Successful treatment of a neonate with vein of Galen aneurysmal malformation

We present a follow-up study on a 5-year-old boy who was born with vein of Galen aneurysmal malformation (VGAM) accompanied by extreme cardiovascular distress. He was successfully treated by the staged endovascular technique during the neonatal stage. Postoperative angiographic study confirmed complete occlusion of the VGAM and the establishment of normal brain perfusion. At 5 years of age, the child is growing normally without neurological deficits or developmental retardation. We suggest that the restoration of normal brain perfusion in neonates with VGAM can cause normal brain development.     

Vein of Galen aneurysmal malformation. Different clinical expressiveness. Three case reports

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebro-vascular disorder in neonates. It is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. It may appear in the neonatal period or afterwards. Three cases of patients affected by VGAM with different clinical expression are presented. Two of them were treated successfully with endovascular embolization. It was not possible to provide the same treatment to the third patient for an intractable congestive heart failure already existing when VGAM was diagnosed.     

Rare basal vein fistula with dilated vein of Galen

The authors present a rare case of arteriovenous fistula (AVF) of the basal vein of Rosenthal draining into a dilated vein of Galen managed by transarterial endovascular embolization. A male infant born at full term following a normal pregnancy and delivery with congestive heart failure, on investigation with MR imaging and MR angiography was found to have a basal vein of Rosenthal fistula with a dilated vein of Galen. His congestive heart failure was treated medically, and the AVF was managed electively at 10 months of age with successful transarterial endovascular embolization. The authors discuss the embryological aspects related to the pathological entity and the various clinical presentations, investigations, and management options. Management is primarily endovascular embolization; microsurgery is performed for a few selected cases, and radiosurgery has a limited role in older patients. Endovascular embolization is a safe and effective way to manage this malformation, with an excellent outcome if the AVF is eliminated by proper embolization at the fistulous point.     

Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation

BACKGROUND: Neonatal presentation of vein of Galen aneurysmal malformations (VGAMs) with intractable cardiac failure is considered a poor prognostic sign. Interventional neuroradiology with embolisation has been shown to control cardiac failure, but there is a perception that neurological outcome in survivors is poor. OBJECTIVE: To determine if aggressive intensive care and anaesthetic management of cardiac failure before urgent embolisation can influence morbidity and mortality. PATIENTS: Nine newborns (four boys, five girls) were diagnosed with symptomatic vein of Galen malformations in the neonatal period during the period 1996-2001. Eight developed intractable high output cardiac failure requiring initial endovascular treatment in the first week of life. RESULTS: The immediate outcome after a series of endovascular procedures was control of cardiac failure and normal neurological function in six (66%) patients, one death from intractable cardiac failure in the neonatal period, and two late deaths with severe hypoxic-ischaemic neurological injury (33% mortality). Clinical review at 6 months to 4 years of age showed five infants with no evidence of neurological abnormality or cardiac failure and one child with mild developmental delay (11%). CONCLUSIONS: Aggressive medical treatment of cardiac failure and early neurointervention combined with modern neuroanaesthetic care results in good survival rates with low morbidity even in cases of high risk VGAM presenting in the immediate perinatal period with cardiac failure. Systemic arterial vasodilators improve outcome in neonates with cardiac failure secondary to VGAM. Excessive beta adrenergic stimulation induced by conventional inotropic agents may exacerbate systemic hypoperfusion.     

A vein of Galen aneurysm with an abnormal drain system: MRI findings

The case of premature girl with a vein of Galen aneurysm is reported. The lesion was detected by ultrasonography. MRI provided complete anatomical information, obviating the need for initial angiography for preoperative evaluation of the malformation. Venous drainage through a persistent embryonic falcine sinus and associated atresia of the straight sinus could be identified, while venous thrombosis and parenchymal damage could be excluded.     

