Congenital giant hairy nevi have an incidence of 1 in 20,000 to 1 in 500,000 newborns, with a prevalence that is roughly equal between genders and appears across all races. Size classification varies, with 'giant' referring to lesions significantly larger than typical congenital nevi, and there is a concern about the potential for malignant transformation, particularly in larger lesions. Management depends on factors such as size and location, with treatment options ranging from monitoring to surgical interventions.

1. Congenital giant hairy
nevus
Faisal Ashfaq

6. Incidence
⚫ Congenital pigmented nevi are present in
approximately 2 to 3% of neonates.
⚫ very large congenital nevi are present in 1 per
20,000 to 1 per 500,000 newborns.
⚫ equal prevalence exists in males and females
⚫ CNN appear in all races, but, paradoxically, the
frequency of small CNN is slightly higher in some
populations such as blacks who are at lower risk of
developing melanoma than whites.

7. Classification on basis of size
⚫ < 10% of these lesions are larger than 3 or 4 cms, a size cut-off
below which the designation small is usually given.
⚫ Large and giant are debatable and confusing terms.
⚫ Definition of ‘giant’ is variable.
⚫ Pers defined ‘giant’ as palm size on the face and twice palm size
elsewhere.
Pers M. Gigantic pigmented nevi. Indications for
operative treatment. Ugeskr Laeger 1963;125:613-9
⚫ According to some : an area of more than 144 square inches is
giant. OR 2 % of body surface area
Greeley PW, Middleton AG, Curtin JW. Incidence of malignancy in
giant pigmented nevi. Plast Reconstr Surg 1965;36:26-37
⚫ others consider a lesion that could not be completely excised and
closed primarily as ‘giant’
Pilney FT, Broadbent TR, Woolf RM. Giant pigmented nevi of the face:
Surgical management. Plast Reconstr Surg 1967;40:469

8. Kopf classification
⚫ small (<1.5 cm in diameter)
⚫ medium (1.5-20 cm in diameter)
⚫ large (>20 cm in diameter

9. Another classification
⚫ Small < 1.5 cm diameter
⚫ Medium 1.5 to 10 cm
⚫ Large 11 to 20 cm
⚫ Giant (G)
⚫ G1 21-30 cm
⚫ G2 31-40 cm
⚫ G3 > 40 cm

10. Why size is important
⚫ giant congenital nevi and malignant
melanoma are associated
⚫ GCMN causes more esthetic problems,
the larger the nevus size the greater the
therapeutic challenge

11. Malignant transformation
⚫ wide controversy .Widely divergent figures range from
1.8% to up to 45%.
⚫ Larger lesions are more prone to develop malignancy
⚫ GCMN located on the vertebral column and scalp has a
greater risk of being associated to meningeal melanosis
which is a potential source of malignant transformation
⚫ One review has calculated an 8.52% incidence of
melanoma developing within nevi larger than 2% of the
total body surface during the first 15 years of life
Quaba AA, Wallace AF. The incidence of malignant melanoma (0 to 15 years of age) arising
in large congenital nevocellular nevi. Plast Reconstr Surg 1986;78:174-9.

12. History
⚫ The presence of a pigmented lesion is noted
at birth or soon thereafter.
⚫ Location and size of a congenital hairy nevus
is variable.
⚫ Small lesions appear more frequently than large
lesions.
⚫ Only 5% of lesions are multiple.
⚫ Coarse surface hairs develop in more than 50%.

