Macrodactyly A rare anomaly

WELCOMEDR. MD. ABDUL AZIZRESIDENT NITORBANGLADESHbigazizabdul89@yahoo.com

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.

FLATT classification according to PATHOLOGY

Type1: Lipofibromatosis

01Type II: Neurofibromatosis

02Type III: Digital hyperostosis

03Type IV: Hemihypertrophy

PRESENTATION:

1History of asymmetry to digits can be present at birth or appearing over time

3Inability to use digits

4Complaints of cosmetic issues

IMAGINGRadiographs

01CT MRI (not typically needed)

02Angiography ( for surgical planning)

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

Macrodactyly A rare anomaly

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