Microdactyly, macrodactyly and hemangioma.ppt

Microdactyly, macrodactyly and hemangioma.

1. MACRODACTYLY, MICRODACTYLY
AND HEMANGIOMA
BY
RAJAH AMINA SULEIMAN
(RN, RM, RNE, RBPN, BNSc., MSc)

2. INTRODUCTION
•Macrodactylyisnonhereditarycongenitaldigitenlargement.
•Congenitalmacrodactylyisararecongenitalmalformationcharacterized
byprogressiveenlargementofallmesenchymalelementsofadigit.
•Themostcharacteristicfindingnotedwasexcessiveovergrowthoffibro-
fattytissuewithunusuallylargefattylobules,fixedbyameshofdense
fibroustissue.
•Othernamesforthisconditionarelocalizedhypertrophy,local
gigantism,andmegalodactyly.

3. INTRODUCTION
•Barsky’sdefinitionofmacrodactylystatesthatphalanges,tendons,nerves,
vessel,subcutaneousfat,fingernails,andskinmustallbeenlarged.
•Thisovergrowthconditioncanaffectoneormultipledigitsoftheupperor
lowerextremities.
•Fingersareinvolvedmorefrequentlythantoes.
•Thedeformitycanpresentunilaterallyorbilaterally.

6. MACRODACTYLY
•There are two types of growth patterns for
macrodactyly:
•Static—the enlargement of the fingers or toes is
present at birth, and the affected digits grow at the
same pace as unaffected fingers or toes.
•Progressive—the affected fingers or toes grow at a
much faster rate than unaffected digits. The affected
fingers or toes can become enormous and severely
impact a child’s ability to perform normal activities.

7. ETIOLOGY
•The cause of macrodactyly remains unknown.
Researchers believe the condition is not inherited, but
may instead be linked to disruptions in prenatal
development.
•Macrodactyly can occur in conjunction with other
conditions and syndromes,
includingneurofibromatosis, lipofibromatous
hamartoma, vascular conditions, and tuberous
sclerosis complex.

8. SIGNS AND SYMPTOMS
•The main symptom of macrodactyly is the appearance of one or more abnormally large
fingers or toes. The larger digits may grow at the same rate as other non-affected
fingers/toes, or have much faster growth.
Other symptoms of macrodactyly may include:
•Abnormal growth in the affected hand or foot
•Stiffness
•Difficulty moving, grasping, or using the affected hand/foot
•Swelling
•Pain

9. MANAGEMENT
•The primary indication to operate on a digit with macrodactyly is to improve
function.
•Epiphysiodeses: Surgery is delayed until the finger(s) reaches the same
length as the same digit in the hand of the parent of the same gender.
Epiphysiodeses, usually of proximal and middle phalanges but also of
metacarpals if necessary, can be used to stop longitudinal growth.
•Circumferential growth, however, needs to be addressed by debulking
procedures that often require removal of the underlying involved digital
nerve as well as skin and subcutaneous overgrowth of an enlarged soft-
tissue segment.

11. MICRODACTYLY
•This is an abnormal smallness or shortness of the fingers or toes.
•It is an inherited pathological condition in which a child is born with
extremely short fingers and/or toes due to the bones being extremely
short.

12. CLINICAL MANIFESTATION
•The finger is small
•Muscles are missing
•Bones are underdeveloped or missing
•There is complete absence of a finger

14. MANAGEMENT
•There is no general, specific management or treatment that is applicable
to all forms of microdactyly.
•Plastic surgery is only indicated if the microdactyly affects hand function
or for cosmetic reasons, but is typically not needed. Physical therapy and
ergotherapy may ameliorate hand function.

15. HEMANGIOMA
•Ahemangiomaorhaemangiomaisusuallybenignvasculartumorderivedfrom
bloodvesselcelltypes.
•Themostcommonformisinfantilehemangioma,knownasa"strawberrymark",most
commonlyseenontheskinatbirthorinthefirstweeksoflife.
•Ahemangiomacanoccuranywhereonthebody,butmostcommonlyappearsonthe
face,scalp,chestorback.
•Theytendtogrowforuptoayearbeforegraduallyshrinkingasthechildgetsolder.

16. TYPES
•Infantilehemangiomasarethemostcommonbenigntumorfoundin
children.
•Theyaremadeupofbloodvessels,oftencalledstrawberrymarks,and
aremorecommoningirlsthaninboys.
•Theyusuallyappearontheskinofinfantsinthedaysorweeksafter
birth.
•Theytendtogrowquicklyforuptoayear.Mostthenshrink
orinvolutewithoutfurtherproblem,howeversomecanulcerateand
formscabswhichcanbepainful

17. TYPES
•Congenital hemangiomas
•Congenital hemangiomasare present on the skin at birth, unlike
infantile hemangiomas, which appear later.
•They are fully formed at birth, meaning that they do not grow after a
child is born, as infantile hemangiomas do.
•They are less common than infantile hemangiomas. Congenital
hemangiomas can be coloured from pink to blue.

18. TYPES
•Congenital hemangiomas are classified into:
•Rapidly Involuting Congenital Hemangiomas (RICH)
•Noninvoluting congenital hemangiomas(NICH).
•Partially involuting congenital hemangiomas(PICH)

21. MANAGEMENT
•Oralbeta blockerssuch aspropranololoratenololworks by narrowing
the hemangioma's blood vessels, stopping them from proliferating and
bringing forward their natural cell death.
•Topical beta blockers such as timolol. They are most helpful for thin
superficial hemangiomas.
•Corticosteroids
•Laser surgery