Penile vascular anomalies:A retrospective single center study and cumulative analysis of studies from China

Objective Penile vascular anomalies(PVAs)or hemangioma can arouse patient concern about aesthetics and cause symptoms like bleeding and sexual dysfunction.However,its low incidence and the deficiency of large-volume studies hinder urologists from making informed decisions.This study aimed to investigate the clinical features and treatment experience of PVAs at the Seventh Medical Center of PLA General Hospital,Beijing,China.Furthermore,by systematically analysis of studies on PVAs in Chinese people,we aimed to provide novel insights on the management of this condition.Methods We retrospectively investigated clinical features and pathology of surgery-treated PVAs at our center.Moreover,by systemically reviewing the literature from PubMed and the three largest medical databases(China National Knowledge Infrastructure,Wan Fang,and Chinese Medical Journal Database)in China,we analyzed the clinical features and various therapies of PVAs in Chinese people.Results Between March 1,2018 and March 1,2023,a total of 356 cases with vascular anomalies were treated with surgery at out center.Only seven(2.0%)cases had lesions involving the perineum and external genitalia.All the seven cases were pathologically benign and demonstrated no recurrence over a follow-up period of 1-52 months(median 14 months).A total of 410 cases from 44 studies were selected in the cumulative analysis.Most patients(92.4%)diagnosed with PVAs were asymptomatic,and 68.8%of the patients were treated with sclerotherapy.As to the pathology,57.1%were venous malformation.Conclusion The most common PVA is venous malformation and the majority of patients are asymptomatic.Sclerotherapy and laser have emerged as viable options for treating small lesions.Surgery still has its role in treating large lesions and obtaining pathology.Although PVAs often relapse or demand multiple treatments,the prognosis is favorable.

Optimal timing for plastic surgical procedures for common congenital anomalies:A review

Apart from listening to the cry of a healthy newborn,it is the declaration by the attending paediatrician in the labour room that the child is normal which brings utmost joy to parents.The global incidence of children born with congenital anomalies has been reported to be 3%-6%with more than 90%of these occurring in low-and middle-income group countries.The exact percentages/total numbers of children requiring surgical treatment cannot be estimated for several reasons.These children are operated under several surgical disciplines,viz,paediatric-,plastic reconstructive,neuro-,cardiothoracic-,orthopaedic surgery etc.These conditions may be life-threatening,e.g.,trachea-oesophageal fistula,critical pulmonary stenosis,etc.and require immediate surgical intervention.Some,e.g.,hydrocephalus,may need intervention as soon as the patient is fit for surgery.Some,e.g.,patent ductus arteriosus need‘wait and watch’policy up to a certain age in the hope of spontaneous recovery.Another extremely important category is that of patients where the operative intervention is done based on their age.Almost all the congenital anomalies coming under care of a plastic surgeon are operated as elective surgery(many as multiple stages of correction)at appropriate ages.There are advantages and disadvantages of intervention at different ages.In this article,we present a review of optimal timings,along with reasoning,for surgery of many of the common congenital anomalies which are treated by plastic surgeons.Obstetricians,paediatricians and general practitioners/family physicians,who most often are the first ones to come across such children,must know to guide the parents appropriately and convincingly impress upon the them as to why their child should not be operated immediately and also the consequences of too soon or too late.

Room for improvement: Patterns of referral misdiagnosis to a vascular anomalies center

Objective: Vascular anomalies are a diagnostic and therapeutic challenge. Errors in diagnosis lead to treatment delay, inappropriate interventions and prolonged suffering. The aim of this study was to analyze patterns of misdiagnosis among patients referred to a vascular anomalies center (VAC). This will better define the problem and may be used to refine and improve referral guidelines for patients with vascular anomalies. Patients and Methods: After IRB approval, we performed a single-center retrospective review of all patients referred to a vascular anomaly between January 1, 2008 and December 15, 2011. Evaluation of both referral and final diagnosis was made. Data regarding accuracy of diagnosis were determined and compared for both vascular tumors and malformations. Results: Mean age was 7.9 ± 7.7 (13 days - 66 years). 42% had a correct diagnosis at the time of referral. Vascular tumors were correctly diagnosed more often than vascular malformations (58% vs 38%). The most common misdiagnosis for infantile hemangioma (IH) was venous malformation (VM). The most common misdiagnosis for VM was IH. Nonspecific and historical terms such as “mass”, “lymphangioma”, and “cavernous hemangioma” frequently appear as the referral diagnosis. Conclusion: Referral misdiagnosis is common. IH and VM are frequently confused and if there is any uncertainty in the diagnosis, these patients should also be referred to a VAC, in addition to the more complicated anomalies. Outdated nomenclature remains prevalent and continued efforts should be made to adhere to International Society for the Study of Vascular Anomalies (ISSVA) classification. Improvements in diagnostic accuracy are likely to greatly improve patients’ care.

