目前分類：漏斗胸知識 (134)
- Jan 14 Fri 2011 11:55
漏斗胸常見的外觀及呼吸情形－－影片
- Dec 01 Wed 2010 14:39
不對稱型凹胸的矯治
不論是傳統手術或是納氏微創手術, 不對稱型的凹胸治療都是一項挑戰, 但納氏手術發展至今, 及技術的成熟, 已有方法讓這類困難的矯治有了良好的結果, 重點就在於仔細的術前評估, 及特殊的 矯正板的弧度設計及支撐點的選擇. 有時斜置的矯治板的方式也能讓不同凹陷程度的矯治.
- Nov 01 Mon 2010 17:11
困難的胸型治療
Dr. Park 所發表的分類表, 仍不足以包括所有的胸形異常, 對特殊的胸形異常, 就需要特別的設計,才能到達良好的結果.
病例一:
如圖所示, 胸部右側嵴形隆起, 在矯治上 需評估各肋骨及胸骨之應力點及製作適合之矯形背架, 才能到達理想效果. 此一病友不需手術, 只配合穿戴式背架即可.
病例二:
雙側不對側凹陷, 前胸不平整, 雙側肋骨呈Z狀曲折, 以改良式納氏手術, 斜置矯正板, 並做波形編穿法, 有良好的整體效果.
- Oct 27 Wed 2010 13:32
Nuss手術前後心臟受壓迫情況改善情形
雖然有許多病友們並沒有臨床症狀, 而是為了胸形外觀而求診, 但是在檢查中卻發現心臟在長期壓迫下已有傳導及心肌病變了,更不用說大部分的病友們多伴有心三尖瓣逆流, 肺動脈瓣逆流, 心二尖瓣脫垂等問題. 這兩張手術前後以胸腔鏡觀察, 可以見到原來被壓的變較扁的心臟, 在矯正板將胸壁抬高後, 已能呈現正常的外觀(包在心包膜內)
- Sep 27 Mon 2010 20:53
移除矯正板的方法(影片)
許多醫師及病友們在問如何移除矯正板, 謹將我們所研發的方式, 介紹如下:
http://www.youtube.com/watch?v=EisidDJGbv8
這種方式比Nuss及Park等使用的方法更"微創", 不但手術時間短, 出血少, 傷口小, 且復原快.
歡迎各醫師們參考使用
http://www.youtube.com/watch?v=EisidDJGbv8
這種方式比Nuss及Park等使用的方法更"微創", 不但手術時間短, 出血少, 傷口小, 且復原快.
歡迎各醫師們參考使用
- Sep 19 Sun 2010 20:33
嬰兒上呼吸道阻塞或狹窄, 易引起胸腔負壓, 造成凹胸
因胸部肺吸氣時有賴橫膈收縮造成胸內負壓,因此需有肋骨支撐住胸廓, 但胸前壁在嬰兒期是由分節的胸骨及肋軟骨形成, 並無足夠的支撐力, 需有右心室的正壓, 才能維持略凸的正常胸形. 漏斗胸可能因嬰兒期胸中膈負壓所引起. Wooler 在Thorax (1969, 24, 557)發表pectus excavatum一文中呼應了這一點, 他說 Dr. Olive Scott 發現一位七個月大嬰兒有先天性的喉部狹窄, 他為這嬰兒做了心導管並測量了右心室壓力, 發現右心室舒張期壓力達到- 18 mm. Hg; 而這嬰兒在日後發展成嚴重漏斗胸.
- Sep 06 Mon 2010 13:56
漏斗胸病人治療指引--2011年1月版
漏斗胸納式手術衛教手冊
這本手冊目的是要幫助您及您的家屬能了解有關漏斗胸及手術的情形，解答您手術前以及術後的疑問。希望經由簡單的解釋能讓您了解整個住院治療的過程。
當您讀完這本手冊，若有問題歡迎您記錄下來，可向醫師及護理人員詢問，我們會竭盡全力為您服務。
什麼是漏斗胸?
漏斗胸是前胸壁肋骨、 胸骨及肋軟骨生長異常，因而造成胸廓向內凹陷（對稱或是不對稱型態），是胸廓異常最常見的一種疾病。發生率約為1/300；男女比約為4：1。大約1/4病人有家族史。往往隨著嬰兒生長，凹陷情況漸漸明顯，而將胸骨向內凹，不但減少了胸腔容量，也壓迫心臟及肺，造成心瓣膜異常，也影響心臟的血液充灌注及心肌異常。此外心及肺的生長空間受限，肺容量亦降低。日常活動或許沒有明顯的影響，但在持久的運動時或年紀漸長時，則可能會影響會變得明顯。可能的日常表現或症狀：凹胸、突腹、垂肩、駝背、脊柱側曲、呼吸短促、運動耐受性差、胸痛、感冒持續久，往往這些症狀年紀越大越明顯。
關於納式手術（Nuss procedure, 漏斗胸微創手術）
漏斗胸應儘早治療，在以往採用傳統(切除肋軟骨及胸骨折斷)手術時，只要是兒童能承受，是越早越好。但在納式手術，因要有彈性適中的肋骨支撐起矯正板，則要考慮到太早(肋骨太軟，不易撐起)或太慢(肋骨太硬，不易抬高)，治療可能外觀效果會較差。
一般來說，納式手術在2歲至45歲間都可使用，但是以5歲至18歲間治療，外觀的效果最好。但對較嚴重且心肺壓迫明顯的病例，則應優先考慮到減緩心肺的壓迫，儘早治療。30歲以上的病患或廣泛型的凹陷，有可能要用到兩條以上的矯正板。
所謂漏斗胸微創手術（納式手術），是因不同於以往切除六到八對肋軟骨的方式，而改用由側面或腋下小切口，置入U形支撐架，將向內凹的胸骨及肋軟骨撐出，不必切除任何組織，等二至三年後胸廓定形後，再由原刀口取出。在胸腔鏡的輔助下，一般來說手術很安全，出血量約10 至30 cc.
納式手術的方式：
1、依病患個別情況，量製適合的矯正板。
2、在胸腔鏡之導引下，以導引器由右側胸壁之小切口進入胸腔，並穿過凹陷的胸骨下方，再由左側胸壁穿出。
3、將矯正板以凹面朝上的方向置入，再翻轉180度，以兩側肋骨支撐，使凹陷之胸壁得以被撐起。
4、將矯正板固定於胸壁上，必要時會使用固定片（stabilizer）與矯正板結合，以增加穩定性。
※與傳統手術不同之處：納式手術不需切除任何組織；刀口小，且位於兩側。
傷口的疼痛：
胸部會有彈性繃帶包紮，大約第二天可移除，傷口不必要每日換藥。手術中即為開始使用PCA（自控式止痛）或硬膜外止痛裝置，疼痛問題也會隨著時間逐漸改善。適當的使用止痛藥物，可以減輕疼痛、增加舒適感。不必擔心影響傷口癒合以及成癮等問題，因為這些藥劑均由麻醉醫師自調配的安全劑量，也會因應個別差異而予以調整劑量，以符合每位病患的需求。
護理人員會使用疼痛臉譜量尺來評估您的疼痛狀況，疼痛的程度分別從0分（完全不痛）到10分（非常疼痛，痛到無法忍受），您可以表達當下的疼痛分數，作為醫師調整止痛藥物的參考。
入院當天
您可能會被安排當天早上辦理住院，我們希望至少有一位親友能在一旁陪伴您，除了給您情緒上支持外，當您在進行手術時，醫師能隨時找到家屬解釋病情。
□手術前檢查：
包含身高、體重、一般身體評估、胸部電腦斷層、肺功能、胸部X光、血液檢查，來了解您目前的狀況‧
□視情況需要會診心臟內科:
做心電圖或心臟超音波檢查，評估手術的安全性。
□填寫同意書:
醫師會向您以及您的家人解釋手術相關事宜，會請您填寫手術以及麻醉同意書。
□選擇止痛方式: PCA(病人自控式止痛) 或 硬膜外疼痛控制劑。
□禁食時間：
依照醫師囑咐的禁食時間，除了開立的藥物以外，不要吃任何東西（包括水），以避免麻醉時發生嘔吐而造成吸入性肺炎。手術前，會為您打上點滴以補充水分及養分。
因手術後可能因疼痛而無法深呼吸，或有說話無力［有時需要分幾個片段才能說完一個句子］難以咳嗽等情形，也易造成痰液蓄積在肺部，引起感染或是肺炎。所以當您手術後，呼吸訓練是相當重要的，我們會在手術前就安排呼吸治療師指導您使用呼吸訓練器Tri-Flow。
。
手術當天
□術前準備:
在確定要送往開刀房前，我們會請您換穿手術衣，並將項鍊、手錶、戒指飾品以及活動假牙取下，並卸除口紅及指甲油。若有貴重用物請交由家人隨身保管。
□在手術房:
由於手術會在全身麻醉下執行，在麻醉醫師評估施打讓您睡著的藥物後，才會進行插管（氣管內管或者放置尿管）及手術，您不會感覺到任何不舒服。
手術時間依照每個病人狀況不同而有些許差異，細節您可以提出跟醫師討論，一般而言大約在1~2小時之內會完成。當您在接受手術時，家屬會在手術室外家屬等候區等候。在您手術完成時，您的家屬可以看到您從開刀房被護送到恢復室或加護病房。
□在加護病房
此時您身上會有的管路包含：
1.鼻用氧氣導管：輔助氧氣給予，當您呼吸平穩而且含氧量穩定並呈現約SPO2（血氧濃度）＞95％，會視情況幫您移除。
2.靜脈導管：在您手上會有軟針（點滴以及自控式止痛（PCA）給予的路徑）。
3.尿管：由於術後需要絕對臥床休息，成年病人多半會主訴沒辦法躺著小便，因而會有膀胱脹尿的情形，所以有必要時在手術進行時會幫您留置尿管。當您醒來後，感覺會有一點點不舒服或有尿意的感覺，都是正常的現象。
※入住加護病房注意事項
一般而言手術完成後會送到加護病房觀察一天，待生命徵象及狀況穩定後，會在第二天轉回一般病房。加護病房探訪時間：
10:00~10:30 17:00~17:30
加護病房病人需要休息及嚴密照護，為避免干擾醫療治療以及避免感染，所以無法讓家屬在加護病房中陪伴。但是若是年紀太小的小朋友，為增加病童安全感，我們會讓媽媽（一位家屬）在旁陪伴。
當會客時間開放時，每一次只能接受2位家屬進入會客，如果有許多家屬前來，須以輪流探視的方式。家屬進入加護病房前，請先洗手（可用門口乾洗手液）再穿上櫃內的黃色隔離服（依照床位號碼取用，以避免感染）。必要時請戴口罩，探視完畢後請記得再洗手一次，才能保護訪客避免感染。
準備用物：由於只會在加護病房住一夜，當您手術後轉至加護病房時，可將盥洗用具（牙膏、牙刷、毛巾、衛生紙…等）交由護理人員，其他用物可放置病房或交由家屬帶回。
