CURRENT CONCEPTS

The Hand in Distal Arthrogryposis

Mohammed A. Alzahrani, MD,* Sebastian Farr, MD†

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Learning Objectives
Upon completion of this CME activity, the learner will understand:
 Clinical features of distal arthrogryposis in the hand.
 Diagnosis and treatment recommendations for distal arthrogryposis in the
hand.
 Outcomes of treatment for distal arthrogryposis in the hand.
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Distal arthrogryposis is the second most common type of arthrogryposis after amyoplasia and
is defined as arthrogryposis that affects hands and feet; it is mostly inherited in an autosomal-
dominant fashion. This review discusses up-to-date background information, clinical features,
and treatment of distal arthrogryposis in hands concentrating on camptodactyly, thumb-in-
palm deformity, and windblown hand deformity, which are the most common and func-
tionally limiting deformities. Treating these deformities should be individualized and follow a
multidisciplinary approach. Most deformities can be initially treated nonoperatively, and if
not responsive, operative treatment may be pursued to improve function. Surgery primarily

From the *Alhada Hospital for Armed Forces, Taif, Saudi Arabia; and the †Department of Corresponding author: Sebastian Farr, MD, Department of Pediatric Orthopaedics and
Pediatric Orthopaedics and Foot and Ankle Surgery, Orthopedic Hospital Speising, Vienna, Adult Foot and Ankle Surgery, Orthopaedic Hospital Speising, Speisinger Strasse 109, 1130
Austria. Vienna, Austria; e-mail: sebastian.farr@oss.at.
Received for publication May 12, 2021; accepted in revised form October 19, 2021. 0363-5023/22/4705-0008$36.00/0
https://doi.org/10.1016/j.jhsa.2021.10.027
No benefits in any form have been received or will be received related directly or indirectly
to the subject of this article.

460 r  2022 ASSH r Published by Elsevier, Inc. All rights reserved.

 HAND IN DISTAL ARTHROGRYPOSIS 461

aims to release soft-tissue contractures, rebalance muscle forces, and may need bony
correction based on the deficits of each case. Current literature suggests that early treatment
leads to better outcomes. However, reported cases are scarce, and no consensus or gold
standard for treatment exists. Therefore, long-term (multicenter) studies are needed to assess
outcomes and standardize the treatment of such deformities whenever possible. (J Hand Surg
Am. 2022;47(5):460e469. Copyright  2022 by the American Society for Surgery of the
Hand. All rights reserved.)
Key words AMC, clasped thumb, distal arthrogryposis, thumb-in-palm, windblown hands.