Vascular anomaly in the levator aponeurosis of neurofibromatosis type 1

Dear Editor,I am Satoru Kase,from the Department of Ophthalmology,Faculty of Medicine and Graduate School of Medicine,Hokkaido University,Sapporo City,Japan.I write to present a case of neurofibromatosis type 1（NF1）showing massive hemorrhage during involutional blepharoptosis surgery.

Vascular injury during laparoscopic cholecystectomy:An oftenoverlooked complication

Laparoscopic cholecystectomy is one of the most frequently performed procedures in gastrointestinal surgery worldwide.Bleeding complications due to vascular injuries represent an important cause of morbidity and mortality,especially when facing major bleeding during laparoscopy,where bleeding control can be technically challenging in inexperienced hands.Interestingly,the reported incidence rate of conversion to open surgery due to vascular lesions is approximately 0%-1.9%,with a mortality rate of approximately 0.02%.The primary aim of this article was to perform an up-to-date overview regarding the incidence and surgical management of vascular injuries during laparoscopic cholecystectomy according to the available scientific evidence.

无分叉颈动脉一例并文献复习

无分叉颈动脉是一种极其少见的颈动脉解剖变异,可能与胚胎期颈内动脉和颈外动脉的发育异常有关。颈部血管超声表现为颈动脉分叉水平未见明显膨大和分叉结构,DSA显示为颈动脉分叉水平无分叉结构,可见“残端样”改变,目前国内外研究报道不多。该研究报道1例无分叉颈动脉患者,中年男性,因头晕1个月就诊,既往有2型糖尿病、高血压病,住院后脑血管造影证实为左侧无分叉颈动脉伴右侧颈内动脉重度狭窄,经支架置入术及降压控糖治疗后症状缓解出院。结合该例临床资料及文献复习对无分叉颈动脉进行分析,旨在提高临床医师尤其是影像诊断医师对无分叉颈动脉的认识,避免误诊漏诊及相关并发症的发生。

椎基底动脉扩张延长症病机的中医“虚气留滞”病机假说阐释

椎基底动脉扩张延长症是一种预后不良的后循环血管变异疾病,目前尚缺乏有效的防治手段。通过对其病理机制、临床特征与转归的总结分析,与中医“虚气留滞”理论相结合,提出了创新病机假说,以“虚气”为发病之本,以“留滞”为发病之标,二者具有互为因果、相互促进的特点,切合疾病螺旋进展、动态演变的模式。并据此形成“扶正护脑以补虚、解毒通络而通滞”的治法,为进一步探索椎基底动脉扩张延长症的中医证治规律与疗效评价方法,为临床实践提供依据,对提高临床疗效,降低其相关的神经系统疾病负担具有重要意义。

法瓦病的诊疗现状

法瓦病(fibro-adipose vascular anomaly,FAVA)是一种复杂的良性脉管类疾病,多见于儿童和青少年,主要临床表现为受累肢体的持续性疼痛和痉挛。由于既往对FAVA的认识不足,造成临床医师对该病的诊治存在较多困惑。为进一步提高临床医师对FAVA的认识,本文对FAVA的发病机制、临床表现、影像学特征及治疗进展进行综述,为FAVA的临床诊治提供参考。

治疗药物监测在西罗莫司用于儿童期脉管异常中的研究进展

西罗莫司(sirolimus,SRL)近年来被广泛应用于治疗脉管异常疾病。由于其治疗窗狭窄,药代动力学参数存在较大的个体内和个体间变异性,需要常规进行治疗药物监测以确保疗效和避免不良反应。因此,本文回顾了SRL的血药浓度监测方法以及不同检测方法之间的对比研究,并讨论SRL在脉管异常群体中应维持的浓度范围。SRL浓度检测方法包括免疫法和色谱法两类,免疫法与色谱法相比存在显著的阳性偏倚,液相色谱串联质谱法是大多数实验室采用的“金标准”方法。SRL的目标血药浓度范围多为5~15 ng/mL或10~15 ng/mL。但脉管异常疾病存在多个分类,SRL在该疾病的临床应用情况较为复杂,治疗药物监测也无可供参考的指南。未来需进一步研究,以获取更多高级别的循证医学证据来指导SRL的治疗性监测及个体化用药。

Overview of peripheral arteriovenous malformations:From diagnosis to treatment methods

