CT淋巴管成像及胸部平扫CT诊断乳糜痰

目的 观察直接淋巴管造影(DLG)后CT淋巴管成像(CTL)及平扫CT诊断乳糜痰的价值。方法 回顾性分析17例乳糜痰患者CTL及胸部平扫CT资料,观察淋巴管异常及胸部其他异常表现。结果 17例CTL均见碘化油异常沉积,分布于颈根部、纵隔区、胸腔和胸壁,以颈静脉角区最为常见,部分可见肺部碘化油反流及多处淋巴管扩张;胸部平扫CT显示肺、胸膜、纵隔及心包等多处异常,以支气管血管束增粗最为常见。结论 CTL对诊断乳糜痰有一定价值;胸部平扫CT可显示乳糜痰患者肺及胸部其他结构异常改变。     

原发性淋巴管发育异常合并Gorham-Stout综合征的影像学表现

目的探讨原发性淋巴管发育异常合并Gorham-Stout综合征(GSS)的影像学特点。方法回顾性分析15例原发性淋巴管发育异常合并GSS患者的临床及影像学资料。15例患者均接受直接淋巴管造影,造影后CT、核医学淋巴显像检查;其中5例接受MR检查。结果直接淋巴管造影发现胸导管出口梗阻8例,下肢、下肢及阴囊、会阴、腹壁乳糜反流7例,锁骨下干、纵隔支气管干反流4例,腹膜后、髂窝淋巴管结构紊乱、纡曲、扩张7例。15例CT均发现多发溶骨性骨质破坏,累及椎体及椎体附件9例,髂骨5例,肋骨6例,肩胛骨1例,锁骨1例,肱骨1例,双侧股骨4例,跟骨、距骨1例;7例脾脏增大,可见多发类圆形低密度影,9例胸腔积液,6例腹腔积液,1例心包积液。核医学淋巴显像发现胸导管出口梗阻8例,下肢、下肢及阴囊、会阴、腹壁乳糜反流6例,锁骨下干、纵隔支气管干反流4例,腹膜后、髂窝淋巴管扩张6例,脾脏多发放射缺损2例。5例MR均发现骨质内多发长T1长T2异常信号,双髂骨3例,腰骶尾椎及椎体附件4例,双股骨2例,跟、距骨1例。结论原发性淋巴管发育异常合并GSS是淋巴系统发育异常的复杂综合征,其影像学表现、尤其是直接淋巴管造影术后CT表现具有特异性,对早期诊断本病非常重要。     

A case of Noonan syndrome with pulmonary and abdominal lymphangiectasia

Noonan syndrome is characterised by a Turner-like phenotype and a normal karyotype. Although it is reported to be associated with abnormalities of the lymphatic system, involvement of the pulmonary lymphatics is rare. We present a case of Noonan syndrome where a whole body scintigraphy revealed lymphangiectasia of the lower extremities, abdomen and lungs.     

Endothelial ERK signaling controls lymphatic fate specification

Lymphatic vessels are thought to arise from PROX1-positive endothelial cells (ECs) in the cardinal vein in response to induction of SOX18 expression; however, the molecular event responsible for increased SOX18 expression has not been established. We generated mice with endothelial-specific, inducible expression of an RAF1 gene with a gain-of-function mutation (RAF1^S259A) that is associated with Noonan syndrome. Expression of mutant RAF1^S259A in ECs activated ERK and induced SOX18 and PROX1 expression, leading to increased commitment of venous ECs to the lymphatic fate. Excessive production of lymphatic ECs resulted in lymphangiectasia that was highly reminiscent of abnormal lymphatics seen in Noonan syndrome and similar “RASopathies.” Inhibition of ERK signaling during development abrogated the lymphatic differentiation program and rescued the lymphatic phenotypes induced by expression of RAF1^S259A. These data suggest that ERK activation plays a key role in lymphatic EC fate specification and that excessive ERK activation is the basis of lymphatic abnormalities seen in Noonan syndrome and related diseases.     