手術後第一~二天(轉回病房)
□檢查: 胸部X光
□移除胸口彈性繃帶
□活動:可坐於床上進食。不可扭轉身體。不可作翻滾（翻身）的動作。
呼吸功能的訓練：
手術後第三~四天
□移除尿管；可能會略有不適，但只需二三秒，不會造成您的痛苦。
□停止點滴；藥物改成口服。
□活動:1. 持續Tri-Flow使用
2.可下床活動，起身時須由他人輔助。不可彎曲胸部及腰部。
手術後傷口仍痛時，怎麼起床：搖高床頭45度，單腳先著地，家屬雙手再由病患背部支托順勢扶起。
手術後第五天(出院)
□辦理出院
□安排回診日期:
□出院後的日常活動
為了維持矯正板穩定，應保持良好的姿勢，勿彎腰駝背，避免趴在桌上睡或俯身洗頭等動作。
隨時保持傷口乾燥。
1週經醫師診察同意後可以洗澡或淋浴。
2-3週-以恢復正常的生活活動。
2個月內避免提重物，尤其是重的學校上課背包。
2個月勿做接觸性、扭轉身體及爆發力量等運動，如括空手道、柔道、網球、高爾夫球等，但可游泳、自行車、慢跑等。
須立即求診的症狀：
體溫超過38.5℃。 持續咳嗽或任何呼吸的問題。
胸痛，尤其在深呼吸時。任何胸部創傷（可能會導致矯正板移位）。
手術傷口部位紅、腫、熱、痛。
漏斗胸之友協會：
部落格： http://tw.myblog.yahoo.com/pectus-excavatum
電話： 秘書邱寶釵 高秋桂 (02) 27713161轉123
這本手冊目的是要幫助您及您的家屬能了解有關漏斗胸及手術的情形，解答您手術前以及術後的疑問。希望經由簡單的解釋能讓您了解整個住院治療的過程。
當您讀完這本手冊，若有問題歡迎您記錄下來，可向醫師及護理人員詢問，我們會竭盡全力為您服務。
什麼是漏斗胸?
漏斗胸是前胸壁肋骨、 胸骨及肋軟骨生長異常，因而造成胸廓向內凹陷（對稱或是不對稱型態），是胸廓異常最常見的一種疾病。發生率約為1/300；男女比約為4：1。大約1/4病人有家族史。往往隨著嬰兒生長，凹陷情況漸漸明顯，而將胸骨向內凹，不但減少了胸腔容量，也壓迫心臟及肺，造成心瓣膜異常，也影響心臟的血液充灌注及心肌異常。此外心及肺的生長空間受限，肺容量亦降低。日常活動或許沒有明顯的影響，但在持久的運動時或年紀漸長時，則可能會影響會變得明顯。可能的日常表現或症狀：凹胸、突腹、垂肩、駝背、脊柱側曲、呼吸短促、運動耐受性差、胸痛、感冒持續久，往往這些症狀年紀越大越明顯。
關於納式手術（Nuss procedure, 漏斗胸微創手術）
漏斗胸應儘早治療，在以往採用傳統(切除肋軟骨及胸骨折斷)手術時，只要是兒童能承受，是越早越好。但在納式手術，因要有彈性適中的肋骨支撐起矯正板，則要考慮到太早(肋骨太軟，不易撐起)或太慢(肋骨太硬，不易抬高)，治療可能外觀效果會較差。
一般來說，納式手術在2歲至45歲間都可使用，但是以5歲至18歲間治療，外觀的效果最好。但對較嚴重且心肺壓迫明顯的病例，則應優先考慮到減緩心肺的壓迫，儘早治療。30歲以上的病患或廣泛型的凹陷，有可能要用到兩條以上的矯正板。
所謂漏斗胸微創手術（納式手術），是因不同於以往切除六到八對肋軟骨的方式，而改用由側面或腋下小切口，置入U形支撐架，將向內凹的胸骨及肋軟骨撐出，不必切除任何組織，等二至三年後胸廓定形後，再由原刀口取出。在胸腔鏡的輔助下，一般來說手術很安全，出血量約10 至30 cc.
納式手術的方式：
1、依病患個別情況，量製適合的矯正板。
2、在胸腔鏡之導引下，以導引器由右側胸壁之小切口進入胸腔，並穿過凹陷的胸骨下方，再由左側胸壁穿出。
3、將矯正板以凹面朝上的方向置入，再翻轉180度，以兩側肋骨支撐，使凹陷之胸壁得以被撐起。
4、將矯正板固定於胸壁上，必要時會使用固定片（stabilizer）與矯正板結合，以增加穩定性。
※與傳統手術不同之處：納式手術不需切除任何組織；刀口小，且位於兩側。
傷口的疼痛：
胸部會有彈性繃帶包紮，大約第二天可移除，傷口不必要每日換藥。手術中即為開始使用PCA（自控式止痛）或硬膜外止痛裝置，疼痛問題也會隨著時間逐漸改善。適當的使用止痛藥物，可以減輕疼痛、增加舒適感。不必擔心影響傷口癒合以及成癮等問題，因為這些藥劑均由麻醉醫師自調配的安全劑量，也會因應個別差異而予以調整劑量，以符合每位病患的需求。
護理人員會使用疼痛臉譜量尺來評估您的疼痛狀況，疼痛的程度分別從0分（完全不痛）到10分（非常疼痛，痛到無法忍受），您可以表達當下的疼痛分數，作為醫師調整止痛藥物的參考。
入院當天
您可能會被安排當天早上辦理住院，我們希望至少有一位親友能在一旁陪伴您，除了給您情緒上支持外，當您在進行手術時，醫師能隨時找到家屬解釋病情。
□手術前檢查：
包含身高、體重、一般身體評估、胸部電腦斷層、肺功能、胸部X光、血液檢查，來了解您目前的狀況‧
□視情況需要會診心臟內科:
做心電圖或心臟超音波檢查，評估手術的安全性。
□填寫同意書:
醫師會向您以及您的家人解釋手術相關事宜，會請您填寫手術以及麻醉同意書。
□選擇止痛方式: PCA(病人自控式止痛) 或 硬膜外疼痛控制劑。
□禁食時間：
依照醫師囑咐的禁食時間，除了開立的藥物以外，不要吃任何東西（包括水），以避免麻醉時發生嘔吐而造成吸入性肺炎。手術前，會為您打上點滴以補充水分及養分。
因手術後可能因疼痛而無法深呼吸，或有說話無力［有時需要分幾個片段才能說完一個句子］難以咳嗽等情形，也易造成痰液蓄積在肺部，引起感染或是肺炎。所以當您手術後，呼吸訓練是相當重要的，我們會在手術前就安排呼吸治療師指導您使用呼吸訓練器Tri-Flow。
。
手術當天
□術前準備:
在確定要送往開刀房前，我們會請您換穿手術衣，並將項鍊、手錶、戒指飾品以及活動假牙取下，並卸除口紅及指甲油。若有貴重用物請交由家人隨身保管。
□在手術房:
由於手術會在全身麻醉下執行，在麻醉醫師評估施打讓您睡著的藥物後，才會進行插管（氣管內管或者放置尿管）及手術，您不會感覺到任何不舒服。
手術時間依照每個病人狀況不同而有些許差異，細節您可以提出跟醫師討論，一般而言大約在1~2小時之內會完成。當您在接受手術時，家屬會在手術室外家屬等候區等候。在您手術完成時，您的家屬可以看到您從開刀房被護送到恢復室或加護病房。
□在加護病房
此時您身上會有的管路包含：
1.鼻用氧氣導管：輔助氧氣給予，當您呼吸平穩而且含氧量穩定並呈現約SPO2（血氧濃度）＞95％，會視情況幫您移除。
2.靜脈導管：在您手上會有軟針（點滴以及自控式止痛（PCA）給予的路徑）。
3.尿管：由於術後需要絕對臥床休息，成年病人多半會主訴沒辦法躺著小便，因而會有膀胱脹尿的情形，所以有必要時在手術進行時會幫您留置尿管。當您醒來後，感覺會有一點點不舒服或有尿意的感覺，都是正常的現象。
※入住加護病房注意事項
一般而言手術完成後會送到加護病房觀察一天，待生命徵象及狀況穩定後，會在第二天轉回一般病房。加護病房探訪時間：
10:00~10:30 17:00~17:30
加護病房病人需要休息及嚴密照護，為避免干擾醫療治療以及避免感染，所以無法讓家屬在加護病房中陪伴。但是若是年紀太小的小朋友，為增加病童安全感，我們會讓媽媽（一位家屬）在旁陪伴。
當會客時間開放時，每一次只能接受2位家屬進入會客，如果有許多家屬前來，須以輪流探視的方式。家屬進入加護病房前，請先洗手（可用門口乾洗手液）再穿上櫃內的黃色隔離服（依照床位號碼取用，以避免感染）。必要時請戴口罩，探視完畢後請記得再洗手一次，才能保護訪客避免感染。
準備用物：由於只會在加護病房住一夜，當您手術後轉至加護病房時，可將盥洗用具（牙膏、牙刷、毛巾、衛生紙…等）交由護理人員，其他用物可放置病房或交由家屬帶回。
手術後第一~二天(轉回病房)
□檢查: 胸部X光
□移除胸口彈性繃帶
□活動:可坐於床上進食。不可扭轉身體。不可作翻滾（翻身）的動作。
呼吸功能的訓練：
手術後第三~四天
□移除尿管；可能會略有不適，但只需二三秒，不會造成您的痛苦。
□停止點滴；藥物改成口服。
□活動:1. 持續Tri-Flow使用
2.可下床活動，起身時須由他人輔助。不可彎曲胸部及腰部。
手術後傷口仍痛時，怎麼起床：搖高床頭45度，單腳先著地，家屬雙手再由病患背部支托順勢扶起。
手術後第五天(出院)
□辦理出院
□安排回診日期:
□出院後的日常活動
為了維持矯正板穩定，應保持良好的姿勢，勿彎腰駝背，避免趴在桌上睡或俯身洗頭等動作。
隨時保持傷口乾燥。
1週經醫師診察同意後可以洗澡或淋浴。
2-3週-以恢復正常的生活活動。
2個月內避免提重物，尤其是重的學校上課背包。
2個月勿做接觸性、扭轉身體及爆發力量等運動，如括空手道、柔道、網球、高爾夫球等，但可游泳、自行車、慢跑等。
須立即求診的症狀：
體溫超過38.5℃。 持續咳嗽或任何呼吸的問題。
胸痛，尤其在深呼吸時。任何胸部創傷（可能會導致矯正板移位）。
手術傷口部位紅、腫、熱、痛。
漏斗胸之友協會：
部落格： http://tw.myblog.yahoo.com/pectus-excavatum
電話： 秘書邱寶釵 高秋桂 (02) 27713161轉123
- Sep 06 Mon 2010 13:55
Dr. Nuss 2010年的成果--發表在Journal of Family Practice
因長期效果良好, 微創手術治療漏斗胸已被廣為接受, 近年來的技術改進, 如配合胸腔鏡之使用及固定片, 肋骨縫合固定及新的儀器設計等, 也降低了併發症發生率.
