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一個早就想做,卻被美國人搶先發表的研究: 在漏斗胸病人接受納氏手術中, 以經食道做心臟超音波檢查, 評估在手術中胸壁被擡起來之前後心臟功能的變化. 發表於American Journal of Surgery (2015) 210,1118-1125
Surgical repair of pectus excavatum relieves right heart chamber compression and improves cardiac output in adult patients-an intraoperative transesophageal echocardiographic study (chestwall.org/ice/incoming/www.chestwall.org/ba_meeting_forum/files/am j of sx 2015 sx rpr of PE releaves r heart compress.output in adult.pdf)
METHODS: A retrospective evaluation was performed of 168 adult patients who underwent a modified Nuss PE repair with intraoperative transesophageal echocardiography from 2011 to 2014. Seventeen patients with prior PE repair undergoing bar removal acted as controls.
RESULTS: Mean age was 33.0 years (range, 18 to 71 years). There was an increase in right atrium
(15.1%), tricuspid annulus (10.9%), and right ventricular outflow tract (6.1%) size after surgery (all P
, .0001). Right ventricular cardiac output measured in a subset of 42 patients improved by 38%. No change
in chamber size or cardiac output occurred before and after bar removal surgery in the control group.
CONCLUSIONS: Surgical correction of PE deformity caused a significant improvement in right heart
chamber size and cardiac output. (成年人在接受納氏手術中以經食道心超音波檢查發現, 比較手術前後, 右心室泵出增加38%;右心房增加15%, 三尖瓣環增加10.9%, 右心室出口增加6.1%)

可見被壓迫的心臟在手術中胸壁被擡起來瞬間, 心功能立即的改善.

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早期漏斗胸的納氏手術, 大多用鋼絲來固定, 但隨著經驗增加, 大多數還是採用固定片, 而不再用鋼絲. 

最近又有一些病友來問是否用鋼絲固定就可以了, 據早期的個人的統計, 鋼絲在體內斷裂的發生率是47%, 但一直沒有其他的論文來支持我的發現,

今天讀到這篇論文, 與我的觀點相合:Am Surg. 2016 Sep; 82(9): 781–782.

 

Complications associated with bar fixation following Nuss repair for pectus excavatum

論文中題到:

Six  had a complication related to bar fixation and position. Five  patients had broken wires, three requiring an operative procedure: Two because of pain, the third for a late pneumothorax (one month postoperatively) caused by the sharp end of the wire protruding into the pleural cavity . Three broken wires that had not caused overt problems or symptoms were found incidentally at the time of bar removal at the end of the period of surgical fixation. 

(本論文有11位病人採用鋼絲固定,)

與固定方式相關的併發症有6例, 其中有5例是因鋼絲斷裂, 其中3位需要外科手術治療,(2位因疼痛, 1位因斷裂鋼絲刺破肋膜腔而引起氣胸), 3條斷裂鋼絲未引起問題, 是意外發現, 

11位用鋼絲固定的病人有5位鋼絲在體內斷裂, 高達45.5%, 與我的統計47%很接近, 有這麼高比率的併發症, 還要用鋼絲嗎?

 


 

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Haller index, 或稱凹陷指數,  常用於凹胸的嚴重度評估, 是由Dr. Haller, Dr. Kramer, 及Dr. Lietman 在1987 創出來的方法, 即用簡單的公式, 即胸腔橫內徑除以內前後徑(胸骨至脊椎的距離), 所得的數值, 如一般人橫內徑是25公分, 前後內徑是10公分, Haller index  即是25/10=2.5 .

當時, 認為大於3.25即有手術矯治的必要.

以現在的觀點看來, 這種方式雖然方便, 但過於粗率, 甚至被認為是手術矯治的標準, 就不合適了. 目前儀器檢查的進步, 以心臟超音波, 心電圖, 電腦斷層, X-光, 及肺功能等等, 即可更精確評估心肺功能的影響, 而且有時, 如下圖, 明顯心臟被壓迫變形, 應儘早矯治, 但Haller index卻只有2.88.  

