MACRODACTY

LY

DR G AVINASH RAO
FELLOW HAND AND
MICROSURGERY
SKIMS

MODERATOR – PROF DR ADIL H WANI

Macrodactyly
 OVERGROWTH (MACRODACTYLY)

 Macrodactyly is a rare congenital anomaly (0.9%) in which there is enlargement of the

finger.
 The index finger is involved most frequently.
 Macrodactyly does not seem to be an inherited condition. Although its cause is uncertain,
three possible factors are strongly suspected:
1. Abnormal Nerve Supply,
2. Abnormal Blood Supply, And
3. Abnormal Humoral Mechanism.
 Some authors have postulated that macrodactyly is an aborted type of neurofibromatosis;
But, other manifestations of this disease usually are not seen in these patients. Recent studies
have implicated a proto-oncogene (somatic mutation of PIK3CA) mutation resulting in
abnormal regulation of growth.
Cui, H., Han, G., Sun, B. et al. Activating PIK3CA mutation promotes osteogenesis of
bone marrow mesenchymal stem cells in macrodactyly. Cell Death Dis 11, 505 (2020).
https://doi.org/10.1038/s41419-020-2723-6

 There is nerve involvement in the affected digits, among which median nerve is
the most frequently involved.
 Aberrant distribution of neurofilament has been reported in the affected nerve
tissues.
 Osseous enlargement, osteochondromatous proliferations, hypertrophic
changes, and ankylosis of innervated joints can be observed in some areas of
innervated bone tissue.
 Mutation of PIK3CA can also be detected in the diseased nerve tissue.
 It encodes the catalytic α-subunit of PI3K (p110α). PI3K catalyzes the
conversion of phosphatidylinositol-4,5-bisphosphate (PIP2) to
phosphatidylinositol (3,4,5)-triphosphate (PIP3)
 True macrodactyly - involves hypertrophy of all the structures of the
digit: the skin, toenail, subcutaneous fat, bones, nerves, and blood
vessels.

Associated with - Proteus syndrome etc

 False macrodactyly - presents as hypertrophy of primarily one tissue
type.

Associated with - Ollier’s disease, Maffucci’s syndrome, vascular

malformation, neuro-fibromatosis, and Milroy’s disease
 Barsky described two types of true macrodactyly:

1. Static enlargement of the digit without progression as the child grows.

2. Progressive enlargement out of proportion to normal growth.

 The Progressive form may not enlarge during infancy but begins to enlarge rapidly
during early childhood; this form frequently is associated with angular deformity.
 Macrodactyly most commonly exists without other conditions, but syndactyly is
associated with macrodactyly in about 10% of patients.
 Macrodactyly involving both the hands and the feet has been reported by Keret, Ger,
and Marks.
 Some patients with neurofibromatosis develop macrodactyly.
 In static macrodactyly, the deformity is present in infancy.
 There usually is diffuse enlargement of the digit; however, the distal and palmar
tissues usually appear more enlarged than the dorsal and proximal tissues.
 The finger grows, but in proportion to normal digital growth.

 Progressive macrodactyly occurs in early childhood as a rapidly enlarging digit,

frequently with an angular deformity that makes the finger banana shaped .
 The skin may be thickened, and the nails may be hypertrophied.
 The phalanges always are involved, and the metacarpals may be enlarged.
 With maturity, the enlarged digit begins to loose motion
 Macrodactyly affects the fingers more frequently than the toes with
concurrent upper and lower extremity involvement being extremely rare.
 In the foot, macrodactyly usually presents in the first, second or third digit;
the second digit is most frequently involved.
 When overgrowth of an adjacent digit is also present, syndactyly is not
unusual.
 Macrodactyly may be suspected to be the result of Proteus syndrome, which
is a rare disorder of skeletal, hamatomatous, and mesodermal
malformations.
 Proteus Syndrome

 In addition to unilateral disproportionate overgrowth, other characteristic

features include connective tissue nevi, dysregulated adipose tissue, and
vascular malformation.