不同於需肋骨切除及胸骨折斷術等傳統手術方式, 微創手術失血量少, 手術時間短, 且不會造成胸壁纖維化等問題. 同時病友們也可更早回復正常生活. 尤其是對兒童, 無切除組織的方式, 不會造成胸壁的纖維化. 其他如吸盤或胸骨下置入磁鐵等其他微創手術方法及凸胸(雞胸)也多以背架方式取代廣泛性切除的傳統方式了.
INDICATIONS FOR SURGERY 手術適應症
無症狀的輕微至中度凹陷病人可先追踨檢查, 有症狀的及有明顯凹陷的病人, 需檢查心肺壓迫情形.
Best Age for Operation:手術的最佳時機
最好是在青春期前, 不但矯治效果最佳, 也不容易復發.同時心肺也能有足夠的空間生長. 近年來有些報告在20-50歲手術都有很好的結果.
手術後, 如能以深呼吸及常規地有氧運動, 刺激肺部, 可減少復發率. 同時, 矯正板移除後, 也要注意挺胸姿勢的保持, 可讓復凹率降至最低.
手術的適應症為符合下列二項:
1. 凹陷指數大於3.25.
2. 心臟呈現受壓迫, 位移, 二尖瓣脫垂, 心雜音, 及傳導障礙.
3.肺功能有限制性/阻塞性障礙.
4. 以前手術失敗病例
� MINIMALLY INVASIVE TECHNIQUES
在使用了胸腔鏡後, 我們在九百個手術中沒有發生過任何心或肺的傷害. 在右側胸腔鏡與兩側胸腔鏡及左側胸腔鏡, 有同樣好的結果.
幾條矯正板呢?
Number of bars
最初, 我們認為一條即足夠支撐, 但後來發現, 在年紀較大的病人,或凹陷範圍較廣的病例, 或馬凡氏症候群, 二條能提供便好的結果. 目前我們約有1/4是用二條矯正板.
At first we thought one support bar was sufficient, but we soon realized that two give better results in older patients or those with a diffuse (“saucer-shaped”) deformity or Marfans Syndrome. Now 26% of patients in our series receive two bars.
矯正板位移可在固定片的使用下明顯地減少發生的機會.
以前傳統手術失敗的病例, 可用微創手術治療. 我曾有74例再手術的經驗. 長期結果非常好的有88%.好的有 6%, 不良的約有6%.
We have done 74 reoperations. The immediate postoperative morbidity is increased, but the long-term results have been good to excellent in 88%, fair in 6%, and failed in 6%.
� COMPLICATIONS OF PECTUS REPAIR併發症
Bar displacement矯正板位移
使用固定片後,由8.9%降到2.3%. 後來更降到0.3%.
對材質過敏.
Nickel allergy
約有2%; 主要是在手術前對金屬或異位性過敏體質者. 可改用鈦金屬材質(台灣尚未引進)
可用低劑量類固醇治療.
植入物感染
Bar infection
發生率小於1%; 治療先用傷口引流及抗生素. 大多不需移除矯正板.
矯正板的動態
� DYNAMICS OF THE BAR
How long the bar stays in place: 要放多久才拿掉?
至少兩年; 最好三年 病人應回診並告知他們運動耐受性改善的情形.
Patients routinely report their exercise tolerance and endurance improves dramatically after the operation.
我們發現明顯地長期FVC(forceful vital capacity)及FVC1(第1秒內的FVC)肺功能改善, 但至少要手術後六個月才看得出來.
We have shown a significant long-term increase in FVC and FEV1, but this improvement takes at least 6 months to manifest.
併發症:
Early complications早期
Death: 0% 死亡率
Cardiac perforation: 0% 心傷害
Pneumothorax: 62.2% 氣胸
Requiring chest tube: 3% 需放胸管
Requiring aspiration: 0.3% 而抽吸
Hemothorax (surgical): 0.8%血胸
Pleural effusion requiring drainage: 0.3%胸腔積水且需引流
Pericarditis: 0.5% 心包膜炎
Pneumonia: 0.6%肺炎
Drug reaction: 3.9%藥物過敏
Transient Horner’s syndrome: 19.7%暫時性交感神經影響
Late complications晚期併發症
Bar displacement: 6%矯正板位移
Requiring revision: 3.2%需重置
- Before stabilizer: 8.9%在使用固定片前
- With stabilizer: 2.3%使用固定片後
- With PDS sutures: 0.1%以PDS線材
Bar infection: 0.6%感染
Requiring early removal: 需提早移除0.2%
Allergy: 2%過敏
Overcorrection: 2.9%過度矯正
Recurrence: 0.9%復發
* 以上為該院 939 位病人的統計
� HOW OUTCOMES IMPACT LIVES生活的影響
Cardiopulmonary function
Patients routinely report their exercise tolerance and endurance improves dramatically after the operation. Two-dimensional echocardiography in patients with severe pectus excavatum has shown cardiac compression causes decreased filling, which then results in decreased stroke volume and decreased cardiac output.22-24 Relieving pressure on the heart and allowing it to resume its normal configuration immediately increases stroke volume.
450
FIGURE 2 Long-term results show good to excellent outcomes
Results are for our cohort of primary surgery patients 1 year or more after bar removal, categorized by patient age at time of surgery.
The pectus deformity results in pulmonary restriction and in severe cases even paradoxical respiration. Correcting the deformity facilitates normal chest function. We have shown a significant long-term increase in forced vital capacity (FVC) and forced expiratory volume in 1 sec (FEV1), but this improvement takes at least 6 months to manifest.25,26
Fast Track
The best results occurred in the 7–12 and 13–18 age groups.
Body image
From a quality-of-life standpoint, patients with pectus excavatum have a poor body image. Their defensive camouflaging with poor posture and lifestyle restrictions—such as avoiding sports and social activities—can lead to depression. Studies have documented marked improvement in self-esteem and quality of life following minimally invasive correction.27,28
451
Long term results from our institution: 95.6% good to excellent
From 1987 through 2006, 939 patients had minimally invasive procedures at our facility, comprising 865 primary repairs and 74 reoperations. The male:female ratio was 4:1. Some 546 patients have had their bars removed, 462 of them for more than a year.
Median age of our cohort was 14.3 years (range 1–31). Echocardiography or CT scan, or both, noted cardiac compression in 84%. Mitral valve prolapse was noted in 14.5%. Preoperative resting pulmonary function testing (PFT) in 767 patients demonstrated FVC below 80% predicted value in 26%, FEV1 in 32%, and forced expiratory flow rate (FEF) of 25–75 in 41%.
We placed a single bar in 73.3% and two bars in the remainder. Most typically had epidural analgesia for 3 days. Median length of stay was 5 days.
Early complications
We reported no deaths nor cardiac perforations (TABLE 3). A small pneumothorax with spontaneous resolution is almost universal after thoracoscopy. Some 173 patients had transient Horner’s syndrome during thoracic epidural administration, but all resolved after catheter removal.
