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1、贲门失弛缓症ACHALASIA,1,Anatomy- esophagus,- Muscular tube - Conduit from the pharynx to the stomach- Length is defined anatomically, from cricoid cartilage to the gastric orifice- Distance from the incisor 40-45 cm (actual length: M 22-28cm F 2cm shorter)- Passes behind aortic arch and left main bronchus
2、.- Enters abdomen through esophageal hiatus 2-4 cm below the diaphragm,2,Course of the esophagus- Neck and upper esophagus: left of midline- Mid-esophagus: right of midline - Lower esophagus: left of midlineThree area of normal constrictions:- Cricopharangeal- Behind the aortic arch - LES (thickenin
3、g of the Circular muscles 4cm),3,- Fixed in position at two places:. Upper: firmly attached to the cricoid cartilage. Lower: Phreno-esophageal ligament to the esophagus whichprovides an air- tight seal between the thoracic and abdominal cavity.(lack of fixation throughout its length allows both tran
4、sverse and longitudinal mobility),4,Vascular supply,ARTERIAL SUPPLYUpper superior and inferior thyroid arteryMiddle Bronchial arteries and esophageal branches directly from aorta Lower L inferior phrenic and gastricVENOUS SUPPLYUpper esophageal venous plexus to azygos veinLower esophageal branches o
5、f the coronary vein, a tributary of the portal vein,5,Structure,- Consists of 3 layers: muscularis externa, submucosa, mucosa,6,Achalasia-historical note,First described more than 300yrs ago Referred to as cardiospasm Thomas Willis (1621-1675) Described a pt starving and unable to swallow Conclusion
6、 was due to lower esophageal narrowing Constructed the first dilator-made of whale bone and sponge First successful treatment of achalasia,7,Achalasia-historical note,1914: Ernst Heller(1877-1964) - First successful cardiomyotomyAnterior and posterior myotomies Extending 8cm or more into esophagus a
7、nd stomach,8,Achalasia-historical note,1918: De Brune Groenveldt and Zaaijer performed modified Heller myotomyanterior onlyOriginal technique was to excessive,9,Achalasia,- Uncommon (0.5-1 in 100,000)- No sex predilection M=F- Majority between ages 20-50s- Ineffective relaxation of the LES combined
8、with loss of esophageal peristalsis impaired esophageal emptying and gradual dilatation- Decrease or loss of myenteric ganglion cells- Slight increase risk of esophageal carcinoma(approx. 10yrs earlier than the general population),10,Achalasia - Presentation,- Dysphagia - delayed and progressive pre
