淺談掌腱膜攣縮症

　　Dupuytrens disease是一種掌腱膜纖維組織增生而造成手指屈曲攣縮的疾病，最多見於無名指，50歲以上發病率最高，男性占90%左右。Xiaflex(collagenase clostridium histolyticum)是一款治療Dupuytrens disease膠原蛋白酶的新藥。 　　掌腱膜攣縮症(Dupuytrens contracture)，又稱作Dupuytrens disease或palmar fibromatosis，是一種掌腱膜纖維組織增生而造成手指屈曲攣縮的疾病(Dupuytrens contracture is caused by underlying contractures of the palmar aponeurosis.This can pull and bend the fingers in toward the palm. You may not be able to straighten them)，以無名指受影響最多見，小指占第二位，中、食、拇指的發病率依次減少，雙手占多數(The ring finger and little finger are the fingers most commonly affected. The middle finger may be affected in advanced cases, but the index finger and the thumb are nearly always spared)。病因不清楚。在歐、美洲發病率高，50歲以上發病率最高，男性占90%左右(Dupuytrens disease occurs most often in people ages 50 and older. At this age, men are affected more often than women)，男性是女性發病率的10倍左右(ten times as likely to develop the condition)。病程緩慢，有時病變停止後又再進展，多數患者無症狀，直到發現手指屈曲伸不直時才去就診，一般也難治癒。 　　掌腱膜(palmar aponeurosis)為一種筋膜結締組織，主要負責手部抓握的功能(The main function of the palmar aponeurosis is to increase grip of the hand)。大部分位於手掌中部(deep palmar fascia)，呈放射狀三角形，尖端朝向手掌近端，與腕橫韌帶(transverse carpal ligament)及掌長肌腱(palmaris longus tendon)相接；遠端到達掌指關節處，然後分成四條纖維束，延伸到食指、中指、無名指、小指的兩側，與淺層筋膜相連；兩側與大、小魚際肌的筋膜相連(The central portion of palmar aponeurosis occupies the middle of the palm, triangular in shape.Its apex is continuous with the lower margin of the transverse carpal ligament, and receives the expanded tendon of the Palmaris longus. Its base divides below into four slips, one for each finger. Each slip gives off superficial fibers to the skin of the palm and finger, those to the palm joining the skin at the furrow corresponding to the metacarpophalangeal articulations, and those to the fingers passing into the skin at the transverse fold at the bases of the fingers)。

　　掌腱膜淺層有許多垂直細纖維和皮膚真皮層相連，深層的細纖維與骨間肌筋膜(interosseous aponeurosis)相連，並形成8個纖維通道，每個通道將血管、神經與蚓狀肌腱和屈指肌腱分別隔開(By this arrangement short channels are formed on the front of the heads of the metacarpal bones; through these the Flexor tendons pass. The intervals between the four slips transmit the digital vessels and nerves, and the tendons of the Lumbricales)。 　　病因不明(The cause of Dupuytrens disease is not known)，可能與下列因素有關：
(一) 炎症、損傷：握工具做事，如使用重槌(sledgehammers)、攀岩愛好者(manual labourers or Rock climbers)、不斷刺激手掌，引起掌腱膜及其週邊組織損傷，促使纖維組織增生而導致攣縮。另外，據統計，並沒有慣用手比非慣用手容易好發之別(It commonly develops in both hands and has no connection to dominant or non-dominant hands)。
(二) 疾病：根據統計，患有癲癇(epilepsy)、糖尿病(diabetes)、心肌梗塞、類風濕、慢性酒精中毒(alcoholism)、吸菸(cigarette smoking)、肝硬化(liver cirrhosis)者，同時有Dupuytrens contracture的發病率較高。癲癇患者發病率比一般人正常人多10倍以上，可能與使用phenytoin有關(epilepsy therapy with phenytoin)。
(三) 遺傳：在北歐國家人民如(people of Scandinavian or Northern European ancestry)或某些家族的發病率較高(It might be inherited, because the disease tends to happen in families)，所以當地又稱作"Viking disease"，對於亞洲人發病率是相對較低的。60~70%患者有遺傳易感性(People with a family history (60 to 70% of those afflicted have a genetic predisposition to Dupuytrens contracture)。
(四) 神經病變：有人質疑是尺神經受某種刺激，造成尺神經支配區域出現一系列病理病變。 　　早期可以看到或摸到手掌接近無名指或小指的皮下小結節(a small lump)，日後結節逐漸形成纖維性索條 (a fibrous cord)，使鄰近的皮膚變厚，出現皮膚皺起橫褶；在皮膚的深層出現邊界不清的硬塊，多無壓痛，如果會痛，可能是第一次發作，久而久之，痛也就不存在(often beginning as a tender lump in the palm. Over time, pain associated with the condition tends to go away, but tough bands of tissue may develop)。因為掌腱膜結締組織主要負責手部抓握的功能，一旦繼發MP joint及PIP joint contracture，必定影響日後戴手套、洗手、抓握或撿東西等日常生活功能(Dupuytrens contracture progresses slowly and is usually painless. As Dupuytrens disease gets worse, a fibrous cord may develop in the tissue of the palm. The cord may extend to one or more fingers, usually the ring or small finger. The cord pull your finger toward your palm. You may find it hard or impossible to do things like put on gloves, wash your hands, or pick up things)。 　　Dupuytrens disease分期，其標準是：