H

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前幾個星期在門診看到一個很特別的病人,一個32歲的病患推著氧氣筒走進診間,一度以為這個病人是不是走錯診間,後來看到他的病歷才知道,原來八年前他曾經來朱醫師門診評估漏斗胸。這個病人在小時候就已經做過傳統矯正手術,但由於效果不好胸廓回凹,八年前來做評估,當時他的凹陷指數已經大於5,而且有很明顯的心肺壓迫,肺功能也有受到影響,那時候就有建議納氏手術矯治,但後來病人就沒有繼續追蹤治療了。

八年後看到這位病人,帶著氧氣筒走進來,舉步維艱,每走一步就會喘。才知道他在兩三年前因為肺炎住院,原本的肺就是處於一個壓迫狀態,又加上肺炎讓整個肺臟纖維化更厲害,雖然當下肺炎得到控制,但肺部纖維化讓他氧合能力變得很差,血氧濃度在靜止的時候最高只有90%,有活動時血氧濃度就掉到70%,日常生活受到很大的影響,更不用想說要出門工作。

評估病人整個狀況,朱醫師跟我都認為現在做納氏手術對他的幫助真的非常有限,因為肺臟纖維化受損的非常厲害,手術本身就是一個很大的風險,就算手術成功,纖維化的肺也無法恢復。看著病人走出診間落寞的背影,我跟朱醫師心中滿滿的感慨跟惋惜,對我們來說這個案例是很可惜的,假如當初八年前有做納氏手術,或許他的人生會有很大的不同。

作者:林介文醫師(三軍總醫院及宏恩醫院)

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最近在門診遇到一些體型偏瘦的病友,有尋求增加食量或是重訓的方式,但效果很有限,經過檢查之後才發現這些病友是廣泛型漏斗胸。

大家聽到漏斗胸直覺就會想到中央凹陷型,就像有一個漏斗放在胸前,但其實這種廣泛型的凹胸比例也很高,而且通常會被忽略,因為整個胸前都是平的或是微凹,這些病友只會覺得是自己太瘦,但其實整個胸廓都是壓扁的狀態。

這類的病友小時候外觀幾乎看不出來有什麼異樣,但到了國小五六年級開始進入快速生長期,胸廓凹陷的狀況也更明顯,剛好這個時候的青少年在叛逆期,自己忽略這個狀況,父母親也無從了解。等到注意到的時候,凹陷指數都已經很嚴重了,而且有明顯的心肺壓迫及脊柱側曲,造成生活及健康的影響。

所以各位朋友或是身邊的人,有這樣體型偏瘦的狀況,甚至合併體力變差或是胸悶心悸等症狀,廣泛型漏斗胸可能就是造成你偏瘦的原因,建議盡早接受完整評估及手術矯正,還給心肺足夠的空間,避免持續壓迫造成心肺功能退化,重建外觀,恢復自信。

作者:林介文醫師(三軍總醫院及宏恩醫院)

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最近跟朱醫師共同照顧一個從澎湖來的一歲多小女孩,一出生就有明顯的凹胸,因為最近凹陷的狀況越來越明顯且小朋友看起來瘦瘦弱弱的(體重只有9公斤),因此有找其他醫師評估,但得到的答案都是再觀察。於是小女孩轉至台灣做進一步的評估,從電腦斷層評估凹陷指數為7.2,心臟超音波發現心臟幾乎完全被推移到左側,有輕微的三尖瓣跟肺動脈瓣的逆流,還有偏高的肺動脈壓。考量到心臟已有壓迫且影響到生長發育,而且早期做完矯正肺的實質會有明顯的生長發育,可以讓肺功能更進步,於是在今年9月初幫小女孩做了納氏胸廓矯正手術,術後恢復非常良好也很順利地出院回家。

過去對於納氏手術的時間點都是落在比較大的小朋友或是成人,但由於現在手術技術不斷的改良進步,手術年紀已經不是很重要的考量因素,跟著朱醫師學習觀摩手術以來,接受矯正的病人從1歲到60歲都有。因此若有凹胸的病友經過評估有合併心肺壓迫或是合併有影響生長發育的小病友,建議盡早接受手術矯正,還給心肺足夠的空間,避免持續壓迫造成心肺功能退化。