综合征的诊断和治疗

Noonan综合征是一种相对常见的常染色体显性遗传病,Noonan综合征遗传异质性很大,涉及多个学科,主要临床特征包括身材矮小、颅颌面畸形、先天性心脏缺陷、认知障碍等,还可以伴发一系列血液系统疾病,患肿瘤风险较普通人群增高。重组人生长激素治疗可协助改善Noonan综合征患者身高,同时需多学科协作,治疗其他系统存在的先天异常。本文总结Noonan综合征的临床特点和相关基因突变,以期为临床提供参考。     

直接淋巴管造影及造影后CT成像在合并乳糜性胸、腹腔积液的淋巴管肌瘤病诊治中的初步应用

目的 探讨直接淋巴管造影及造影后CT成像在合并乳糜性胸、腹腔积液的淋巴管肌瘤病(LAM)诊治中的作用。 方法 收集我院经病理或影像学典型表现证实的12例LAM患者的直接淋巴管造影及造影后CT资料,并与手术相对照。 结果 CT示12例LAM均伴有乳糜性胸腔积液,5例伴有腹腔积液,2例伴有盆腔积液。12例LAM直接淋巴管造影及造影后CT均不同程度出现腹膜后和(或)髂淋巴管扩张,5例于直接淋巴管造影时出现胸导管扩张,1例胸导管回流不畅,5例胸导管未显影,1例部分显影。12例LAM中11例接受手术治疗均取得良好疗效。 结论 直接淋巴管造影和造影后CT成像可为合并乳糜性胸、腹腔积液的LAM的诊断和治疗提供重要依据。     

CT淋巴管造影对乳糜尿的诊断价值

目的 探讨CT淋巴管造影对直接淋巴管造影术后乳糜尿的诊断价值.方法 回顾性分析11例乳糜尿患者的核素淋巴显像、直接淋巴管造影、CT淋巴管造影并经手术证实的所有资料.结果 11例乳糜尿患者中,核素淋巴显像发现胸导管扩张8例,腰干增宽5例,肾盂显影7例;直接淋巴管造影发现胸导管扩张10例,腰干淋巴管纡曲、扩张11例,对侧腰干、腹膜后淋巴管反流4例,向肾盂反流10例;CT淋巴管造影发现胸导管扩张9例,右淋巴管扩张1例,腰干、腹膜后、髂、盆腔淋巴管纡曲、扩张11例,对侧腰干、腹膜后淋巴管反流10例,肾盂、肾窦反流11例.结论 CT淋巴管造影可以发现更多病变淋巴管,并清晰显示其周围解剖关系,对核素淋巴显像和直接淋巴管造影的低空间分辨力起到补充作用.     

骨淋巴管瘤CT淋巴管造影及常规CT表现

目的 探讨骨淋巴管瘤CT淋巴管造影（CTL）及常规CT表现。方法 回顾性分析79例病理证实或临床综合诊断为骨淋巴管瘤患者的临床及影像学资料。79例均接受直接淋巴管造影及造影后CT平扫,16例接受CT平扫及增强检查,分析其CTL和常规CT表现。结果 骨淋巴管瘤CT表现为骨内单发或多发低密度影,多位于脊柱（n=71）及骨盆（n=73）。79例中,囊状病变37例,筛网状病变18例,24例二者均存在;28例患者所有骨病变均可见硬化边,47例患者部分骨病变可见硬化边,4例患者所有骨病变均未见硬化边。骨内病变增强CT扫描均未见强化。CTL检查中,24例病变内可见碘化油沉积。结论 骨淋巴管瘤CTL及常规CT表现较具特征性,且常伴淋巴系统其他异常改变,有助于诊断。     