Late complications
Fifty-three patients had bar displacement, 28 of which warranted repositioning. The incidence of bar displacement dropped from 8.9% to 2.3% after stabilizers were introduced.
Six patients had infections, and two required early bar removal at 18 months. Two percent had bar allergies. Several patients had a history of metal allergy and so received titanium bars. The others were diagnosed postoperatively and treated with indomethacin or steroids. Three patients required early bar removal, two of whom received a titanium bar. Symptoms resolved in all three after bar removal.
Three percent developed a moderate overcorrection of their deformity and 0.4% developed a true carinatum deformity.
Overall results and follow-up
We evaluate patients postoperatively at 6 months for the first year, and then yearly. We classify results thus:
Excellent—total repair of the pectus excavatum and resolution of all associated symptoms.
Good—minor persistence or irregularity of the anterior chest wall with resolution of associated symptoms.
Fair—mild residual pectus excavatum without complete resolution of associated symptoms.
Poor—moderate recurrence and associated symptoms or the need for another repair or both.
Initial cosmetic and functional results were excellent in 92% of patients and good in 7.7%. One patient had a fair result, and two failed. Our long-term results in 459 patients who had their bar out for more than 1 year were excellent in 83.3%, good in 12.3%, fair in 1.7%, poor 2.7%. The best results occurred in the 7–12 and 13–18 age groups.
DN, MAK, RJO
不同於需肋骨切除及胸骨折斷術等傳統手術方式, 微創手術失血量少, 手術時間短, 且不會造成胸壁纖維化等問題. 同時病友們也可更早回復正常生活. 尤其是對兒童, 無切除組織的方式, 不會造成胸壁的纖維化. 其他如吸盤或胸骨下置入磁鐵等其他微創手術方法及凸胸(雞胸)也多以背架方式取代廣泛性切除的傳統方式了.
INDICATIONS FOR SURGERY 手術適應症
無症狀的輕微至中度凹陷病人可先追踨檢查, 有症狀的及有明顯凹陷的病人, 需檢查心肺壓迫情形.
Best Age for Operation:手術的最佳時機
最好是在青春期前, 不但矯治效果最佳, 也不容易復發.同時心肺也能有足夠的空間生長. 近年來有些報告在20-50歲手術都有很好的結果.
手術後, 如能以深呼吸及常規地有氧運動, 刺激肺部, 可減少復發率. 同時, 矯正板移除後, 也要注意挺胸姿勢的保持, 可讓復凹率降至最低.
手術的適應症為符合下列二項:
1. 凹陷指數大於3.25.
2. 心臟呈現受壓迫, 位移, 二尖瓣脫垂, 心雜音, 及傳導障礙.
3.肺功能有限制性/阻塞性障礙.
4. 以前手術失敗病例
� MINIMALLY INVASIVE TECHNIQUES
在使用了胸腔鏡後, 我們在九百個手術中沒有發生過任何心或肺的傷害. 在右側胸腔鏡與兩側胸腔鏡及左側胸腔鏡, 有同樣好的結果.
幾條矯正板呢?
Number of bars
最初, 我們認為一條即足夠支撐, 但後來發現, 在年紀較大的病人,或凹陷範圍較廣的病例, 或馬凡氏症候群, 二條能提供便好的結果. 目前我們約有1/4是用二條矯正板.
At first we thought one support bar was sufficient, but we soon realized that two give better results in older patients or those with a diffuse (“saucer-shaped”) deformity or Marfans Syndrome. Now 26% of patients in our series receive two bars.
矯正板位移可在固定片的使用下明顯地減少發生的機會.
以前傳統手術失敗的病例, 可用微創手術治療. 我曾有74例再手術的經驗. 長期結果非常好的有88%.好的有 6%, 不良的約有6%.
We have done 74 reoperations. The immediate postoperative morbidity is increased, but the long-term results have been good to excellent in 88%, fair in 6%, and failed in 6%.
� COMPLICATIONS OF PECTUS REPAIR併發症
Bar displacement矯正板位移
使用固定片後,由8.9%降到2.3%. 後來更降到0.3%.
對材質過敏.
Nickel allergy
約有2%; 主要是在手術前對金屬或異位性過敏體質者. 可改用鈦金屬材質(台灣尚未引進)
可用低劑量類固醇治療.
植入物感染
Bar infection
發生率小於1%; 治療先用傷口引流及抗生素. 大多不需移除矯正板.
矯正板的動態
� DYNAMICS OF THE BAR
How long the bar stays in place: 要放多久才拿掉?
至少兩年; 最好三年 病人應回診並告知他們運動耐受性改善的情形.
Patients routinely report their exercise tolerance and endurance improves dramatically after the operation.
我們發現明顯地長期FVC(forceful vital capacity)及FVC1(第1秒內的FVC)肺功能改善, 但至少要手術後六個月才看得出來.
We have shown a significant long-term increase in FVC and FEV1, but this improvement takes at least 6 months to manifest.
併發症:
Early complications早期
Death: 0% 死亡率
Cardiac perforation: 0% 心傷害
Pneumothorax: 62.2% 氣胸
Requiring chest tube: 3% 需放胸管
Requiring aspiration: 0.3% 而抽吸
Hemothorax (surgical): 0.8%血胸
Pleural effusion requiring drainage: 0.3%胸腔積水且需引流
Pericarditis: 0.5% 心包膜炎
Pneumonia: 0.6%肺炎
Drug reaction: 3.9%藥物過敏
Transient Horner’s syndrome: 19.7%暫時性交感神經影響
Late complications晚期併發症
Bar displacement: 6%矯正板位移
Requiring revision: 3.2%需重置
- Before stabilizer: 8.9%在使用固定片前
- With stabilizer: 2.3%使用固定片後
- With PDS sutures: 0.1%以PDS線材
Bar infection: 0.6%感染
Requiring early removal: 需提早移除0.2%
Allergy: 2%過敏
Overcorrection: 2.9%過度矯正
Recurrence: 0.9%復發
* 以上為該院 939 位病人的統計
� HOW OUTCOMES IMPACT LIVES生活的影響
Cardiopulmonary function
Patients routinely report their exercise tolerance and endurance improves dramatically after the operation. Two-dimensional echocardiography in patients with severe pectus excavatum has shown cardiac compression causes decreased filling, which then results in decreased stroke volume and decreased cardiac output.22-24 Relieving pressure on the heart and allowing it to resume its normal configuration immediately increases stroke volume.
450
FIGURE 2 Long-term results show good to excellent outcomes
Results are for our cohort of primary surgery patients 1 year or more after bar removal, categorized by patient age at time of surgery.
The pectus deformity results in pulmonary restriction and in severe cases even paradoxical respiration. Correcting the deformity facilitates normal chest function. We have shown a significant long-term increase in forced vital capacity (FVC) and forced expiratory volume in 1 sec (FEV1), but this improvement takes at least 6 months to manifest.25,26
Fast Track
The best results occurred in the 7–12 and 13–18 age groups.
Body image
From a quality-of-life standpoint, patients with pectus excavatum have a poor body image. Their defensive camouflaging with poor posture and lifestyle restrictions—such as avoiding sports and social activities—can lead to depression. Studies have documented marked improvement in self-esteem and quality of life following minimally invasive correction.27,28
451
Long term results from our institution: 95.6% good to excellent
From 1987 through 2006, 939 patients had minimally invasive procedures at our facility, comprising 865 primary repairs and 74 reoperations. The male:female ratio was 4:1. Some 546 patients have had their bars removed, 462 of them for more than a year.
Median age of our cohort was 14.3 years (range 1–31). Echocardiography or CT scan, or both, noted cardiac compression in 84%. Mitral valve prolapse was noted in 14.5%. Preoperative resting pulmonary function testing (PFT) in 767 patients demonstrated FVC below 80% predicted value in 26%, FEV1 in 32%, and forced expiratory flow rate (FEF) of 25–75 in 41%.
We placed a single bar in 73.3% and two bars in the remainder. Most typically had epidural analgesia for 3 days. Median length of stay was 5 days.
Early complications
We reported no deaths nor cardiac perforations (TABLE 3). A small pneumothorax with spontaneous resolution is almost universal after thoracoscopy. Some 173 patients had transient Horner’s syndrome during thoracic epidural administration, but all resolved after catheter removal.
Late complications
Fifty-three patients had bar displacement, 28 of which warranted repositioning. The incidence of bar displacement dropped from 8.9% to 2.3% after stabilizers were introduced.
Six patients had infections, and two required early bar removal at 18 months. Two percent had bar allergies. Several patients had a history of metal allergy and so received titanium bars. The others were diagnosed postoperatively and treated with indomethacin or steroids. Three patients required early bar removal, two of whom received a titanium bar. Symptoms resolved in all three after bar removal.
Three percent developed a moderate overcorrection of their deformity and 0.4% developed a true carinatum deformity.
Overall results and follow-up
We evaluate patients postoperatively at 6 months for the first year, and then yearly. We classify results thus:
Excellent—total repair of the pectus excavatum and resolution of all associated symptoms.
Good—minor persistence or irregularity of the anterior chest wall with resolution of associated symptoms.
Fair—mild residual pectus excavatum without complete resolution of associated symptoms.
Poor—moderate recurrence and associated symptoms or the need for another repair or both.
Initial cosmetic and functional results were excellent in 92% of patients and good in 7.7%. One patient had a fair result, and two failed. Our long-term results in 459 patients who had their bar out for more than 1 year were excellent in 83.3%, good in 12.3%, fair in 1.7%, poor 2.7%. The best results occurred in the 7–12 and 13–18 age groups.
