口颌面部动静脉畸形诊治指南

动静脉畸形(arteriovenous malformations,AVMs)是由于胚胎期脉管系统发育异常而导致动、静脉直接吻合所形成的血管团块,内含不成熟的动脉和静脉,而且血管团块中没有毛细血管,动静脉之间存在不同程度的直接交通.在血管瘤与血管畸形中,动静脉畸形相对少见,仅占1.5%左右,口腔颌面部是最好发的部位,占...     

颌面部血管瘤合并动静脉畸形：病例报告及文献复习

血管瘤和血管畸形虽是婴幼儿的常见病,但血管瘤能否与动静脉畸形合并发生却一直存在争议。作者报告1例颌面部巨大、多发性血管瘤伴动静脉畸形的新生儿病例,经α-干扰素皮下注射治疗,血管瘤基本消退,而动静脉畸形依然存在。该病例报告证实,血管瘤可与动静脉畸形并存发生,且干扰素治疗重症血管瘤具有显著疗效。     

CT诊断颌骨动静脉畸形的价值探讨

目的：比较颌骨动脉畸形的CT和X线平片表现特征,评价CT对颌骨动静脉畸形的诊断价值。方法：具有完整CT和X线平片资料的颌骨动静脉畸形患者12例,其中上颌骨5例,下颌骨7例,拍摄同骨华特位、曲面体层片和下颌骨骨正、侧斜位片,CT采用横断位和冠状位两种扫描方式。结果：颌骨动静脉畸形在CT上均表现为骨质膨隆,骨髓腔间隙增大,骨皮质完整,其内骨小梁结构消失,其间未见明显分隔或分隔纤细,5例上颌骨动静脉畸形均匀以上颌牙曹局限性的单状扩张表现为主,7例下凳骨动静脉畸形有3种表现形式;局限单囊状、弥散囊状形均以上颌牙槽骨局限性的单囊状扩张表现为主,7例下颌骨动静脉畸形有3种表现形式：局限单囊状、弥散单囊状和多个小囊状。而X线平片的表现具有多样性,结论：CT有助于颌骨动静脉畸形的早期,及时诊断,为进一步行局部穿刺介入治疗提供导向和依据。     

下颌骨动静脉畸形3例临床治疗体会

目的总结下颌骨动静脉畸形的诊断和治疗经验。方法3例下颌骨动静脉畸形1例在瘤腔填塞后行部分下颌骨切除,2例在介入治疗后行部分颌骨切除或瘤腔刮治。结果3例下颌骨动静脉畸形的不同治疗方案获得疗效满意程度不同,术后随访12-36个月,均未发现口腔内渗血或出血。结论颈外动脉术中暂时阻断、介入栓塞和手术治疗相结合是治疗下颌骨动静脉畸形的有效方法。     

无水乙醇栓塞治疗颅面部动静脉畸形的现状与进展

无水乙醇栓塞治疗动静脉畸形已初步取得了令人满意的临床效果,并展现出良好的应用前景,从而为彻底治愈动静脉畸形提供了一条崭新的途径。但是,无水乙醇栓塞治疗动静脉畸形是一项极具挑战性的工作,如应用不当,会引起一系列并发症,并可导致严重后果。本文对无水乙醇栓塞治疗动静脉畸形的机制、技术要点、栓塞治疗后的病理生理变化、栓塞并发症及其防治作一综述。     

颅颌面动静脉畸形的分型和治疗

目的：研究颅颌面部动静脉畸形的临床分型、探索安全、有效的治疗措施。方法：通过多种检查方法,将38例动静脉畸形病例分为单纯型、同侧吻合型、复杂吻合型3种,并应用介入栓塞、结扎相应血管、手术切除等方法治疗。结果：病变全部彻底切除,术后随访6个月至7年,无1例复发。结论：根据不同的分型,处理相应的滋养血管,彻底切除病变组织是一种安全、有效的治疗动静脉畸形的方法。     

影像学技术在口腔颌面部动静脉畸形体积测量中的应用

颌面部动静脉畸形对患者会造成极大的危害,其主要治疗方法为介入栓塞技术,这种方法对术前病灶体积影像学测定提出了较高的要求.数字减影的血管造影、计算机断层扫描、磁共振和超声都可清晰地显示颌面部动静脉畸形病灶.相比较而言,目前认为磁共振技术不但能更充分地显示颌面部动静脉畸形病灶,也能利用计算机软件充分提取病灶信息,所得到病灶体积测量值与实际体积较为接近,有一定的临床意义.     