胸导管结扎术治疗原发性乳糜胸失败病例临床特征分析

  目的  对胸导管结扎术(thoracic duct ligation, TDL)治疗原发性乳糜胸失败病例的临床特征进行总结。  方法  本研究为回顾性分析。研究对象为2007年5月至2021年10月首都医科大学附属北京世纪坛医院淋巴外科收治的经外院TDL治疗失败的原发性乳糜胸患者。对其入院后临床表现及直接淋巴管造影(结合淋巴管造影后CT影像学资料)结果进行总结。  结果  共入选38例符合纳入和排除标准的原发性乳糜胸患者。TDL治疗后, 50.0%(19/38)的患者胸腔积液引流量增加, 36.8%(14/38)的患者出现了新症状。直接淋巴管造影结果显示, 所有患者的胸导管结扎均较彻底, 但患者普遍存在不同程度的淋巴回流障碍, 表现为淋巴管扩张(94.7%, 36/38)和不同形式的淋巴管反流(92.1%, 35/38)。  结论  TDL治疗失败的原发性乳糜胸患者的渗漏点应位于乳糜池及其属支淋巴管与结扎点之间, 治疗失败的原因可能与胸导管结扎加重了原本存在的淋巴回流障碍有关。建议原发性乳糜胸患者于TDL术前均行淋巴管造影检查, 以指导治疗方案的制订。     

MSCT直接淋巴管造影在弥漫性肺淋巴管瘤病中的诊断价值

目的 探讨MSCT淋巴管造影在弥漫性肺淋巴管瘤病(DPL)中的诊断价值.方法 收集经临床和病理证实的DPL患者18例,由2名医师分别对其淋巴管造影表现及其发生率进行分析.结果 18例DPL患者的胸部表现包括:对比剂反流18例(100％)、淋巴管扩张18例(100％)、纵隔软组织肥厚18例(100％)、中轴支气管血管束增厚18例(100％)、小叶间隔增厚15例(83.33％)、叶间裂增厚15例(83.33％)、双肺磨玻璃影14例(77.78％),以及胸腔积液17例(94.44％)、胸膜增厚9例(50.00％)、胸膜外软组织增厚11例(61.11％)、心包积液和心包增厚16例(88.89％)、纵隔淋巴结肿大8例(44.44％)、胸腔和纵隔心包积气4例(22.22％)、胸廓塌陷3例(16.67％);胸外异常表现包括:腹膜后对比剂反流和淋巴管扩张4例(22.22％)、腹腔积液1例(5.56％)、腹膜后和脾脏囊性淋巴管瘤各1例(5.56％)、颈部淋巴管扩张8例(44.44％)、胸导管出口梗阻8例(44.44％)、颈部多发淋巴结10例(62.50％)、腋窝多发淋巴结16例(88.89％)、下肢淋巴水肿1例(5.56％)、颜面部水肿1例(5.56％)和骨骼异常3例(16.67％).结论 MSCT直接淋巴管造影能通过显示对比剂的异常分布和堆积明确判断有无淋巴液反流和淋巴管扩张及增多,为诊断和治疗DPL提供重要依据.     

CT淋巴管成像诊断原发性乳糜尿

目的 观察CT淋巴管成像(CTL)诊断原发性乳糜尿的价值。方法 分析37例原发性乳糜尿,比较CTL与直接淋巴管造影(DLG)所见淋巴管形态、淋巴回流,泌尿系统、胸腹盆部淋巴管异常及腹盆腔、腹膜后、肺、纵隔及骨骼等其他异常;以Kappa检验评价二者诊断原发性乳糜尿的一致性。结果 CTL显示对侧髂淋巴反流、对侧腰干反流及支气管纵隔干反流优于DLG(P均<0.05),诊断一致性中等(Kappa均>0.40);二者显示同侧及对侧肾脏淋巴反流几乎完全一致(Kappa均>0.80),而CTL能进一步显示肾脏及肾周异常淋巴管分布;DLG显示颈干及锁骨下干反流优于CTL(P<0.05),二者显示同侧髂、腰干及腹膜后淋巴管纡曲扩张差异均无统计学意义(P均>0.05),诊断一致性中等及以下(Kappa均<0.50)。CTL显示对比剂异常反流至胸部14例、腹盆部36例,5例复杂性淋巴管畸形及8例淋巴管瘤等;DLG仅显示7例胸部及5例腹盆部对比剂异常反流。结论 CTL显示肾脏反流、肾周异常淋巴管分布及对侧髂、腰干反流具有重要价值,而DLG显示胸导管末端反流更具优势;联合应用...     