DN, MAK, RJO
- Aug 09 Mon 2010 21:32
最近發現一些年輕漏斗胸病友們心臟已有缺血性變化
多年來, 許多醫師及病友們, 常以為漏斗胸只是外觀的問題, 最近我與心臟科醫師合作研究,發現許多廿多歲的年輕漏斗胸病友們心臟已有缺血性變化及心右側傳導束阻斷; 其實在1998著名的醫學雜誌Circulation 中已有德國的一篇病例報告. 僅摘譯如下:
Circulation. 1998;98:605-606
漏斗胸合併右側臥時心肌下缺氧
Pectus Excavatum With Inferior Ischemia in Right Lateral Position
Thomas A. Heitzer, MD; ; Helmut Wollschläger, MD
From the Medizinische Klinik III, Kardiologie, Universität Freiburg, Germany.
Correspondence to Thomas Heitzer, MD, Universitätskrankenhaus Eppendorf, Abteilung für Kardiologie, Martinistr 52, 20246 Hamburg, FRG.
一56歲漏斗胸之女性近兩年來右側臥時有漸漸加重的胸痛. 心電圖在右側臥時呈現ST段升高,但仰臥時則恢復正常. 胸部電腦斷層顯示漏斗胸嚴重胸骨凹陷,壓迫至上行段主動脈. 心冠狀動脈造影顯示仰臥時正常, 但右側臥時則呈現心臟右冠狀動脈近端被壓迫. 手術治療後, 病人不再有心絞痛.
A 56-year-old woman with pectus excavatum presented with a 2-year history of increasing chest pain only while she was lying on her right side. The ECG showed ST-segment elevation in inferior leads (A) when the patient was in the right lateral position and became normal when she turned onto her back. A CT of the thorax (B) showed the funnel-chest deformity and severe sternal depression, with close contact to the proximal part of the ascending aorta. Coronary angiography showed no coronary artery disease or any abnormality in supine position (D), but revealed proximal compression of the right coronary artery only when the patient lay on her right side (C).
Surgical treatment with repair of the chest deformity was successfully performed and made the patient angina free.
Circulation. 1998;98:605-606
漏斗胸合併右側臥時心肌下缺氧
Pectus Excavatum With Inferior Ischemia in Right Lateral Position
Thomas A. Heitzer, MD; ; Helmut Wollschläger, MD
From the Medizinische Klinik III, Kardiologie, Universität Freiburg, Germany.
Correspondence to Thomas Heitzer, MD, Universitätskrankenhaus Eppendorf, Abteilung für Kardiologie, Martinistr 52, 20246 Hamburg, FRG.
一56歲漏斗胸之女性近兩年來右側臥時有漸漸加重的胸痛. 心電圖在右側臥時呈現ST段升高,但仰臥時則恢復正常. 胸部電腦斷層顯示漏斗胸嚴重胸骨凹陷,壓迫至上行段主動脈. 心冠狀動脈造影顯示仰臥時正常, 但右側臥時則呈現心臟右冠狀動脈近端被壓迫. 手術治療後, 病人不再有心絞痛.
A 56-year-old woman with pectus excavatum presented with a 2-year history of increasing chest pain only while she was lying on her right side. The ECG showed ST-segment elevation in inferior leads (A) when the patient was in the right lateral position and became normal when she turned onto her back. A CT of the thorax (B) showed the funnel-chest deformity and severe sternal depression, with close contact to the proximal part of the ascending aorta. Coronary angiography showed no coronary artery disease or any abnormality in supine position (D), but revealed proximal compression of the right coronary artery only when the patient lay on her right side (C).
Surgical treatment with repair of the chest deformity was successfully performed and made the patient angina free.
- Jul 26 Mon 2010 22:52
論文摘要--漏斗胸的微創內視鏡手術治療
看到 Jacobs八年前的論文, 當時的手術數量及經驗也不多, 雖說是改良式納氏手術, 可是看來仍與Donald Nuss 的方法原汁原味
Eur J Cardiothorac Surg. 2002 May;21(5):869-73.
Minimally invasive endoscopic repair of pectus excavatum.
漏斗胸的微創內視鏡手術治療
Jacobs JP, Quintessenza JA, Morell VO, Botero LM, van Gelder HM, Tchervenkov CI.
Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/University of South Florida College of Medicine, Cardiac Surgical Associates,
摘要:
Abstract
OBJECTIVE: We report our initial 3 years 4 months' single institution experience in 31 consecutive patients with pectus excavatum treated with minimally invasive endoscopic pectus excavatum repair utilizing a modification of the 'Nuss' technique.
目的:報告三年四個月中單一機構之31例漏斗胸以改良式納氏微創手術經驗.
METHODS: Under general anesthesia, a curved steel bar is individually shaped for each patient to match the ideal chest wall shape and is placed through an endoscopically created retrosternal tunnel between two bilateral midaxillary line 2-cm incisions. The tunnels initially go along the outside of the rib cage, under the pectoral muscles. At the level of the sternum, these tunnels go retrosternal and communicate with each other. The steel bar is passed with the convexity facing posteriorly, within a protective flat silastic drain. Under endoscopic guidance, the curved steel bar is passed through one tunnel, under the sternum, and out the other tunnel. Once positioned, the bar is turned over, thereby correcting the deformity. An epidural catheter provides perioperative pain relief.
方法:在全身麻醉下, 由內視鏡輔助下, 由兩側鎖骨中線間產生胸骨後通道, 並由腋下之2公分刀口, 再置入以個別量製之弧形之金屬矯正板. 置入後, 再翻轉, 而矯正凹陷異常. 手術後以硬膜外止痛.
RESULTS: Minimally invasive endoscopic pectus excavatum repair has been performed on 31 patients (age: range 4.4-31.0 years, median 15.0 years, mean 14.5 years). Median hospital length of stay is 4 days (range 3-10 days, mean 4.6 days). Pneumothorax occurred in five patients requiring tube thoracostomy in three. One patient developed delayed bilateral pleural effusions requiring drainage. Two patients developed evidence of sterile seroma formation at the skin incision several months after minimally invasive repair of pectus excavatum. These seromas resolved with non-interventional conservative medical treatment. No other complications occurred.
結果:31位病人, 年齡由4.4歲至31歲, 平均14.5歲. 平均住院日由3至10天, 平均4.6天. 氣胸發生於5位病人, 其中有三人需置入胸管. 一位病人發生延遲性雙側肋膜積水而需引流, 兩位病人發生切口下積液, 無其他併發症發生.
CONCLUSION: The minimally invasive endoscopic pectus repair is safe and effective and currently our procedure of choice for primary pectus excavatum in all ages. Endoscopic visualization facilitates the safe creation of the retrosternal tunnel. Short-term results have been excellent. Further follow-up will be necessary to determine long-term results.
結論: 微創漏斗胸治療對任何年紀來說均是安全及有效的治療方法. 內視鏡能便利手術的安全進行. 短期看來, 效果良好, 未來需長期追踪其療效.
Eur J Cardiothorac Surg. 2002 May;21(5):869-73.
Minimally invasive endoscopic repair of pectus excavatum.
漏斗胸的微創內視鏡手術治療
Jacobs JP, Quintessenza JA, Morell VO, Botero LM, van Gelder HM, Tchervenkov CI.
Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/University of South Florida College of Medicine, Cardiac Surgical Associates,
摘要:
Abstract
OBJECTIVE: We report our initial 3 years 4 months' single institution experience in 31 consecutive patients with pectus excavatum treated with minimally invasive endoscopic pectus excavatum repair utilizing a modification of the 'Nuss' technique.
目的:報告三年四個月中單一機構之31例漏斗胸以改良式納氏微創手術經驗.
METHODS: Under general anesthesia, a curved steel bar is individually shaped for each patient to match the ideal chest wall shape and is placed through an endoscopically created retrosternal tunnel between two bilateral midaxillary line 2-cm incisions. The tunnels initially go along the outside of the rib cage, under the pectoral muscles. At the level of the sternum, these tunnels go retrosternal and communicate with each other. The steel bar is passed with the convexity facing posteriorly, within a protective flat silastic drain. Under endoscopic guidance, the curved steel bar is passed through one tunnel, under the sternum, and out the other tunnel. Once positioned, the bar is turned over, thereby correcting the deformity. An epidural catheter provides perioperative pain relief.
方法:在全身麻醉下, 由內視鏡輔助下, 由兩側鎖骨中線間產生胸骨後通道, 並由腋下之2公分刀口, 再置入以個別量製之弧形之金屬矯正板. 置入後, 再翻轉, 而矯正凹陷異常. 手術後以硬膜外止痛.
RESULTS: Minimally invasive endoscopic pectus excavatum repair has been performed on 31 patients (age: range 4.4-31.0 years, median 15.0 years, mean 14.5 years). Median hospital length of stay is 4 days (range 3-10 days, mean 4.6 days). Pneumothorax occurred in five patients requiring tube thoracostomy in three. One patient developed delayed bilateral pleural effusions requiring drainage. Two patients developed evidence of sterile seroma formation at the skin incision several months after minimally invasive repair of pectus excavatum. These seromas resolved with non-interventional conservative medical treatment. No other complications occurred.
結果:31位病人, 年齡由4.4歲至31歲, 平均14.5歲. 平均住院日由3至10天, 平均4.6天. 氣胸發生於5位病人, 其中有三人需置入胸管. 一位病人發生延遲性雙側肋膜積水而需引流, 兩位病人發生切口下積液, 無其他併發症發生.
CONCLUSION: The minimally invasive endoscopic pectus repair is safe and effective and currently our procedure of choice for primary pectus excavatum in all ages. Endoscopic visualization facilitates the safe creation of the retrosternal tunnel. Short-term results have been excellent. Further follow-up will be necessary to determine long-term results.
