Neural Crest Cell Failure as Embryogenesis for Fusiform Aneurysm of the Anterior Communicating Artery: Case Report and Review of the Literature.

BACKGROUND: Pure fusiform aneurysms of the anterior communicating artery (AcomA) are rare. We report a unique case of a patient with an AComA fusiform aneurysm in the setting of several unusual cranial neurocristopathies, including a hypoplastic internal carotid artery (ICA), persistent craniopharyngeal canal, transsphenoidal encephalocele, and ectopic, duplicated pituitary gland. We also review the literature on cranial base neurocristopathies and AComA fusiform aneurysms. CASE DESCRIPTION: This 46-year-old patient had a history of short stature, osteoporosis, obesity, cleft lip, decreased libido, congenital left eye blindness, headaches, and chronic nasal congestion. Magnetic resonance imaging revealed a 25 × 25 × 33 mm heterogenous soft tissue mass with an ectopic pituitary gland extending transsphenoidally and a duplicated pituitary stalk. A hormone panel revealed undetectable insulin-like growth factor 1 and growth hormone, central hypogonadism, and elevated prolactin. Before presentation, computed tomography angiography (CTA) had revealed a congenitally hypoplastic right ICA and 4.7 × 10.7 mm fusiform aneurysm of the AComA. Digital subtraction angiography confirmed stable morphology after 9 years. Nonoperative management of aneurysm and cephalocele was elected, with repeat CTA in 1 year. CONCLUSIONS: This case provides evidence that inherent arterial wall defects can contribute to fusiform aneurysm formation in the AComA. We propose that small AComA fusiform aneurysms without sclerotic or symptomatic features can be safely observed by describing the longest reported conservative management for this type of aneurysm. A high degree of suspicion for cerebrovascular anomalies should be maintained in patients who present with cranial neurocristopathy.

Concurrent basilar artery double fenestration with aneurysm and vertebral artery dissection: case report and literature review of rare cerebrovascular abnormalities.

Many disorders can cause aneurysm and/or dissection of the cerebral arteries, including fibromuscular dysplasia (FMD), connective tissue disorders, cerebral vasculitis, infection, and vascular malformations. Arterial fenestration is a rare congenital finding that can also cause aneurysms, and can rarely dissect and bleed. Treatment of aneurysm and dissection with subarachnoid hemorrhage can be very complicated, and requires case-by-case analysis of the risks and benefits of antithrombotic therapy. To the authors' knowledge, no case of double fenestration of the basilar artery has been reported. This report presents a case of concurring vertebral artery dissection and double fenestration of the basilar artery with aneurysm. The fenestration and FMD are considered possible main contributing causes of this presentation. A literature review of cerebrovascular fenestration and FMD is provided and the relationship between the 2 is considered. Lastly, the use of antithrombotic therapy in the setting of subarachnoid hemorrhage, dissection, and stent placement is discussed.

Heart failure in the new born; vein of Galen aneurysmal malformation.

We narrate the case of a term baby who presented at 30 h of age in congestive cardiac failure due to vein of Galen aneurysmal malformation. A successful interventional radiology embolisation of the feeding vessels to the aneurysm was performed on day 5. Twenty hours following the procedure, the baby developed significant intraventricular haemorrhage with extension into the brain parenchyma; care was reoriented on day 7 of life after discussion with the parents. Vein of Galen aneurysmal malformation is a rare congenital vascular malformation. It usually manifests in the newborn period with high-output cardiac failure. The death rate is higher in those who present early in the neonatal period. The management of this condition requires a multidisciplinary approach.

[Help, I have crooked fingers - or the solution to a series of medical phenomena]. / Hilfe, ich habe krumme Finger - oder die Lösung einer Reihe medizinischer Phänomene.

We describe the case of a 23-year old man, who is himself a student of human medicine and who performed, thanks to his knowledge gained during his studies, a presumptive diagnosis of the Ehlers-Danlos-syndrome on himself. This fact was previously underdiagnosed by several physicians, who were consulted due to the manifestation of symptoms.

Acute intermittent porphyria presenting with a subarachnoid haemorrhage.

A 47-year-old man presented with abdominal pain, neck stiffness, severe transient hypertension and unusually dark urine. Cerebrospinal fluid investigations and angiography confirmed the diagnosis of a subarachnoid haemorrhage. Porphyrin studies on the patient and his family demonstrated that the family has acute intermittent porphyria. This is the second case report of an acute hepatic porphyria presenting with a subarachnoid haemorrhage. Acute transient hypertension during the attack of porphyria caused the rupture of an intracranial arterial aneurysm.