Cardiopulmonary rehabilitation in a patient with Noonan syndrome

Noonan syndrome, an autosomal dominant disease occurring with an incidence of 1 in 1,000 to 1 in 2,500 live births, is characterized by its particular cardiovascular abnormalities, including pulmonic valve stenosis, pulmonary artery stenosis, and, more rarely, septal defects and coarctation of the aorta. The case of a 20-year-old man admitted for inpatient cardiopulmonary rehabilitation after pulmonic valve repair, left pulmonary artery angioplasty, and pectus excavatum repair is presented. His endurance was markedly decreased, thus limiting his ability to perform activities of daily living and reducing his exercise tolerance. With participation in a comprehensive cardiopulmonary rehabilitation program, he experienced marked improvement with independence in his activities of daily living and an increase in his metabolic equivalent levels from to 2.8 to 5.4. After inpatient rehabilitation, he underwent left pulmonary stent placement before being discharged home. Subsequent outpatient cardiopulmonary rehabilitation has continued to improve significantly his overall exercise tolerance. Given that Noonan syndrome is viewed as the most common syndrome associated with congenital heart disease after Down syndrome, physiatrists must be familiar with its presentation, its associated abnormalities, and the treatment approach to optimize the patient's cardiopulmonary, musculoskeletal, and psychological status.     

Retroperitoneal lymphatics on CT and MR

We report the CT and MRI appearances of dilated retroperitoneal lymphatic channels in six patients. In two patients, these dilated channels resembled a mass of confluent low-density lymph nodes on CT. On MR urography the lymphatic channels in all six patients were seen as a meshwork of multiple tubular, tortuous, fluid-filled structures in the retroperitoneum of the abdomen and pelvis. On axial T1W images, these channels were seen as numerous, interconnected small, nodular and streaky intensities and as a cloak of diffuse homogenous hyperintensity on T2W axial images. The lymphatic nature of these abnormalities was confirmed at surgery in one patient. In another patient, the calibre and number of the dilated retroperitoneal channels reduced following anti-filarial therapy. The remaining four patients presented with chyluria.     

Imaging of the lymphatic system: new horizons

The lymphatic system is a complex network of lymph vessels, lymphatic organs and lymph nodes. Traditionally, imaging of the lymphatic system has been based on conventional imaging methods like computed tomography (CT) and magnetic resonance imaging (MRI), whereby enlargement of lymph nodes is considered the primary diagnostic criterion for disease. This is particularly true in oncology, where nodal enlargement can be indicative of nodal metastases or lymphoma. CT and MRI on their own are, however, anatomical imaging methods. Newer imaging methods such as positron emission tomography (PET), dynamic contrast‐enhanced MRI (DCE‐MRI) and color Doppler ultrasound (CDUS) provide a functional assessment of node status. None of these techniques is capable of detecting flow within the lymphatics and, thus, several intra‐lymphatic imaging methods have been developed. Direct lymphangiography is an all‐but‐extinct method of visualizing the lymphatic drainage from an extremity using oil‐based iodine contrast agents. More recently, interstitially injected intra‐lymphatic imaging, such as lymphoscintigraphy, has been used for lymphedema assessment and sentinel node detection. Nevertheless, radionuclide‐based imaging has the disadvantage of poor resolution. This has lead to the development of novel systemic and interstitial imaging techniques which are minimally invasive and have the potential to provide both structural and functional information; this is a particular advantage for cancer imaging, where anatomical depiction alone often provides insufficient information. At present the respective role each modality plays remains to be determined. Indeed, multi‐modal imaging may be more appropriate for certain lymphatic disorders. The field of lymphatic imaging is ever evolving, and technological advances, combined with the development of new contrast agents, continue to improve diagnostic accuracy. Published in 2006 by John Wiley & Sons, Ltd.     

直接淋巴管造影后CT及增强CT对小肠淋巴管扩张症的诊断价值

目的探讨直接淋巴管造影后CT和增强CT对小肠淋巴管扩张症(IL)的诊断价值。方法回顾性分析28例IL患者的影像学资料。所有病例均接受直接淋巴管造影后再接受腹盆部CT平扫,4例于淋巴管造影前接受腹部增强CT检查,5例淋巴管造影及CT平扫后接受腹部增强CT扫描。结果患者中肠壁增厚、肠腔扩张15例,肠系膜水肿21例,胸、腹腔积液16例。直接淋巴管造影后CT显示不同程度淋巴管异常:对比剂漏入肠腔或分布于肠壁7例,肠系膜淋巴管扩张10例、碘油分布于胃壁1例、对比剂反流至肝门、脾门、胰腺周围、对侧髂淋巴管及胸腔21例。9例增强扫描患者中,肠壁分层强化呈"晕轮征"3例,肠黏膜明显线状强化7例。结论直接淋巴管造影后CT和增强CT对IL的诊断有重要价值,可观察胸、腹腔积液及脏器情况,并可用于复查。     