結論: 微創漏斗胸治療對任何年紀來說均是安全及有效的治療方法. 內視鏡能便利手術的安全進行. 短期看來, 效果良好, 未來需長期追踪其療效.
- Jul 25 Sun 2010 23:06
漏斗胸病人的運動呼吸困難--醫學論文摘錄
Exertional dyspnoea in patients with pectus excavatum
漏斗胸病人的運動呼吸困難
Paolo T. Pianosia
aPaolo T Pianosi MD is in the Department of Pediatrics, Divisions of Pediatric Allergy Immunology and Pulmonology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA
Available online 4 December 2009.
摘要:
大多數漏斗胸病人的運動耐受性及運動呼吸困難在手術後有改善. 而這些症狀改善的原因被推論為胸腔容積變大及壓迫的緩解. 但是呼吸肌的運動卻從未被認為是症狀改善的原因之一. 本文提出以一項新發展的方法可用來評估運動時之呼吸限制, 畫出在運動時最大通氣流速-容積的方法, 來解釋手術後改善的現象,
Abstract
Most patients with pectus excavatum report better exercise tolerance and less exertional dyspnoea following surgical repair of their chest wall deformity. The reason(s) for this symptomatic improvement remain speculative but include a reversal of deconditioning and improved stroke volume. Improved function or efficiency of the respiratory muscle pump has never been assessed as a potential reason for reduced dyspnoea. An emerging approach to assessing ventilatory constraint during exercise is to plot the tidal flow–volume loops obtained during exercise within the maximal volitional flow–volume envelope obtained at rest. This paper summarises a review of pulmonary function in patients with pectus excavatum before and after surgery, and then illustrates how this newer technique can be applied in order to determine the ventilatory limitation to exercise in this population.
Keywords: dyspnoea; exercise; pectus excavatum; ventilation
漏斗胸病人的運動呼吸困難
Paolo T. Pianosia
aPaolo T Pianosi MD is in the Department of Pediatrics, Divisions of Pediatric Allergy Immunology and Pulmonology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA
Available online 4 December 2009.
摘要:
大多數漏斗胸病人的運動耐受性及運動呼吸困難在手術後有改善. 而這些症狀改善的原因被推論為胸腔容積變大及壓迫的緩解. 但是呼吸肌的運動卻從未被認為是症狀改善的原因之一. 本文提出以一項新發展的方法可用來評估運動時之呼吸限制, 畫出在運動時最大通氣流速-容積的方法, 來解釋手術後改善的現象,
Abstract
Most patients with pectus excavatum report better exercise tolerance and less exertional dyspnoea following surgical repair of their chest wall deformity. The reason(s) for this symptomatic improvement remain speculative but include a reversal of deconditioning and improved stroke volume. Improved function or efficiency of the respiratory muscle pump has never been assessed as a potential reason for reduced dyspnoea. An emerging approach to assessing ventilatory constraint during exercise is to plot the tidal flow–volume loops obtained during exercise within the maximal volitional flow–volume envelope obtained at rest. This paper summarises a review of pulmonary function in patients with pectus excavatum before and after surgery, and then illustrates how this newer technique can be applied in order to determine the ventilatory limitation to exercise in this population.
Keywords: dyspnoea; exercise; pectus excavatum; ventilation
- Jul 22 Thu 2010 18:25
非為廣泛所採用手術的危險
由於以往以傳統手術治療漏斗胸的結果不甚理想, 許多醫師也嘗試改良, 因而許多改良式手術也被發展出來. 雖然, 醫學就是這樣進步的, 但手術的設計及材料的使用,仍要以病人的健康為最大考量; 許多病友們詢問不同的手術方式, 我們的建議, 最廣為醫界所接受的手術方式, 衛生署所核可的材料, , 才是經過驗證, 也是最安全的. 謹再以三例說明:
例一.
小茹在五歲時因嚴重漏斗胸而接受改良式Ravitch手術, 手術由胸前正中切口進行, 所不同者, 0胸骨下端以骨材固定物支撐, 大約在手術三年後, 小茹的胸部又再發生凹陷, X光發現, 原來之植入支撐物已斷成兩截. .
經Nuss手術治療, 已成功移除原植入物, 並將凹陷的胸壁撐起. .
檢討: 植入物本非用來支撐胸骨及前胸壁用, 故在形狀及材質及強度均不適合.
例二..
李先生早年因漏斗胸而接受手術, 將金屬網狀物綴補在胸前, 數年後, 胸壁復凹, 經檢查, 凹陷的胸壁中有一向內凹陷的金屬網狀物, 由於網狀物已與胸壁組織長合, 不易移除. 也難有再度的矯正機會..
例三
最離譜者, 竟發現植入物是樹脂石膏, 除了折斷外, 更引起周圍組織嚴重發炎反應. 
回覆病友們的問題,.
手術矯正經多年的發展, 已具有一定的治療水準, 但對創新且未廣被接受的手術方式, 仍需謹慎考量.
- Jul 21 Wed 2010 09:30
手術的時機
手術的時機:
最佳的年紀是在6至15歲之間, 但因病情, 由1至50歲的病友們都可接受手術.
漸漸加重的凹胸及駝背等等
症狀漸漸加重, 如運動能力及耐受力, 胸痛, 及呼吸短促等等及其他的症狀..
臨床檢查發現明顯凹胸合併心臟受壓迫及位置偏移與肺受壓迫症狀症狀
無症狀的病人接受運動訓練及姿勢改善, 六個月後重新評估, 如有症狀, 則可能評估手術之必要.
有症狀的病人接受電腦斷層檢查, 肺功能檢查, 及心臟檢查; 依檢查結果, 決定是否手術; 大致上, 如Haller index指數大於3.2, 有肺塌陷, 肺功能不良, 心臟受壓迫, 二尖瓣脫垂, 心雜音, 及心房心室傳導延遲等等.
病人如有如馬凡症候群, Ehlers-Danlos或波蘭症候群等.
History of progressive worsening of the pectus excavatum.
History of the symptoms related but not limited to exercise intolerance, chest pain and shortness of breath.
Clinical evaluation showing severe pectus with the demonstration of cardiac displacement and pulmonary compromise.
Asymptomatic patients are given an exercise program to correct their posture and are reevaluated every 6 months to follow their progress. Patients will be moved into the symptomatic group as indicated by their symptoms.
Symptomatic patients are sent for a CT scan, pulmonary-function studies and cardiology examinations. Surgery is supported by the results of the objective criteria obtained from these exams. The criteria includes a CT index (Haller index) of 3.2 or greater, atelectasis, abnormal pulmonary function, cardiac compression, mitral valve prolapse, heart murmurs and A-V conduction delay. These patients may have other abnormalities, such as Marfan's syndrome, Ehlers-Danlos or Poland's syndrome.
最佳的年紀是在6至15歲之間, 但因病情, 由1至50歲的病友們都可接受手術.
漸漸加重的凹胸及駝背等等
症狀漸漸加重, 如運動能力及耐受力, 胸痛, 及呼吸短促等等及其他的症狀..
臨床檢查發現明顯凹胸合併心臟受壓迫及位置偏移與肺受壓迫症狀症狀
無症狀的病人接受運動訓練及姿勢改善, 六個月後重新評估, 如有症狀, 則可能評估手術之必要.
有症狀的病人接受電腦斷層檢查, 肺功能檢查, 及心臟檢查; 依檢查結果, 決定是否手術; 大致上, 如Haller index指數大於3.2, 有肺塌陷, 肺功能不良, 心臟受壓迫, 二尖瓣脫垂, 心雜音, 及心房心室傳導延遲等等.
病人如有如馬凡症候群, Ehlers-Danlos或波蘭症候群等.
History of progressive worsening of the pectus excavatum.
History of the symptoms related but not limited to exercise intolerance, chest pain and shortness of breath.
Clinical evaluation showing severe pectus with the demonstration of cardiac displacement and pulmonary compromise.
Asymptomatic patients are given an exercise program to correct their posture and are reevaluated every 6 months to follow their progress. Patients will be moved into the symptomatic group as indicated by their symptoms.
Symptomatic patients are sent for a CT scan, pulmonary-function studies and cardiology examinations. Surgery is supported by the results of the objective criteria obtained from these exams. The criteria includes a CT index (Haller index) of 3.2 or greater, atelectasis, abnormal pulmonary function, cardiac compression, mitral valve prolapse, heart murmurs and A-V conduction delay. These patients may have other abnormalities, such as Marfan's syndrome, Ehlers-Danlos or Poland's syndrome.
- Jun 22 Tue 2010 20:57
手術後的運動--From 阿倫(假名)
朱醫生認為我恢復狀況滿良好的，所以希望我寫一些心得分享手術後的一些生活和其他小撇步。
但是因為我自己也不覺得自己很專業，而且很多撇步都是以前的網友分享過的，所以這篇文章實在寫的很惶恐阿～
我相信病友們剛做完手術精神很差希望快點恢復的應該不少，這就要靠有氧運動啦！
而且大部分都很瘦的漏斗胸病友們也應該希望自己可以順利壯點！這就要靠重訓啦！
剛手術完生活上很多小事情都很不方便，這就要靠網友們熱情分享自己的小撇步啦！
這邊就歸納一些我採用的方法，並且有請朱醫生和香港峰教練幫忙確認和修改，
期望能以這篇文章為大家帶來科學又有用的方法，快快恢復元氣！ 轟轟！！！
______________________
運動有氧
______________________
呼吸練習器:每天有空就做。
散步&爬樓梯:出院後 一日0.5~1小時 一週三次。
騎腳踏車:術後兩週 一日15~30mins 一週三~四次。（單車要選身體不是傾前那種）
游泳:術後一月 一日15~30mins 一週二~三次。蛙式為佳，自由式較易拉扯到固定器。
(這點峰教練覺得為了減少固定器位移的風險，最好還是考慮三個月後再游 )
慢跑:??? (怕會震動到矯正板，遲遲不敢跑。術後一月有用滑步機取代了。)
打球:???(這裡指籃球，我還不敢打，等術後三月後再考慮。但是為了固定器還是盡量能免就免。)
心得:
基本上我就是一週固定運動三到四天，一次約一小時。
隨著離手術後的日子增加來改變運動項目，慢慢加強強度和時間～
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運動重訓
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大腿:術後兩週 一週兩次 動作->蹲舉(建議使用機器式, 替代動作以下圖所示...)
