贲门失弛缓症的处理.ppt

贲门失弛缓症ACHALASIAAnatomy-esophagusn n-Muscular tube-Conduit from the pharynx to the-Muscular tube-Conduit from the pharynx to the stomachstomachn n-Length is defined anatomically,from cricoid-Length is defined anatomically,from cricoid cartilage to the gastric orificecartilage to the gastric orificen n-Distance from the incisor 40-45 cm(actual-Distance from the incisor 40-45 cm(actual length:M 22-28cm F 2cm shorter)length:M 22-28cm F 2cm shorter)n n-Passes behind aortic arch and left main-Passes behind aortic arch and left main bronchus.bronchus.n n-Enters abdomen through esophageal hiatus 2-Enters abdomen through esophageal hiatus 2-4 cm below the diaphragm4 cm below the diaphragm n nCourse of the esophagusCourse of the esophagusn n-Neck and upper esophagus:-Neck and upper esophagus:left of midline left of midlinen n-Mid-esophagus:right of-Mid-esophagus:right of midline midlinen n-Lower esophagus:left of-Lower esophagus:left of midline midlinen nThree area of normalThree area of normal constrictions:constrictions:n n-Cricopharangeal-Cricopharangealn n-Behind the aortic arch-Behind the aortic archn n-LES(thickening of the-LES(thickening of the Circular muscles 4cm)Circular muscles 4cm)n n-Fixed in position at two places:-Fixed in position at two places:n n.Upper:firmly attached to the cricoid cartilage.Upper:firmly attached to the cricoid cartilagen n.Lower:Phreno-esophageal ligament to the.Lower:Phreno-esophageal ligament to the esophagus whichesophagus whichn nprovides an air-tight seal between the thoracic and provides an air-tight seal between the thoracic and abdominal cavity.abdominal cavity.n n(lack of fixation throughout its length allows both(lack of fixation throughout its length allows both transverse and longitudinal mobility)transverse and longitudinal mobility)Vascular supplyn n ARTERIAL SUPPLY ARTERIAL SUPPLYn n Upper superior and inferior thyroid Upper superior and inferior thyroid artery arteryn n Middle Bronchial arteries and Middle Bronchial arteries and esophageal branches directly from aorta esophageal branches directly from aortan n Lower L inferior phrenic and gastric Lower L inferior phrenic and gastricn n VENOUS SUPPLY VENOUS SUPPLYn n Upper esophageal venous plexus Upper esophageal venous plexus to azygos vein to azygos veinn n Lower esophageal branches of Lower esophageal branches of the coronary vein,a tributary of the the coronary vein,a tributary of the portal vein portal veinStructuren n-Consists of 3 layers:muscularis externa,submucosa,mucosaAchalasia-historical noten n First described more than 300yrs agon n Referred to as cardiospasmn n Thomas Willis(1621-1675)n n Described a pt starving and unable to swallow Described a pt starving and unable to swallown n Conclusion was due to lower esophageal narrowing Conclusion was due to lower esophageal narrowingn n Constructed the first dilator-made of whale bone Constructed the first dilator-made of whale bone and sponge and spongen n First successful treatment of achalasia First successful treatment of achalasiaAchalasia-historical noten n1914:Ernst Heller1914:Ernst Hellern n(1877-1964)-First(1877-1964)-First successful successful cardiomyotomy cardiomyotomyn nAnterior and posteriorAnterior and posterior myotomies myotomiesn n Extending 8cm or more Extending 8cm or more into esophagus and into esophagus and stomach stomachAchalasia-historical notenn 1918:De Brune Groenveldt and Zaaijer performed modified Heller myotomyn n anterior onlyn n Original technique was to excessiveAchalasian n-Uncommon(0.5-1 in 100,000)-Uncommon(0.5-1 in 100,000)n n-No sex predilection M=F-No sex predilection M=Fn n-Majority between ages 20-50s-Majority between ages 20-50sn n-Ineffective relaxation of the LES combined with-Ineffective relaxation of the LES combined with loss of esophageal peristalsis impaired loss of esophageal