Congenital hand anomalies

1. Congenital Hand Anomalies
Dr Sushil Sharma

2. Swanson classification
1. Failure of formation of parts
2. Failure of differentiation or separation of
parts
3. Duplication
4. Overgrowth
5. Undergrowth
6. Congenital constriction ring syndrome
7. Generalized skeletal abnormalities &
syndromes

3. Overgrowth (Macrodactyly)
• Rare congenital anomaly (Incidence : 0.9%)
• Most frequently involved : Index finger
• Etiology : Uncertain
– Abnormal nerve supply, blood supply & humoral mechanism
• Associated with syndactyly (10%)
• Two types (Barsky)
– Static
• Deformity is present in infancy.
• Diffuse enlargement of the digit. Distal and palmar tissues more enlarged
– Progressive
• Associated with angular deformity
• Occurs in early childhood as a rapidly enlarging digit (banana shaped
finger)
• Clinical Features
– Enlarged digit
• Thickened skin & hypetrophied nails
• Phalanges always involved. (Metacarpal may be enlarged)
– Unilateral, and multiple digits are affected two to three times as
often as single digits.
– If the thumb is involved, a characteristic abduction and
hyperextension deformity
– Loss of motion
– Trophic ulcers

4. Treatment - Macrodactyly
• No role of conservative treatment
• Indications for surgery
– Enlargement (Cosmetic)
– Angulation (Obstructed grasp & pinch)
– Carpal tunnel syndrome
– Causalgia
• Debulking
– Most common procedure
– As much excess tissue as possible is excised from one half of
the digit; 3 months later, the other half is debulked
– Digital nerves be stripped of one half their fascicles (to
prevent recurrence)
• Physeal arrest after the digit has reached estimated
adult length
– Holes drilled through the physes
– Resection of the physes
– High-speed drill for epiphysiodesis of all phalanges
• Digital shortening
– Amputation of the distal phalanx
– Amputation is used to provide relief only as a last resort
• Complications
– Recurrence
– Flap necrosis

5. Undergrowth
• Deficiency in any of its anatomical parts—osseous,
musculotendinous, or ectodermal
• Hypoplastic thumb
– Incidence : 1.3% - 3.6%
– Shorter than normal or, in the most severe manifestation,
totally absent.
– Types:
• short thumb
• adducted thumb
• abducted thumb
• floating thumb
• absent thumb
• clasped thumb.

6. Short thumb
• The normal thumb extends to about the level of the
proximal interphalangeal joint of the index finger; a
thumb is considered “short” if its length is less than
this
• Hypoplasia of any or all osseous components
• Associated with syndrome
– Metacarpal is short and slender : Fanconi, Holt-Oram
syndrome.
– Metacarpus is short and broad : Hand-foot-uterus
syndrome, myositis ossificans progressiva.
– A slender distal phalanx may be associated with
Fanconi or Holt-Oram syndrome.
• Shortening of the proximal phalanx of the thumb may
be associated with brachydactyly.
• The thumb may be radially deviated (“hitchhiker's
thumb”) or very short and stubby (“potter's thumb”
or “murderer's thumb”).
• Treatment
– Deepening of the web space
– Two-limb or four-limb Z-plasty.

7. Adducted thumb
• Due to absence or partial absence of the
thenar muscles
– Lack a functional FPL
– The radial collateral ligament of the
thumb & MCP joint may be deficient.
• Shortened & tapered thumb with
flattened thenar eminence and a
deficient first web space.
• Unilateral involvement
• Autosomal dominant transmission
• Treatment
– Correction of the adduction contracture
• Two-limb or four-limb Z-plasty
• Sliding dorsal flap raised from the radial
side of the index finger.
– Restoration of opposition
• ring flexor superficialis tendon
opponensplasty
• abductor digiti quinti opponensplasty

8. Abducted thumb
• Extremely rare deformity
• Due to abnormal insertion of the FPL
muscle into an otherwise normal EPL
muscle
• Associated with
– Deficiencies in the thenar musculature
– Adduction contracture of the first
metacarpal with web space deficiency
• Types
– A : Stable CMC joint
– B : Unstable CMC joint
• Treatment
– Release of the bifurcated tendon insertion
and reattachment to the metacarpal neck
– Release of the tendon distally and
reattachment to the distal phalanx
– Release of the anomalous slip to the EPL
muscle, with an ulnarward shift of the EPL
at the metacarpophalangeal joint

9. Floating thumb (Pouce Flottant)
• Small, slender thumb arises from the
radial border of the hand.
• Thumb originates more distally than
usual, and there is neither extrinsic
nor intrinsic muscle function
• There are two phalanges, a
fingernail, no metacarpophalangeal
joint, and no first metacarpal
• Trapezium and scaphoid also often
are absent
• Treatment
– Amputation is the treatment of
choice, followed by index finger
pollicization

10. Absent thumb
• Most severe hypoplastic thumb
deformity
• Associated with
– Radial ray deficiency
– Holt-Oram syndrome
– Trisomy 18
– Rothmund-Thomson syndrome
• Treatment
– Pollicization or recession of the
index finger
– Best time for pollicization : 6-12
months
– Recession is preferable in an older
child with a strong lateral pinch
between the index and long fingers

