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An 8-year-old boy presenting with left-angle paralysis, tremor in upper and lower extremities, and diplopia was diagnosed with hemorrhage from a mesencephalic cavernous hemangioma. He underwent hemangiomectomy through the occipital transtentorial approach 4 weeks post-hemorrhage, after which Holmes tremor (HT) markedly reduced. A year later, hemangioma has not recurred; he is now independent in his daily activities. Early intervention in the subacute stage allows for the complete removal of brainstem cavernomas (BSCs), with minimal risk of complications or sequelae. Proper timing and surgical approach for BSCs can prevent re-bleeding and improve HT after an initial hemorrhage, without any lasting negative consequences., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: We aimed to determine the surgical indications and postoperative outcomes among pediatric patients with lobar cerebral cavernous malformations (CCMs)., Methods: We retrospectively reviewed pediatric patients operated on for lobar CCM between March 2010 and August 2021. Indications for surgery included (1) intracranial hemorrhage, (2) symptomatic superficially located lesion, and (3) asymptomatic CCM in non-eloquent area in case of strong parental preferences. Patients presenting with seizures were assessed using Engel Epilepsy Surgery Outcome Scale., Results: Twenty-one patients were included. The predominant symptoms were seizures (57.1%), headaches (33.3%), and focal neurological deficits (23.8%). Patients were qualified for surgery due to symptomatic intracranial hemorrhage (47.6%), drug-resistant epilepsy (28.6%), and focal neurological deficits (9.5%). Three patients (14.3%) were asymptomatic. A gross total resection of CCM with the surrounding hemosiderin rim was achieved in all patients. The mean follow-up was 52 months. No patient experienced surgery-related complications. In all individuals with a preoperative first episode of seizures or focal neurological deficits, the symptoms subsided. All six patients with drug-resistant epilepsy improved to Engel classes I (67%) and II (33%)., Conclusion: Surgical removal of symptomatic lobar CCMs in properly selected candidates remains a safe option. Parental preferences may be considered a sole qualifying criterion for asymptomatic lobar CCM excision., (© 2024. The Author(s).)

