The normal vascularization of the intradural filum terminale in man

Summary The arterial and venous blood-supply of the intradural filum terminale was studied microscopically in 18 fresh cadavers after removing the dorsolumbar spinal cord in one piece, with the roots and the filum in their dural sheath. The arteries were examined after manual injection of the artery of the lumbar enlargement, while study of the veins was made without injection since their bluish-black color made them easily identifiable. After gross examination, each specimen was fixed and then sectioned at 12 different levels from the medullary conus to the bottom of the dural sac for histologic study. The distribution of the vascularization the filum terminale appeared constant. A single artery, the artery of the filum, arises from the termination of the anterior spinal axis, either by trifurcation or from the proximal part of one of the 2 branches of the anastomotic ansa of the conus. The artery travels in front of the filum, with rapidly diminishing caliber; rarely, it can be followed into the sacral canal. The vein of the filum travels in front of that structure but behind the artery, as in the cord; its caliber is uniform but varies from subject to subject. It traverses the dura below and continuous with the anterior spinal vein above. No vessels were found on the dorsal aspect of the filum. While the artery of the filum is of a caliber proportional to that of the filum and appears to be a nutrient vessel, the vein has a caliber unrelated to that of the filum and appears rather as an intradural drainage route continuous with the anterior spinal vein. Several cases of disease of the filum terminale confirm this anatomic appearance and also show that, because of the existing hyperpressure in the vein of the filum, the posterior spinal vein also shares in the drainage of the latter and that entire system may function in both ascending and descending directions.

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La vascularisation normale du filum terminale intradural chez l'homme

Résumé La vascularisation artérielle et veineuse du filum terminale intradural a été étudiée sur 18 cadavres frais, sous microscope, après prélèvement en monobloc de la moelle épinière dorso-lombaire, des racines et du filum dans leur étui dural. L'examen des artères a été fait après injection manuelle de l'artère du renflement lombaire, tandis que l'étude des veines s'est faite sans injection compte tenu d'une coloration bleu-noir spontanée qui les rendent aisément identifiables. Après étude macroscopique, chaque pièce a été fixée, puis coupée à 12 niveaux différents depuis le cône médullaire jusqu'au fond du cul-de-sac dure-mérien, pour étude histologique. La distribution de la vascularisation du FT apparaît constante. Une artère unique, l'artère du FT, naît de la terminaison de l'axe spinal antérieur, soit par trifurcation, soit de la partie proximale d'une des 2 branches de l'anse anastomotique du cône. L'artère chemine devant le FT; son calibre diminue rapidement; rarement, elle a pu être suivie jusque dans le canal sacré. Une veine, la veine du FT, chemine en avant du FT mais en arrière de l'artère, comme au niveau médullaire. Son calibre est uniforme mais variable d'un sujet à l'autre. Elle traverse la dure-mère en bas; elle se continue avec la veine spinale antérieure en haut. Aucun vaisseau n'a été retrouvé à la face dorsale du FT. Si l'artère du FT a un calibre qui est proportionnel à celui du filum et apparaît comme un vaisseau nourricier, la veine a un calibre sans aucun rapport avec le volume de celui-ci et apparaît davantage comme une voie de drainage intradural en continuité avec la veine spinale antérieure. Quelques cas de pathologie du FT confirment cet aspect anatomique et montrent aussi qu'en raison de l'hyperpression veineuse régnant dans la veine du FT, la veine spinale postérieure participe également au drainage de celle-ci et que l'ensemble peut fonctionner dans les 2 sens ascendant ou descendant.

     

Noninvasive three-dimensional computed tomographic angiography in preoperative detection of intracranial arteriovenous malformations

Ｃｏｎｖｅｎｔｉｏｎａｌｉｎｔｒａ ａｒｔｅｒｉａｌｄｉｇｉｔａｌｓｕｂｔｒａｃｔｉｏｎａｎｇｉｏｇｒａｐｈｙ (ＤＳＡ)ｕｓｅｄｔｏｂｅｒｅｇａｒｄｅｄａｓｔｈｅｇｏｌｄｓｔａｎｄａｒｄｆｏｒｄｉａｇｎｏｓｉｓｏｆｉｎｔｒａｃｒａｎｉａｌａｒｔｅｒｉｏｖｅｎｏｕｓｍａｌｆｏｒｍａｔｉｏｎｓ(ＡＶＭｓ) Ｒｅｃｅｎｔｌｙ ,ｎｅｗｔｅｃｈｎｉｑｕｅｓｏｆｎｅｕｒｏｉｍａｇｉｎｇａｎｄｃｏｍｐｕｔｅｒａｓｓｉｓｔｅｄｔｈｒｅｅ ｄｉｍｅｎｓｉｏｎａｌｒｅｃｏｎｓｔｒｕｃｔｉｏｎｍａｋｅｉｔｐｏｓｓｉｂｌｅ…     