11. Clasp thumb• Thumb is positioned in adduction and extreme flexion at
the MCP joint.
• Imbalance between the flexors and extensors of the thumb
– Hypoplasia or absence of the EPB muscle
– EPL may be absent.
• Clinical feature
– Thumb flexed into the palm
– No active extension at the MCP joint is shown after prolonged
observation and particularly by age 3 months
• Types
– Group 1 : deficient extension only
• Most common; Usually bilateral
• Sex linked inheritance
– Group 2 : flexion contracture combined with deficient extension
– Group 3 : hypoplasia of the thumb, including tendon and muscle
– Group 4 : deformities that do not fit easily any of the other three
categories
• Treatment
– Conservative : Early splinting in extension and abduction. (Group 1)
• Plaster splint changed every 6 weeks and continued for 3 to 6 months.
– Operative (Tendon transfer for inadequate EPL)
• PL, BR, ECRL, EIP, and flexor superficialis muscles
• Chondrodesis of MCP joint

12. Hypoplastic hands & digit
• Incidence
– Hypoplasia of the entire hand : 0.8%
– Brachydactyly (Short fingers) : 5.2%
• The most common hypoplastic bony segment :
middle phalanx (brachyphalangia).
• Autosomal dominant trait
• Seen in malformation syndromes
– Treacher Collins
– Bloom
– Cornelia de Lange, Holt-Oram, Silver, and Poland
syndromes
• Treatment
– Lengthening procedures (If shortening > 1cm)
• Improve the appearance of the metacarpal row
• Increase grip strength.
– Extraperiosteal toe-phalanx transplantation as
an interpositional or terminal graft for the
extremely hypoplastic digit

13. Constriction band syndrome (streeter’s
dysplasia)
• Incidence : 2%
• Etiology- constricting amniotic band &
intrinsic causes have been proposed
• Patterson clasification-
a. Simple constrictions
(partial/circumferential)
b. Constrictions with distal deformity
(lymphedema +/-)
c. Constrictions with acrosyndactyly
(Fenestrated syndactyly)
d. Intrauterine amputation
• Treatment
– Excision of band and the defect should be
closed with multiple Z-plasty
– Release of acrosyndactyly

14. Camptodactyly (arched finger)
• Painless, progressive flexion contracture of
PIP joint
– MCP joint hyperextended
• Due to imbalance in flexors
& extensors
• Incidence <1%
• Autosomal dominant trait
• Little finger (>70% cases)
• 3 types:
type I : Newborn (M=F, 80%)
type II : Adolescent females
type III : Multiple digits/with
syndromes
• Treatment : Not satisfactory results
– Conservative : Splinting until extension achieved
– Operative : Release of FDS tendon & transfer into
extensor apparatus

15. Congenital trigger thumb
• Stenosing tenosynovitis of FPL
tendon at A1 pulley .
• Fixed flexion of IPJ (thumb locked in
flexion)
• “Notta node” – palpable nodule
over flexor aspect of MCP joint of
thumb proximal to A1 pulley.
• Frequently B/L.
• Treatment
– Spontaneous resolution (30%
cases)
– Observation and gentle
manipulation
– Release of the A1 pulley should be
performed at about age 2 years
– Release of A3 pulley
– Resection of FDS

16. Kirner’s deformity
• Progressive palmar radial curvature
of the distal phalanx of little finger
• Seen at 8 to 10 years of age
• Deformity usu bilateral and
symmetrical
• Progressive deformity, not painful
• Distortion & widening of physeal
plate along with curvature of the
diaphysis of the distal phalanx.
• Radiograph: Broadened epiphysis
with irregularity in metaphysis
• Treatment: Multiple opening wedge
osteotomy

17. Madelung deformity
• Abnormality of the palmar ulnar part of the
distal radial physis
• Progressive ulnar and volar tilt at distal
radial articular surface with dorsal
subluxation of the distal ulna.
• Incidence : 1.7% (F>M)
• Commonly bilateral
• Autosomal dominant pattern
• Types:
– Posttraumatic
– Dysplastic (dyschondrosteosis or diaphyseal
aclasis)
– Genetic (Turner syndrome)
– Idiopathic
• Deformity
– Radius curved, with its convexity dorsal and
radial
– Ulna subluxated dorsally, enlarged ulnar head,
decreased ulnar length
– Carpus subluxated ulnarward and palmarward
into the distal radioulnar joint

18. Madelung deformity - Treatment
• Indication of surgery: Severe deformity
or persistent pain due to ulnocarpal
impingement of the carpus
• Distal radial osteotomy with ulnar
shortening (Milch recession)
• Osteotomy combined with Darrach
excision of the distal ulnar head
– The radial osteotomy may be a closing
or opening wedge as needed for
alignment
• Resection of the abnormal portion of
the radial physis and insertion of fat as
a form of surgical prophylaxis

19. Thank You