Prenatal ultrasonographic diagnosis of vein of Galen aneurysms--report of two cases.

Aneurysms of the vein of Galen (AVG) represent less than 1% of all intracranial arteriovenous malformations. Two cases of prenatal diagnosis made by color Doppler ultrasonography at 32 weeks of gestation are reported. Both cases presented with antenatal mild cardiomegaly and both developed severe cardiac failure in the neonatal period. Embolization was unsuccessful and both infants died. These cases highlight the need for a careful evaluation of the time and mode of delivery; embolization must be performed after a fully informed decision.

Segmental unfused basilar artery with kissing aneurysms: report of three cases and literature review.

Basilar artery "fenestration" is the result of a failed fusion of the bilateral longitudinal neural arteries and can be associated with a saccular aneurysm, which typically arises at the proximal juncture of the unfused segment. "Kissing" aneurysms at this site, i.e. two aneurysms arising from the proximal junction of the unfused segment of the basilar artery pointing anteriorly and posteriorly are reported to be exceedingly rare. We present three patients with this rare condition, all of them being treated by endovascular techniques.

Vein of Galen aneurysm in an adult: case report.

Vein of Galen aneurysm is a rare pathology, representing less than 1% of intracranial vascular malformations. We report on a 65-year-old man who experienced a generalized tonic-clonic seizure. Brain imaging showed a large calcified expanding mass in the pineal region, confirming the diagnosis of a vein of Galen aneurysm. Because of the spontaneous thrombosis of the malformation, there was no need for microsurgical or endovascular treatment and he has been regularly followed since that.

Vein of Galen aneurysm in an adult: case report / Aneurisma da veia de Galeno em adulto: relato de caso

Vein of Galen aneurysm is a rare pathology, representing less than 1 percent of intracranial vascular malformations. We report on a 65 years-old man who experienced a generalized tonic-clonic seizure. Brain imaging showed a large calcified expanding mass in the pineal region, confirming the diagnosis of a vein of Galen aneurysm. Because of the spontaneous thrombosis of the malformation, there was no need for microsurgical or endovascular treatment and he is been regularly followed since that.

Aneurisma da veia de Galeno é patologia rara, representando menos de 1 por cento das malformações vasculares intracranianas. Apresentamos o caso de um homem de 65 anos que teve episódio de crise convulsiva tônico-clônica generalizada. Exames de imagem evidenciaram grande processo expansivo calcificado na região pineal, confirmando o diagnóstico de aneurisma trombosado de veia de Galeno. Devido à trombose espontânea da malformação, foi excluída a possibilidade de tratamento endovascular bem como microcirúrgico, mantendo-se o acompanhamento clínico.

Confirmation of communication between deep venous drainage and the vein of galen after treatment of a vein of Galen aneurysmal malformation in an infant presenting with severe pulmonary hypertension.

Vein of Galen aneurysmal malformations (VGAM) are characterized by multiple arteriovenous connections draining into a markedly enlarged median draining vein. This ectatic vein is not the vein of Galen, but its embryonic precursor, the median prosencephalic vein of Markowski. During normal development, the posterior portion of the median prosencephalic vein persists as the vein of Galen, while its anterior portion regresses in parallel with the formation of the internal cerebral veins (ICV). It has been traditionally thought that, in children with a VGAM, the deep venous system does not connect to and, a fortiori, does not drain into the ectatic median prosencephalic vein/vein of Galen. This report describes a case of successfully treated VGAM in which the drainage of an ICV into the vein of Galen was only demonstrated by follow-up MR imaging and venography. The potential implications of this finding for the management of VGAMs are discussed.

Neonatal cavernous carotid artery aneurysm: case report.

Neonatal intracranial aneurysms are rare. The authors report the case of a 4-week-old girl who presented with left-eye ptosis and proptosis. Computerized tomography scanning and magnetic resonance imaging demonstrated a mass involving the left cavernous sinus and middle cranial fossa. Cerebral angiography revealed a large complex left cavernous carotid artery (CA) aneurysm. The patient underwent endovascular treatment in which detachable coils and N-butyl cyanoacrylate glue were used to achieve complete obliteration of the aneurysm. To the authors' knowledge, this is the first reported neonatal intracranial aneurysm originating from the cavernous CA and treated endovascularly. The authors review the literature on neonatal intracranial aneurysms.

