2. • Rare
• Mostly sporadic
• Familial association is occasionally observed.
• Form between weeks 9 and 20 week of gestation
• Giant CMN 1 in 20,000 live births
3. EMBRYOLOGY
• 8 -10 wks of gestation, melanoblasts migrate
from the neural crest to skin, leptomeninges,
eyes, and ears
• Differentiate into dendritic melanocytes.
• Abnormalities in neuroectodermal
development and arrested migration or
diffrentiation of melanoblasts result in the
formation of a CMN.
4. TYPES OF NEVI:
• SMALL NEVI : <1.5 cm
• MEDIUM SIZED NEVI : 1.5 and 19.9 cm.
• GIANT NEVI : >20 cm
• In an infant, 9 cm scalp and 6 cm over the trunk
5.
6. CHARACTERISTICS
• Initially hairless, pale brown, flat
• Hyperpigmentation, hairs may develop,
elevated
• Surface can be wrinkly, pebbly or
cerebriform appearance.
• May be associated with multiple
smaller satellite lesions dispersed over
the trunk, extremities or head and
neck
• Satellite lesions in 80% of giant CMN.
7. CHARACTERISTICS
• Giant CMN may be associated with
Spina bifida
Scoliosis
Elephanitiasis
Clubfoot
Cranial osseous hypertrophy
8. • Common sites: posterior trunk,
extremities, head and neck
• Giant nevi anatomic patterns, bathing
trunk and glove- stocking
distributions.
• kissing nevus
9. HISTOLOGIC FEATURES OF CMN
• Nevus cells within the middle to deep reticular dermis and
subcutaneous tissue or deeper structures
• Nevus cells extending between collagen bundles in the reticular
dermis (Indian files) and around sebaceous glands, sweat glands
and hair follicles
• Infiltration of arrector pili muscle
• Perifollicular and perivascular distribution of nevus cells
resembling an inflammatory reaction.
11. Malignant Transformation:
• Melanoma in small CMN 0% - 5%
• Melanoma in Giant CMN 0% - 42%
• 70% of melanomas in giant CMN occur by age 13, 50% arising in the first 3
years of life, 10% in childhood and 10% by puberty.
• Risk factors: 1) size (diameter> 20 cm) 2) young age (3 to 5 years) 3)
multiple lesions (three or more)
• Melanoma in Giant CMN deep to the dermal-epidermal junction or
extracutaneously e.g the central nervous system or retroperitoneum
• In small CMN: epidermis.
• Ulceration, bleeding, uneven pigmentation, change in shape, focal growth
or pain -> Biopsy
12. Neuro-cutaneous Melanosis
• Melanocytes present in the leptomeninges
• Dysregulation in proliferation and migration of melanoblasts in the
CNS
• Morbidity and mortality from seizures, hydrocephalus, cranial nerve
palsies, developmental delay, and other signs of CNS irritation.
• Risk factors: 1. CMN in the midline of the trunk or skull 2. multiple
satellite nevi (>20)
• MRI at 4 and 6 months of age prior to normal myelination of the
brain, which will obscure visualization of deposits of melanin.
13. history
Duration
Color, flat, hairy
With time became more pigmented, elevated and increased hair growth.
other lesion on body
history of fits or any other neurological symptoms
recent change in behaviour of lesion, any pain, irregularity of borders, ulceration,
bleeding
Systemic review on history
Social behaviour
PAST MEDICAL AND SURGICAL HISTORY
FAMILY HISTORY
SOCIO ECONOMIC HISTORY
17. TISSUE EXPANSION:
• Mechanical Creep (cellular stretch) 70%
• Biological Creep (cellular proliferation) 30%
• Stress relaxation
• Mechanism of tissue creep:
1. Disruption of elastin fibres
2. Realignment of collagen
3. Fluid displacement
4. Migration of local tissue
18. ADV:
1. Replacement of tissue of similar color and texture
2. Sensate skin coverage with skin appendages
3. Limited donor site deformity
• Expanded flap has superior vascularity
• Epidermal thickening, dermal thinning
19. • Capsule forms around the expanded
1. Inner Zone: Fibrin layer with macrophages
2. Central Zone: Fibroblast and myofibroblasts between collagen
bundles
3. Transitional Zone: Losse collagen fibres, few blood vessels
4. Outer Zone: Loose collagen fibres, established vascular layer
21. Planning
• Incision at the border of the lesion
• 10 – 20% expansion at the time of placement
• Use larger ports for easier palpability and to avoid flipping of port
• Pocket incision is closed in a watertight fashion
• Expansion begins after 7-10 days, depending upon the condition of
the flaps
• Should be filled till the skin is tense, not painful or cause skin
compromise
• Weekly expansion
23. Planning
• Expanded flaps can be advanced, transposed or rotated.
• In face and trunk, expander is placed subcutaneously
• In forehead and scalp, it is placed in subfascial plane
• When fully expanded, wait for 3-4 weeks before the 2nd stage
24. Contraindications
• In the vicinity of an immature scar
• In presence of infection
• In irradiated skin
• Under the skin graft
26. Scalp
• 2nd most common site
• CMN, scar alopecia, craniofacial abnormalities
• Temporary cranial moulding occurs, correct within 3-4 months
• Serial expansion to distribute expansile forces evenly over the hair
follicles
• Subgaleal plane
• Port placement in pre auricular region produces least migration
• 50% scalp can be covered without significant hair thinning
27.
28. Forehead
• Less than 2/3rd involved = expander
• Entire forehead = FTG
• Complications: Brow asymmetry, brow ptosis, altered hair direction,
hairline asymmetry
• Principles:
1. Mid forehead lesion = Bilateral expansion
2. Hemi forehead lesion = Serial expansion
3. Supra orbital, Temporal nevi = Expansion and transposition from
medial normal skin
4. Mild temporal nevi = expanded parietal skin
5. Brow elevation = interposing non hair bearing forehead skin
29. Face and neck
• Hide scars in natural creases
• Tension in middle and lower face can cause Lower lip droop, oral
incompetence, asymmetric smile. Specially for expanded cervical
flaps
• Expanded lateral cheek, neck, post auricular region
• Expanded FTGs
30.
31. Abdomen
• Easily expanded
• Expanded ftgs
• Expanded flaps for anterior thigh defects
• Expanded free TRAM flap
• Upper abdomen and anterior trunk -> breast distortion
32. Back
• For CMN of back and buttock
• Aesthetic and functional position of scar
33. Extremities
• Unfavorable site
• Proximal non circumferential defects = expanded flaps from the back
or shoulder
• Proximal circumferential = expanded free TRAM flap
• Distal circumferential mid – lower forearm = expansion of flank
• Fingers, webs and hands = expanded ftg from abdomen/groin
• Lower limb = lacks flexibility, expanded free flaps, expanded local
flaps serial excision, FTGs
34. Breast
• Post mastectomy reconstruction
• Poland syndrome, unilateral breast hypoplasia
• Expanders, expander implants