13. Family history
⚫ vast majority of LCMN do not show familial
occurrence
Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: Definitionand review of the literature.
J Am Acad Dermatol 1991; 24: 747-55
⚫ Genetic mosaicism probably originates
virtually always from a postzygotic mutation,
which would explain why LCMN is usually
sporadic

14. Physical examination
⚫ Size
⚫ Borders sharp regular
irregular blends with surrounding skin
⚫ Surface textured with and without hair
⚫ Shape round oval
⚫ Color black 80%, brown 16%, mottled 4%.
⚫ Distribution single lesion less than 5% are
multiple
⚫ Associated Findings neurofibromatosis
leptomeningeal melanocytosis

15. Location of GCMN is
important in :
⚫ Eyelides Divided nevus of eyelids :As a marker of embryonal
development of nevus (< 24 w. gestation).
⚫ Limbs GCMN in the limbs : As a cause of limb hypotrophy
Ruiz-Maldonado R. et al J Pediatr 1992; 120: 906-11
⚫ Scalp as a marker of neurological alterations and
neurocutaneous melanosis
Ruiz- Maldonado R. et al Dermatology 1997; 195: 125-128
GCMN on the scalp often undergo spontaneous depigmentation
⚫ Perineum GCMN of genital and perineal area as a special
clinico-pathologic variant
Alvarez-Mendoza et al Ped Develop Pathol 2001; 4: 73-81
⚫ GCMN in mucocutaneous, orificial location, palms and soles as
therapeutic challenges.

16. Associated abnormalities with
large CMN
⚫ leptomeningeal melanocytosis
(presents with epilepsy, mental retardation, or focal
neural deficiency).
⚫ spina bifida occulta
⚫ hypertrophy of cranial bones
⚫ prognathism
⚫ high arched palate
⚫ scoliosis
⚫ atrophy of the involved limb
⚫ Cutis marmorata

17. DIFFERENTIAL DIAGNOSIS
⚫ Acquired nevomelanocytic nevus
⚫ Becker nevus:
⚫ Café-au-lait macules
⚫ Congenital blue nevus
⚫ Dysplastic melanocytic nevi
⚫ Lentigo
⚫ Mongolian spots
⚫ Nevus sebaceous
⚫ Nevus spilus
⚫ Pigmented epidermal nevi

20. Therapeutic possibilities
⚫ observation
⚫ split skin grafts
⚫ serial surgical excision
⚫ (subcutaneous inflatable expansors)
⚫ dermoabrassion
⚫ chemical pell
⚫ lasers.

21. Principles of management
⚫ Management depends on size, location, and
propensity for malignant transformation.
⚫ Aesthetic considerations are important.
⚫ Surgical treatment of giant or large CNN is
addressed at age 6 months.
⚫ Removal of smaller lesions is delayed until
adolescence.
⚫ Management of small lesions includes close
monitoring with photographic documentation.

22. Observation
Arguments supporting observation :
⚫ Malignant transformation in GCMN may
develop from extracutaneous melanocytes
(meninges).
⚫ Most treatments are traumatic and results are
often poor.

23. ⚫Surgical excision with
reconstruction is the
mainstay of treatment

25. Tissue expansion
⚫ recommended for head, neck, or torso
⚫ reconstructive outcome in the head and neck, is far
superior to that obtained with skin grafting.
⚫ For large lesions requiring more than one set of
tissue expanders, a subsequent set of expanders
can be inserted at the time of the initial flap
advancement.
⚫ waiting period of 2 weeks is usual
⚫ Even if a filling delay is necessary, serial expansion
is preferable, as it avoids the need for a repeated
operation for insertion of tissue expanders

26. Advantages of tissue
expanders
⚫ reduced donor-site morbidity
⚫ coverage with adjacent skin of similar texture
and color.

27. Disadvantages of tissue
expanders
⚫ may be a need for serial tissue expansion
requiring multiple operating room and office
visits.
⚫ widened scars
⚫ flap necrosis
⚫ hematoma
⚫ mechanical failure of tissue expander
⚫ infectionexposure or extrusion of the
expander or filler dome

28. Skin grafts
⚫ for lesions involving the extremities distal to the knee or elbow
joint.
⚫ Tissue expansion in this context has greater morbidity with less
potential gain because of the restricting circumference of skin
envelope.
⚫ Skin grafts are also preferred for reconstruction of the eyelids or
ears.
⚫ In these areas, the use of expanded flaps or serial excision
results in distortion of involved structures.
⚫ A full-thickness skin graft is always preferable to a split-thickness
graft, because of both an improved aesthetic outcome and less
subsequent graft contracture.
⚫ An expanded full-thickness graft should be considered if donor
skin limitations are problematic