Hennekam综合征一例并文献复习

Hennekam综合征是以淋巴管扩张、淋巴水肿、特征性颜面部异常、生长发育迟缓为主要临床表现的一种常染色体隐性遗传性疾病。该文报道1例Hennekam综合征患者,其以腹泻、水肿、手足搐搦为主要临床表现,伴有特征性面容,生长发育迟缓及精神运动发育迟滞,经胶囊内镜检查发现小肠淋巴管扩张。在给予低脂、高蛋白、补充中链甘油三酯饮食后患者病情好转。对临床难以解释的慢性腹泻、面容异常、生长发育迟缓、精神运动发育迟滞的患者,需警惕该综合征的可能,应尽早行内镜、病理活组织及淋巴显像检查以明确诊断。     

The first reported case of Noonan syndrome complicated with hepatocellular carcinoma

Noonan syndrome is a genetic multisystem disorder and is associated with mutation of genes encoding the proteins in the RAS‐MAPK pathway. We reported the first case of Noonan syndrome complicated with hepatocellular carcinoma.     

Spinal cord arteriovenous malformation in a person with congenital lymphatic abnormalities.

Spinal cord arteriovenous malformations have been described in association with a variety of congenital diseases affecting the vasculature, including Klippel-Trenaunay-Weber syndrome, Rendu-Osler-Weber syndrome and others, but rarely in association with lymphatic abnormalities. We report the case of a young man with congenital lymphedema and arteriovenous malformations of one lower extremity and a spinal cord arteriovenous malformation. Awareness of the possible presence of a central nervous system arteriovenous malformation in individuals with pre-existing arteriovenous and lymphatic abnormalities may be helpful in their diagnosis and management.     

盆腹腔淋巴切除术后淋巴漏的诊治现状

淋巴漏是妇科盆腔术后常见的并发症,对于存在肥胖、贫血、术中出血过多及淋巴结肿大等高危因素的患者,应给予足够重视。淋巴漏发生后应首选保守治疗,包括禁食、营养支持、药物治疗等; 对于保守治疗不佳的难治性患者,可考虑手术或介入治疗,目前超声引导下结内淋巴管造影术不失为一种可选择的方法。本文回顾盆腹腔淋巴系统的解剖及生理,分析妇科术后淋巴漏的形成原因、高危因素、治疗方法及预防措施,以期为临床医生提供指导和帮助。     

Poor prenatal detection rate of cardiac anomalies in Noonan syndrome.

BACKGROUND: The wide variation and nonspecific nature of many of the associated ultrasonographic findings complicate prenatal diagnosis of Noonan syndrome. The aim of the present study was to define the rate of prenatal diagnosis of heart malformations in cases diagnosed postnatally with Noonan syndrome. METHODS: English-language literature review of 29 cases of Noonan syndrome examined prenatally with confirmed postnatal diagnosis and four case reports from our center. RESULTS: Cases were evaluated for cervical spine pathologies, cardiac anomalies and other pathological findings, including hydrops fetalis and polyhydramnios. Cardiac anomalies were suspected in only nine of 33 cases; three of these were associated with cystic hygroma. Cardiac anomalies were eventually diagnosed in 31/33 cases postnatally. Polyhydramnios was diagnosed in 19/33 cases in the third trimester, and hydrops fetalis was detected in eight of 33. Cystic hygroma was present in a total of nine cases at mid-trimester. CONCLUSIONS: Noonan syndrome is characterized by late-onset and progressive pathologies, particularly the associated cardiac anomalies, which develop through the course of gestation and postnatal life. This complicates or precludes prenatal diagnosis at mid-trimester or at any time in the prenatal period, and partly explains the low rate of detection of fetal cardiac lesions in this syndrome.