作者:林介文醫師(三軍總醫院及宏恩醫院)

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納式手術在漏斗胸矯正已經是一個非常成熟而且常見的手術,但還是有很多病友會對手術有疑慮或是害怕其風險及併發症。今年荷蘭有發表研究就是在分析年輕人跟成年人在納式手術後的比較。此篇研究收集了327個病人,年輕人組(24歲以下)272人,成年人組(24歲以上)55人。從滿意程度跟成功率來看幾乎沒有差別,在重大併發症方面發生率很低,也幾乎沒有差別。唯一差別比較大的是術後慢性疼痛,在成人組發生比例較高,大約是14%,年輕人大約是3%左右。合理的解釋是因為成人骨質較硬且胸廓已經固定,所以在撐起來的時候,疼痛會比年輕人明顯。因此若有漏斗胸的病友,尤其在20幾歲之前,骨質相對較有彈性, 早期發現及早矯正,會發生術後慢性疼痛的機會也會比較低。

還有一個比較有趣的發現,這些病人開刀的原因除了運動上有不適之外,在年輕人常見的原因是因為外觀造成心理社會的影響,在成年人身上多半都是因為造成身體的不適,如胸悶心悸等等,這跟我們在診間觀察到的病友狀況還蠻相近的。

另外,此篇研究也提到,成人骨質較硬因此也需要多支矯正板來做支撐,可以分散支撐力量達到比較好的矯正效果。也可以利用改良式納式手術,例如胸骨懸吊技術(目前朱醫師跟我都會使用的技術),去增加手術的安全性。

參考文章出處: Ann Thorac Surg. 2021 Sep;112(3):905-911.

作者:林介文醫師(三軍總醫院及宏恩醫院)

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最近有病友詢問真空鐘形吸引器(Vacuum Bell)的效果,雖然目前沒有什麼大型的研究,不過在去年(2020)有一篇文章發表: 收集了15個病人,使用了真空鐘形吸引器治療至少有六年以上。在凹陷深度有九成以上的改善,但只有平均改善0.87公分左右,而在漏斗胸凹陷指數(Haller index),卻幾乎沒有改善(before:4.4 => after: 4.3)。在電腦斷層中可以發現所謂凹陷深度的改善,只是皮膚皮下脂肪增厚的結果,對整個胸廓骨架幾乎沒有影響。

以下面那張圖來說明,雖然看起來外觀有改善,但從電腦斷層來看,心臟還是受到壓迫,凹陷指數幾乎沒有改變,唯一改變的是最上面那條白線到胸骨的距離,而這段距離就是增厚的皮下脂肪。

011.jpg

雖然真空鐘形吸引器是一種非侵入性的治療,但它必須長時間的使用,而且只適用於對稱型的凹胸,雖然外觀可能看起來有進步,但只是皮下脂肪增厚的假象,更重要的是對於整個胸廓骨架及心肺壓迫程度幾乎沒有改善。

 

作者:林介文醫師(三軍總醫院及宏恩醫院)

參考文章出處: Pediatric Surgery International volume 36pages1465–1469 (2020), “The vacuum treatment for the pectus excavatum thickened subcutaneous fat of the chest wall and is effective in preteenagers”

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很多家裡有漏斗胸小朋友的家長都有一個共通的困擾:到底要不要開刀和什麼時候開刀??其實經過醫生的評估之後假如有明顯的心肺壓迫,就會建議盡早手術。何謂明顯心肺壓迫,例如心臟超音波有明顯肺動脈壓高,瓣膜逆流,以及容易喘,感冒拖很久不易痊癒,甚至影響到生長發育。最近有一篇日本的研究指出在年輕人身上(尤其是小孩)做完漏斗胸的矯正,術後肺的容積跟重量都有明顯的增加。也就代表在胸廓矯正之後,肺的實質會有明顯的生長發育,也可推估肺功能會有進步,因此若評估之後需要手術的病童家長,及早手術對小朋友會有更大的助益。

作者:林介文醫師(三軍總醫院及宏恩醫院)

參考文章出處: Interact Cardiovasc Thorac Surg. 2021 Aug 23;ivab203, “Can surgical repair for pectus excavatum contribute to lung growth?"