＂(室)頭要指著第二腳指上落都是一樣,你圖沒有做到,留意留意~＂ From 峰
二頭&三頭&前臂:術後兩週 一週兩次 動作->立式二頭彎舉 法式臥推 前手臂伸屈肌動作
(例)立式二頭彎舉
"拳頭的方向性大部分是反過來，開始時手指公向前，完成動作之後才向內" From 峰(但我是習慣圖中那樣啦 XD)
肩膀&下腹:術後一月 一週兩次 動作->立式前平舉 坐式俯身飛鳥 直立划船 抬腿動作
(例)坐式俯身飛鳥
下腹方面盡量還是和其他軀幹部位一樣，在3~6月後做比較好，到時候峰教練有個推薦動作如下網址～
ＰＬＡＮＫ： http://www.youtube.com/watch?v=9Ar2iRusnnc
胸&背&上腹:???我在峰教練那邊得到的建議是盡量先不要練習軀幹部份，但胸我還是有用飛鳥偷偷練啦～
心得:
每一個動作都先使用100下訓練法約一個多月
（說明網址： http://blog.roodo.com/jinxiu/archives/2555775.html ）
打算這動作習慣後才漸進使用大重量鍛鍊～
其他沒有說明的動作ｇｏｏｇｌｅ都很容易搜尋到的～
最後峰教練有個愛心叮嚀是＂記得過肩的動作不可以做!!!轉屈身體的不可以做!!＂
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其他雜項
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飲食:一天五餐並多攝取一點點蛋白質，一開始有喝雞精和人蔘，後來有吃點維他命和消化酵素～
起床方式:為了平躺更舒服我是用守晨教我的方法佈置，起床則一手抓住自己在床腳綁的繩子一手抓自己的腳。
上班日:視情況術後10天可恢復上班，我自己是做資訊方面的研究，都在電腦前所以工作沒有大問題～
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自身舉例
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術後一週:每天都練呼吸練習器，並且一週散步三次左右。
術後兩週:一週踩腳踏車和手部和大腿練習各三輪，都是低強度高次數。
術後一月:騎腳踏車的時間改成游泳或是滑步機，訓練部位多了肩膀和下腹。
術後兩月:＂打算＂開始慢跑並且提升訓練的強度。
術後三月:＂希望＂可以開始練習軀幹部位和打籃球啦～
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- Jun 11 Fri 2010 20:42
由三個兒童漏斗胸手術治療中心, 以胸部電腦斷層檢查對漏斗胸兒童肺容積的評估
此次日本的太平洋小兒外科醫學會中,, 有一篇由兩岸的華人資料合併所做的分析報告.是由北京兒童醫院, 長庚醫院, 及朱志純醫師的病人們的資料分析得出, 僅在此列出摘要, 由於是華人的跨兩岸資料, 可供大多數病友們參考
A Cross-sectional Study of Lung Volume Development in Pectus Excavatum Patients: Estimating the Total Lung Volume from Chest CT using Three-dimensional Volumetric Reconstruction
(跨機構漏斗胸病人肺容積發育評估--以胸部電腦斷層三度容積重建來估算全肺容積)
這一篇是由 我, 北京兒童醫院, 及長庚醫院的病人電腦斷層分析, 計算其肺容量的結果
Background/Purpose: This study quantified the lung volume development of pectus excavatum (PE) patients using chest computed tomography (CT) three-dimensional volumetric reconstructions. The technique permits current and retrospective analyses of data from different institutions.
Patients and Methods: We analyzed the records of PE patients who underwent chest CT preoperatively between 2005 and 2009 at three institutions. All patients were Chinese. A window of –992 to –198 Hounsfield units was chosen for calculating the CT total lung volume (TLV). The data were compared with the data for 73 microtia and other chest-wall tumor patients studied during the same period as a control group.
所有納入評估的病人均為2005至2009年間接受胸部電腦斷層之有漏斗胸之華人, 以其胸部電腦斷之三度空間重組計算, 與同時期非漏斗胸的病人對照組比較.
Results: In total, 377 PE patients with Haller pectus index (PI) ≥3.2 were identified for this study. Compared with reported TLV data for 1050 normal children and our control group, we found little evidence of a decreased TLV in PE patients at any age for either sex. The mean PI did not change significantly between the ages of 3 and 27 years. The PI was inversely correlated with the TLV (p < 0.001).
凹陷指數在3.2以上者377人納入評估; 對照組則有1050人, 全肺容積(Total Lung Capacity, TLC), 不論男女, 在3至27歲間, 皆無明顯差別.
Conclusion: Our cross-sectional study provides evidence that the TLV of PE patients is within the normal range in children and adolescents.
結論: 兒童及青春期之漏斗胸患者之全肺容積在正常範圍.
感言:
一. 即使臨床上有明顯的生長遲滯, 呼吸困難, 呼吸道感染不易健癒等等肺功能問題, 但僅憑胸部電腦斷層, 只能有解剖學上的評估, 只要橫膈向下壓迫腹部, 造成腹部凸起, 在全肺容量的減少則可由代償, 在電腦斷層上則不會呈現明顯變化,所以即使兒童病患不易配合肺功能檢查的, 也不能依賴電腦斷層來評估肺功能
.
二. TLC全肺容量, 即使在明顯凹陷的病人, 仍不會有明顯變化, 最好用FVC(forced vital capacity)來評估, 可是不幸的是, 我國兵役檢查漏斗胸部分, 就是以TLC來評斷.
A Cross-sectional Study of Lung Volume Development in Pectus Excavatum Patients: Estimating the Total Lung Volume from Chest CT using Three-dimensional Volumetric Reconstruction
(跨機構漏斗胸病人肺容積發育評估--以胸部電腦斷層三度容積重建來估算全肺容積)
這一篇是由 我, 北京兒童醫院, 及長庚醫院的病人電腦斷層分析, 計算其肺容量的結果
Background/Purpose: This study quantified the lung volume development of pectus excavatum (PE) patients using chest computed tomography (CT) three-dimensional volumetric reconstructions. The technique permits current and retrospective analyses of data from different institutions.
Patients and Methods: We analyzed the records of PE patients who underwent chest CT preoperatively between 2005 and 2009 at three institutions. All patients were Chinese. A window of –992 to –198 Hounsfield units was chosen for calculating the CT total lung volume (TLV). The data were compared with the data for 73 microtia and other chest-wall tumor patients studied during the same period as a control group.
所有納入評估的病人均為2005至2009年間接受胸部電腦斷層之有漏斗胸之華人, 以其胸部電腦斷之三度空間重組計算, 與同時期非漏斗胸的病人對照組比較.
Results: In total, 377 PE patients with Haller pectus index (PI) ≥3.2 were identified for this study. Compared with reported TLV data for 1050 normal children and our control group, we found little evidence of a decreased TLV in PE patients at any age for either sex. The mean PI did not change significantly between the ages of 3 and 27 years. The PI was inversely correlated with the TLV (p < 0.001).
凹陷指數在3.2以上者377人納入評估; 對照組則有1050人, 全肺容積(Total Lung Capacity, TLC), 不論男女, 在3至27歲間, 皆無明顯差別.
Conclusion: Our cross-sectional study provides evidence that the TLV of PE patients is within the normal range in children and adolescents.
結論: 兒童及青春期之漏斗胸患者之全肺容積在正常範圍.
感言:
一. 即使臨床上有明顯的生長遲滯, 呼吸困難, 呼吸道感染不易健癒等等肺功能問題, 但僅憑胸部電腦斷層, 只能有解剖學上的評估, 只要橫膈向下壓迫腹部, 造成腹部凸起, 在全肺容量的減少則可由代償, 在電腦斷層上則不會呈現明顯變化,所以即使兒童病患不易配合肺功能檢查的, 也不能依賴電腦斷層來評估肺功能
.
二. TLC全肺容量, 即使在明顯凹陷的病人, 仍不會有明顯變化, 最好用FVC(forced vital capacity)來評估, 可是不幸的是, 我國兵役檢查漏斗胸部分, 就是以TLC來評斷.
- May 31 Mon 2010 09:28
2010/5 日本泛太平洋小兒外科醫學會報告(兒童漏斗胸在納氏手術後生長曲線的改進)
Improvement of Growth Development Curve in Nuss procedure for Pediatric Pectus Excavatum
兒童漏斗胸在納氏手術後生長曲線的改進
I. Background/Purpose
發現在兒童漏斗胸以納氏手術治療後, 在其生長曲線上, 不論身高及體重都有顯的增進. <br>
In treating pediatric pectus excavatum patients with the Nuss procedure, we found that there were improvements in body weight (BW) and body height (BH) in the growth curve of these children. Herein, we want to describe improvements in the growth curve in pediatric patients who underwent the Nuss procedure for pectus excavatum.