peristalsis impaired esophageal emptying and gradual dilatationesophageal emptying and gradual dilatationn n-Decrease or loss of myenteric ganglion cells-Decrease or loss of myenteric ganglion cellsn n-Slight increase risk of esophageal carcinoma-Slight increase risk of esophageal carcinoman n(approx.10yrs earlier than the general population)(approx.10yrs earlier than the general population)Achalasia-Presentationn n-Dysphagia-delayed and progressive presentation(mean 2 years)n n-Exacerabated by emotional stress or cold fluidn n-60-90%report spontaneous or forced regurgitation of undigested foodn n-10%will have pulmonary complicationn n-Chest pain(heartburn)-30-50%resolves with MyotomyAchalasia-Diagnosisn n-CXR:air fluid levels-CXR:air fluid levelsn n-Barium swallow:dilated esophagus with Birds-Barium swallow:dilated esophagus with Birds beak deformity.(pseudoachalasia from extrinsic beak deformity.(pseudoachalasia from extrinsic mass maymass may mimic the classic achalasia appearance)mimic the classic achalasia appearance)n n-Manometry:-Manometry:gold standardgold standardn n.Elevated LES pressure(greater than 35mmHg).Elevated LES pressure(greater than 35mmHg)n n.Incomplete sphincter relaxation.Incomplete sphincter relaxationn n.Complete absence of peristalsis.Complete absence of peristalsisn n-Endoscopy:dilated esophagus with tightly-Endoscopy:dilated esophagus with tightly closed LESclosed LESn n gentle pressure will admit the scope with a gentle pressure will admit the scope with a pop“.pop“.AchalasiaAchalasiaAchalasia-Treatmentn n Palliation of dysphagia is the key relieve functional obstruction of distal esophagusn n-pharmacotherapyn n-botulinum toxinn n-esophageal dilationn n-operative myotomyAchalasia-algorithmAchalasia-Treatmentn n Pharmacotherapy:(poorly absorbed and short lived,best reserved as adjunct to other therapies)n n-Nitratesn n-Ca+channel blockersn n-Anticholinergicsn n-OpiodsBotulinum Toxin TherapyAchalasia-Treatmentnn Botox injection:n n-Bind to cholinergic nerves and irreversibly inhibit Acetyl Choline releasen n-60-85%of patient get relief but 50%get recurrent symptoms within 6 months.n n-Endoscopically injectedn n-For pt who are not candidates for other therapiesAchalasia-Treatmentnn Botox injection cont.n n-Advantages:safety,ease of administration,-Advantages:safety,ease of administration,minimal side effects minimal side effectsn n-Disadvantages:expensive,need for multiple-Disadvantages:expensive,need for multiple injections,and efficacy decreased with repeated injections,and efficacy decreased with repeated injectioninjectionn n-Cause obliteration of the dissection planes-Cause obliteration of the dissection planes between submucosa and muscular layer which will between submucosa and muscular layer which will make subsequent surgery more difficult and make subsequent surgery more difficult and increase risk of perforation.increase risk of perforation.Pneumatic DilatorAchalasia-Treatmentn n Esophageal dilation(under fluroscopy)n n-Standard nonoperative therapy-Standard nonoperative therapyn n-Break the muscle fibers-Break the muscle fibersn n-For pts with limited life expectancy-For pts with limited life expectancyn n-Can have repeated dilatation-Can have repeated dilatationn n-60-80%success rate,5yr recurrence rate 50%-60-80%success rate,5yr recurrence rate 50%n n-Efficacy is decreased after second dilatation-Efficacy is decreased after second dilatationn n-Perforation rate 2%-Perforation rate 2%n n-PPI reduces the need for repeat dilatation-PPI reduces the need for repeat dilatationEsophageal myotomyAchalasia Surgical treatmentn n-Excellent results in 90-95%Excellent results in 90-95%n n-Gold standard Gold standardn n-1914-Ernest Heller-double myotomy 1914-Ernest Heller-double myotomyn n-Modified by Zaaijer-single myotomy Modified by Zaaijer-single myotomyn n-Worlds largest