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剛收到 CWIG (Chest Wall International Group)的通知, Johns Hopkins 兒童醫療中心的名小兒外科醫師 

Dr. Alec Haller 醫師過世了.

https://www.hopkinsmedicine.org/johns-hopkins-childrens-center/ways-to-give/endowments-funds/j-alex-haller.html

哲人已逝, 但他在1987年所創立的Haller index 卻將永遠在我們領域中被應用著.

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病例三,

25歲男性

三年前在南部做過非正統的納氏手術, 以骨板取代矯正板, 植入胸腔, 骨板右側滑入胸腔內.

檢討:

1. 植入骨板太短, 置入點偏外, 導致安全長度不夠, 滑入胸腔.

2.骨板非設計用來矯治凹胸, 其中孔洞多, 植入後不易取出.

解決方式:

以懸吊方式將前胸壁拉高, 將骨板重新放置於支撐肋骨上, 再以一固定器跨在上下兩肋骨上做為支撐點.

1234.jpg

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失敗病例之一.

男性19

為廣泛型凹胸, 在南部手術, 放置一條12吋矯正板及右側固定器,  手術後發生矯正板翻轉90度,

檢討:

1.     廣泛型凹陷只用一條矯正板, 無法分散張力, 亦難讓胸型有正常外觀.

2.     右側雖有固定器, 伯因一條矯正板承受張力過大, 作無法支撐, 造成矯正板90度翻轉.

解決方案: 

          再次手術, 調整原矯正板位置並加上第二條矯正板, 並以加上兩個固定器, 加強穩定性, 得到良好效果

圖左側為再次手術前, 右側為再次手術後.

111.jpg

112.jpg

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朱醫師嶨2015年榮任CWIG(Chest Wall international Group)--世界胸壁醫學會, 的台灣代表, 成

網址改成

http://www.chestwall.org/2015,世界漏斗胸(凹胸)大師們全到了.

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http://www.nownews.com/n/2014/11/29/1525432

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chest-CT  

Dear colleagues,                                   September 28  2014   Eindhoven, the Netherlands

 

Let me first introduce myself , because you may not all know me.

 

I am a cardiothoracic surgeon  at Catharina Hospital , Eindhoven , the Netherlands.

Mostly doing all cardiothoracic procedures , except congenital cardiac procedures.

 

Having learned from my teachers, how to do the classical surgical correction for thoracic wall deformities, I have added these operations to my specialties . My technique for pectus excavatum is a mixture of Daniel ( Nashville , Tennessee 1958) and Sanger , Tayler and Robicsek (1963) .

And with these operation my patients were satisfied.

Some 5 years ago , a girl of 15 years of age came with her mother to my outpatient clinic and ask for a Nuss bar , because her cousin had such an operation with good results.

I told her that i never did or even saw a Nuss bar , but that this technique looked to me an attractive alternative to my own one.

 

Promising her and her mother to we delved into this technique we went to a chest wall meeting in Ismir 2009 , where we met Dr Pilegaard.

After visiting him in Denmark he helped us with our first 2 cases, and from there we went on.

 

Because my fellow cardiac surgeons asked me what the fate was of the mammary arteries I did a study about this matter which was recently published : Patency of the internal mammary arteries after removal of the Nuss bar: an initial report ( pub  Interactive cardiovasc Thoracic Surgery , 2014 , Vol 19(1) pp 6-9 . Now we can visualize the mammary arteries with MRI .

 

Having done only 150 Nuss bar procedures I am a rookie in the group .So far my results are excellent , having only few complications , in line of the current era. Knowing that age limits for the Nuss Bar procedure is shifting forward  I started operating on adults as well.

 

 

I would ask the group now for some advice  here is my case:

 

58 year old female with symptoms that fitted with pectus excavatum in adult age; physical condition less,etc.

Haller index was 6.5 . see picture  1-3 for preoperative imaging.

 

On the 8 th of October 2013 I put in two Nuss bars , 12 and 11 inch with lateral side bars only on the left.