29. Dermal allografts
⚫ may be useful in creating a thicker neodermis
when a splitthickness graft is required.

30. ⚫ Integra is a bilayered dermal regeneration system.
⚫ The dermal replacement layer is made of a porous matrix of
fibers that consist of cross-linked bovine collagen and shark
glycosaminoglycan (chondroitin-6-sulfate). This layer serves as a
matrix for migration of fibro-blasts, macrophages, lymphocytes,
and capillaries derived from the wound bed. The migration and
eventual replacement of this layer by the patient’s own fibroblasts
and collagen network and eventual degradation of the synthetic
matrix lead to the generation of a neodermis that is histologically
very close to normal human dermis.
⚫ The second layer of Integra consists of an epidermal substitute
created from synthetic polysiloxane polymer (silicone). This is a
temporary layer that provides wound closure, relieves metabolic
stresses due to fluid and electrolyte losses, provides a barrier
against microorganisms, and delays the need for an autograft.

31. Microsurgical techniques
⚫ may be useful if reconstruction of the face as
one aesthetic unit is required.
Siebert, J. W., and Longaker, M. T. Salvage reconstruction of an extensive facial deformity
due to congenital giant hairy nevus. Plast. Reconstr. Surg. 102: 2414, 1998.

32. ⚫ The best outcome is often achieved through
the use of multimodality therapy, particularly
for lesions involving the face or those
involving contiguous anatomic regions

33. ⚫ Leaving residual nevus in the genital and
perineal regions results in a more predictable
reconstructive outcome.

34. PHENOL
⚫ Phenol peel is potentially toxic (heart, kidney)
partially removes pigment but not hair, risk of
scaring.

35. Lasers
⚫ requires multiple procedures (and anaesthetics)
⚫ does not remove all melanocytes
⚫ Often re-pigments
⚫ has a very limited effect on hair growth.
⚫ lightening of pigmented giant congenital nevi using
these techniques may make it more difficult to
monitor the resultant lesion for signs of malignant
transformation,because alteration in pigmentation of
the lesion can no longer be followed reliably.

36. Curettage
⚫ based on the observation that in the first few weeks
of life of infants with GCMN, there is a cleavage
plane between upper dermis that contains most of
pigmented nevus cells and deeper dermis
⚫ best undertaken in first 2 weeks of life later on,it
progressively becomes difficult
⚫ leaves residual dyschromia
⚫ has no effect on hypertrichosis.
⚫ The effect of treatment on the malignant potential is
not known, but it is assumed to diminish the risk as
fewer melanocytes remain.

37. Dermabrasion
⚫ was originally discovered by accident, when
obstetric forceps sheared off part of a giant
congenital hairy naevus at birth and the area
healed without re-pigmentation.
⚫ This technique is most successful early in life
and removes the melanocytes before they
migrate deeper within the dermis during the
first year of life.

38. Frank Pilney et al recommendations
⚫ 1. Complete excision and coverage of the defect with a single sheet
of medium-thickness skin.
⚫ 2. Treatment before the age of 18 months, when a full drum of skin
can cover most of the defect, using the thorax or abdomen as the
donor site.
⚫ 3. Meticulous haemostasis, as any loss of skin graft defeats the
purpose of using a solid sheet and leads to scarring, contracture and
distortion.
⚫ 4. If the upper and lower eyelids are involved, temporary
tarsorrhaphy is used for 3 weeks.
⚫ 5. If the eyebrow is involved, leave two or three rows of hairs,
brought through the graft, to avoid the need for later reconstruction.
⚫ 6. Placement of suture lines in natural skin folds whenever possible.
Pilney FT, Broadbent TR, Woolf RM. Giant pigmented nevi of the
face: surgical management. Plast Reconstr Surg 1967; 40:469-74.

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