 Macrodactyly associated with Proteus syndrome should be differentiated

from isolated macrodactyly because it has progressive nature, poor
prognosis and high associated rates of recurrence
 Later in life, symptoms of carpal tunnel syndrome may develop, with complaints of paresthesias
and hypesthesias.
 Trophic ulcers also may develop over the involved digit.
 Involvement usually is 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 results.
 It generally is believed that all the tissues of the involved finger are enlarged; however, some
authors have noted sparing of the tendons and vessels.
 The nerves that innervate the involved territory are characteristically enlarged.
 In a rare type of macrodactyly (hyperostotic), there may be osteocartilaginous deposits around the
joints;
 a traumatic cause for this condition has been reported.
Macrodactyly of index finger and thumb
 Treatment
 There are no satisfactory nonsurgical methods of controlling macrodactyly.
 Attempts to compress the digit with elastic wrapping have been unsuccessful.
 Indications for surgery include enlargement, angulation, carpal tunnel syndrome, and
causalgia.
 For a progressively enlarging digit, a debulking procedure usually is needed. With this
procedure, as much excess tissue as possible is excised from one half of the digit; 3
months later, the other half is debulked. This procedure may be required several times
during the growth period.
 Tsuge proposed that the disproportionate growth is a result of excessive neural input
and recommended that the digital nerves be stripped of one half their fascicles at the
time of debulking.
 He also recommended complete excision of the enlarged digital nerves during debulking as
the most effective way to control progressive macrodactyly, believing that this causes only
minimal neural impairment in children.
 Kelikian recommended segmental resection of the tortuous digital nerves with end-to-end
repair.
 Physeal arrest by drilling holes through the physes, resection of the physes, or epiphysiodesis
of all phalanges frequently is recommended after the digit has reached estimated adult length.
 Various methods of digital shortening also have been described, including simple amputation
of the distal phalanx and filleting of the distal phalanx, with transfer of the nail and matrix
onto the end of the middle phalanx, with or without some of the underlying distal phalanx
 In the angulated finger, closing wedge osteotomies through then proximal or middle
phalanx are necessary for correction.
 Tan et al. performed middle phalangectomy in one patient with macrodactyly as their
preferred surgical option.
 Millesi described a complicated technique for shortening the enlarged thumb, in which
parts of the distal and middle phalanges are removed and the distal interphalangeal
joint is preserved.
 Shortening procedures, however, are prone to stiffness and development of
contractures.
 Amputation is used to provide relief only as a last resort in an adult with a severe and
bothersome deformity.
 Although not routinely performed, long finger pollicization has been reported for
severe nonreconstructable macrodactyly of the index finger and thumb.
 The most common complication is recurrence, which is expected after debulking.
Flap necrosis is a major surgical complication, and some authors have
recommended excision of the overlying skin and replacement with a full-thickness
skin graft to avoid this problem.
 Careful attention to flap design may help prevent skin necrosis. Operating on only
one side of the finger at a time minimizes the risk of circulatory disturbance.
 DEBULKING (TSUGE)

 Under tourniquet control, make a midlateral incision the length of the involved digit.
 Identify and dissect out the digital nerve.
 Excise all excessive adipose tissue.
 If the digital nerve is grossly enlarged, half the fascicles may be stripped and excised as
recommended by Tsuge. If the digital nerve is excessively tortuous, a section can be
resected and an end-to-end repair performed as described by Kelikian.
 Resect matching sections of the volar half of the distal phalanx and the dorsal half of the
middle phalanx and reduce the fragments. Remove excessive skin, close the incision and
apply a bulky hand dressing.
 No particular postoperative protection is required.
 Debulking of the opposite side of the digit can be done 3 months after the first procedure.
A, Recurrent macrodactyly in 6-year-old child 2 years
after debulking procedure of ring finger and
amputation of long finger.
B, Intraoperative photograph shows enlargement of
digital nerve.
C, Wound closure after debulking.
A, Matching sections (shaded areas) of volar half of distal phalanx and dorsal half of
middle phalanx are removed.
B, Distal phalanx is reduced on middle phalanx, with preservation of dorsal skin
bridge, but removal of excess soft tissue.
C, Soft-tissue closure is completed, accepting some excess dorsal soft tissue.
 EPIPHYSIODESIS

 Under tourniquet control, make a midlateral incision the length of the entire finger.
 Identify the physes of the proximal, middle, and distal phalanges, and perform
epiphysiodesis of these with a high-speed burr or curet and cautery.
 Close the incision and apply a finger splint, which is worn for 3 weeks.
 DIGITAL SHORTENING (BARSKY)

 Under tourniquet control, make an L-shaped incision beginning at the midlateral aspect of
the proximal interphalangeal joint and extending distally to a level just proximal to the
germinal matrix Carry the incision transversely across the dorsum of the finger.
 Remove the distal half of the middle phalanx and the proximal part of the distal phalanx.
 Using a rongeur, sharpen the distal end of the remaining middle phalanx to a point to fit
into the medullary canal of the distal phalanx (Fig. 79-58B).
 Place the distal phalanx onto the middle phalanx and fix it with a Kirschner wire to recess
the finger.
 Excess volar soft tissue can be removed at a later stage.
 Close the incision and apply a finger splint to be worn for 3 weeks.
A, L-shaped midlateral and dorsal incisions allow removal of excess dorsal tissue, distal
half of middle phalanx, and proximal portion of distal phalanx (shaded area).
B, Bone ends are preparedfor pencil-cone reduction.
C, Distal phalanx is reduced on middle phalanx and secured with Kirschner wire.
 THUMB SHORTENING (MILLESI)

 Under tourniquet control, excise the distal half of the nail and nail matrix and the

underlying distal phalangeal tuft.

 Through a dorsal longitudinal incision overlying the proximal and distal phalanx, remove

the middle third of the distal phalanx and the middle third of the overlying nail and matrix.

 Remove the middle third of the proximal phalanx by making parallel oblique osteotomies.

 Reduce the two remaining longitudinal components of the distal phalanx and pin them

with a transverse Kirschner wire.

 Reduce the distal and proximal fragments of the proximal phalanx in a shortened fashion

and pin them with an oblique Kirschner wire.

 Close the wound by carefully approximating the skin edges and the nail matrix,
leaving the Kirschner wires protruding through the skin.
 Apply a thumb splint.
 POSTOPERATIVE CARE. The splint is worn for 3 weeks.
 The Kirschner wires are removed when the osteotomy incisions are healed, usually
by 4 to 6 weeks.
A, Removal of distal half of nail and distal phalanx, preserving eponychial tissue.
B, Reduction osteotomies performed through dorsal incision.
C, Remaining bone reduced and pinned.
AMPUTATIONS
Thank you