The where wider than I usually use, but I felt this was a more solid construction in this particular case.

The procedure itself was very easy and quick , like I was operating a 18 year old girl.

Being proud that also could do Nuss bars  in patients over 50 years of age , the first disappointment came soon : lot of pain , despite epidural post op and a lot of pain killers.

Post operative x-rays are shown in picture 4 and 5.

 

On May 2014 I saw her , just before she went on a holiday to Portugal.

There the situation got really worse: she had fever, elevated CRP , x-rays are shown in picture 6.

Diagnosed as pneumonia , treated with antibiotics I saw her back in the Netherlands ( ct scan and x-rays are in figure 9).It is always helpful to provide your patients with the last x-rays of the bar in place , so that where ever they might travel to , local physicians can compare the position of the bar , that is what I always do. The all have cellphone so I mail the pictures to them. Bars Did not looked displaced  neither on x-rays or ct scan.

 

Because she had so much pain , and my suspicion that the lower bar at the right side might have caused pleural irritation , we finally decided to remove the lower bar. This gave some release of the  pain.

When removing the lower bar I saw that the bar did not enter the thoracic cavity but only pushed 5 rib ( which you can see on the ct scan , picture 10 d)

 

What was the cause of her fever , elevated CRP and pain??

Was it allergy? Or bad surgery? I know from an article from the clinic of Nuss ( When it is not an infection : metal allergy after the Nuss procedure repair of pectus excavatum. J ped surg 2007  Jan ;4291) 93-7 that allergy is possible.

There might be other causes for these symptoms.

 

She will visit me soon , and has ask me to consider the possibility to remove the other bar and to convert  to a classical procedure.

She still trust me , but ask me to discuss this matter with some experts, which  is done by  this email.

 

Questions:

Was the initial procedure good?

What was the cause of the fever and pain  and elevated CRP in Portugal.

Was it the right decision to remove the lower bar.

Might this be a case of allergy to nickel?

 

What to do now?

Testing for Ni allergy?

If positive , is removing the upper bar and placing 2 new titanium ones an option.

Convert to classical operation?

Is a partial incision in the sternum like described by Ravenni , 2013 an option to have less forces and pain ?

 

 

Yours sincerely, Ted Elenbaas , CWIG , member

 

Cardiothoracic Surgeon , Catharina Hospital Eindhoven, the Netherlands

Dr. 朱志純 發表在 痞客邦 留言(3) 人氣()

1.

Dear Professor Robicsek,

 

Thank you so much for your comment. The nuggets of advice are engraved in my mind.

I know what you are worrying about. And I agree that it is not an option if a surgical technique places the patients’ lives in jeopardy.

 

However, I would like to tell you this fact, based on my experience and other specialists’ around the world through the past decade: the Nuss repair of PE is extremely safe and just nice and simple for de novo pectus patients who have no adhesions or other obstacles. If we follow the line correctly, we will enjoy the full benefits of chest wall integrity.

 

The case I described and problematic cases of others are all complicated ones than a pure PE. I have been trying to be ready for any challenges that I might meet during the procedure, and fortunately, I have no mortality or morbidity causing any serious disability. In cases of PE with a previous other operation, if we sensibly utilize the existing midline incision, we would be able to stay safe.

 

Among others, the major difference between Ravitch and Nuss is cartilage resection: namely, "Resection vs. Remodeling." I would like to preserve the cartilages intact; try not to cut them away. I think they are innocent but just arranged in a wrong direction for some reason. Thus, I would like to lead them grow to the right direction and let them do their jobs until the end of their lives.

 

It has been great to talk with you on this pro-con. Thank you again for your words of wisdom.

 

Sincerely,

 

Hyung Joo Park, MD, PhD, FCCP

Professor and Chairman

Department of Thoracic and Cardiovascular Surgery

Seoul St. Mary's Hospital

College of Medicine, The Catholic University

Seoul, South Korea

 

2.

Because of sever adhesios after sternotomy and redo operation ,Ravich procedure in this age is the safest method in this patient


Professor M. Aghajanzade, MD

Dr. 朱志純 發表在 痞客邦 留言(0) 人氣()

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