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MACRODACTYLY, MICRODACTYLY
AND HEMANGIOMA
BY
RAJAH AMINA SULEIMAN
(RN, RM, RNE, RBPN, BNSc., MSc)
INTRODUCTION
• Macrodactyly is nonhereditary congenital digit enlargement.
• Congenital macrodactyly is a rare congenital malformation characterized
by progressive enlargement of all mesenchymal elements of a digit.
• The most characteristic finding noted was excessive overgrowth of fibro-
fatty tissue with unusually large fatty lobules, fixed by a mesh of dense
fibrous tissue.
• Other names for this condition are localized hypertrophy, local
gigantism, and megalodactyly.
INTRODUCTION
• Barsky’s definition of macrodactyly states that phalanges, tendons, nerves,
vessel, subcutaneous fat, finger nails, and skin must all be enlarged.
• This overgrowth condition can affect one or multiple digits of the upper or
lower extremities.
• Fingers are involved more frequently than toes.
• The deformity can present unilaterally or bilaterally.
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.
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,
including neurofibromatosis, lipofibromatous
hamartoma, vascular conditions, and tuberous
sclerosis complex.
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
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.
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.
CLINICAL MANIFESTATION
• The finger is small
• Muscles are missing
• Bones are underdeveloped or missing
• There is complete absence of a finger
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.
HEMANGIOMA
• A hemangioma or haemangioma is usually benign vascular tumor derived from
blood vessel cell types.
• The most common form is infantile hemangioma, known as a "strawberry mark", most
commonly seen on the skin at birth or in the first weeks of life.
• A hemangioma can occur anywhere on the body, but most commonly appears on the
face, scalp, chest or back.
• They tend to grow for up to a year before gradually shrinking as the child gets older.
TYPES
• Infantile hemangiomas are the most common benign tumor found in
children.
• They are made up of blood vessels, often called strawberry marks, and
are more common in girls than in boys.
• They usually appear on the skin of infants in the days or weeks after
birth.
• They tend to grow quickly for up to a year. Most then shrink
or involute without further problem, however some can ulcerate and
form scabs which can be painful
TYPES
• Congenital hemangiomas
• Congenital hemangiomas are 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.
TYPES
• Congenital hemangiomas are classified into:
• Rapidly Involuting Congenital Hemangiomas (RICH)
• Noninvoluting congenital hemangiomas (NICH).
• Partially involuting congenital hemangiomas (PICH)
MANAGEMENT
• Oral beta blockers such as propranolol or atenolol works 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

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Microdactyly, macrodactyly and hemangioma.ppt

  • 1. MACRODACTYLY, MICRODACTYLY AND HEMANGIOMA BY RAJAH AMINA SULEIMAN (RN, RM, RNE, RBPN, BNSc., MSc)
  • 2. INTRODUCTION • Macrodactyly is nonhereditary congenital digit enlargement. • Congenital macrodactyly is a rare congenital malformation characterized by progressive enlargement of all mesenchymal elements of a digit. • The most characteristic finding noted was excessive overgrowth of fibro- fatty tissue with unusually large fatty lobules, fixed by a mesh of dense fibrous tissue. • Other names for this condition are localized hypertrophy, local gigantism, and megalodactyly.
  • 3. INTRODUCTION • Barsky’s definition of macrodactyly states that phalanges, tendons, nerves, vessel, subcutaneous fat, finger nails, and skin must all be enlarged. • This overgrowth condition can affect one or multiple digits of the upper or lower extremities. • Fingers are involved more frequently than toes. • The deformity can present unilaterally or bilaterally.
  • 4.
  • 5.
  • 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, including neurofibromatosis, 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.
  • 10.
  • 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
  • 13.
  • 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 • A hemangioma or haemangioma is usually benign vascular tumor derived from blood vessel cell types. • The most common form is infantile hemangioma, known as a "strawberry mark", most commonly seen on the skin at birth or in the first weeks of life. • A hemangioma can occur anywhere on the body, but most commonly appears on the face, scalp, chest or back. • They tend to grow for up to a year before gradually shrinking as the child gets older.
  • 16. TYPES • Infantile hemangiomas are the most common benign tumor found in children. • They are made up of blood vessels, often called strawberry marks, and are more common in girls than in boys. • They usually appear on the skin of infants in the days or weeks after birth. • They tend to grow quickly for up to a year. Most then shrink or involute without further problem, however some can ulcerate and form scabs which can be painful
  • 17. TYPES • Congenital hemangiomas • Congenital hemangiomas are 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)
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  • 21. MANAGEMENT • Oral beta blockers such as propranolol or atenolol works 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