Congenital anomalies of hand & leg.

Question 1

incidence

Answer 1

sporadic

Question 2

etiology

Answer 2

genetic, teratogenic/idiopathic

may have syndromic association with anomalies elsewhere in the body

Question 3

most common anomalies

Answer 3

syndactyly

polydactyly

Question 4

syndactyly

Answer 4

Most prevalent in the Western Hemisphere & classified as failure of differentiation of parts / structures (fusion of adjacent digits & can involve part / whole of the length of involved digits)

simple: fusion is limited to skin and soft tissues only
complex: includes bone fusion

apert’s syndrome: a severe form of syndactyly

treatment:
surgical separation of the digits within the first year of life. Local flaps & full-thickness skin grafts are necessary to achieve full coverage of the separated digits.

complete: whole fingers
incomplete: parts of fingers

Question 5

polydactyly

Answer 5

a duplication of digits (more prevalent in the African continent).
Very variable in its clinical presentation & ranges from simple skin tags to a complete supernumerary digit (usually the thumb).

treatment: removal of the extra appendage

Question 6

three types of polydactyly

Answer 6

The extra digit is outside the thumb or big toe (preaxial polydactyly). When the digit is outside the thumb, it’s also called radial polydactyly. When it’s outside the big toe, it’s also called tibial polydactyly.

The extra digit is outside the little finger or little toe (postaxial polydactyly). When the digit is outside the little finger, it’s also called ulnar polydactyly. When it’s outside the little toe, it’s also called fibular polydactyly.

The extra digit is between other fingers or toes (central polydactyly)

Question 7

Brachydactyly

Answer 7

longitudinal growth failure of digits.

Partial / complete longitudinal growth deficits affecting the radius / ulna

produce radial / ulnar “club hands”.

Question 8

radial club hand (brachydactyly)

Answer 8

manus valgus

more common and can be associated with other anomalies, such as thrombocytopenia, Fanconi anemia/ VACTERL complex (vertebral defects, anal atresia, cardiac defects, trachea-esophageal fistula, esophageal atresia, renal dysplasia, limb abnormalities)

Question 9

Poland syndrome

Answer 9

a condition characterized by ipsilateral chest wall & limb hypoplasia.

causes brachydactyly

Question 10

treatment of brachydactyly

Answer 10

lengthening and realignment of existing structures

Question 11

Constriction Band Syndrome

Answer 11

intrauterine amniotic bands wrap around the limbs

These can act like tourniquets & threaten the viability of digits, limbs & other parts.
Often results in congenital amputation.

It is important to differentiate congenital amniotic bands from acquired bands that may occur in infants because of neglected external ligatures, termed the hair-thread-tourniquet syndrome.

Question 12

clinodactyly

Answer 12

A deviation of digits toward the radial / ulnar direction.
This usually involves the distal phalanx & commonly seen with the presence of a triangular middle phalanx termed the delta phalanx.

Treatment: if the problem is affecting function, done by corrective osteotomy.

Question 13

camptodactyly

Answer 13

A congenital flexion deformity of digits occurring in the sagittal plane.

Most common & severe in the small finger at the PIP joint

Treatment:

• depends on severity
• most don’t require surgery
• non surgical - Initially by serial splinting / external fixation.
• If this does not correct the deformity - surgical correction by Z-plasties of the skin.
  Usually accompanied by deficient skin - surgical correction may require skin grafts.