Stage	Contracture
0	0 (healthy)
N	0 (feel nodules / cords)
N/1	0-5 degrees (beginning contracture)
1	6 ~ 45 degrees
2	46 ~ 90 degree
3	91 ~ 135 degree
4	> 135 degree.

　　具體必須根據病變程度選擇適當治療方案，如果患者症狀較輕，或只有無痛性的小結節出現，且未引起手功能障礙者，可暫不作處理，可以包括以下幾種：

分期	N	N/1	1	2	3	4
放射線治療 (radiation therapy)	*
Xiaflex 注射			*
不處理			*
手部手術 ( hand surgery)			*	*	*	*

1. 藥物治療：對有初期結節，可進行類固醇結節內注射(When you first get the disease, your doctor may have you try steroid shots)。實驗顯示，Xiaflex或Xiapex可有效治療Dupuytrens contracture情況，是一款目前FDA唯一認可用於Dupuytrens contracture患者的膠原蛋白酶的藥物(A medicine called collagenase clostridium histolyticum may be injected to try to dissolve some of the tight tissue)，可分解膠原蛋白中的肽腱(which breaks peptide bonds in collagen)，減少組織攣縮功效。
2. 物理治療：包括放射線治療(radiation therapy)、超短波等可以暫時使局部攣縮的組織軟化，症狀得到短期緩解，但日後可復發。可適當做finger stretches,及穿戴手部splint。手術後做物理治療可增加手部的肌力與肌張力(physical therapy for the affected hand will be implemented to help increase strength and function)。
3. Needle aponeurotomy：一種門診局部麻醉手術即可的治療方式，不需切開皮膚而曝露傷口、手部功能恢復快速、術後併發症少、也無須做復健等好處，但有無法移除結節，並且容易復發的缺點(Advantage：minimal intervention without incision、done in the office under local anesthesia、very rapid return to normal activities without need for rehabilitation。Disadvantage：the nodules are not removed and might start growing again)。不僅適用於Dupuytrens contracture的早期階段，也可用於手指嚴重彎曲的患者(Needle aponeurotomy may be performed on fingers that are severely bent, stage IV, and not just on early stage)。
4. 手術治療：
   　　手術目的是儘可能地恢復手在最佳功能狀態(The goal of treatment for Dupuytrens disease is to keep your hand working as best as it can)。
   (1) 掌腱膜切斷術：簡單手術只針對病程較長、有全身性嚴重疾病如心臟病、糖尿病，難以耐受較大的手術的老年患者。
   (2) 掌腱膜切除術(fasciotomy)：切除攣縮的索條(cord)及攣縮組織，不作廣泛切除。只適用於病情嚴重、單純腱膜切斷手術無效，而無法忍受較大手術患者。至於，掌腱膜徹底切除術則適用能耐受手術患者，除了切除攣縮的掌、指腱膜組織之外，並將已粘連組織與皮下脂肪墊一併切除，然後再進行皮瓣移植(skin grafts are needed to correct the overlying skin)。
   (3) 截指術(amputation)：針對病程較長、反覆、較嚴重的在小指或無名指屈曲攣縮，並關節有明顯改變，嚴重影響功能而無法矯正者，或手術後出現手紙方面併發症者(In extreme cases, amputation of fingers may be needed for severe or recurrent disease, or after complications in surgery)。

　　手術無法治愈Dupuytrens disease，因為未受影響的fascia有可能發展而導致復發。據統計，約有15.7%患者可出現術後併發症，其包括：digital nerve injury(3.4%)、digital artery injury(2%)、infection(2.4%)、hematoma(2.1%), and complex regional pain syndrome(5.5%)、painful flare reactions in(9.9%)、wound healing complications(22.9%)。

掌腱膜攣縮症、膠原蛋白、筋膜結締組織、神經病變、放射線治療、臨床醫學資訊館