先天性心脏病心脏停跳和不停跳下心内直视手术的临床分析

目的　对比分析心脏停跳和不停跳心内直视手术治疗先天性心脏病心内畸形的疗效。方法　5 5 8例患者分别采用心脏停跳手术 2 31例 ,不停跳手术 32 7例。心脏不停跳心内直视手术仍常规建立体外循环 ,置左心房引流管 ,降温至 (32± 1)℃并维持 ,仅阻断上下腔静脉 ,不阻断主动脉 ,维持灌注压在 8kPa左右 ,心脏跳动下进行畸形矫正 ,方法同停跳下手术。结果　不停跳组早期死亡 7例 (死亡率 2 .14 % ) ,停跳组早期死亡 5例 (死亡率 2 .17% ) ,其余均痊愈出院 ,随访 3～ 2 3月 ,恢复良好。结论　心脏不停跳心内直视手术 ,是一种有效的心肌保护方法 ,并可简化操作 ,缩短体外循环时间     

Mosaic pathogenic variants in AKT3 cause capillary malformation and undergrowth

Capillary malformations are slow-flow vascular malformations that affect the microcirculation including capillaries and post capillary venules and can be associated with growth differences. Specifically, the association of capillary malformations with undergrowth is a vastly understudied vascular syndrome with few reports of genetic causes including PIK3CA, GNAQ, and GNA11. Recently, a somatic pathogenic variant in AKT3 was identified in one child with a cutaneous vascular syndrome similar to cutis marmorata telangiectatica congenita, undergrowth, and no neurodevelopmental features. Here, we present a male patient with a capillary malformation and undergrowth due to a somatic pathogenic variant in AKT3 to confirm this association. It is essential to consider that mosaic pathogenic variants in AKT3 can cause a wide spectrum of disease. There is a need for future studies focusing on capillary malformations with undergrowth to understand the underlying mechanism.     

Reversible tonsillar prolapse in vein of Galen aneurysmal malformations: report of eight cases and pathophysiological hypothesis

We report eight cases of vein of Galen aneurysmal malformation (VGAM) assoicated with a Chiari type I malformation. In four cases magnetic resonance imaging (MRI) or computed tomography performed in the neonatal period did not demonstrate the posterior fossa anomaly, which appeared on later scans. In the other cases the MRI was performed in infancy and the anomaly was already present. We compared the venous phases of the posterior fossa angiograms and the MRI in these patients. In all eight cases, the angiograms showed a reflux in the cerebellar veins, via the petrous vein, associated with a uni-or bilateral stenosis or thrombosis of the distal posterior dural sinuses. Furthermore, in two cases the posterior fossa returned to normal on MRI following endovascular treatment, while in three cases the herniation of the cerebellar tonsils decreased after the embolization. Tonsillar prolapse becomes irreversible when the venous outlet is incapable of taking the flow even when the VGAM has been treated adequately. In eight additional cases of VGAM for which MRI and angiogram studies were available and in which stenosis or thrombosis of posterior dural sinuses was present without tonsillar prolapse, no reflux into the cerebellar veins was shown. We suggest that the posterior fossa hydrovenous congestion is a result of inadequate venous drainage and that the tonsillar descent is reversible if adequate venous drainage is reconstituted following therapeutic embolization of the fistula. Tonsillar prolapse is not a consequence of mass or raised intraventricular pressure. Our observation suggests that in some other conditions, the Chiari I malformations may be secondary to early hydrovenous dysfunction of the posterior fossa.     