Microbial seeding of a cerebral cavernous malformation is an extremely rare occurrence with only 3 cases reported in the literature thus far. Campylobacter fetus is an opportunistic pathogen that rarely causes neurological infection with only 3 cases of C. fetus cerebral abscesses and 38 cases of C. fetus meningitis reported in the literature. There have been no cases of cerebral cavernous malformation seeding by C. fetus reported to date. We report the first case of cerebral cavernous malformation seeding by C. fetus, a case occurring in a previously healthy 16-year-old female who presented with suspected left cerebellar cavernous malformation with subacute hemorrhage. She underwent a suboccipital craniectomy for the resection of the cavernous malformation with additional intraoperative findings suggestive of cerebral abscess. Following positive blood and CSF cultures and surgical pathology results, the patient was diagnosed with C. fetus meningoencephalitis with co-infected left cerebellar cavernous malformation. This is the fourth reported case of microbial seeding of a cerebral cavernous malformation, and to our knowledge, the first case of a C. fetus-infected cavernous malformation. Compared to previous reports, the clinical events of this case strongly support the presence of a preexisting lesion that was secondarily seeded versus de novo formation as a result of prior infection., (© 2023. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Cavernous malformations (CM) have long been considered congenital of central nervous system, while the mechanism of CMs detailed development process associated with genetic factors remains unclear. We reported an uncommon case which suffered spinal cord cavernous malformations. In this work, representative samples were obtained, and the sequenced results were described for the first time. A 9-year-old boy was found oblique shoulder with slightly weakness of left limbs; MRI indicated spinal cord cavernous malformations (CMs) located at the C4-C6 vertebral level. On genetic analysis, a shared mutation of PIK3CA (p.H1047R) in CMs and associated developmental venous anomalies (DVAs) was detected, with a different abundance (2% and 7%, respectively), and a somatic mutation of MAP3K3 (p.I441M) was detected in the CM tissue samples. This case provides better knowledge of the formation history and genetic triggers of the DVA-associated CMs. This evidence allows us to speculate the developmental history of the CM lesion: The DVA with PIK3CA mutation might be genetic precursor, and then the associated CM could be derived from terminal cell population of the DVA by acquiring a somatic mutation in MAP3K3., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Intracranial cavernous malformations (CMs) are rare vascular malformations of the central nervous system in children. Infantile patients, being a developmentally vulnerable age group, pose a special challenge for management of these lesions. We pooled data from infantile patients diagnosed at our institution and individual cases published in the literature to provide input towards therapeutic decision-making., Methods: A systematic search of PubMed, MEDLINE, Embase, and Scopus was performed in accordance with PRISMA guidelines to identify all reported cases of intracranial CMs in the literature for infantile patients aged ≤ 2 years. In addition, cases from our institution diagnosed between 2010 and 2020 were also included. Individual cases were pooled and analyzed for clinical presentation, natural history, and outcomes from conservative and surgical management., Results: A total of 36 cases were included, of which 32 were identified from the literature. Median age at presentation was 14 months (range: 2 days to 24 months) months; 53% (n = 19) were females. Most cavernomas (64%, 23/36) were supratentorial, while 30% (n = 11) were located in brainstem and 5.5% (n = 2) in the cerebellum. With the exception of one patient, all cases were reported to be symptomatic; seizures (n = 15/31, 48.3%) and motor deficits (n = 13/31, 42%) were the most common symptom modalities. A total of 13 patients were managed conservatively upon initial presentation. No symptomatic hemorrhages were observed during 26 total person-years of follow-up. A total of 77% (28/36) underwent surgery; either upfront (23/28, 82%) at initial presentation or following conservative management. Among 12 patients who had preoperative seizures, 11/12 (91.6%) achieved seizure freedom post-resection. Among 7 patients who presented with hemiparesis preoperatively, 5 (71%) demonstrated some improvement, while 1 remained unchanged, and another patient with a brainstem cavernous malformation had worsening of motor function postoperatively. Postoperative recurrence was noted in 3 cases (3/27, 11%)., Conclusion: Annual risk of repeat hemorrhage may be low for infantile patients with intracranial cavernous malformations; however, better follow-up rates and higher number of cases are needed to make a definitive assertion. Surgical resection may be associated with high rates of epilepsy cure and provide improvement in neurological function in a select number of cases., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Cavernous malformations (CMs) are either congenital or acquired vascular lesions comprised of sinusoid spaces filled with either blood or its breakdown products. They possess a relatively reduced risk of hemorrhage, yet placement within the posterior fossa and especially the brainstem heightens their likelihood to rupture, making them a likely cause of permanent and debilitating neurological deficit, as well as a veritable surgical challenge. Although the incidence of rupture varies with age among reported case series, it is undoubtable that the severity of this occurrence is the highest while the brain is as its most vulnerable period, i.e. during infancy., Case Presentations: We present two patients, both female, 6.5- and 5-months-old respectively, who presented with brainstem hemorrhage from CM. They suffered from a sudden onset of hemiparesis and were subjected to surgical removal of their lesions and resulting hematomas. Both patients were discharged in a favorable neurological status and are currently alive and in good health., Conclusion: Microsurgical treatment of brainstem CMs in infants is not only possible with minimal deficit, but also advisable if the lesions are symptomatic. Nevertheless, this requires substantial patience and experience to prevent significant loss of blood and injury to the structures of the posterior fossa. We argue that the safest method to prevent further damage from brainstem CM rebleed is to remove these lesions shortly after the initial hemorrhage.

Purpose: Pediatric intramedullary spinal cord cavernous malformation (ISCM) is a rare vascular disease with unclear natural history and long-term outcomes. We aim to determine the demographics, hemorrhagic risk, and long-term outcomes of this rare entity., Methods: A retrospective review of clinical data and treatment outcomes of pediatric patients treated with ISCM in our institution from 3/2000 to 3/2017 was conducted. In addition, we performed a systematic review of the literature on pediatric ISCM., Results: Eighteen consecutive pediatric patients were included, with an average age of 12.9 ± 4.7 years (range: 4-18 years) and 66.7% being male. Locations were equally distributed in cervical and thoracic segments, with mean extension of 1.3 ± 0.7 segments. Clinical manifestation included extremity weakness (n = 15, 83.3%), pain (n = 10, 55.6%), sensory disorders (n = 8, 44.4%), sphincter disturbance (n = 6, 33.3%), muscular atrophy (n = 3, 16.7%), and spinal deformity (n = 1, 5.6%). Most patients presented with acute symptoms (n = 11, 61.1%), and 7 (38.9%) of them had severe neurological deficits. The annual retrospective hemorrhagic risk was 7.7 per patient-year. Two patients received conservative management, with one improved neurologically and the other remained unchanged. Total resection was achieved in 12 (75%) of the 16 surgical cases, with 8 patients (50%) improved their clinical outcomes, 7 patients (43.8%) remained unchanged, and 1 (6.3%) worsened. During follow-up, one patient had relapse of ISCM., Conclusion: Pediatric ISCM appears to have higher hemorrhage risk than their adult counterparts, and they can benefit from surgery whether in the acute phase of neurological deterioration or after clinical recuperation.