experience Worlds largest experiencen n-Brazil,Chagas disease-endemic Brazil,Chagas disease-endemicn n-1 in 8 inhabitants,in which 5%develops achalasia 1 in 8 inhabitants,in which 5%develops achalasian n-Traditionally trans-thoracic or trans-abdominal-Traditionally trans-thoracic or trans-abdominaln n-Now minimally invasive Laparoscopic/-Now minimally invasive Laparoscopic/n n Thoracoscopic Thoracoscopicn n-Robotic Heller myotomy-Robotic Heller myotomyAchalasia Surgical treatmentn n Indications:Indications:n n Younger than 40yrs old(group which PD is 50%effective)Younger than 40yrs old(group which PD is 50%effective)n n High risk of perforation High risk of perforationn n Esophageal diverticula Esophageal diverticulan n Previous surgery of GE junction Previous surgery of GE junctionn n Tortuous or dilated distal esophagus Tortuous or dilated distal esophagusn n Recurrent symptoms despite Botox or PD therapyRecurrent symptoms despite Botox or PD therapyn n Personal choice of therapyPersonal choice of therapyn n Lower risk of perforation Lower risk of perforationn n Better long term outcome Better long term outcomen n Decrease chance of re-intervention Decrease chance of re-interventionAchalasia Surgical treatmentn n Expose mucosal surfaceExpose mucosal surfacen n Length of myotomyLength of myotomyn n Cephalad:1-2 cm beyond the dilated esophagus Cephalad:1-2 cm beyond the dilated esophagusn n Caudal:1-2 cm into the gastric musculature or Caudal:1-2 cm into the gastric musculature or when transverse veins are encountered when transverse veins are encounteredn n Check for perforationCheck for perforationn n Meythlene blue Meythlene bluen n Air AirComplicationsn n Intra-opn n Mucosa perforationn n Post-op:n n Dysphagia-adhesion,inadequate myotomyn n GERD-long myotomy,nerve damagen n Delay perforation-inadequate myotomyAchalasia Surgical treatmentn n Which esophageal technique should be used?n n Any role for anti-reflux procedure?概念n n 贲门失弛缓症是一种食管动力学功能障碍性疾病。n n 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n n 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。病因n n 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。n n 目前,对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。临床表现n n 大多数患者起病缓慢，起病时症状不明显。突然起病者多 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。与情绪紧张有关。n n(一 一)吞咽困难：是该病最突出的的表现。其程度常有差异。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 通常液体吞咽困难者占60 60，固体吞咽困难者占，固体吞咽困难者占98 98。很。很少有食管癌的从固体到 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。流食到液体的规律性吞咽困难的发病过程。n n(二 二)食管反流：未消化食物的食管内潴留及反流是该病另 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 一常见症状，占总数的6o 6o 90 90。n n(三 三)胸痛：胸痛：1 1 3 3 1 1 2 2 的病人伴有胸痛。常在进食后突发，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。并时常迫使病人停止进食。n n(四 四)其他症状：部分病人可出现烧心症状，多发生于疾病 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 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。体重减轻、营养不良、贫血等症状。非手术治疗方法n n 1 药物治疗n n 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。n n 1 1 1 1 钙离子拮抗剂 钙离子拮抗剂 可干扰细胞膜的钙离子内流，解除平滑肌 可干扰细胞膜的钙离子内流，解除平滑肌痉挛，可松弛 痉挛，可松弛LES LES，有效解除吞咽困难及胸骨后疼痛。侯延，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 丽等报道，硝苯毗啶舌下含服能降低LES LES 静 静l l 卜压、食管收缩 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，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，使吞咽困难改善。常用量为 使吞咽困难改善。常用量为10 10 20 nag 20 nag，每日，每日3 3 次。硫氮卓酮、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 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。功能不全、房室传导阻滞和房颤、房扑的患者，应忌用。n n 1 1 2 2 硝酸盐类硝酸盐或亚硝酸盐类药物在体内降解产生 硝酸盐类硝酸盐或亚硝酸盐类药物在体内降解产生NO NO，松弛，松弛I Es I Es，从而缓解，从而缓解AC AC 患者临床症状 患者临床症状_2 J _2 J。实验证明硝酸。实验证明硝酸甘油、甘油、硝酸异戊 硝酸异戊 酯应用后 酯应用后l5 nfin l5 nfin 起效，起效，LES LES 可从 可从6.12 kPa(46 6.12 kPa(46 mmHg)mmHg)下降到 下降到2 2 0 kPa(15 mmHg)0 kPa(15 mmHg)，持续，持续90 min 90 min。常用药物：。常用药物：硝酸甘油 硝酸甘油0 0 3 3 0 0 6 mg 6 mg 每日 每日3 3 次餐前 次餐前15min 15min 舌下含服，硝酸异 舌下含服，硝酸异山梨酯 山梨酯5 5 10 mg 10 mg 餐前 餐前10 10 20 min 20 min 舌下含服每日 舌下含服每日3 3 次，疗程不宜 次，疗程不宜过长，一般为 过长，一般为2 2 周，以防止产生耐药性。