included in the idiopathic camptodactyly series.6

D
ISTAL ARTHROGRYPOSIS (DA), defined as ar-
throgryposis affecting the hands and feet,
refers to a large group of specific and het-
erogeneous syndromes.1 It is the second most com-
mon form of arthrogryposis after amyoplasia and
accounts for approximately 20% of arthrogryposis
cases (Fig. 1).1 Distal arthrogryposis is typically in-
herited in an autosomal-dominant manner, and
several DA types are associated with gene mutations
in sarcomeric muscle proteins, such as troponin,
myosin, and tropomyosin. Distinct gene mutations
may lead to different types of DA, and one DA
may be caused by mutations in more than one gene,
suggesting varying clinical expression and gene
penetrance (Table 1).2
Primary diagnostic criteria for upper and lower
limbs have been established, of which 2 or more are
required to be present to diagnose DA (Table 2).
However, when a first-degree family member (ie, a
parent or sibling) fulfills these diagnostic criteria,
other family members with at least one major diag-
nostic criterion are considered affected.2
The main hand deformities found in DA and dis-
cussed in this review article include camptodactyly of
multiple fingers, thumb-in-palm deformity, and
windblown hands (WBHs).2 According to the latest
version of the Oberg-Manske-Tonkin classification, it
is classified as III.C.i.b.3
CAMPTODACTYLY
Camptodactyly may be defined as a non-traumatic
proximal interphalangeal joint flexion contracture
with ineffective active extension, intrinsic and
extrinsic tendon shortening, and skin defects in the
palmar aspect of the fingers.4 Its true incidence is
unknown but it reportedly occurs in approximately
1% of the population and all variants of arthrogry-
posis.4,5 Syndromic camptodactyly (such as arthrog-
ryposis) has rarely been mentioned in isolation but is
Syndromic camptodactyly is more severe, affects
multiple fingers, and often occurs bilaterally (Fig. 2).6
In general, functional limitations are caused by a
decrease in range of motion with blocking of the
palm during grasping and limited finger extension
(Fig. 2).4 Proximal interphalangeal joint deformities
may be associated with compensatory meta-
carpophalangeal (MP) joint extension, distal inter-
phalangeal joint extension (boutonniere deformity),
and WBH.4,5 The most common presenting
complaint is dissatisfaction with appearance, fol-
lowed by functional limits (eg, typing, wearing
gloves, writing, and playing piano).5,6
Non-operative treatments like stretching, exer-
cising, serial casting, dynamic orthosis fabrication,
and static orthosis fabrication are the mainstay of
therapy.5 Continuous re-evaluation and orthotic
adaptions are necessary throughout childhood, espe-
cially during periods of accelerated growth, such as
puberty. Operative treatments are reserved for pa-
tients who are unresponsive to non-surgical treatment
and have functional limitations and proximal inter-
phalangeal joint flexion deformities > 50e60 . Sur-
gical treatment provides better results when a
stepwise intraoperative approach is followed during
soft-tissue release. We found the algorithm shown
in Figure 3 to be useful in practice.5 The contrain-
dications for this soft-tissue surgery are flattening of
the condyles of the proximal phalanx and the inability
to adhere to postoperative therapy and/or orthoses
wear. Bony Procedures like arthrodesis and/or
osteotomy are indicated if bony changes like flat-
tening and parrot beaking of proximal phalanx head
are present.5 Families should be counseled that the
finger will not become completely normal and that
some flexion deformity is likely to persist.4 Previous
studies show inconsistent outcomes regarding the
recurrence rate of camptodactyly in general. A recent
study showed good results with corrective and

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462 HAND IN DISTAL ARTHROGRYPOSIS

FIGURE 1: Types of congenital contractures. Reprinted with permission from Bamshad et al.2

shortening osteotomy of the proximal phalanx in Type 2 - flexed CMC joint and extended MP joint
syndromic camptodactyly types.6 Although some Type 3 - flexed CMC and MP joints
studies showed improved outcomes after syndromic
Each type may further be divided as A or B
camptodactyly surgeries, recurrence has been
based on the possibility of passive correction of the
mentioned in about a quarter to half of patients.7,8
MP joint.
Based on this classification, the authors suggested
THUMB-IN-PALM DEFORMITY an algorithm for treatment. The first step is to assess
the CMC. If it is extended, no correction is neces-
This is often called “clasped thumb” and is character-
sary, but if it is flexed, a correction osteotomy
ized by supination, adduction, and flexion contracture
should be performed at the metacarpal base. The
of the thumb with tight first web space and extensor
second step is to assess the MP joint. If it is
tendon weakness. The patient may adapt some func-
extended, no correction is necessary, but if it is
tion; however, pinch or grasp may be heavily affected
flexed, then an extensor indicis proprius to extensor
with the thumb opposing only the palm, but not the
pollicis longus transfer should be done if it is
fingers.9e11 It commonly includes interphalangeal joint
passively correctable. If it is not passively correct-
flexion, MP joint flexion, and carpometacarpal (CMC)
able, a chondrodesis should be performed (Figs. 4,
joint extension. However, patients with arthrogryposis
5). The osteotomy at the base of the metacarpal
may demonstrate different contractures than those with
bone aims to produce abduction, extension, and
classic deformities.9 These include an abnormally
pronation as per intraoperative assessment. The
contracted first web space, palmar skin, and intrinsic
eventual goal is to provide a thorough opposition to
musculature, attenuated extrinsic tendons, and joint
reach the other fingers. Shortening may be added at
stiffness.9 Contracture severity and type vary largely
times to overcome volar contracture. This may also
among patients. Treatment is nonsurgical or surgical
be achieved by chondrodesis.9,11
(only in cases of functional impairment) and must be
Repositioning of the thumb requires a thorough
individualized through a multidisciplinary approach
release of fibrous bands and lengthening of the first
comprising a team of surgeons and occupational and
dorsal interosseous and adductor muscles. In certain
physical therapists.9
cases, the flexor pollicis longus (FPL) tendon may
Zlotolow et al9 provided a new classification for
have to be lengthened by Z-lengthening in the fore-
this deformity:
arm, intramuscular tenotomy, or by tenotomy and
Type 1 - extended CMC joint and flexed MP joint suturing it into the index tendon if the FPL is

