Case Discussion <Brief History > This 68-year-old woman suffered from intermittent postprandial vomiting and acid regurgitation for about 20 years. Chest X-ray at OPD showed dilated proximal esophagus with air fluid level (fig 1). Upper GI studies (fig 2) revealed dilated esophagus with beak-like narrowing. Esophageal manometry (fig 3, fig 4 ) revealed elevated basal LES (lower esophageal sphincter) pressure (38-44 mmHg) and incomplete relaxation of LES. Aggravating postprandial vomiting was found in recent one month. Vomiting occurred even when she was just drinking water. Dysphagia, mid-chest pain, and epigastralgia accompanied. Dyspnea developed for one week with cough, chills, and sticky sputum. Therefore, she was sent to the ER, where leukocytosis with left shift was disclosed. Under the impression of achalasia with suspected aspiration pneumonia, she was admitted for further management. On physical examinations at our ER, the body temperature was 38.0℃, blood pressure 126/80 mmHg, pulse rate 84 /min, and the respiratory rate 24 /min. Her consciousness was clear. The conjunctivae were not pale and sclerae were not icteric. Light reflex of the pupils was prompt and symmetric. The neck was supple and there was no lymphadenopathy or jugular vein engorgement. The chest wall was symmetrically expanded. Diffuse crackles at left lower to middle lung field were noted on ascultation. The heart beats were regular. No heart murmur was audible. The abdomen was flat and soft on palpation. There was no tenderness or rebound tenderness. The extremities were free movable and there was no cyanosis, clubbing or pitting edema. The peripheral pulse was palpable. <Lab> 1. CBC WBC RBC HB HCT MCV PLT  Seg Eos. Baso. Mono. Lym K/μL M/μL g/dL  % fL K/μL % % % %   9.14 3.93 11.6 36.3 92.4 187.0  84.7 0.3 0.2 7.0 7.8 2. BCS +e- Alb  Glo  T-BIL D-BIL AST ALT BUN CRE Na K g/dL g/dL mg/dL mg/dL U/L U/L mg/dL mg/dL mmol/L mmol/L 3.6 2.6 0.6 0.2 18.0 8.0 26.0 0.7 141.0 3.7 <Course and Treatment> Chest X-ray on admission (fig 5 ) showed left lower lobe pneumonia. Antibiotic with Unasyn was prescribed empirically for possible aspiration pneumonia. Her symptoms improved gradually. Then, she received surgical treatment for achalasia with modified Heller esophagomyotomy. After operation, her dysphagia resolved and she was discharged two weeks later. <題目解析> 這個個案以漸進式的Dysphagia來表現，之後因aspiration pneumonia 而入院治療，其barium swallowing study 為典型的鳥嘴形狀，乃高度懷疑診斷是achalasia，一般而言最常見的symptoms為Dysphagia (80.3%) 、Regurgitation (74.2%)、Weight loss (52.6%)及Chest pain (46.7%)。另外aspiration pneumonia也是其常見的併發症之一，約10-33%，為了確立診斷，安排了食道的manometry study，由結果圖中可以清楚的看出下食道括約肌的壓力升高，而整體的食道則呈現缺乏蠕動波的型態，故至此可以確立診斷為achalasia，隨後病人接受新式的modified laparoscopic Heller myotomy。關於achalasia的治療，可先從藥物治療開始，例如nifedipine及Isosorbide dinitrate等，而內科治療尚可選擇用Balloon Dilation，例如Rigiflex dilator等，或endoscopic Botulinum toxin injection，而外科治療則以Heller myotomy為主，可以較有效地改善症狀，療效也較能持久，但相對地，治療的risk也較高。 