周，以防止产生耐药性。n n 1 3 局部麻醉剂2 普鲁卡因60 mL 于餐前15 20 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。n n 1 4 抗胆碱能药物解痉灵10 20 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。n n116 6 胃肠动力药物胃肠动力药物ACAC患者晚期常继发食管运动明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显减弱，排宅延迟，故可采用胃肠动力药物胃复安安5510 nag10 nag每日每日44次口服，或多潘立酮次口服，或多潘立酮l020 nagl020 nag每日每日 4 4次口服，增加次口服，增加LESPLESP和食管下端的蠕动，缩和食管下端的蠕动，缩短食管与酸性反流物的接触时间。短食管与酸性反流物的接触时间。n n117 7 注射肉毒杆菌毒素注射肉毒杆菌毒素(BT)BT(BT)BT能阻断神经肌肉接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。头处突触前膜乙酰胆碱的释放而使肌肉松弛麻痹。以缓解以缓解ACAC患者临床症状。据报道内镜下行患者临床症状。据报道内镜下行LESLES内内注射注射AA型型BTBT初治有效率为初治有效率为828255。本方法不良。本方法不良反应轻微、操作简便、痛苦小、安全可靠。对无反应轻微、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。法手术、无法行气囊扩张的患者更为适宜。n n 2 扩张治疗n n 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。手术治疗n n 开放式食管下括约肌 开放式食管下括约肌(Heller(Heller 肌 肌)切开术 切开术n n 开放式 开放式Heller Heller 肌切开手术分为经腹和经胸 肌切开手术分为经腹和经胸2 2 种，手术的目的 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，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 常用的是改良Heller Heller 手术。手术适应证包括临床诊断的贲 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。门失弛缓症，无黏膜病变，无手术禁忌证均可手术治疗。n n 手术要点是经胸或经腹暴露扩张、狭窄的病段食管，根据 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 狭窄长度，沿食管纵轴垂直切开食管侧肌层约6 cm 6 cm，胃底，胃底侧 侧 1 1 3 cm 3 cm，完全切断狭窄环，并在黏膜外剥离被切开的，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 肌层，使其达到食管周径的1 1 2 2。蒋俭等。蒋俭等 报道开放手术术 报道开放手术术后症状改善率为 后症状改善率为96 96 9 9。早期并发症主要为食管穿孔，。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 晚期主要为胃食管反流，发生率50 50 以上。以上。腔镜下食管下括约肌(Heller 肌)切开术n n 1991 1991 年 年Shimi Shimi 等 等 率先施行腹腔镜 率先施行腹腔镜Heller Heller 肌切开术，肌切开术，n n 1992 1992 年 年Pellegrini Pellegrini 等 等 首次施行胸腔镜 首次施行胸腔镜Heller Heller 肌切开术。肌切开术。n n Patti Patti 等 等 回顾了近十年来贲门失弛缓症治疗的变化趋势，总 回顾了近十年来贲门失弛缓症治疗的变化趋势，总结出腔镜下 结出腔镜下Heller Heller 肌切开术手术具有传统手术的有效性，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 手术操作简便、创伤小、缩短术后住院13 13 和康复时间，降 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 低术后死亡率，并发症和开放手术相当，腔镜下Heller Heller 肌 肌切开手术已经成为手术治疗首选。切开手术已经成为手术治疗首选。Robe Robe 等 等 报道 报道36 36 例腹腔 例腹腔镜 镜Heller Heller 肌切开术，手术优良率 肌切开术，手术优良率94 94 4 4，术中黏膜穿孔发，术中黏膜穿孔发生率 生率8 8 3 3，术后胃食管反流发生率仅为，术后胃食管反流发生率仅为8 8 3 3。刘隆。刘隆等 等 报道 报道25 25 例腹腔镜 例腹腔镜HellerDor HellerDor 手术，术后 手术，术后92 92 的患者吞 的患者吞咽功能恢复良好。咽功能恢复良好。机器人辅助微创手术n n随着手术机器人达芬奇、宙斯的出现，机器人腹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。腔镜手术很快应用到外科各个领域。20002000年年77月月MelvinMelvin等等 报道首例机器人辅助腹腔镜食管报道首例机器人辅助腹腔镜食管HellerHeller肌切开术。他们认为机器人腹腔镜手术具有三维肌切开术。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。更稳定，准确性更高的优点。20052005年年HorganHorgan等等 报报道机器人辅助腹腔镜食管贲门括约肌切开术比普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，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。且手术前安置机器的时间比较长，手术总时间长。目前存在的争论n n 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。n n 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。是否需要联合抗反流手术n n Heller Heller 肌切开术是否联合抗反流手术是目前争论的主要问 肌切开术是否联合抗反流手术是目前争论的主要问题。反对常规使用抗反流手术的人认为单纯 题。反对常规使用抗反流手术的人认为单纯Heller Heller 肌切开 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 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。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 Dempsey 等 等 对比 对比29 29 例 例Heller Heller 肌切开联合 肌切开联合Dor Dor 折叠术和 折叠术和22 22 例单 例单纯 纯Heller Heller 肌切开，肌切开，2 2 组病人在症状的改善、术后吞咽困难及 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 烧心的症状评分均一样，提示Dor Dor 前折叠对手术疗效无明 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 显影响。认为需要联合抗反流手术的学者认为Heller Heller 肌层 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 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。的主要原因。Mahhaner Mahhaner 等 等 州报道单纯 州报道单纯Heller Heller 肌切开术后 肌切开术后20 20年胃食管反流的发生率可达到 年胃食管反流的发生率可达到78 78。抗反流手术可有效降。抗反流手术可有效降低手术后胃食管反流率，低手术后胃食管反流率，Richards Richards 等 等 在一项随机对照试验 在一项随机对照试验中比较了 中比较了HellerDor HellerDor 手术与单纯 手术与单纯Heller Heller 手术疗效，发现前 手术疗效，发现前者术后病理性胃食管反流仅为 者术后病理性胃食管反