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 HAND IN DISTAL ARTHROGRYPOSIS 463

TABLE 1. Types of DA1,2

Major Clinical
DA Type Syndrome Gene Defect(s) OMIM Code Features

DA1 TNNI2, TPM2, MYBPC1, 108120 Only hand and feet are
MYH3 affected (adducted
thumbs, ulnar deviation
of MP joints, normal
facies)
DA 2A Freeman Sheldon syndrome MYH3 193700 Whistling face syndrome
(distinctive facies,
flexion and ulnar
deviation of the fingers)
DA2B Sheldon Hall syndrome TNNI2, TNNT3, TPM2, 601680 Distinctive facies, flexion
MYH3 and ulnar deviation of
the fingers, vertical talus
DA3 Gordon syndrome PIEZO2 114300 Cleft palate, finger
contractures, clubfoot
DA4 609128 Scoliosis, finger
contractures
DA5 PIEZO2, ECEL1 (for DA5 108145 Limited ocular motility,
without ptosis, finger
ophthalmoplegia) contractures
DA6 108200 Sensorineural hearing loss,
finger contractures
DA7 Trismus pseudo-camptodactyly MYH8 158300 Inability to fully open
mouth, facultative
camptodactyly
DA8 Autosomal- dominant multiple MYH3 178110 Multiple pterygiums,
pterygium syndrome finger contractures
DA9 Congenital contractural arachnodactyly/ FBN2 121050 Ear deformity, finger
Beals syndrome contractures
DA10 Congenital plantar contractures 187370 Toe walking, short
Achilles tendon, elbow
contracture

ECEL1, neuronal endopeptidase; FBN2, fibrillin-2; MYBPC1, myosin binding protein C1; MYH3, embryonic myosin heavy chain 3; MYH8, fetal
myosin heavy chain 8; PIEZO2, piezo type mechanosensitive ion channel component 2; TNNI2, troponin I subunit; TNNT3, troponin T subunit;
TPM2, tropomyosin 2, or b-tropomyosin (1,2). Also see: OMIM. Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute of
Genetic Medicine, Johns Hopkins University (Baltimore, MD). Available at: https://omim.org/ 2021

nonfunctional.9e11 Several techniques have been

TABLE 2. Established Diagnostic Criteria of DA2
described to address the 2-plane contracture (thumb-
Major Criteria Major Criteria index interval and thumb flexion aspect), such as the
(Upper Limb) (Lower Limb) index rotation flap and modified dorsal rotation
Camptodactyly Talipes equinovarus advancement flap.10,11 A simple Z-plasty may be
Pseudocamptodactyly* Calcaneovalgus deformities sufficient for mild cases, but care should be taken as it
Hypoplastic and/or absent Vertical talus may create new tension in the adjacent aspect of the
flexion creases Metatarsus adductus defect.10
Overriding fingers
Ulnar wrist deviation
Studies show improved outcomes after surgery in
most patients from a functional perspective.11,8
*Limited passive proximal interphalangeal joint extension with wrist However, other studies showed up to half of the
hyperextension.
cases developing recurrence over time.7

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464 HAND IN DISTAL ARTHROGRYPOSIS

FIGURE 2: Young patient with mild ulnar deviation of wrist and the middle, ring, and little fingers and multiple bilateral finger
contractures. Note the missing joint creases and impaired fist grip due to stiffness.

FIGURE 3: Surgical soft-tissue release steps in managing camptodactyly.5 DIP, distal interphalangeal joint; FDS, flexor digitorum
superficialis; PIP, proximal interphalangeal joint.

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 HAND IN DISTAL ARTHROGRYPOSIS 465

FIGURE 4: A patient with DA and WBHs. Note the ulnar finger deviation in the native radiograph, tight first web space (arrowhead),
MP flexion contracture of the thumb and the index, middle, ring, and little fingers. Previous extensor indicis proprius transfer and FPL
lengthening were not strong enough to achieve sufficient active thumb extension. The finger deformity is well-maintained and
correctable using orthosis.

