Syndactyly - Syndactyly

Japanese: 合指症 - ごうししょう(英語表記)Syndactyly
Syndactyly - Syndactyly

What is the disease?

Hand development progresses during the 4th to 8th week of fetal development. If this process is disrupted by intrinsic (chromosomal or genetic abnormalities) or extrinsic factors, various congenital hand anomalies occur. Syndactyly is when some or all of the adjacent fingers are not aligned. Healing ( Yugo ) Syndactyly occurs in 1 in every 3,000 births, and is most prevalent in the middle and middle fingers. Ring finger ( guard ) (ring finger) syndactyly tends to be the most common. Embryologically, syndactyly is classified as a differentiation disorder of the fingers (inadequate separation), and depending on the severity, it is divided into cutaneous syndactyly (fusion of only the skin) and bony syndactyly (fusion of the bones as well).

What is the cause?

It may occur alone or as part of a congenital malformation syndrome (such as Poland syndrome). Craniosynostosis ( Body armored vehicle ) A group of congenital anomalies associated with this condition (such as Apert syndrome) are caused by mutations in genes encoding growth factor receptors that control bone development.

Testing and diagnosis

Syndactyly is evident at birth. First, the patient is examined to determine whether there are any other physical abnormalities. An orthopedic specialist is consulted to determine whether and when surgery is necessary. X-rays are taken to evaluate the shape of the phalanges.

Treatment methods

Finger separation surgery is usually performed between 8 months and 3 years of age. In most cases, Skin flap ( Hiben ) The interdigital space is then created using a stent, the fingers are separated using a zigzag skin incision, and the skin defect is then covered with a skin graft. Some cases require a two-stage operation, osteotomy, or bone graft.

What to do if you notice an illness

See a pediatric orthopedic specialist.

Miyabi Mizuguchi

Syndactyly
Syndactyly
(Musculoskeletal system diseases (including trauma))

What is the disease?

Adjacent fingers touch each other Healing ( Yugo ) Syndactyly is an abnormality where the fingers are stuck together (Figure 3). It is seen in the area between the middle finger and the ring finger.

What is the cause?

Hands and fingers are thought to be formed between 6 and 8 weeks of fetal development, and this is thought to be an abnormality in development at this time. Polydactyl ( Plus ) Merged into Multiple Fingers ( Tagoshi ) There are also cases where Constriction ring syndrome ( Government ) Other conditions such as syndactyly may also cause syndactyly, but these are different diseases.

How symptoms manifest

Depending on whether the bones are fused, it is divided into bony syndactyly and cutaneous syndactyly. The extent of fusion can be either fused over the entire finger or the tip is separated.

Testing and diagnosis

X-rays will be taken to check the condition of the bones.

Treatment methods

In cases of simple cutaneous syndactyly, surgery is performed to separate the affected area at 1 to 2 years of age. Skin grafting is usually required.

It can be technically difficult in cases of bony syndactyly or syndactyly of three or more fingers.

What to do if you notice an illness

Please visit a hospital with a specialized orthopedic or plastic surgeon and consult with them.

Shigeharu Okuzumi

Figure 3 Syndactyly
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Figure 3 Syndactyly


Source: Houken “Sixth Edition Family Medicine Encyclopedia” Information about the Sixth Edition Family Medicine Encyclopedia

Japanese:

どんな病気か

 手の発生は胎生4~8週に進行します。内因(染色体異常や遺伝子異常)または外因によりこの過程が損なわれると、さまざまな手の先天異常が生じます。このうち合指症とは、隣接する指同士の一部または全部が癒合(ゆごう)した(くっついた)ものを指します。合指症は出生3000人に対し1人の頻度でみられ、なかでは中指・環指(かんし)(薬指)間の合指が最も多い傾向にあります。発生学的には、合指症は指の分化障害(分離不全)に分類されており、その程度により、皮膚性合指(皮膚だけの癒合)と骨性合指(骨も含めた癒合)に分けられます。

原因は何か

 単独で生じることもありますが、先天奇形症候群(ポーランド症候群など)の症状の一部としてみられることもあります。合指症と頭蓋骨早期癒合症(ずがいこつそうきゆごうしょう)を合併する先天異常の一群(アペール症候群など)は、骨の発生を制御する成長因子の受容体の遺伝子の変異に起因しています。

検査と診断

 合指症は出生時に、すでに明らかです。まず体にほかの異常が合併していないかどうか、診察を受けます。整形外科の専門医を受診し、手術の可否、時期について相談します。X線撮影で指骨の形態を評価します。

治療の方法

 指の分離手術はたいてい生後8カ月~3歳で行われます。多くの場合、皮弁(ひべん)を用いて指間を形成し、ジグザグ皮切により指を分離し、皮膚欠損を移植によりおおいます。二段階に分けた手術や骨切り術、骨移植術を要する症例もあります。

病気に気づいたらどうする

 小児の整形外科専門医の診察を受けます。

水口 雅

合指症
ごうししょう
Syndactyly
(運動器系の病気(外傷を含む))

どんな病気か

 隣り合った指が互いに癒合(ゆごう)している(くっついている)異常が合指症です(図3)。中指~薬指間などにみられます。

原因は何か

 手や指が形づくられるのは胎生6~8週とされ、このころの発生異常と考えられ、多指(たし)に合併した多合指(たごうし)の場合もあります。次項で述べる絞扼輪症候群(こうやくりんしょうこうぐん)などでも合指を示す場合がありますが、別の疾患です。

症状の現れ方

 骨が癒合しているかどうかで骨性合指、皮膚性合指に分けます。癒合範囲については、指全体にわたって癒合している場合と、先端は分離している場合とがあります。

検査と診断

 骨の状態をみるためにX線検査を行います。

治療の方法

 単純な皮膚性合指では1~2歳で分離手術を行います。この際、通常は植皮が必要です。

 骨性合指、3指以上の合指などでは技術的に難しい場合があります。

病気に気づいたらどうする

 専門の整形外科医や形成外科医のいる病院を受診して相談してください。

奥住 成晴

図3 合指症
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図3 合指症


出典 法研「六訂版 家庭医学大全科」六訂版 家庭医学大全科について 情報

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