6
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Congenital Bathing Trunk Nevus with Meningomyelocele

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Sir, A 3-day-old female neonate, first born to non-consanguineous parents with unsupervised antenatal period was brought with paucity of movements of the lower limbs and a black skin lesion with ulcerated mass protruding through the midline in the lower back noted since birth. She did not have urinary dribbling or retention. At the time of presentation, child was noted to have elevated hyperpigmented lesion with thick hair measuring 15 × 10 cm involving entire posterior torso suggestive of congenital bathing trunk nevus surrounding the meningomyelocele as shown in Figure 1. Initial screening in form of the ultrasound of cranium was normal. Child underwent meningomyelocele repair. Currently child is asymptomatic; and, magnetic resonance imaging (MRI) brain has been planned on follow up. Figure 1 Hyperpigmented giant hairy nevus surrounding the meningomyelocele with satellite lesion noted over buttock area Congenital bathing trunk nevus is a rare lesion with female preponderance and an incidence of one in 2–5 lakh births. Though named congenital as it is noticed soon after birth, it is not hereditary and occurs due to somatic mutations in NRAS (chromosome 1p13) and BRAF gene.[1] A strong association exists between congenital giant melanocytic nevus, neurofibromas, lipomas, and neural tube defects (NTD).[2] Congenital nevus is said to be giant when it covers >2% of body surface area or 5 cm in neonates or 20 cm in adults. Lesions may be small in infants and grow proportionately as the body grows. It can occur anywhere on the body with predilection over trunk and is associated with pigmented satellite lesions. It may be flat or raised, hypo- or hyperpigmented, and color may change over time with majority (95%) being associated with thick hair in the lesion. Age of presentation varies; adult presentation is seen when they develop multiple nodules (lipoma, neurofibroma) or when it ulcerates (sclerotic variant of melanoma) while adolescents and girls present for cosmetic reasons. Neurocutaneous melanosis (NCM) and malignant melanoma are the two most important complications.[3] NCM is characterized by proliferation of melanocytes present in the meninges of brain and spinal cord and present as headache, seizures, movement abnormalities, and brain tumors. Risk of malignant melanoma is 2–5 times higher and occurs in the first 5–10 years of life.[4] High-risk lesions for malignant transformation include those with size >20 cm, satellite lesions and multiple (>3) lesions. Bathing trunk nevus needs continuous follow-up to monitor for signs of malignant transformation. Surgical excision of high-risk lesions is best performed in early childhood.[5] Many non-surgical procedures have been tried and each has its own limitations. Serial excision with tissue expansion is the mainstay of treatment of bathing trunk nevus. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

          Related collections

          Most cited references 5

          • Record: found
          • Abstract: found
          • Article: not found

          Congenital nevocytic nevi and malignant melanomas.

          The subject of the relationship between congenital nevocytic nevi and malignant melanomas has many controversial aspects. Data are insufficient to come to firm conclusions concerning how often melanomas supervene in such nevi and to determine the prognosis of those melanomas. What data are available indicate that malignant melanomas develop in a substantial percentage of large (giant) nevocytic nevi. This lends support to those who recommend surgical excision of such large lesions, when feasible. On the other hand, the information currently available concerning the association of malignant melanomas with medium- and small-sized congenital nevocytic nevi (i.e., those less than 20 cm in larges diameter) is scanty. Because of this lack of data on congenital nevocytic nevi, our current approach is to take into consideration the probability of cosmetic improvement by surgical removal in conjunction with education of the patient and/or family that malignant melanomas have been known to occur in congenital nevocytic nevi but are probably rare except in nevi of large size. Such information provided to the patient and/or family will often help in making the decision for removal or continued observation. A Congenital Nevocytic Nevus Registry has been established in the Oncology Section of the Skin and Cancer Unit at New York University Medical Center in the attempt to begin a long-term prospective study which might eventually provide some meaningful information concerning the natural history of such lesions, including the incidence of malignant melanomas.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Congenital melanocytic nevi: treatment modalities and management options.

            Congenital melanocytic nevi can be cosmetically disfiguring, give rise to melanoma, and suggest the presence of neurocutaneous melanocytosis. Management decisions must be tailored for each patient and each nevus, taking into consideration the risk for developing malignancy, risk for developing symptomatic neurocutaneous melanocytosis, cosmetic implications of having the nevus, cosmetic implications of any resultant surgical scars from their removal, adverse effects that the nevus may have on psycho-social development, and the adverse effects and long-term sequelae of any surgical intervention. The advantages and disadvantages of different modalities used in the treatment of congenital melanocytic nevi are discussed. Organizational flow diagrams are presented to help clinicians in managing patients with different sized congenital melanocytic nevi.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Syndromes associated with melanocytic nevi.

              Melanocytic nevi have been reported in association with several congenital syndromes. This review describes the clinical and cutaneous manifestations of six syndromes associated with congenital melanocytic nevi, two associated with acquired nevi, and six associated with melanocytic nevi in which insufficient evidence exists to classify them as congenital or acquired. It is important to recognize these associations to evaluate and counsel patients with melanocytic nevi. Early recognition will also facilitate timely intervention.
                Bookmark

                Author and article information

                Journal
                Indian Dermatol Online J
                Indian Dermatol Online J
                IDOJ
                Indian Dermatology Online Journal
                Wolters Kluwer - Medknow (India )
                2229-5178
                2249-5673
                Mar-Apr 2019
                : 10
                : 2
                : 186-187
                Affiliations
                Department of Pediatrics, PGIMER, Chandigarh, India
                Author notes
                Address for correspondence: Dr. Vijai Williams, Department of Pediatrics, PGIMER, Chandigarh - 160 012, India. E-mail: vijaiwilliams@ 123456gmail.com
                Article
                IDOJ-10-186
                10.4103/idoj.IDOJ_256_18
                6434768
                Copyright: © 2019 Indian Dermatology Online Journal

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                Categories
                Letters to the Editor

                Dermatology

                Comments

                Comment on this article