繼續教育考題 1. (D) 請問有關Achalasia的Chest X-ray表現何者為非? A absence of gastric air bubble, B a tubular mediastinal mass beside aorta C air-fluid level in mediastinum D right diaphragm elevation 2. (B) 請問以下何者非Achalasia的特徵? A 下食道括約肌(LES; lower esophageal sphincter)的壓力增加 B LES的壓力下降 C LES的放鬆無法完全 D 食道不蠕動(aperistalsis) 3. (C) 老年人的achalasia要小心為Secondary achalasia, 尤其因惡性腫瘤引起的secondary achalasia 更為大宗, 請問其機制以下何種為非? (1) 由食道及胃交界處的腫瘤引起 (2) 因為食道肌肉退化 (3) 副腫瘤症候群 (paraneoplastic syndrome) (4) 因毒素累積過多 A (1), (2) B (2), (4) C (1), (3) D (1), (2), (3) 4. (D) 請問以下何者較不須考慮 secondary achalasia? A 吞嚥困難時間小於一年者 B 年紀大於60歲者 C 體重下降者(>15磅) D 年輕時曾有反復嗆到的經驗 5. (B) 請問有關Achalasia的鋇劑吞嚥法(barium swallowing study)何者為是? (1)鋇劑吞嚥法(barium swallowing study) 通常誤診率相當高, 不能拿來當做鑑別診斷的工具 (2)正確性高, 一般說來可有95% 正確性 (3) 其特徵為放鬆的食道, 但其尾端為鳥嘴狀(beak-like narrowing) (4) 有時候也會看到整段都緊縮的食道 A (1), (3) B (2), (3) C (2), (3), (4) D (1), (3), (4) 6. (A) Pneumatic dilation也是另外一種治療Achalasia的方法,請問以下何者為對? (1)一次的pneumatic dilation 成功率(response)大約是60~85% (2) 連續三次的pneumatic dilation成功率大概只有70% (3) Esophageal perforation是最常見的併發症(complication) (4) Acute myocardial ischemia是最常見的併發症 (5) 有些人會有reflux esophagitis的併發症 A (1), (3), (5) B (2), (3), (5) C (1), (4), (5) D (2), (4), (5) 7. (D) 請問下列敘述有關achalasia的epidemiology何者正確? (1)好發於男性 (2)好發於女性 (3) 男女ㄧ樣多 (4) 通常只有固狀食物吞不大下去 (dysphagia) (5) 不論是固狀或液狀食物都吞不大下去 A (1), (4) B (2), (4) C (3), (4) D (3), (5) 8. (C) Achalasia會增加罹患esophageal cancer 的機會，請問以下敘述何者為錯? (1)主要是以鱗狀細胞癌(squamous cell carcinoma)為主 (2)主要是以腺癌(adenocarcinoma)為主 (3) ㄧ般平均在第一次診斷Achalasia後約三年內會得esophageal cancer A (1) B (1), (3) C (2), (3) D (1), (2) 9. (B) 關於Achalasia的藥物治療可用以下哪些? (1) nitrates (2) ACEI (3)β-blocker (4) Calcium-channel blocker A (1), (3) B (1), (4) C (2), (3) D (2), (4) 10. (D) Achalasia的手術治療方式, 以下何種為是? A Nissan fundoplication B Toupet fundoplication C Hill repair D Heller myomectomy 答案解說 (D)Achalasia的Chest X-ray表現應為absence of gastric air bubble, a tubular mediastinal mass beside aorta, 及air-fluid level in mediastinum (B)Achalasia的特徵為lack of primary peristasis, increased resting LES pressure, incomplete or only brief LES relaxation (C)Secondary achalasia, 尤其因惡性腫瘤引起的secondary achalasia 更為大宗, 其機制為： 由食道及胃交界處的腫瘤引起以及副腫瘤症候群 (paraneoplastic syndrome) (D)secondary achalasia通常表現於吞嚥困難時間小於一年者、年紀大於60歲者以及體重下降者(>15磅) (B)Achalasia的鋇劑吞嚥法(barium swallowing study)其正確性高, 一般說來可有95% 正確性，特徵為 dilated esophagus with fluid level, tapering of distal esophagus to narrow “bird beak-like”, aperistalsis on fluoroscopy (A)Pneumatic dilation治療Achalasia：一次的pneumatic dilation 成功率(response)大約是60~85% ，Esophageal perforation是最常見的併發症(complication) (D)achalasia：男女ㄧ樣多，dysphagia是固狀或液狀食物都吞不大下去 (C)Achalasia會增加罹患esophageal cancer 的機會，主要是以鱗狀細胞癌(squamous cell carcinoma)為主  (B)Achalasia的藥物治療可用nitrates， Calcium-channel blocker (D)Achalasia的手術治療方式, 為Heller myomectomy