9、sentation (mean 2 years)- Exacerabated by emotional stress or cold fluid- 60-90% report spontaneous or forced regurgitation of undigested food- 10% will have pulmonary complication- Chest pain ( heartburn) - 30-50% resolves with Myotomy,11,Achalasia - Diagnosis,-CXR: air fluid levels- Barium swallow
10、: dilated esophagus with Birds beak deformity. (pseudoachalasia from extrinsic mass may mimic the classic achalasia appearance)- Manometry: gold standard. Elevated LES pressure (greater than 35mmHg). Incomplete sphincter relaxation. Complete absence of peristalsis- Endoscopy: dilated esophagus with
11、tightly closed LES gentle pressure will admit the scope with a pop“.,12,Achalasia,13,Achalasia,14,Achalasia - Treatment,Palliation of dysphagia is the key relieve functional obstruction of distal esophagus - pharmacotherapy - botulinum toxin - esophageal dilation - operative myotomy,15,Achalasia- al
12、gorithm,16,Achalasia - Treatment,Pharmacotherapy: (poorly absorbed and short lived, best reserved as adjunct to other therapies) - Nitrates - Ca+ channel blockers - Anticholinergics - Opiods,17,Botulinum Toxin Therapy,18,Achalasia - Treatment,Botox injection:- Bind to cholinergic nerves and irrevers
13、ibly inhibit Acetyl Choline release- 60-85% of patient get relief but 50% get recurrent symptoms within 6 months.- Endoscopically injected- For pt who are not candidates for other therapies,19,Achalasia - Treatment,Botox injection cont.- Advantages: safety, ease of administration, minimal side effec
14、ts- Disadvantages: expensive, need for multiple injections, and efficacy decreased with repeated injection- Cause obliteration of the dissection planes between submucosa and muscular layer which will make subsequent surgery more difficult and increase risk of perforation.,20,Pneumatic Dilator,21,Ach
15、alasia - Treatment,Esophageal dilation (under fluroscopy) -Standard nonoperative therapy -Break the muscle fibers -For pts with limited life expectancy -Can have repeated dilatation -60-80% success rate, 5yr recurrence rate 50% -Efficacy is decreased after second dilatation -Perforation rate 2% -PPI
16、 reduces the need for repeat dilatation,22,Esophageal myotomy,23,Achalasia Surgical treatment,- Excellent results in 90-95%- Gold standard- 1914 - Ernest Heller- double myotomy- Modified by Zaaijer- single myotomy- Worlds largest experience-Brazil, Chagas disease-endemic-1 in 8 inhabitants, in which