Based on the latest classification by the International Society for the Study of Vascular Anomalies in 2018,vascular malformations(VMs)can be categorized into simple,combined,VMs of major named vessels,and VMs associated with other anomalies.Simple VMs include lymphatic,venous,capillary,and arteriovenous malformations(AVMs).AVMs represent disorders of direct arteriovenous shunts caused by the absence of a capillary bed between the involved arteries and veins.This abnormal vascular communication causes arterial blood to accumulate in the venous vessels,thus resulting in venous hypertension and characteristic clinical manifestations,such as pulsation,tremors,and elevated temperature.AVMs can occur sporadically or as manifestations of syndromic lesions and are considered among the most complex and challenging VMs.The diagnosis and treatment of AVMs can vary depending on the lesion location and associated clinical symptoms,thus complicating their management.Herein,we discuss peripheral AVMs in terms of their clinical manifestations,imaging examinations,and staging systems to provide a comprehensive reference for the treatment,evaluation methods,and follow-up procedures for this vascular anomaly.

两例犬血管环异常的CT血管造影诊断和手术治疗效果

本文阐述了两例犬血管环异常(vascular ring anomaly,VRA)的CT血管造影(CT angiography,CTA)诊断及手术治疗。通过术前对患犬进行CTA诊断,进行经左侧第4肋间开胸动脉韧带切断的手术治疗,并在术后对病例1进行食道球囊扩张。CTA显示两只犬均存在持久性右主动脉弓(persistent right aortic arch,PRAA),并分别伴有右侧颈动脉异位发育和持久性左前腔静脉。手术治疗后,食道狭窄基本得到纠正,返流消失。CTA可对VRA进行更精确地诊断,并有助于制订具体手术方案;犬PRAA的手术治疗效果良好。

头颈部脉管性疾病的诊断与治疗

脉管性疾病包括血管瘤和脉管畸形,约60%发生于头颈部。该病虽然治疗方法较多,但由于其发病机制不甚明了,临床治疗仍面临挑战。疾病分类对治疗方法的选择起决定性作用,基于文献和我们的经验,本课题组将脉管性疾病进行分类,提出综合序列治疗的理念及其治疗原则。相关研究成果获上海市科技进步一等奖和国家科技进步二等奖。

脑发育性静脉异常的影像学诊断

目的:分析脑发育性静脉异常的CT、MRI和DSA表现,提高对本病的认识。方法:17例脑发育性静脉异常(DVA)患者中15例行MRI检查、CT检查6例、DSA检查4例。对DVA在不同影像检查的表现进行分析,对DVA的部位、分型、引流方式以及合并的其它异常进行探讨。结果:17例患者有21个DVA,其中3例多发,4例患者合并有海绵状血管瘤。21个DVA中幕上7个,幕下14个,根据其位置分为浅型(皮层和皮层下区)12个和深型9个,其引流静脉中单支引流14例,多支引流3例。增强CT、增强MRI和DSA静脉期均能清晰显示其特征性表现既"水母头"状扩张的髓静脉汇入粗大的引流静脉干。结论:增强CT、MRI以及DSA检查均能正确诊断和评价DVA,DSA是诊断DVA并与其它血管畸形鉴别的金标准;多种序列组合使MRI能更全面的评价DVA及其合并的其它脑实质异常,是DVA检查的首选方法。

儿童下肢血管畸形的介入治疗

目的研究儿童下肢血管畸形的介入治疗方法、疗效及并发症。方法对8例儿童下肢血管畸形进行经导管栓塞术及结合局部注射硬化剂治疗：蔓状血管瘤3例，海绵状血管瘤5例。结果栓塞后临床症状、体征迅速改善，术后0.5～1．5年随访，有效率100％，治愈率68％，并发症：病变部位疼痛加重，局部皮色皮温改变，软组织肿胀等。结论采用血管内介入治疗下肢血管畸形是一种微创安全、疗效显著的方法。

产前超声心动图冠状切面在胎儿先天性心血管畸形诊断中的应用价值

目的探讨超声心动图冠状切面在胎儿先天性心血管畸形诊断中的应用价值。方法选取2014年1月至2017年7月于首都医科大学附属北京安贞医院小儿心脏中心产前超声心动图检出并经产后证实的先天性心血管畸形胎儿70例，包括不同类型先天性血管环51例、主肺动脉间隔缺损7例、单纯性完全性肺静脉异位引流12例，回顾分析其产前常规超声切面、血管气管切面及冠状切面对先天性心血管畸形的超声图像特征，总结冠状切面对提高胎儿不同类型畸形的诊断准确性的价值。结果①加用冠状切面后，镜面有位主动脉弓形成的血管环及右位主动脉弓伴迷走左锁骨下动脉之左锁骨下动脉的显示率均明显提高，对右位主动脉弓伴左位动脉导管与双主动脉弓的鉴别性提高，但对双主动脉弓诊断无明显差别；②冠状切面可清晰显示主肺动脉间隔缺损长度及与2组半月瓣间的距离；③冠状切面可显示异位引流的静脉弓走行，并通过叠加彩色多普勒显示是否存在梗阻。结论超声心动图冠状切面可提供辅助、甚至关键的诊断信息，对提高胎儿先天性心血管畸形诊断的准确性具有重要的临床价值。