WINDBLOWN HANDS hypertrophy, hyperlaxity syndromes, Digito-talar

The WBH deformity was first reported in 1897 in a
man who was 50 years old and had MP and inter-
phalangeal joint contractures and ulnar deviation. It
had been described using Brissaud’s phrase (“devia-
tion en coup de vent,” meaning “windblown fin-
gers”), and is also known as congenital arthrogrypoid
ulnar drift hands.12e14 This deformity is rare and,
therefore, there is a lack of cases reported in the
literature.14 It is widely believed that this deformity is
a feature of patients with DA.15 Windblown hands
often present at birth and become more prominent as
the child grows, especially during puberty.12e16
Windblown hands are characterized by flexion con-
tractures of MP joints with ulnar deviation of fingers
and associated thumb-in-palm deformities, campto-
dactyly, and digital overlap (Fig. 6).12,13,17 Interpha-
langeal joint extension and clinodactyly have also
been mentioned as features of WBHs.17
Although WBHs are flexible in the first few
years of life, they may cause secondary stiffness
with soft tissue and skeletal changes if they are not
treated properly.17 As many authors have previ-
ously stated, WBHs are likely a symptom of
different etiologies and not a syndrome on its own.
13
Apart from DA, other disorders associated with
WBHs include DA associated with upper limb
dysmorphisms, Waardenburg syndromes, Pena-
Shokeir phenotypes, Ashley syndromes, and Oto-
palato-digital syndrome type 2.12,15,17 Syndromes
such as Freeman Sheldon syndrome and Escobar
syndrome are considered as DA syndromes and not
as isolated syndromic WBHs (Table 1).1,17 The
deformity may also be seen in association with
some occuptions, ulnar nerve injury, Dupuytren
contracture, and rheumatoid arthritis. However, the
classical features are found almost exclusively in
the congenital type.
Zancolli and Zancolli18 had divided WBHs into 3
types depending on the involvement of tissue
contractures:
Type 1 - resembles mainly fascial and cutaneous
deformity
Type 2 - includes the skin abnormalities of type 1
in addition to finger and wrist flexor tendon
shortening
Type 3 - ligamentous, capsular, and bony de-
formities are present as well13
It has been found that mid-palmar fascia, inter-
digital ligaments, natatory ligaments, Cleland’s liga-
ments, and Grayson’s ligaments are all involved in
the structural development of WBHs. Of note, this

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466 HAND IN DISTAL ARTHROGRYPOSIS
FIGURE 5: The same patient from Figure 4 received soft-tissue release as described by Mahmoud et al23 and MP chondrodesis of the
thumb.
grading system cannot be done without surgical
exploration, and all these structures need to be
released during correction of the deformity.12
Etiology
The precise etiology of congenital WBHs is un-
known; however, studies have shown a sporadic
occurrence while others have found an autosomal-
dominant inheritance pattern with variable pene-
trance that frequently occurs in certain families.13,15
A report suggested that this deformity is caused by
the persistence of atavistic intrinsic musculature
originating from the palmar aponeurosis and inserting
distally into the extensor mechanism or by impaired
rotation of extremities during embryological devel-
opment.16 Zancolli and Zancolli had attributed the
ulnar drift and flexion deformities to “malformation
of the retaining ligaments, or retinaculum cutis,”
namely the mid-palmar fascia and natatory ligaments.
They had demonstrated that these cords on the ulnar
side might be the underlying cause of ulnar devia-
tion.18 Other authors have also found extrinsic
tightness in the form of extensor tendon underde-
velopment and tightness of flexor tendons.15e17
J Hand Surg Am.
Some have suggested that the cause of thumb
deformity is related to weak or absent thumb exten-
sors and/or instability of the MP joint and that the
abnormality of the thumb consequently leads to
pushing of other fingers to the ulnar side.15 In one
study, the biopsies of 2 patients who had Freeman
Sheldon syndrome had shown fatty infiltration,
fibrosis, atrophy, and thickened contracted joint
capsules, which were suggested to be the causes of
the deformities.13 In another study, Vanĕk et al19 had
observed similar findings through biopsies and elec-
tromyograms of Freeman Sheldon syndrome cases
and had concluded that a congenital myopathy
seemed to be the primary cause of the deformities.
Some cases have been described to be associated
with muscle hypertrophy, in which anomalous
musculature acts on the proximal phalanx and leads
to ulnar deviation of the fingers.13 In these cases, no
thumb deformity is present, and they are usually
unilateral; treatment comprises resection of the
aberrant muscles.13 There are also a few other
studies that have shown another cause, such as a
primary ossification disorder leading to finger
deviation.16
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 HAND IN DISTAL ARTHROGRYPOSIS 467