术前辅助性动脉栓塞在颌面部动静脉畸形手术治疗中的应用

目的：才探讨经导管动脉栓塞术在颌面部动静脉畸形手术治疗中的应用价值。方法：对17例颌面部动静脉畸形DSA检查,明确病变和主要供血动脉,选择性注入栓塞剂以减少或阻断患区血供,5－7d后再行根治性手术切除。结果：经术前辅助性栓塞,7例术中平均失血量550mL,明显低于传统手术,根治性切除后经半年至6年随访,未见复发,结论：术前辅助性动脉栓塞能明显减少术中出血,减低手术难度,为口腔颌面部动静脉畸形的根治性切除创造了条件。     

颌骨动静脉畸形的栓塞治疗

目的：总结直接穿刺结合经血管内介入栓塞治疗颌骨动静脉静脉畸形的经验。方法：收治凳骨动静脉畸形患者6例,均进行了介入栓塞治疗。采用的栓塞材料为附凝血棉纤毛的螺圈,聚乙烯醇泡沫微粒和二氰基丙烯酸对丁酯。数字减影颈动脉造影在PHILIPSV300下完成。结果6例颌骨动静脉畸形患者中4,例急性出血得到了快速、有效控制,1例慢性渗血的右下 骨动静脉畸形患者,介入栓塞治疗,拔除松动的右下凳第一磨牙,有效地控制了出血,另1例伴局部软组织搏动性膨隆的上凳骨动静脉畸形患者,介入治疗后膨隆的搏动性得到明显改善,栓塞治疗后分别随访3－24个月,均未发现有口腔内渗血或出血。随访的X线片上,病灶区可见新骨形成。结论：局部穿刺结合经血管内介入栓塞治疗颌骨动静畸形是一种安全、有效的治疗方法。     

颌骨动静脉畸形的治疗

目的：总结颌骨动静脉畸形的诊断、治疗的经验。方法：7例颌骨动静脉畸形病例先行CT或MRI检查，了解颌骨破坏情况，7例均行栓塞治疗，栓塞材料为PVA颗粒(250～500um)，栓塞后一周内进行手术，切除颌骨病变组织。结果：7例经PVA栓塞，异常血管团块消失、搏动、杂音消失、软组织肿胀得到改善。口内渗血及动脉出血得到控制。手术后随访，无1例复发。结论：血管造影栓塞后行手术切除病变组织是治疗颌骨动静脉畸形有效而可靠的治疗方法。     

Vascular malformations of the orbit (lymphatic,venous, arteriovenous): Diagnosis,management and results

Vascular malformations are often found inside the orbit. Isolated venous malformations (frequently misnamed as cavernous hemangiomas) are the most frequent among these. However, also lymphatic and arteriovenous malformations can affect the orbit. The complex anatomy of the orbit and the fact that its content easily suffers from compartmental syndrome explain why treating orbital vascular malformations can be challenging and technically demanding.In this study, two institutions have retrospectively collected their cases, consisting in a total of 69 vascular malformations of the orbit. Each type of malformation has been evaluated separately in terms of diagnosis, indications for treatment, techniques and outcomes. Moreover, the authors have analyzed in detail venous malformations, identifying three different types, named orbital venous malformation (OVM) 1, 2 and 3. These behave differently from each other, and a prompt differential diagnosis is mandatory to pose correct indications, minimize risks and improve results.Overall, surgery was the technique of choice for OVM1, microcystic lymphatic malformations (LM) and arteriovenous malformations (AVM). A pure transnasal approach with mass removal and reconstruction of the medial wall with polyethylene sheets was chosen for OVM1 (intra- or extraconal) located in the medial or superomedial compartment.Sclerotherapy had a role in treating macrocystic LM and OVM3.     

Ankyloglossia superior syndrome: case report and review of publications

Ankyloglossia superior syndrome is a rare malformation that consists of a fibrous or osseous connection between the tip of the tongue and the hard palate, and additional congenital anomalies such as cleft palate, gastrointestinal malformations, and deformed limbs. We present the case of a 5-year-old boy with ankyloglossia superior syndrome that comprised the complete complex malformation. We reviewed previous publications and summarise the different theories of its genesis.     

Syndromology: an updated conceptual overview. IV. Perspectives on malformation syndromes

In Part IV, the terms sequence and syndrome are defined and the difference between them is emphasized. Malformations and sequences are non-specific; each may occur as an isolated defect and each may occur as a component part of various syndromes. True malformation syndromes are characterized by embryonic pleiotropy in which a pattern of developmentally unrelated malformations occurs, that is, the malformations that make up the syndrome occur in embryonically non-contiguous areas. It is emphasized that syndrome diagnosis is never made from obligatory anomalies, but from the overall pattern of defects. Finally, the relationships among etiology, pathogenesis, and the phenotype are discussed.     

Anterior convex lateral orbital wall: distinctive morphology in Apert syndrome

Bony malformations of the orbit and alterations to the soft tissue in Apert syndrome contribute to ophthalmic dysfunction. Recognised structural malformation of the sphenoid and ethmoid sinuses, together with corresponding deformities in the anterior and middle cranial base, are characteristic. Our aim was to explore the underlying structural components of disfigurement and the consequent development of the orbit in patients with Apert syndrome over time by studying 18 preoperative computed tomographic (CT) scans of affected patients and 36 scans from controls. Cephalometric measurements related to the orbit were collected, and analysed with Materialise software. The patients with Apert syndrome had larger than normal external orbital horizontal angles between the ages of 6 months and 2 years. The inside horizontal angle was narrower at 16.36° before 6 months, and continued to decrease into adulthood. The ethmoid and sphenoid side angles in affected patients consistently increased, starting at 7.93% and 14.68% of the external horizontal angle, respectively, during the first 6 months of age, and becoming 20.55% and 11.69%, respectively, in adulthood. In unaffected patients, both angles were less than 3% of the external horizontal angle overall. The orbital vertical angle also changed synchronously, with increasingly wide lateral orbits and shortened anteroposterior orbits. The anterior protrusion of the lateral orbital wall resulted from superior and posterior rotation of a curved, greater wing of the sphenoid, while the widened median orbital wall was caused by the widened ethmoid sinus. These resulted in bony deformities of the orbit, which predisposed to the visual impairments of Apert syndrome.     