MRI在合并大血管异常的小儿复杂型先天性心脏病诊断中的应用

目的探讨MRI对复杂型先天性心脏病(CCHD)患儿大血管异常的诊断价值。方法 22例CCHD患儿接受心脏大血管MRI检查,所有患儿行手术治疗并证实诊断。结果手术证实22例CCHD患儿中法洛四联症11例、内脏心房异位综合征4例、血管环2例、先天性主动脉缩窄5例。CCHD患儿合并的大血管异常在MRI上全部得到明确诊断。结论 MRI能够准确诊断CCHD患儿的大血管异常。

异常体动脉供血正常左肺下叶的CT表现

目的探讨异常体动脉供血正常左肺下叶的CT表现,提高对本病的认识。方法回顾性分析8例确诊为异常体动脉供血正常左肺下叶及25例确诊为肺隔离症的CT表现,并对比分析两者的病灶位置、形态、与支气管关系、供血动脉来源及直径等。结果 8例异常体动脉供血正常左肺下叶与25例肺隔离症的胸痛、反复咳嗽咳痰及发热等症状的发生率差异有统计学意义(P<0.01)。两者的病灶部位、密度及形态差异有统计学意义(P<0.01)。病灶与正常支气管相通发生率差异有统计学意义(P<0.01)。异常体动脉供血正常左肺下叶直径为11.88±1.13mm,肺隔离症供血动脉直径为5.96±0.98mm,两者差异有统计学意义(P<0.01)。结论异常体动脉供血正常左肺下叶与肺隔离症CT表现不同,CT增强扫描可确诊异常体动脉供血正常左肺下叶。

双源CT在颌面部脉管性病变诊断中的应用

目的评价双源CT对颌面部脉管性疾病的的诊断价值。方法回顾性分析24例颌面部脉管性疾病患者的双源CT影像资料,原始数据应用MIP、MPR、VR及双能去骨后处理技术,其中13例儿童受检者使用虚拟平扫技术获得平扫图像。结果 24例颌面部脉管性疾病患者中,18例为血管瘤,分别发生在腮腺咬肌区6例,颊部3例,眶部2例,耳前2例,鼻部2例,唇部3例,CT平扫表现为皮下类圆形软组织密度肿块,其中3例可见静脉石,15例增强后可见持续不同程度明显强化,3例强化不明显或轻度强化;6例为血管畸形,表现为病变部位不规则明显强化异常血管团,MPR、VR可见迂曲增粗的供血动脉、引流静脉及侧支。结论双源CT去骨技术及后处理技术可以清楚显示颌面部病变及异常血管的细节,对诊断颌面部脉管性疾病具有诊断价值,同时双能虚拟平扫技术可减低儿童受检者的放射剂量。

平阳霉素联合地塞米松瘤体内注射治疗婴幼儿脉管性疾病

目的:应用平阳霉素(Pinyangmycin,PYM)联合地塞米松(Dexamethasone,DXM)瘤体内注射治疗婴幼儿脉管性疾病并观察其临床疗效。方法:102例婴幼儿脉管性疾病采用PYM、DXM联合行瘤体内直接注射,间隔时间2周,4～6次为一个疗程。结果:经6～24个月随访,治愈59例(57.84%),基本治愈30例(29.41%),有效9例(8.82%),治愈和基本治愈率87.25%。结论:PYM与DXM联合瘤腔内注射治疗婴幼儿脉管性疾病,具有疗程短、疗效好、副作用小、安全可靠等优点。

儿童周围血管畸形的血管内治疗

目的研究儿童外周血管畸形的血管内治疗方法及疗效.方法对83例周围血管畸形患儿实施血管内治疗.本组病例术前或术中分别诊断为:动静脉瘘23例,蔓状血管瘤32例,海绵状血管瘤28例.治疗方法:①经导管栓塞术,经导管释放弹簧圈或明胶海绵栓塞治疗;②导管栓塞术结合局部注射硬化剂治疗.结果栓塞治疗后造影证实,动静脉瘘和血管瘤均得到有效闭塞,临床症状、体征迅速改善,术后1～3年观察随访,治疗有效率100%,治愈率68.7%.无并发症.结论采用血管内治疗方法治疗儿童外周血管畸形,是一种微创而安全、疗效特别显著的方法.