Arteriovenous malformations of the vein of Galen

Vein of Galen arteriovenous malformations encompass a diverse group of vascular anomalies that share a common feature: dilatation of the vein of Galen. Although clinical presentations are highly variable, depending on age of presentation, signs and symptoms overlap between age groups. The association of heart failure and cranial bruit constitutes the most striking clinical presentation in neonates. However, less severe and fulminant modes of presentation are frequent in older infants, children, and adults. Treatment approaches consist of symptomatic treatment of heart failure on the one hand and of surgery or endovascular treatment on the other. The results of the latter have improved in recent years, opening up a broad spectrum of new possibilities. We present the case of an asymptomatic 15-day-old neonate who presented an arteriovenous malformation of the vein of Galen and who was treated with endovascular occlusion of the arterial afferents. An excellent result was obtained with no evidence of neurological abnormalities.     

颈内静脉搭桥Rex手术治疗小儿肝外门静脉梗阻的初步探讨

目的 探讨颈内静脉搭桥Rex手术(肠系膜上静脉-门静脉左支吻合术)治疗小儿肝外门静脉梗阻的手术效果.方法 2014年10月至2015年6月共收治肝外门静脉梗阻8例,行颈内静脉搭桥Rex手术治疗,随访1～9个月,评价手术效果.手术方法:取上腹正中纵行切口,首先切断肝圆韧带,并沿其向Rex窝分离,解剖出门静脉左支矢状部3 cm;沿结肠中血管找到肠系膜上静脉,分离出3 cm;取左侧颈部横切口,切取颈内静脉7～9 cm.将颈内静脉在门静脉左支和肠系膜上静脉之间搭桥行两个端侧吻合.结果 8例中男5例,女3例,平均年龄4岁(1岁4个月至7岁),表现为消化道出血6例,脾功能亢进6例,出血合并脾亢4例.术前肝功能及凝血大致正常,胃镜检查7例食管胃底静脉曲张,1例无曲张.超声及CT均诊断门静脉海绵样变性;行经肝静脉逆行门静脉系统造影,显示门静脉左支矢状部存在7例,未显示1例(术中证实为闭锁).7例手术探查Rex窝内有血流,完成Rex手术,1例门静脉左支闭锁,改行Warren手术.吻合后经肠系膜上静脉造影显示搭桥血管通畅,肝内门静脉系统显影,肝外侧枝曲张血管明显减少或未显示.吻合后门静脉压力明显降低,吻合前PVP为(27.00±3.74)mmHg,吻合后PVP为(19.00±2.71)mmHg,吻合前后PVP压力差为(8.00±3.42)mmHg,P=0.001.术后随访,全部患儿无再次出血,6例术前脾亢患儿5例得到缓解,1例未缓解;3例于术后半年复查胃镜,食管胃底静脉曲张由重度转为轻-中度;超声检查门静脉血流均通畅,无血栓及狭窄,术后门静脉左支直径为(7.10±2.47)mm(3.3～10.5 mm).结论 Rex手术对于小儿门静脉梗阻具有良好治理效果,在降低门静脉压力的同时,恢复了肝脏血流,是EHPVO根治性手术方式,应该作为治疗小儿肝前性门静脉高压的首选手术方法.     

Congenital absence of the portal vein: Case report and MR demonstration

Congenital absence of the portal vein (CAPV) is a rare anomaly that results from aberrant venous development in early embryonic life. The intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava (IVC), the left renal vein, or the left hepatic vein. We describe a case where prenatal ultrasonography demonstrated an unusual C-shaped vessel between the umbilical vein and a dilated IVC, and failed to show a portal vein. Ultrasonography and magnetic resonance imaging and magnetic resonance angiography at 4 months of age again found no portal vein. The superior mesenteric vein drained into the left renal vein.     