In this case, we describe an evident hemorrhagic brainstem cavernous malformation successfully treated with a planned sequence of surgical evacuation of the hematoma followed by postoperative propranolol therapy. In contrast to common practice, the cavernoma itself was not resected. A nearly 3-year-old male presented with altered mental status, gait disturbance, and facial palsy. CT and MRI demonstrated a large acute pontine hematoma. A large nearby vein suggested cavernous malformation. He was initially treated conservatively but a repeat CT scan demonstrated further expansion of hematoma and he was taken emergently to the OR. Due to the sensitive location of the hematoma in the pons, we planned to evacuate the hematoma without resecting any of the presumed cavernoma. Instead, we planned to treat the cavernoma with propranolol. Postoperatively, the patient's condition improved and was still improving at hospital discharge 2 weeks later. Six-month follow-up MRI showed no cavernoma with only hemosiderin at the site of the evacuated hematoma. This is the first reported case of a hemorrhagic brainstem cavernous malformation treated with a planned sequence of hematoma evacuation followed by propranolol without an attempt to resect the cavernoma.

Background and Purpose: To test the hypothesis that the prevalence of cerebral cavernous malformation (CCM) associated with developmental venous anomalies (DVAs) increases with age, we studied the age-related prevalence of DVA-associated CCM among patients with DVAs., Materials and Methods: Patients with DVAs on contrast-enhanced MRI exams performed over a 2-year period were included in this study. A single neuroradiologist reviewed all imaging exams for the presence of CCMs. Baseline demographic data collected included age, gender, presence of CNS neoplasm, history of cranial radiation, and history of seizure. Patients were divided into age groups based on decade of life. Cochran-Armitage trend tests were performed to determine if increasing age was associated with CCM prevalence., Results: A total of 1689 patients with DVAs identified on contrast-enhanced MRI were included. Of these patients, 116 (6.9%) had a cavernous malformation associated with the DVA. There was a significant positive association between age and the prevalence of DVA-associated CCM (P = 0.002). The prevalence of DVA-associated CCM was 0.8% for the 0-10 age group, 1.6% for the 11-20 age group, 7.5% for the 21-30 age group, 9.5% for the 31-40 age group, 6.1% for the 41-50 age group, 6.3% for the 51-60 age group, 7.4% for the 61-70 age group, and 11.6% for the >70 age group (P < .0001)., Conclusions: Our study demonstrated an age-related increase in prevalence of DVA-associated cavernous malformations among patients with DVAs. These findings suggest that DVA-associated cavernous malformations are acquired lesions.

Purpose: While cavernous angioma (CVA) after cranial irradiation has been documented, its development after high-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) has not. We present a patient with desmoplastic/nodular medulloblastoma (DNMB) associated with anhidrotic ectodermal dysplasia (AED) who developed CVA 2 years after high-dose chemotherapy and PBSCT., Methods: A 1-year-old boy with ingravescent vomiting was admitted to our institute. He presented with a large head, a depressed nasal bridge, low-set ears, thick lips with peg-shaped teeth, hypohidrosis, sparse hair, thin atrophic skin, scaly dermatitis with frontal bossing, and a bulging anterior fontanel. Neuroradiological examination revealed multiple cerebellar masses with heterogeneous enhancement and speckled calcifications and severe obstructive hydrocephalus. The histological diagnosis of surgical specimens was DNMB, and he underwent postoperative multiple-drug chemotherapy with autologous PBSCT. The outcome was favorable and he did not undergo radiotherapy., Results: After 2 years, intracranial hemorrhage was detected at his regular radiological check-up and he again underwent surgery. The histological diagnosis was CVA., Conclusions: To our knowledge, this is the first report of AED-associated DNMB and CVA.

Purpose: Optic pathway cavernous malformations (CMs) are extremely rare, accounting for less than 1 % of all intracranial CMs. We report a case of optic pathway CM mimicking optic glioma because the initial magnetic resonance (MR) images did not disclose hemorrhagic findings such as popcorn-like lesion or hemosiderin ring., Methods: A 20-year-old woman presented with subacute left visual acuity loss and visual field defect and was referred to our hospital. Initial MR imaging findings were suggestive of optic glioma. Second MR imaging demonstrated hemorrhagic findings, but the hemorrhage was considered to be intratumoral hemorrhage from left optic pilocytic astrocytoma. She underwent radiochemotherapy and intravenous administration of corticosteroids but her symptoms deteriorated. Third and fourth MR imaging revealed enlargement of the hematoma. Therefore, the radiation therapy was interrupted at a delivered dose of 16.2 Gy, and craniotomy was performed to preserve residual right visual field. The lesion was totally removed and the histological diagnosis was CM. Her right visual field was preserved, but not improved., Results: The present case and previous cases suggest that optic pathway CMs sometimes do not initially manifest with signs of hemorrhage, so CMs should be included in the differential diagnosis of optic pathway lesions., Conclusions: Histological confirmation with preparation for total resection should be considered for rapidly progressive cases even if the neuroimaging findings are compatible with optic glioma.