Experimental study and clinical use of poly(vinyl acetate) emulsion as liquid embolisation material

A new material, an emulsion of poly(vinyl acetate) was experimentally developed and clinically used to overcome several disadvantages in currently used liquid embolisation materials. The emulsion microparticles, 0.3–0.7 m in size, possessed cationic charge on the surface and hence aggregated immediately on contact with fluids containing anions. This inert polymer has the advantage that it does not induce a deleterious reaction in living tissue. Moreover, its medium is water and it is not adhesive, like the cyanoacrylates. Several concentrations of emulsion were injected into the renal arteries of dogs. For the investigation of tissue reactions and the possibility of recanalisation, the emulsion was injected into rats both subcutaneously and into the renal arteries. The renal artery injections in dogs showed adequate radiopacity and consistent complete occlusion. The lower the concentration of the emulsion, the smaller the arteries which could be occluded. Even at very low concentrations, however, venous occlusion did not occur. Histological study of the embolised rat kidney revealed no detectable damage in the vessel wall and no recanalisation for up to 6 months. The subcutaneously injected PVAc emulsion elicited mononuclear cell infiltration and gradual centripetal fibrosis, without any deleterious effect on the surrounding tissue. A cerebral arteriovenous malformation (AVM) was embolised using the material. Histology of the resected nidus showed findings similar to those in the animal experiments.     

Cerebral arteriovenous malformations in children

The treatment of cerebral arteriovenous malformations (AVM) or vascular anomalies are challenging neurosurgical procedures for an anaesthetist. Large AVMs are uncommon in children. Only 18% of AVMs become symptomatic before the age of 15 yr. This series reviews the experience at this institution during the period of 1982 to 1992. The symptoms at the time of presentation are varied and include haemorrhage (50%), seizures and hydrocephalus (36%) or congestive cardiac failure (18%). Symptoms of congestive heart failure predominate in the new-born whilst neurological symptoms, such as stroke, seizures or hydrocephalus occur more commonly in infants and older children. Approximately one third of AVMs in childhood present acutely. Radiological investigations, e.g., CT scan, MRI and cerebral angiography are essential to identify the precise location of the lesion. Therapeutic intervention in the acute presentation may involve craniotomy for evacuation of haematoma and treatment of increased intracranial pressure (ICP). Control of seizures and congestive heart failure may take priority and allow time to plan the elective procedures of embolization and surgical excision of the AVM. Operative intervention is hazardous and peroperative complications can be expected in more than 50% of patients. The morbidity and mortality associated with cerebral AVM are high, especially in infants who present in the neonatal period with congestive cardiac failure. The overall mortality in this series was 20%. Children presenting with intracranial arteriovenous malformations require a multidisciplinary approach. The successful management of anaesthesia either for embolization or surgical resection necessitates an understanding of the disciplines of paediatric and neuroanaesthesia. Special care and specific attention to detail may contribute to reduce the high morbidity and mortality encountered in these compromised children.     

Single forebrain ventricle without prosencephaly: agenesis of the corpus callosum with dehiscent fornices

Coalescence of the cerebral ventricles with formation of a single forebrain ventricle is described in an unusual case of agenesis of the corpus callosum with dehiscent fornices and severe hydrocephalus. The cerebral hemispheres were fully cleaved. The detached fornices were widely separated from the thalami. The membrana tectoria was retroverted over the midbrain and cerebellum, where it joined the fornices and merged with a curved membranous ependymal dome which, at a great distance, circumvented the thalami as it extended toward the anterior commissure. Other anomalies included arhinencephaly, multiple subependymal heterotopias, and Dandy-Walker malformation. Similar malformations have been described as interhemispheric cysts, as (holo)prosencephaly, or as midline telencephalic dysgenesis. The basic features of prosencephaly and agenesis of the corpus callosum are reviewed and compared to the present case.     

静脉内支撑治疗布-加综合征

目的：评估静脉内支撑治疗布－加（Ｂｕｄｄ－Ｃｈｉａｒｉ）综合征的应用价值。方法：①下腔静脉完全梗阻者,根据阻塞长度置入可扩张性内支撑架;②门静脉高压者,在下腔静脉球囊扩张的同时行颈内静脉肝内门体分流术（ＴＩＰＳＳ）治疗后再置入内支撑架;③肝静脉狭窄或闭塞者,扩张肝静脉远端后再植入内支撑架。结果：所有患者均获得操作成功。手术后临床症状明显改善,下腔静脉压差和门静脉压力显著下降。随访８例下腔静脉内支撑和肝内门体分流道通畅良好。结论：静脉内支撑治疗布－加综合征疗效良好