Ultrasound detection of congenital Arteriovenous aneurysm of the great cerebral vein of Galen

Using combined echoencephalography and Doppler flow determination the diagnosis Arteriovenous aneurysm of the great cerebral vein of Galen could be made in two infants. Without vascular surgery one patient died, the other recovered completely. CT scanning confirmed the diagnosis. Invasive methods such as cerebral angiography were avoided.Case histories, neuropathological findings, ultrasound method and results are presented. Pathogenesis, clinical signs, treatment and prognosis are discussed. With the ultrasound method presented, the nature and location of the vascular anomaly were demonstrable, thus additional higher risk diagnostic methods could be avoided or planned more purposefully.     

Vein of Galen aneurysmal malformation associated with an endoglin gene mutation

A child with vein of Galen aneurysmal malformation (VGAM) presented with cardiac failure in the neonatal period. The family history revealed his mother to have hereditary hemorrhagic telangiectasia. The child underwent an endoglin genetic analysis after the newborn period, which eventually demonstrated an endoglin mutation. The pathogenesis of VGAM is currently unknown. The findings of this case suggest that an endoglin mutation might be linked with VGAM.     

Spontaneous resolution of marked dilatation of cerebral duro-venous system in newborn presenting with fetal hydrops

A newborn with antenatal diagnosis of fetal hydrops at 36 wk of gestation, presented with congestive heart failure (CHF) and generalized edema. Computed tomographic angiography showed marked dilatation of cerebral duro-venous system including vein of Galen (VOG), straight sinus, torcula and transverse sinus without evidence of arteriovenous fistulae at the vein of Galen. Dilatation of duro-venous system resolved with concomitant improvement in biventricular function and CHF with decongestive therapy. Such entity should be differentiated from more serious conditions like VOG malformation and venous sinus thrombosis.     

Rapid normalization of marked dilatation of the cerebral duro-venous system in a newborn infant mimicking a great vein of Galen varix

A newborn infant with a marked dilatation of the cerebral duro-venous system is presented. The patient was diagnosed as having a vein of Galen aneurysmal varix by a cranial ultrasound examination immediately following delivery. Computed tomographic angiography on the following day, however, showed a marked dilatation of the cerebral duro-venous system, including the great vein of Galen, superior sagittal sinus, torcular herophili and transverse sinuses. There were no arteriovenous fistulas at the vein of Galen. Dilatation of the duro-venous system and concomitant heart failure subsided rapidly after intravenous administration of indomethacin for the treatment of the patent ductus arteriosus on the fourth day of life. Dilatation of the duro-venous system in a newborn infant should be differentiated from any form of vein of Galen aneurysm.     

Calcification of an aneurysm of the vein of Galen

A 3.5 month old boy was diagnosed as having hydrocephalus secondary to an aneurysm of the vein of Galen. Cerebral angiography 2 1/2 years later showed the aneurysm to be completely occluded and CT demonstrated it to be thrombosed and completely calcified. Complete calcification of an aneurysm of the vein of Galen has only been described on two previous occasions and never in a child this young.     

婴幼儿肺静脉异位引流术后肺静脉狭窄的再干预治疗

目的分析婴幼儿肺静脉异位引流术后肺静脉狭窄(pulmonary vein stenosis,PVS)的原因,总结再干预治疗的经验。方法回顾性分析2012年5月至2019年12月郑州大学附属儿童医院胸心外科收治的13例肺静脉异位引流术后PVS患儿的临床资料。其中,男8例,女5例;年龄为(7.2±4.6)个月,范围在6~31个月;体重为(6.9±3.4)kg,范围在5.0~17.6 kg;完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)术后12例,部分性肺静脉异位引流(partial anomalous pulmonary venous connection,PAPVC)术后1例。13例患儿再干预治疗前均给予心脏超声及心脏CT血管造影检查。其中,吻合口处狭窄7例,单支肺静脉狭窄4例(肺静脉于左房开口处狭窄2例,肺静脉远端狭窄1例,肺静脉近端狭窄1例),双支肺静脉狭窄(均为左侧)2例。9例采用Sutureless技术,3例采用牛心包补片扩大,1例球囊扩张后效果差后行牛心包补片扩大。结果13例患儿均顺利完成手术,住院期间死亡2例。其中,1例经体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)辅助治疗3 d后因颅内出血死亡;1例因原有脑部疾病,家属拒绝使用ECMO辅助治疗,后放弃治疗。另11例患儿随访3~36个月,无延迟死亡。1例采用的Sutureless技术和1例采用中心包补片扩大患儿术后随访3年,肺静脉流速为1.6~1.9 m/s,现随访观察中。结论儿童PVS多见于肺静脉畸形术后,尤其是TAPVC术后,应早期再干预治疗。Sutureless技术能明显改善PVS的预后。     