TABLE 3. Recommended Surgical Procedures to

Address WBH12,13,15,23
Deformity Procedures

First web space Syndactyly and

contracture contracture release (by
Z-plasty, full thickness
skin graft, and dorsal
rotational flap)
FPL lengthening, extensor
pollicis longus plication
or tendon transfer in
cases of weak extensor
pollicis longus
Adductor pollicis release
First metacarpophalangeal
joint chondrodesis if
instability present
Opposition and derotation
osteotomy
Ulnar finger deviation Crossed intrinsic tendon
transfer or release
Recentralization of
extensor tendons
Metacarpal osteotomy
FIGURE 6: Another patient with WBHs received a dorsal K-wire fixation
advancement flap for first web space deepening. The ulnar drift of Flexion contractures Release of all contracted
the fingers in a resting position aggravates during active exten- tissues including skin
sion, whereas no deviation is present during gripping. and subcutaneous tissue
Flexor tendon lengthening
by fractional
Diagnosis lengthening at the
forearm or
The diagnosis is usually made clinically. It starts with Z-lengthening
a thorough history, including prenatal, natal, and
postnatal events in addition to developmental and
family history. General examination should focus on
Life expectancy and mental ability are usually
any dysmorphic features on physical examination,
unaffected.
and an upper limb functional assessment should be
performed. Interphalangeal joints are usually found
flexed, while the MP joints are always flexed. The Treatment
thumb is adducted into the palm with the above- Patients with WBHs usually present to tertiary
described flexion contracture and narrow first web referral centers because of functional or cosmetic
space (Fig. 4).16 There may be limitations in prona- complaints. A study has shown that cosmetic
tion and supination, and active supination may appearance is the main reason for seeking medical
aggravate the finger deformities.12,16 There is atrophy advice despite functional limitations.14 Treatment
of the thenar and hypothenar muscles and thin palmar should ideally be done in a multidisciplinary
skin. There is also webbing of various degrees.16 A setting.15 Thumb deformity and first web space
decrease in pinch and grip power are the main find- contracture are among the most functional limiting
ings caused by ulnar finger deviation (which in turn anomalies in WBHs.12 Due to the rarity of cases,
causes decentralization of flexor tendons) and thumb there is debate regarding the effectiveness of surgical
deformities.20 There may be associated foot, cranio- versus nonsurgical management, appropriate timing
facial, spine, and limb deformities. Moreover, of surgery, and potential relapses after treatment.
hyperlaxity syndromes and features should be ruled Consequently, there is no consensus or gold standard
out. Radiological studies may help assess any bony treatment for WBHs. Long-term studies are required
changes. Genetic studies help determine and secure but are difficult to accomplish due to the rarity of the
the diagnosis of any associated DA syndromes.1 deformity.14,21 Early orthosis fabrication is effective

