Congenital Nevi Treatment & Management

Updated: Apr 05, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Medical Care

The management of congenital melanocytic nevi depends on a number of factors, including the size of the lesion, the location of the lesion, the age of patient, the effect on cosmesis, and the potential for malignant transformation.

Although the risk of malignant transformation in small and medium-sized congenital melanocytic nevi has not been established, many physicians agree that the risk is probably not significant enough to warrant the prophylactic removal of all of these lesions. However, some patients may desire removal of these lesions to improve cosmesis. [44] Until evidence is presented on which to base definitive treatment guidelines, many physicians are managing small and medium-sized congenital melanocytic nevi with baseline photography and regular follow-up, with frequency dependent upon the clinician's experience and practice. [45]


Surgical Care

Surgical removal of congenital melanocytic nevi is performed for two main reasons, (1) to improve the cosmetic appearance of the patient and (2) to reduce the likelihood of malignant transformation. The increased risk of malignant transformation associated with giant congenital melanocytic nevi is well established. Ideally, these lesions are removed whenever possible. Children with congenital melanocytic nevi of the eyelids and periorbital region may also benefit from early treatment to prevent or minimize disturbance of eyelid function. [46] Barriers to removal may include the size of the lesion and its proximity to vital structures. Several different procedures are available to remove congenital melanocytic nevi. [47] Serial excision can be an effective approach. [48] After excision of a giant congenital melanocytic nevus, recurrent nevi may be evident. [49]

Surgical excision of giant congenital melanocytic nevi, depending on the size and location of the lesion, may be challenging. [50] Often, the size of the lesion necessitates a staged excision. Tissue expanders, tissue grafts, and tissue flaps are often necessary to close the large defects following excision. Cultured skin replacements have also been used in the closure of surgical wounds. [51] Because the melanocytes in such cases may extend deep into underlying tissues (including muscle, bone, and central nervous system), removing the cutaneous component may not eliminate the risk of malignancy. Excision may be facilitated by tissue expansion. Immediate expansion using a Foley catheter may be used in single-stage reconstruction of head and neck defects; it has the advantage of omnidirectional expansion. [52] Alternative strategies may be mandated. [50]

Curettage of the lesions may be performed during the neonatal period, [53] but long-term studies suggest the nevus will, in part, recur. This is likely due to those components of the epidermis that are deep to the level of curettage.

Laser treatment of the lesions has been performed with a number of different types of lasers, including the following systems:

  • High-energy pulsed carbon dioxide laser [54, 55, 56, 57]

  • Erbium:YAG laser [55, 58]

  • Normal-mode ruby laser [55, 59, 60]

  • Q-switched ruby laser [55, 60, 61]

  • Switched alexandrite laser [57, 59, 62]

Because of the lack of penetrance to deeper tissue levels, long-term recurrence is also an issue with these techniques. Laser treatment of congenital melanocytic nevi also remains controversial for a variety of reasons. The effects of sublethal laser fluences on the risk of malignant transformation of melanocytes are uncertain. Additionally, while the destruction of more superficial melanocytes may improve cosmesis, if melanoma does develop in a laser-treated lesion, it may be more likely to occur deeper in the tissue, where it may evade clinical detection until it reaches a more advanced stage.



Because of the risk of neurocutaneous melanosis in patients with giant congenital nevi or multiple smaller congenital nevi, consultation with a neurologist, pediatrician, or both may be useful to detect possible early neurologic manifestations of the disease. Even in the absence of malignancy, neurocutaneous melanosis may cause problems such as obstructive hydrocephalus.



No specific dietary recommendations are necessary for patients with congenital nevi.



No specific activity restrictions are necessary for patients with congenital nevi. However, because of the increased risk for the development of melanoma, especially in patients with giant congenital nevi, proper protective measures should be taken to minimize ultraviolet light exposure. Maintaining the ability to take part in normal activities should be a consideration when planning surgical removal of a congenital nevus.

Affected children may be stigmatized with psychological maladjustment and need special monitoring and support. [63]