17、 5% develops achalasia- Traditionally trans-thoracic or trans-abdominal- Now minimally invasive Laparoscopic /Thoracoscopic- Robotic Heller myotomy,24,Achalasia Surgical treatment,Indications: Younger than 40yrs old (group which PD is 50%effective) High risk of perforation Esophageal diverticula Pre
18、vious surgery of GE junction Tortuous or dilated distal esophagus Recurrent symptoms despite Botox or PD therapyPersonal choice of therapy Lower risk of perforation Better long term outcome Decrease chance of re-intervention,25,Achalasia Surgical treatment, Expose mucosal surface Length of myotomy C
19、ephalad: 1-2 cm beyond the dilated esophagus Caudal: 1-2 cm into the gastric musculature or when transverse veins are encountered Check for perforation Meythlene blue Air,26,Complications, Intra-op Mucosa perforation Post-op: Dysphagia- adhesion, inadequate myotomy GERD- long myotomy, nerve damage D
20、elay perforation- inadequate myotomy,27,Achalasia Surgical treatment,Which esophageal technique should be used? Any role for anti-reflux procedure?,28,29,30,概念,贲门失弛缓症是一种食管动力学功能障碍性疾病。特点是下食管括约肌不能松弛，食管体缺乏正常的蠕动波，食管排空受阻造成食管腔内食物淤积而扩张根据本病在X线上的解剖学改变又被称为巨食管症或贲门痉挛。,31,病因,本病病因不清。可能与基因遗传、自身免疫、病毒感染、社会心理因素有关。目前,对
21、其发病机制普遍接受神经源性学说，即病人食管壁肌间神经丛内神经节细胞减少或缺如，而控制食管环型肌松弛的氮能神经和血管活性肠肽(VIP)免疫阳性神经纤维减少或消失，从而导致LES不能正常松弛。,32,临床表现,大多数患者起病缓慢，起病时症状不明显。突然起病者多与情绪紧张有关。(一)吞咽困难：是该病最突出的的表现。其程度常有差异。通常液体吞咽困难者占60 ，固体吞咽困难者占98。很少有食管癌的从固体到 流食到液体的规律性吞咽困难的发病过程。(二)食管反流：未消化食物的食管内潴留及反流是该病另一常见症状，占总数的6o90。(三)胸痛：1312的病人伴有胸痛。常在进食后突发，并时常迫使病人停止进食。(四
22、)其他症状：部分病人可出现烧心症状，多发生于疾病早期和吞咽困难以前。重症、病程较长时，可出现明显的体重减轻、营养不良、贫血等症状。,33,非手术治疗方法,1 药物治疗药物治疗包括局部麻醉剂、钙离子拮抗剂、硝酸盐类药物、抗胆碱药物、镇静药物、胃肠动力药、中药治疗等。药物治疗作用轻微，而且作用时间短暂，因此，仅用于贲门失弛缓症的早期、老年高危病人或拒绝其他治疗的病人。,34,11钙离子拮抗剂 可干扰细胞膜的钙离子内流，解除平滑肌痉挛，可松弛LES，有效解除吞咽困难及胸骨后疼痛。侯延丽等报道，硝苯毗啶舌下含服能降低LES静l卜压、食管收缩振幅和自发性收缩频率，同时也能改善食物在食管中的排空，使吞咽困
23、难改善。常用量为1020 nag，每日3次。硫氮卓酮、异博定疗效不如硝苯吡啶，且不良反应日月显，尤其对有心功能不全、房室传导阻滞和房颤、房扑的患者，应忌用。12 硝酸盐类硝酸盐或亚硝酸盐类药物在体内降解产生NO，松弛I Es，从而缓解AC患者临床症状_2 J。实验证明硝酸甘油、 硝酸异戊酯应用后l5 nfin起效，LES可从6.12 kPa(46 mmHg)下降到20 kPa(15 mmHg)，持续90 min。常用药物：硝酸甘油0306 mg每日3次餐前15min舌下含服，硝酸异山梨酯510 mg餐前1020 min舌下含服每日3次，疗程不宜过长，一般为2周，以防止产生耐药性。,35,13
24、局部麻醉剂2普鲁卡因60 mL于餐前1520 min口服，有助于LES松弛，可能与该药抑制兴奋活动过程，而使LES松弛有关。14 抗胆碱能药物解痉灵1020 nag次，肌注或静推，可阻断M 胆碱能受体，使乙酰胆碱不能与受体结合而松弛平滑肌，改善食管排李，可扶疗效。其他药物山莨菪碱、阿托品等疗效不大，不良反应可见口干、尿潴留、心悸。应用较少。,36,16 胃肠动力药物AC患者晚期常继发食管运动明显减弱，排宅延迟，故可采用胃肠动力药物胃复安510 nag每日4次口服，或多潘立酮l020 nag每日 4次口服，增加LESP和食管下端的蠕动，缩短食管与酸性反流物的接触时间。17 注射肉毒杆菌毒素(BT
