Giant Congenital Melanocytic Nevus Coexistent with Chiari II Malformation

Abstract

Background: Giant congenital melanocytic nevus (GCMN) and Chiari II malformation (CM II) are distinct rare clinical entities. No previous report of this condition is to be found in the medical literature.

Material and Methods: We report the case of a male infant seen initially for a GCMN and an upper thoracic myelomeningocele. MRI incidentally revealed cerebellum and brain stem elongate via the foramen magnum. The infant’s physical examination included spastic quadriparesis and hypotonia. An operation for myelomeningocele repair and suboccipital craniectomy with resection of C₁ and cerebellar tonsils was done. The signs and symptoms of the patient were improved after surgery. The GCMN was not treated according to the parents’ wished, but a schedule of long-term monitoring was recommended.

Conclusion: This case report illustrates that CMNs can coexist with CM II. The clinician who diagnoses a patient with CMNs should remember that this entity can be associated with other pathologies of the central nervous system. Earlier diagnosis and surgical intervention improves the prognosis. Long-term monitoring and follow-up should be recommended for patients with CMNs.

Key words

congenital melanocytic naevus - myelomeningocele - Chiari II malformation - central nervous system

References

• 1 Arisoy AE, Akbasak A, Oram Y. et al . Giant congenital nevomelanocytic nevus andintracerebral calcified lesion.  Int J Dermatol. 1996;  35 877-878
• 2 Bourlond J, Bourlond A, Rousseau C. Retrolumbar subcutaneous ependymoma and giant bathing-trunk nevocellular nevus.  Int J Dermatol. 1994;  33 488-492
• 3 Faillace WJ, Okawara SH, MacDonald JV. Neurocutaneous melanosis with extensive intracerebral and spinal cord involvement. Report of two cases.  J Neurosurg. 1984;  61 782-785
• 4 Foster RD, Williams ML, Barkovich AJ. et al . Giant congenital melanocytic nevi: the significance of neurocutaneous melanosis in neurologically asymptomatic children.  Plast Reconstr Surg. 2001;  107 933-941
• 5 Ishikawa T, Nishi T, Shimada H. et al . Two cases of melanose neurocutanee with development of malignantmelanoma: A microspectrophotomeric and electron microscopy study.  Gann. 1975;  66 277-289
• 6 Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: definition and review of the literature.  J Am Acad Dermatol. 1991;  24 747-755
• 7 Kasantikul V, Shuangshoti S, Pattanaruenglai A. et al . Intraspinal melanotic arachnoid cyst and lipoma in neurocutaneous melanosis.  Surg Neurol. 1989;  31 138-141
• 8 Komatsu S, Takaku A, Suzuki J. Neurocutaneous melanosis with chronic subdural hematoma on the left side and later on the right side-a case study.  Rinsho Shinkeigaku. 1974;  14 855-860
• 9 Leaney BJ, Rowe PW, Klug GL. Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report.  J Neurosurg. 1985;  62 148-152
• 10 Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review.  Pediatr Neurosurg. 2007;  43 236-248
• 11 MacCune AB, Cohen BA, Gartner JC. Congenital choroid plexus papilloma and giant nevomelanocytic nevus: report of a case.  J Am Acad Dermatol. 1990;  22 849-851
• 12 MacLone DG, Knepper PA. The cause of Chiari II malformation: a unified theory.  Pediatr Neurosci. 1989;  15 1-12
• 13 Mena-Cedillos CA, Valencia-Herrera AM, Arroyo-Pineda AI. et al . Neurocutaneous melanosis in association with the Dandy-Walker complex, complicated by melanoma: report of a case and literature review.  Pediatr Dermatol. 2002;  19 237-242
• 14 Morioka M, Hamada J, Ohmori Y. et al . Spinal arachnoid cyst containing nevus cells in a patient with a large congenital melanocytic nevus: case report.  Neurosurgery. 2004;  55 983-984
• 15 Novotny  Jr  EJ, Urich H. The coincidence of neurocutaneous mela-nosis and encephalofacial angiomatosis.  Clin Neuropathol. 1986;  5 246-251
• 16 Ruiz-Maldonado R, Tamayo L, Laterza AM. et al . Giant pigmented nevi: Clinical, histopathologic, and therapeutic considerations.  J Pediatrics. 1992;  120 906-911
• 17 Salomão JF, Cavalheiro S, Matushita H. et al . Cystic spinal dysraphism of the cervical and upper thoracic region.  Childs Nerv Syst. 2006;  22 234-242
• 18 Shah PA, Hassan I, Nafee A. Bathing trunk naevus and neurofibromatosis type 1: a unique association of Friedreich's ataxia.  J Indian Med Assoc. 2004;  102 327-328
• 19 Tulipan N. Intrauterine myelomeningocele repair.  Clin Perinatol. 2003;  30 521-530
• 20 Heuzen EP van, Kaiser MC, Slegte RG de. Neurocutaneous mela-nosis associated with intraspinal lipoma.  Neuroradiology. 1989;  31 349-351

Correspondence