Anesthetic management of a neonate with vein of Galen aneurysmal malformations and severe pulmonary hypertension.

Vein of Galen aneurysmal malformations (VGAMs) are rare congenital vascular malformations and excessive arteriovenous shunt causes intractable congestive high-output heart failure in the neonate. We report a case of successful staged transcatheter embolizations for a neonate with congestive heart failure and pulmonary hypertension (PH). Heart failure was dramatically relieved as the staged procedure progressed. Although transcatheter embolizations is essential for the treatment, inhaled nitric oxide (iNO) was helpful as a bridge treatment to reduce right-to-left shunt before the initial emergency embolization. Endovascular embolization is a less invasive therapy than open cranial surgery and allows hemodynamic stability. Perioperative iNO can be used to manage PH in VGAMs.

Ruptured cerebral aneurysm in the early stage of life--a congenital origin?

We report the case of a large fusiform aneurysm of the anterior cerebral artery in a 3-month-old girl presenting with generalized seizures and complicated by a cardiorespiratory arrest needing resuscitation. The native cerebral CT scan revealed a diffuse subarachnoid hemorrhage (Fischer III grade); CT angiography showed an aneurysm of the distal A1 segment of the anterior cerebral artery (ACA). Successful treatment was achieved through surgical trapping of the aneurysm and sacrifice of the distal A1 segment. A histological study of the aneurysmal wall revealed the absence of elastic fibers in an otherwise fibromuscular media and showed no signs of previous hemorrhage. Intracranial aneurysms of the ACA are very rare in the early stage of life and their pathogenesis is not clear. This case is of interest as the location and shape of the aneurysm, the absence of relevant familial and medical history, and the histopathological findings raise the hypothesis of a congenital origin. It further describes the value of CT angiography for studying aneurysms in the very young.

[Frequency of vascular variations and anomalies accompanying intracranial aneurysms]. / Intrakranyal anevrizmalara eslik eden vasküler varyasyon ve anomalilerin sikligi.

PURPOSE: Investigation of the sites of intracranial aneurysms and incidence of associated congenital variations or anomalies. MATERIALS AND METHODS: One hundred and ninety cerebral angiography examinations that were positive for aneurysm were evaluated retrospectively. Fourteen cases with vasospasm were excluded and the remaining 176 patients were assessed for the location of the aneurysm and co-incidental vascular variations and/or anomalies. There were two observers in the study, one of whom was an experienced vascular radiologist. RESULTS: The most frequent locations of aneurysms were the supraclinoid internal carotid artery (32%, n=74), anterior communicating artery (30%, n=68), and middle cerebral artery bifurcation (23%, n=52). Twenty-eight patients (17%) had multiple aneurysms. Ninety-one (52%) patients had a vascular anomaly or variation. Hypoplasia or agenesis of A1 segment of the anterior cerebral artery was found in 48 patients, an azygous anterior cerebral artery in 1, a frontoorbital artery in 1, a duplicated middle cerebral artery in 1, early bifurcation of the middle cerebral artery in 9, persistent fetal origin of the posterior cerebral artery in 26, fenestration of P1 segment of posterior cerebral artery in 1, agenesis of P1 segment of posterior cerebral artery in 1, fenestration of vertebral artery in 1, double trunk visualization of superior cerebellar artery in 2 patients. CONCLUSION: Due to an increased hemodynamic stress, congenital anomalies of the intracranial arteries predispose to the formation of saccular aneurysms. Nomalies such as A1 hypoplasia or agenesis, azygous anterior cerebral artery, accessory middle cerebral artery and persistent trigeminal artery are detected more frequently in patients with cerebral aneurysms compared to the normal population. The location of aneurysms and the frequency of arterial variations and anomalies in this study correlated well with data found in previous studies.

A newborn with cardiac failure secondary to a large vein of Galen malformation.

We report the case of a term newborn with vein of Galen aneurysm. No antenatal diagnosis was done. The infant was admitted secondary to cardiac failure, with cardiomegaly and pulmonary hypertension. Physical examination was remarkable for acrocyanosis, microcephaly, cranial bruit, bilateral neck swelling, coarse breath sounds, and a hyperdynamic precordium with continuous murmur. Head ultrasound demonstrated a large, midline venous structure. Computed tomographic scan confirmed vein of Galen malformation.