Introduction: Cavernous angiomas (CA) are congenital intraparenchymal vascular malformations that contain sinusoidal spaces lined by a single-layer endothelium, separated by collagenous stroma with no intervening brain parenchyma. Despite the congenital origin of CA, they rarely present in the neonatal and prenatal period. In this paper, we present a case report of a neonatal suprasellar CA that presented with a bleed. We also present a literature review focusing on specific features of intracranial CA in the neonatal and fetal age groups., Case Report: A 27-day-old neonate presented with a left eye ptosis for 2 days, followed by a generalized seizure. A head computed tomography revealed a suprasellar hematoma with intraventricular and subarachnoid extension. Brain magnetic resonance imaging revealed hemorrhages of various ages. Magnetic resonance angiography did not reveal any vascular malformation. Surgical exploration of the suprasellar mass revealed a capsulated dense hematoma. Postoperatively, the neonate was weaned of artificial ventilation over a protracted period and remained hemiparetic with signs of third nerve palsy. Pathology revealed a CA. CA presenting as a suprasellar bleed with subarachnoid and intraventricular extension is very rare especially among neonates. To the best of our knowledge, 20 cases of CA have been reported in the neonatal and fetal period in the English literature. Neonatal CA in general and suprasellar location in particular are extremely rare lesions. Neonatal/fetal CA seems to present more aggressively and have a worse prognosis compared to those presenting at a later age.

Introduction: Giant cavernous malformations (GCMs) constitute an uncommon entity in the diagnostic armamentarium of the neurosurgeon. We report a 3-year-old boy with a GCM in the peri-trigonal region and review 13 other paediatric cases previously reported in literature., Case: A 3-year-old boy presented with right-sided hemiparesis and features of raised intracranial pressure of short duration. Computed tomography showed a large left peri-trigonal mass with a bleed. Magnetic resonance imaging showed associated perilesional edema and mass effect, rendering the lesion a tumefactive appearance. He underwent total excision of the lesion, with subsequent recovery of hemiparesis. Histopathology was reported as a cavernoma., Discussion: The clinico-radiological presentation of GCM is discussed based on a case report and relevant cases in literature. Good surgical outcome can be expected with total microsurgical excision., Conclusion: A giant cavernous malformation should be one of the differentials of a lesion with a tumefactive clinico-radiological presentation in children. There should be a high index of suspicion for such a diagnosis, especially when the lesion does not enhance with contrast. This is the third case report in paediatric literature describing a periventricular location of this unusual lesion.

Introduction: Less than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit., Case Material: A 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8-T11), intramedullary edema (T6-L1), and an ImC at T9-T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4-C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma., Conclusion: With adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.

Objective: Cavernous malformations (CMs) are vascular malformations affecting any part of the central nervous system. CMs in the pediatric age group are known to be different from those of adults both in origin and clinical characteristics. In this paper, we report our experience in managing CMs in pediatric patients., Materials and Methods: Between January 1994 and December 2006, 33 patients younger than 20 years of age with intracranial CMs were treated with microsurgery or radiosurgery. We retrospectively reviewed the presentation and treatment of these 33 patients (18 boys and 15 girls; average age 11.6 years)., Results: The most common symptom at presentation was seizure (19 children, 57.6%). The most common type of CM, classified on the basis of magnetic resonance imaging, was type II (18, 54.5%). The supratentorial compartment was the most frequent location (27, 71.8%), and only six CMs (18.2%) were observed in the infratentorial compartment. Microsurgery was performed on 25 patients (75.8%), and radiosurgery was performed on eight patients (24.2%). The overall post-treatment results were positive. Only two children (6.1%) had persistent presurgical neurological signs, although the treatment ameliorated them. No progression of the preoperative neurological signs or onset of new neurological deficits was seen in any of the patients. In our patient group, the microsurgical removal of CMs resulted in the prevention of recurrent hemorrhage and the control of seizure disorders in all cases., Conclusions: Our results suggest that pediatric patients with symptomatic CMs should be treated surgically because of the risk of recurrent hemorrhaging and the general benefits of CM removal.

We report two cases with extratemporal cavernous angioma (CA) and coexisting ipsilateral hippocampal sclerosis. Classically dual pathology is defined as the association of hippocampal sclerosis with an extrahippocampal lesion. Subtle changes in hippocampus might be overlooked in the presence of an unequivocal extrahippocampal abnormality. Seizure outcome after epilepsy surgery in cases with dual pathology is less favourable if only one of the lesions is removed. Dual pathology must always be considered in diagnostic imaging of patients with intractable epilepsy and CA.