25、) BT能阻断神经肌肉接头处突触前膜乙酰胆碱的释放而使肌肉松弛麻痹。以缓解AC患者临床症状。据报道内镜下行LES内注射A型BT初治有效率为825 。本方法不良反应轻微、操作简便、痛苦小、安全可靠。对无法手术、无法行气囊扩张的患者更为适宜。,37,2 扩张治疗扩张治疗包括球囊扩张、支架治疗等。禁忌证包括病人不能合作、合并严重心肺疾患或其他严重疾病、严重器官衰竭无法耐受治疗、局部水肿严重、狭窄严重致导丝无法通过等 。,38,手术治疗,开放式食管下括约肌(Heller肌)切开术开放式Heller肌切开手术分为经腹和经胸2种，手术的目的是彻底切开食管下括约肌，以消除吞咽困难症状。目前，常用的是改良He
26、ller手术。手术适应证包括临床诊断的贲门失弛缓症，无黏膜病变，无手术禁忌证均可手术治疗。手术要点是经胸或经腹暴露扩张、狭窄的病段食管，根据狭窄长度，沿食管纵轴垂直切开食管侧肌层约6 cm，胃底侧 13 cm，完全切断狭窄环，并在黏膜外剥离被切开的肌层，使其达到食管周径的12。蒋俭等 报道开放手术术后症状改善率为969 。早期并发症主要为食管穿孔，晚期主要为胃食管反流，发生率50 以上。,39,40,腔镜下食管下括约肌(Heller肌)切开术,1991年Shimi等 率先施行腹腔镜Heller肌切开术，1992年Pellegrini等 首次施行胸腔镜Heller肌切开术。Patti等 回顾了近
27、十年来贲门失弛缓症治疗的变化趋势，总结出腔镜下Heller肌切开术手术具有传统手术的有效性，手术操作简便、创伤小、缩短术后住院13和康复时间，降低术后死亡率，并发症和开放手术相当，腔镜下Heller肌切开手术已经成为手术治疗首选。Robe等 报道36例腹腔镜Heller肌切开术，手术优良率944 ，术中黏膜穿孔发生率83 ，术后胃食管反流发生率仅为83 。刘隆等 报道25例腹腔镜HellerDor手术，术后92 的患者吞咽功能恢复良好。 ,41,机器人辅助微创手术,随着手术机器人达芬奇、宙斯的出现，机器人腹腔镜手术很快应用到外科各个领域。2000年7月Melvin等 报道首例机器人辅助腹腔镜食
28、管Heller肌切开术。他们认为机器人腹腔镜手术具有三维图像对病变的识别更容易、清楚，机械臂比人臂更稳定，准确性更高的优点。2005年Horgan等 报道机器人辅助腹腔镜食管贲门括约肌切开术比普通腹腔镜食管贲门肌切开手术更安全。但机器人腹腔镜食管贲门括约肌切开术需要昂贵的仪器，且手术前安置机器的时间比较长，手术总时间长。,42,目前存在的争论,目前，存在的争论主要为是否需要联合抗反流手术，抗反流手术的方式和既往治疗对手术效果的影响等。抗反流手术基本有三类：全胃底折叠术、部分胃底折叠术和贲门固定术。,43,是否需要联合抗反流手术,Heller肌切开术是否联合抗反流手术是目前争论的主要问题。反对常
29、规使用抗反流手术的人认为单纯Heller肌切开术后反流并不高，术后出现胃食管反流可以用药物很好控制，并且抗反流手术可能造成术后持续的吞咽困难或复发。Dempsey等 对比29例Heller肌切开联合Dor折叠术和22例单纯Heller肌切开，2组病人在症状的改善、术后吞咽困难及烧心的症状评分均一样，提示Dor前折叠对手术疗效无明显影响。认为需要联合抗反流手术的学者认为Heller肌层切开破坏食管下段肌层原本的生理功能，会导致术后严重的反流，而胃食管反流是引起贲门失弛缓症手术晚期失败的主要原因。Mahhaner等 州报道单纯Heller肌切开术后20年胃食管反流的发生率可达到78 。抗反流手术可
30、有效降低手术后胃食管反流率，Richards等 在一项随机对照试验中比较了HellerDor手术与单纯Heller手术疗效，发现前者术后病理性胃食管反流仅为9，1 (222)，而单纯Heller肌切开手术术后病理性胃食管反流为476(1021)。,44,抗反流的方式,1、 Dor前折叠(前壁180胃底折叠)Dor前折叠手术不用解剖食管后组织，对胃食管膜的破坏比较小，且前折叠将胃底覆盖在膨出的食管黏膜上，可以预防手术食管黏膜破口所致的食管瘘。Mineo等 在一组81例开放HellerDor手术中观察到术后病理性反流率仅为77 。Dor前折叠并不增加术后吞咽困难比例，如Harold等 报道Hell
31、erDor手术后96 的病人吞咽功能良好。常用于配合Heller手术。,45,2、 Toupet后折叠(食管左、后、右壁270胃底折叠术)Toupet后折叠手术在食管后方将黏膜外肌分开缝合，保持黏膜外肌持续分开，有效降低手术后病理性反流率，抗反流效果与Dor手术相当甚至更佳 。Arain等 比较了41例HellerDor和23例HellerToupet手术疗效，认为在术后症状评分、吞咽困难的改善、病人对手术效果的评价以及质子泵抑制剂的应用方面无明显差异，但后折叠手术操作较前折叠困难，手术时间更长。,46,3、 Nissen全折叠 （全胃底折叠360）Nissen全折叠可有效缓解Heller肌切
32、开病人术后胃食管反流。Falkenback等 报道在附加Nissen全折叠的Heller肌切开病人术后24 h反流比单纯Heller肌切开病人低很多。但由于贲门失弛缓病人食管蠕动功能欠佳，Nissen全折叠手术在食管运动过程中形成障碍而导致术后吞咽困难缓解率偏低，因此，有作者认为认为Nissen全折叠手术对贲门失弛缓症病人不适合 。,47,鉴于传统Nissen手术有较多的机械性并发症如包饶部分滑脱、缝合裂开、胃胀气、嗳气困难或呕吐以及难于掌握等，Donahue将折叠缝合改为2.0cm或更短，且包饶较松弛称为短松Nissen手术（short floppy Nissen手术），这种手术应用较广。,48,4、Hill手术（经腹后固定术）将食管裂孔疝加以缝合后，将食管胃连接部缝合固定于弓状韧带上。手术难度大，术中需要测压指导缝合，采用者较少。,49,
