobe is demonstrated in Video 1. Cerebral angiography showed major supply from both superior cerebellar arteries and minor supply from a right anterior inferior cerebellar artery-posterior inferior cerebellar artery variant. Venous drainage was through a single ectatic vermian vein draining toward the torcula...

BACKGROUND AND PURPOSE: The traditionally described Dandy-Walker malformation comprises a range of cerebellar and posterior fossa abnormalities with variable clinical severity. We aimed to establish updated imaging criteria for Dandy-Walker malformation on the basis of cerebellar development. MATERIALS AND METHODS: In this multicenter study, retrospective MR imaging examinations from fetuses and children previously diagnosed with Dandy-Walker malformation or vermian hypoplasia were re-evaluated, using the choroid plexus/tela choroidea location and the fastigial recess shape to differentiate Dandy-Walker malformation from vermian hypoplasia...

Recent studies (Alaou-Ismaili et al. 2020; Kilic et al. Eur J Radiol 56:212-219, 2005) among experienced sub-specialized neurosurgeons described divergent perceptions of surgical risk for venous sacrifice in posterior fossa surgery. Three galenic veins stood out as controversial in venous risk assessment and underexplored in the literature: the internal occipital vein (IOV), the precentral cerebellar vein (PCV), and the superior vermian vein (SVV). We have conducted a narrative review based on a systematic literature search to analyze terminology and anatomic descriptions and to suggest a coherent synthesis of published data on these veins...

Pediatric posterior fossa arteriovenous malformations (AVMs) are rare entities that pose significant cumulative lifetime risk of rupture and require treatment. Microsurgical resection remains a good option for definitively treating posterior fossa AVMs in one setting. The drawback of endovascular embolization is the lower rates of nidus obliteration. Although stereotactic radiosurgery is a safe alternative, it takes several years to achieve the treatment goal all the while predisposing the patient to the risk of AVM rupture...

Neonatal epilepsy, cerebellar dysgenesis and facial dysmorphisms may be associated with de novo PACS2 missense pathogenic variants (EIEE 66) (OMIM #618067). Here, we report a toddler boy with neonatal-onset seizures, developmental delay with hypotonia, facial dysmorphisms and prominence of the cisterna magna, mild inferior vermian and cerebellar hypoplasia. A nextgeneration epilepsy gene panel revealed a known pathogenic PACS2 missense variant, p.Glu209Lys, that was inherited from his mildly affected mother...

The inositol 1,4,5-triphosphate receptor type 1 ( ITPR1 ) gene encodes an InsP3 -gated calcium channel that modulates intracellular Ca2+ release and is particularly expressed in cerebellar Purkinje cells. Pathogenic variants in the ITPR1 gene are associated with different types of autosomal dominant spinocerebellar ataxia: SCA15 (adult onset), SCA29 (early-onset), and Gillespie syndrome. Cerebellar atrophy/hypoplasia is invariably detected, but a recognizable neuroradiological pattern has not been identified yet...

Antenatal posterior fossa cystic lesions are intimidating due to overlapping imaging features of benign and severe malformations. Sonographic assessment of the posterior fossa with good resolution median sagittal and axial views, either primary or secondarily reconstructed, plays the lead role in antenatal evaluation, further enhanced when sequential assessments are added. We present 10 cases of fetal posterior fossa cystic lesions diagnosed in the first and second trimesters that were sequentially analyzed and followed up till delivery or termination...