口腔颌面部静脉畸形诊治：经验与进展

静脉畸形是临床上最常见的先天性血管畸形,可发生于全身各个部位。其病变位置及范围的多样性,使得静脉畸形的诊断和治疗富有挑战性。目前,口腔颌面部静脉畸形的治疗方法主要包括硬化治疗、手术治疗、激光治疗等。本文通过对近几年文献的回顾,结合作者经验,对静脉畸形诊断和治疗的相关进展进行了总结。     

射频消融术用于治疗面颈部脉管畸形

目的:观察射频消融技术治疗面颈部脉管畸形的效果.方法:回顾2009- 07～2010- 10间32 例患者的临床资料.32 例患者中16 例为静脉畸形,10 例为动静脉畸形,6 例为淋巴管畸形.患者接受单纯射频消融治疗或射频消融辅助治疗.通过临床观察、B超、磁共振、三维CT血管造影等评价治疗效果.随访时间3～12 个月...     

Dandy Walker Syndrome with Tessier 7 Cleft-a Rare Case Report and a Surgical Note on the Use of Vermilion Flap and Lazy W-Plasty

The Dandy-Walker syndrome is a malformation of the brain that involves the mal-development of the cerebellum, associated with a cystic enlargement of this area, and frequently hydrocephalus. This malformation occurs in ~1 in 30,000 babies. It is seen mostly in females. Developmental anomalies like cleft lip, cleft palate, and cardiac malformation, orthopaedic and urinary structural abnormalities may also occur in 30% of the individuals. We report a case of Dandy Walker syndrome with Tessier 7 facial cleft with paramedian cleft palate in a 6 month old child. Surgical methods used to correct this anomaly include commissuroplasty, myoplasty of the orbicularis oris, and closure of the cleft cheek. Authors report a vermilion square flap technique that combines a lower lip mucocutaneous vermilion border flap with a lazy W-plasty to ensure a natural commissure and skin closure.     

Oral vascular malformation in a patient with hereditary hemorrhagic telangiectasia: a case report

Hereditary hemorrhagic telangiectasia (HHT) is an inherited mucocutaneous disease characterized by recurrent epistaxis, lesions on skin and oral mucosa, and arteriovenous malformations of the soft tissues. This article describes the treatment of a 64‐year‐old woman with a bleeding nodule, which was diagnosed as an arteriovenous malformation of the gingival mucosa. She was treated using sclerotherapy. Patients with HHT can be treated in the dental office and vascular malformations of these patients can be successfully managed with sclerotherapy, which eliminates the need for invasive surgical procedures and the possibility of postsurgical complications.     

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目的：评价口腔颌面部脉管畸形的手术治疗的临床疗效。方法对2008年6月至2012年10月中国医科大学口腔医学院口腔颌面外科收治的112例口腔颌面部脉管畸形患者,均采用手术切除治疗,病变切除后直接拉拢缝合83例,邻近皮瓣转移修复19例,中厚皮片移植修复10例。随访观察术后效果。结果术后病理进一步证实,静脉畸形67例、微静脉畸形8例、淋巴管畸形4例、淋巴-静脉畸形24例、动静脉畸形9例。术后1例患者发生皮片感染,化脓部分坏死,患区给予湿敷换药后创面二期愈合。随访6个月至5年,治愈105例,其余7例于术后3～10个月复发,其中1例淋巴-静脉畸形、4例动静脉畸形、2例静脉畸形。复发的静脉畸形患者,病变内注射平阳霉素后均治愈;其他复发患者均再次手术切除后治愈。结论手术切除治疗口腔颌面部脉管畸形在外形和功能上均可获得满意疗效,而且安全。     

Oral vascular malformation in a patient with hereditary hemorrhagic telangiectasia: a case report

Hereditary hemorrhagic telangiectasia (HHT) is an inherited mucocutaneous disease characterized by recurrent epistaxis, lesions on skin and oral mucosa, and arteriovenous malformations of the soft tissues. This article describes the treatment of a 64-year-old woman with a bleeding nodule, which was diagnosed as an arteriovenous malformation of the gingival mucosa. She was treated using sclerotherapy. Patients with HHT can be treated in the dental office and vascular malformations of these patients can be successfully managed with sclerotherapy, which eliminates the need for invasive surgical procedures and the possibility of postsurgical complications.