Cardiac failure caused by arteriovenous malformation in the area of Galen's vein. Treatment by endovascular embolization

The authors report a case, in a 7-month-old girl, of cerebral arteriovenous malformation with a vein of Galen ectasia, complicated by a congestive heart failure and hydrocephalus. The clinical diagnosis was carried out by fontanelle auscultation and confirmed by: cerebral echography. D?ppler exploration, computerized tomography and cerebral angiography. A cerebral embolization permitted reduction of cardiac insufficiency and stabilisation of the hydrocephalus.     

Magnetic resonance demonstration in the newborn of generalized cerebral venous dilation with spontaneous resolution.

The authors present serial magnetic resonance (MR) images of an infant with cardiac failure and generalized cerebral venous dilation, which was initially misdiagnosed in the first week of life on cranial ultrasound as a vein of Galen malformation. At 3 months of age, repeat MR imaging demonstrated complete resolution of this marked cerebral venous distension with no evidence for cerebral injury. This case illustrates the value of MR in the identification of this disorder and its distinction from more serious conditions, such as vein of Galen malformation and venous sinus thrombosis. Complete resolution of the venous dilation and the lack of definable parenchymal injury suggest a good prognosis for this disorder.     

Developmental deep venous system anomaly associated with congenital malformation of the brain

We report a rare case of developmental deep venous system anomaly. The great vein of Galen and the straight sinus were absent. Both internal cerebral veins and the basal veins of Rosenthal drained into a large frontal interhemispheric falcine vein, which eventually drained into the superior sagittal sinus. The patient also had an associated neuronal migration anomaly.     

Diagnosis of an aneurysm of the great cerebral vein of Galen using duplex sonography

The combination of two-dimensional sonography of the skull and pulsed Doppler measurements (= Duplex-scan-technique) was used to detect an aneurysm of the vein of Galen in a three months old infant, who presented with clinical signs of congestive heart failure. Excellent correlation of sonography with computed tomography and intravenous digital subtraction angiography could be demonstrated. A short review of the pathological and clinical findings of this lesion is given. The value of Duplex-scanning in the diagnostic approach of arterio-venous malformations of the brain in comparison with other radiological methods is discussed.     

Arteriovenous malformation of the vein of Galen in three neonates: emphasis on associated early ischaemic brain damage

 Vein of Galen malformation is a rare intracranial disorder in newborns. In recent years the survival rate has improved due to improvement in endovascular treatment of this abnormality. We describe three neonates with a vein of Galen malformation for whom treatment was not attempted because of associated severe cerebral damage, of antenatal origin in two and of perinatal origin in the other. Autopsy was performed in two neonates. Periventricular leukomalacia was present in both cases, associated in one case with cortical infarction, gliosis and atrophy. Conclusion We recommend careful evaluation of asso ciated cerebral damage prior to attempted treatment of the vein of Galen malformation. Received: 30 November 1995 and in revised form: 10 June 1996 / Accepted 8 July 1996     

Aneurysmal dilatation of the portal vein

In the following study, a case involving an aneurysmal dilatation of the portal vein is reported. The 5-year-old boy had a 2 month history of intermittent abdominal pain. A segmental dilatation of the portal vein was noted in the sonographic examination. The portal vein aneurysm diagnosis was later confirmed by superior mesenteric angiography and computed tomography. In our review of the literature, less than 30 cases of portal venous aneurysm have been reported. To our knowledge, this is the youngest case with such an anomaly.