macrodactyly a rare anomaly
TRANSCRIPT
WELCOMEDR. MD. ABDUL AZIZRESIDENT [email protected]
MACRODACTYLY a rare
anomaly
DEFINITION
A Hamartomous Enlargement Of Soft Tissue And Underlying Bone.
Macrodactyly was first loosely coined by Feriz when he described lymphomatous degenerative lesions and called them “macrodystrophia lymphometosa progresiva.”
In the foot, the most common presentation of this pathology consists of excessive fibro-fatty tissue on the plantar aspect which causes dorsal curling of the toes.
Must be distinguished from other forms of Enlarged digits1. neurofibromatosis2. multiple enchondromatosis3. vascular malformations
Associtaed With
• Proteus syndrome, • Banayan-Riley-Ruvalcabe,• Maffuccin, • Ollier’s disease, and• Milroy’s disease.
EPIDEMIOLOGYVery rare
90% unilateral
70% involved more than one digit
Index finger most commonly involved
Affect toesSlight male predominace
PATHOPHYSIOLOGY
Etiology unknown
No genetic correlation
known to date
Median nerve most commonly
involved
CLASSIFICATION: Funtional ClassificationStatic
macrodactyly: enlarged in proportion to the rest of the digits
1Progressive macrodactyly: enlarged out of proportion to the rest of the digitsPedal macrodactyly tends to be of the progressive type.
2
FLATT classification according to PATHOLOGY
Type1: Lipofibromatosis
01Type II: Neurofibromatosis
02Type III: Digital hyperostosis
03Type IV: Hemihypertrophy
04
PRESENTATION:
1History of asymmetry to digits can be present at birth or appearing over time
2Pain
3Inability to use digits
4Complaints of cosmetic issues
IMAGINGRadiographs
01CT MRI (not typically needed)
02Angiography ( for surgical planning)
03
Treatment Options: Aim is the reconstruction of a pain-free functioning foot.
Non operative: mild case
(observation)
Operative: unrewarding! 1.Staged debulking
2.Epiphysiodesis3.Osteotomies & Shortening procedure
4.Amputation
BEAWRE
Ampuatation may be the best options but an adjacent digits
may start overgrowing!
tAKE HOME MESSAGE
• Macrodactyly is a congenital disease in which the soft tissue, bone, or fat of the digits affected is enlarged.
• Due to the rarity of the condition, the prevalence is unknown.
• There is not much known about this deformity, including the etiology.
• Surgical options vary, and include but are not limited to debulking of the soft tissue and fat, partial osteotomies, and amputations.
• However, each deformity must be treated on a case by case basis.
REFERENCESApley’s system of orthopaedics and fractures
01The foot and ankle journal
02Internet 03