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468 HAND IN DISTAL ARTHROGRYPOSIS
TABLE 4. Outcome Assessment According to
Wood and Biondi12
Grade Description
Excellent Hand with normal
function and cosmesis
Good Hand with almost normal
function and satisfactory
appearance
Fair Some remaining
contractures interfering
with normal function
Poor Contracted hand that
cannot function in
activities of daily living
and may limit progression severity and simplify
surgical procedures.15 However, thumb adduction
contractures are challenging to correct by orthoses.14
Orthosis fabrication and nonsurgical treatments
require a great deal of patient/parent compliance.
Some studies have shown unsuccessful results with
orthosis fabrication but still recommend it as a
reasonable modality before surgical treatment.12,22
If necessary, surgery needs to correct all de-
formities in the WBH, including adduction and
flexion deformities of the thumb, ulnar deviation of
the fingers, and flexion contracture of the MP joints
(Table 3). All contracted components (eg, retinacular
cutis, subcutaneous fibrous cords, dynamic compo-
nents) should be addressed.13,20 Surgical treatment is
recommended before the age of 2 or 3 years.14 Early
surgeries are technically demanding but associated
with less adaptive behaviors, more achievable and
better corrections, and do not require bony
procedures.14
Outcomes
Wood et al12 had developed a simple assessment and
classification system primarily used for postoperative
evaluation (Table 4). Other hand functional scores,
such as the Disability of the Arm, Shoulder and Hand
(DASH), QuickDASH, and Pediatric Outcomes Data
Collection Instrument, are not applicable for
WBHs.15 Wood et al12 had reported 7 cases of WBHs
treated surgically with different techniques such as
dorsal rotational flap, Z-plasty flap, and skin grafts for
the thumb and other fingers to manage skin defects.
They had also performed intrinsic release and tendon
lengthening. Tendon transfers had been used to
improve the weakness of thumb extension. Interest-
ingly, no excellent results were reported according to
J Hand Surg Am.
their classification, and 2 cases had a recurrence.
However, the results of operated hands were better
and more satisfactory than those of the patients who
had refused surgery (4 patients).
Gavashkar et al14 had treated 23 hands in 18 pa-
tients. Indications were difficult in grasping and
holding objects, inability to perform tasks like writing
and sports, and difficulty in perineal care. Extensor
tendon centralization had been performed by plication
of sagittal radial bands, and ulnar drift had been cor-
rected by cross-intrinsic transfer. One adolescent case
with bony abnormalities necessitated corrective sub-
capital osteotomy of the thumb’s metacarpal bone.
First, web space contractures were released, and
transpositional or rotational flaps covered defects.
Metacarpophalangeal joint fusion was performed for
the 2 adolescents who had difficulty in perineal care,
and a groin graft covered their skin defects. Most
patients had excellent functional and cosmetic re-
sults. The authors had recommended surgery before
the age of 2 years; however, recurrence had occurred
more frequently in this age group. The authors had
concluded that the functional outcomes were better
in cases with bony interventions.
Neser et al15 had attempted to establish an
intraoperative algorithm to help manage WBHs.
They suggested that early diagnosis, corrective
manipulation, and orthotic therapy limit the pro-
gression of the severity and simplify the surgical
procedure and that orthosis fabrication mainly en-
courages first web space opening. Therefore, the
authors had recommended addressing the first web
space by Z-plasty and adductor pollicis release if
needed and that MP joint chondrodesis with K-wire
fixation should be performed first in unstable cases.
Then, the extensor pollicis longus should be
addressed by plication or extensor indicis proprius
transfer. Subsequently, the ulnar deviation should
be corrected by crossed intrinsic tendon transfer
(ulnar-sided lateral bands from the index, middle,
and ring fingers are transferred and attached to the
radial lateral bands to minimize ulnar forces).
Ülkür et al20 had reported 2 adult patients (4
hands) in whom skin and retinaculum cutis release
and defect coverage with side finger flaps had been
performed. Moreover, first web space and muscle
release, extensor tendon centralization, crossed
intrinsic transfer, FPL lengthening, and radially
closing wedge osteotomy of all the metacarpals
had been added. The results showed improved grip
and thumb-to-index pinching powers in all oper-
ated hands. However, the authors suggested cor-
recting WBHs in the early stages of life.
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 HAND IN DISTAL ARTHROGRYPOSIS
Kalliainen et al21 had reported 18 hands, one of
which was treated by early orthosis fabrication with
good results. Two patients had refused surgery, and
the remaining 15 hands had been treated surgically,
of which some had undergone preoperative orthosis
fabrication to improve thumb-index web space. The
authors recommended surgical treatment before 3
years of age to minimize the development of articular
and bony deformities and limit maladaptive learned
behaviors. No patients achieved excellent results
(normal function and appearance), 5 hands achieved
good results, and 10 hands achieved fair results.
Better results were obtained with tendon centralizing,
intrinsic crossed transfer, and intrinsic release of the
thumb. The authors had recommended first meta-
carpal realignment and dorsal flaps to widen the first
web space.
SUMMARY
The main features of DA in hands are thumb-in-
palm, camptodactyly, and WBH deformities.
Treatment of DA should be individualized and
covered by multidisciplinary teams to achieve
optimal results. Most arthrogrypotic hand deformity
surgeries result in better outcomes if performed
during the early years of life using bony and/or soft-
tissue interventions.
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