Reply to Y. Khakoo et al

Abstract

We thank Drs Khakoo and Marghoob for their interest in our article. We would like to take the opportunity to respond to the comments brought forward by them. Khakoo and Marghoob criticized that our article might mislead readers to believe that neurocutaneous melanosis (NCM) is a generally fatal disease. This is certainly not our intention, and we are fully aware of the heterogenous nature of this disease. Our article—and this is indicated in the title—describes only a case report of a child with a symptomatic NCM and correlating Dandy-Walker malformation leading to death. Children with this rare defect do have a poor life expectancy, as already demonstrated by several other case reports. In our article, we differentiate between symptomatic and asymptomatic NCM and clearly cite that “asymptomatic cerebral melanosis is assumed to occur in as many as 25% of infants with giant nevi,” which is concordant with the figures contemplated by Khakoo and Marghoob. Khakoo and Marghoob also comment that small melanin deposits in the brain may be missed on magnetic resonance imaging scans. This can certainly occur; however, it is unclear whether these small deposits will become symptomatic and whether the detection of these small deposits is leading to any therapeutic consequences. Lastly, we absolutely agree that for the best management of patients with NCM, “a multidisciplinary team approach” is necessary. We therefore concluded our article, which was written by an interdisciplinary board of dermatologists, pediatricians, neurosurgeons, and neuropathologists, by saying that “interdisciplinary care of patients with symptomatic NCM by pediatricians, dermatologists, and neurosurgeons is essential.”