II. Patients and Methods <br>
<br>
116 children aged 3 to 17 with pectus excavatum received the Nuss procedure between June 2005 and September 2009. Indications for surgery included two or more of the following: progression of deformity; exercise intolerance or restrictive disease on pulmonary function test; Haller index > 3.25; mitral valve prolapse; or heart compression. All surgeries were performed in a uniform manner by the same pediatric surgeon. Height and weight data for all subjects were collected 1 day before surgery (OP); 6 months postoperatively (6M) and 1 year postoperatively (1Y) and compared with the pediatric development chart. Exclusionary criteria included: age above 14 years prior to surgery; previous thoracic surgery; absence of complete data for weight and height within 1 year; and rank below the lowest (< 3%) or above the highest (> 97%) percentiles on the pediatric development chart. <br>
III. Results<br>
<br>
A total of 116 pediatric patients with pectus excavatum received the Nuss procedure in our hospital; 30 were included in this study. The average BW (average growth development percentile) at OP, 6M, and 1Y were 27.97 kg (49.17%), 30.67 kg (57.42%), and 32.19 kg (60.42%) respectively. The average BH (average growth development percentile) at OP, 6M, and 1Y were 130.15 cm (57.25%), 130.68 cm (61.84%), and 137.33 cm (65.32%) respectively. Compared with the OP and the 6M and with the OP and 1Y in the BW growth development percentile, the P values were 0.01 and < 0.01. In comparison with the OP and the 6M and the OP and 1Y in the BH growth development percentile, the P values were 0.02 and < 0.01. Both differences were significant. IV. Discussion The Nuss procedure [1] is the most revolutionary method for treating patients with pectus excavatum [2, 3]. This minimally invasive procedure corrects the pectus excavatum physically instead of destructing the costo-cartilage structure. The improvement in BH may be due to the correction of the deformity and directing it to a relative normal thoracic structure while improvement in BW may possibly be due to the decrease the cardiopulmonary compression and decrease in the respiratory effort of the body.
Conclusion:
Pectus excavatum in pediatric patients can be treated safely and successfully by using the Nuss procedure. The procedure also has the benefit of improvement of the growth development curve in pediatric patients who were preoperatively in the normal or lower growth percentile in the growth development chart. Fig. 1 The average growth development percentile in BW at OP, 6M and 1Y. (** p<0.01) Fig. 2 The average growth development percentile in BH at OP, 6M and 1Y. (* p<0.05, ** p<0.01)
兒童漏斗胸可相當安全及成功地用納氐手術治療. 這手術方法也對兒童病患的生長有明顯的幫助.
- May 09 Sun 2010 00:08
挑戰--骨翻轉手術(Wada procedure) 失敗病例的矯治
今天下午為小王(假名)進行納氏胸廓矯治手術(Nuss procedure)。與往常不同，心中壓力很大，因為今天的手術很特殊，雖然已在電腦上模擬了無數次，但心中仍然有點不踏實。小王的情形就是這類的＂疑難雜症＂。雖然曾為不少傳統Ravitch手術失敗的病友們再行矯治手術，但畢竟沒有人報告過用Nuss手術來矯治Wada手術（胸骨翻轉手術）失敗的病例；這是一項挑戰，更是一項契機，讓我能有機會去改進這項手術。
小王在十年前曾接受過胸骨翻轉手術。這項手術是由Wada等人在1970年發表的方法，主要的理念是將胸前壁切下來，再翻轉，將凹入的內面轉向前方而向外凸出。隨著Wada來台，這項手術曾流行了好一陣子。但隨後，許多醫師也發現在手術後，因為前胸壁的血液循環已被完全斷絕，而漸漸纖維化，原來翻轉向前凸起的胸則因纖維化而又漸漸凹回去了。小王來門診時，抱著很大的期望，或許在尋求治療的過程中遭受到了太多的挫折，對他深切地期盼，我答應他一定儘力為他矯治。
在經檢查後，我有點懊惱答應地太快了；我發現小王凹陷的前胸壁上除了一道橫向弧形刀疤外，胸骨及肋軟骨均已嚴重纖維化了。不但失去了彈性，也因胸壁對心包膜及肋膜等均已粘黏在一起，手術中無法用胸腔鏡導引，危險性增加了許多。但是，在胸骨下多做一個切口，或許就可以解決這個問題。
小王在麻醉前清醒時最後的一句話是：＂朱醫師，就交給你了！＂雖然他儘量表現地很輕鬆，但我知道他心中有著許多的焦慮，但我卻已有了答案，而這些焦慮，也將在手術後消逝。在為他消毒敷單後，我在他胸骨下方的上腹部做了一個約3公分的縱向切口，再由這切口用手指的柔軟指尖將胸骨與其下的纖維組織剝離，再把心臟及肋膜推開，空出胸骨與心肺中間的間隙；再由胸部兩側的切口置入矯正板，穿過經指尖造成的空隙。由於整個胸壁都凹陷，範圍廣，加上嚴重地纖維化而失去彈性，用了兩根矯正板才能將胸前壁抬起。整個手術大約用了八十分鐘，失血約20 cc。用了上腹部切口，以手指取代了胸腔鏡，讓困難變成容易。每位病友們都很特殊，都有不同的難題要解決，但是"Nothing is impossible."多用點心就行。
註 : 手術後第一天, 小王居然可坐起來, 談笑自若, 也不覺得有多疼. 居然可以上網回應! 不知是否因以前的胸骨翻轉手術時將所有神經打斷, 所以反而不痛? 沒有別的醫師的經驗或報告可供參考, 只能依理推斷了.
- Mar 11 Thu 2010 11:36
到日本發表論文--漏斗胸兒童在納氏手術後生長曲線的改進
經我們病友們的資料分析, 發現漏斗胸兒童在手術治療後, 身高及體重增長皆有明顯的作用, 這項發現在以前並未有人發表過, 也代表著手術治療對兒童健康及發育的影響, 我們將這成果投出, 並將於今年五月份於日本神戶召開之大平洋小兒外科醫學會(PAPS. Pacific Association of Pediatric Surgeons) 第43次年會的學術會議上發表. 題目為: Improvement of Growth Development Curve in Nuss procedure for Pedicatric Pectus Excavatum." (漏斗胸兒童在納氏手術後生長曲線的改進). 謹謝謝大家, 也以此獻給病友們, 做為對本協會的第一項成果.
- Nov 30 Mon 2009 09:50
小尤的問題--手術後工作困擾
大家好:
我今年28歲,男生 開刀快一年半了,開刀前在科技業工作的RD,後來留職停薪,公司沒辦法等我,我就回到我的家鄉台中,做補習班的課輔(想當老師沒資格),薪水當然很低,最近年輕健康的工作也不好找,我只有大學畢業,突然想找比較拼的工作,比如像責任制的工作,但是鋼板就是感覺怪怪的(心態衝不起來),
我想請問有開完刀的網友,拿掉鋼板前,從事哪方面的工作,
謝謝!
我今年28歲,男生 開刀快一年半了,開刀前在科技業工作的RD,後來留職停薪,公司沒辦法等我,我就回到我的家鄉台中,做補習班的課輔(想當老師沒資格),薪水當然很低,最近年輕健康的工作也不好找,我只有大學畢業,突然想找比較拼的工作,比如像責任制的工作,但是鋼板就是感覺怪怪的(心態衝不起來),
我想請問有開完刀的網友,拿掉鋼板前,從事哪方面的工作,
謝謝!
- Nov 26 Thu 2009 17:10
漏斗胸原因分析
「為何我會有漏斗胸？」，這是在門診初次見到大家時，最常被提出的問題，謹就這問題，做個簡單的分析整理。
一。單純漏斗胸：超過一半以上的漏斗胸患者是單純的漏斗胸，其化異常，也無親人有類似情形。
二。家族性漏斗胸：在世界文獻中，最高發現有43%的漏斗胸患者是家族性的。
三。馬凡氏徵候群（Marfan's syndrome）: 一種第15對染色體基因(FBN1)異常(15q21.1)，屬顯性遺傳，即父母中有任何一人，即可導至臨床疾病，主要影響纖維組織成份，對身體多處都會影響，其特徵為身才瘦高，肢體長，心臟瓣膜、主動脈、眼、肺、骨、軟骨及各結締組織都常受影響。
四。軟骨症(rickets):維生素缺乏症，會影響鈣的代謝及骨生長，在台灣很罕見。br>
五。波蘭徵候群(Poland syndrome):可能是約在肧胎約46天大時發生鎖骨下動脈血流不良所引起之一側胸壁及同側上肢肌及骨骼發育不良。發生率約為1/10.000~1/100,000; 男性約為女生之三倍，而右側為左側之兩倍。
六。氣管軟骨軟化症(tracheomalasia)
~待續
一。單純漏斗胸：超過一半以上的漏斗胸患者是單純的漏斗胸，其化異常，也無親人有類似情形。
二。家族性漏斗胸：在世界文獻中，最高發現有43%的漏斗胸患者是家族性的。
三。馬凡氏徵候群（Marfan's syndrome）: 一種第15對染色體基因(FBN1)異常(15q21.1)，屬顯性遺傳，即父母中有任何一人，即可導至臨床疾病，主要影響纖維組織成份，對身體多處都會影響，其特徵為身才瘦高，肢體長，心臟瓣膜、主動脈、眼、肺、骨、軟骨及各結締組織都常受影響。
四。軟骨症(rickets):維生素缺乏症，會影響鈣的代謝及骨生長，在台灣很罕見。br>
五。波蘭徵候群(Poland syndrome):可能是約在肧胎約46天大時發生鎖骨下動脈血流不良所引起之一側胸壁及同側上肢肌及骨骼發育不良。發生率約為1/10.000~1/100,000; 男性約為女生之三倍，而右側為左側之兩倍。
六。氣管軟骨軟化症(tracheomalasia)
~待續
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