- Discussion:
- a hamartomatous enlargement of soft tissue & underlying bone;
- can be static, growing commensurately w/ hand, or progressive, growing faster than the rest of the hand;
- involvement in the hand is more common than involvement in the foot;
- "nerve territory-oriented macrodactyly":
- some authors believe that digital nerves cause disproportionate growth of the finger (and that excision in children
will reduce growth with minimal neurologic sequelae;
- enlargement of the nerve supply to affected digits that more commonly follow the distribution of the median nerve than that of ulnar nerve;
-
associated conditions:
-
neurofibromatosis: as fat & fibrous tissue are found in peripheral nerves of both dz's;
- lipofibromatosis
- references:
-
Lipofibroma of the median nerve in the palm and digits of the hand.
- Lipofibromatous hamartomas of the median nerve.
LG Warhold et al.
J. Hand. Surg. Vol 18-A. 1993. p 1032-1037.
- Lipofibromatous hamartoma of nerve.
PC Amadio et al.
J. Hand Surg. Vol 13-A. 1988. p 67-75.
- hemi-hypertrophy;
- Wilm's tumor, adrenal carcinoma, and hepatoblastoma: the association of hemihypertrophy w/ intra-abdominal tumors is particularly
strong with Beckwith-Wiedemann syndrome;
- macrodactyly simplex congenita:
- 10% of cases;
- enlargement includes skin, subQ tissue, nerve, joint, and bone (tendons and blood vessels are of normal size);
- most often phalanges are involved and metacarpals are spared;
- macromelia;
- proteus syndrome;
-
diff dx:
-
AV malformation
- congenital lymphangioma;
- macrodystophia lypomatosa progressive:
- rare form of localized gigantism characterized by a congenital and progressive overgrowth of all
the mesenchymal elements in digit, with a disproportionate increase in the fibroadipose tissue;
- Treatment:
- staged debulking, dealing with one side of digit or hand at a time, because blood supply to the skin of the enlarged digits is poor;
- in the first stage, defatting is performed on one side of digit, removing up to 20% of its thickness (convex side of digit is adressed first);
- in the second stage, perform similar defatting procedure and consider bone shortening;
- in severe cases, consider removal of an entire phalanx (digit must be stabilized with a K wire, extensor tendon shortened, and flexor tendon left alone);
- appropriately timed epiphysiodesis of the involved bones is performed during growth;
- alternatively, later bone resections and fusions can be carried out;
- thumb reduction has been performed by excising the central third and attaching the two lateral portions of bone side to side;
- in the report by J. Augusto Bertelli et al., the author used a hemi-Bruner zigzag incision was made on the palmar and dorsal
aspects of the ulnar sides of the digit, with incisions aligned to match the zigzags;
- an en bloc dissection included skin, fatty tissue, neurovascular bunldes, and longitudinal resection of 1/3 of the
articular surfaces and bone (proximal, middle, and distal phalanges);
- tip of the finger and part of the nail were also removed, which allowed digit shortening;
- collateral ligament of the proximal IP joint was harvested from the portion of the digit removed;
- transverse shortening was additionally achieved by resection of the physis of the distal phalanx and the DIP joint;
-
transverse retinacular ligament of the extensor tendon was sutured to the flexor tendon sheath;
- 6 months later, the patient underwent debulking of the skin on the opposite side (w/ preversation of the NV bundle);
-
Case Example: by
Jan Van Der Bauwhede MD
c 1993-1997
Jan Van Der Bauwhede MD
Macrodactyly.
PC Dell.
Hand Clinics. Vol 1(3). 1985. p 511-524.
Macrodactyly.
Barsky, A. J.: J. Bone Joint Surg. 49A:1225, 1967.
Current Concepts Review. Hemihypertrophy.
Concepts and Concepts.
RT Ballock MD. et al.
JBJS Vol 79-A.
No 11. Nov 1997. p 1713.
Macrodactyly.
PP Kotwal and M. Farooque.
JBJS Vol 80-B. No 4. July 1998. p 651.
Hemidigital resection with collateral ligament transplantation in the treatment of macrodactyly: A case report.
J. Augusto Bertelli et al.
J.Hand. Surg. 2001. Vol 26-A. p 623.
Surgical treatment of macrodactyly in older children and adults.
