Schreml, S. and Gruendobler, B. and Schreml, J. and Bayer, M. and Ladoyanni, E. and Prantl, L. and Eichelberg, G. (2008) Neurocutaneous melanosis in association with Dandy-Walker malformation: case report and literature review. CLINICAL AND EXPERIMENTAL DERMATOLOGY, 33 (5). pp. 611-614. ISSN 0307-6938, 1365-2230
Full text not available from this repository. (Request a copy)Abstract
Neurocutaneous melanosis (NCM) is a rare congenital noninheritable phacomatosis characterized by large and/or numerous cutaneous congenital melanocytic naevi (CMN) in combination with melanocytic leptomeningeal tumours. Dandy-Walker malformation (DWM) consists of a cystic dilatation of the fourth ventricle communicating with the posterior fossa, and a high insertion of the tentorium and hypoplasia/aplasia of the cerebellar vermis (partially caused by Zic1(+/-) Zic 4(+/-) on 3q2). An association of NCM and DWM is very rare, with only 15 previously reported cases to our knowledge. We present an 8-year-old girl with multiple CMN and DWM. A ventriculoperitoneal shunt operation was performed when she was 1 day old. Her neurological symptoms to date comprise headaches, nausea and vomiting as a result of ventriculoperitoneal shunt dislocation at the age of 4 years. The diagnosis is provisional asymptomatic multiple CMN-type NCM in association with DWM.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | COMPLEX; GENES; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Dermatologie und Venerologie Medicine > Lehrstuhl für Pathologie Medicine > Zentren des Universitätsklinikums Regensburg > Zentrum für Plastische-, Hand- und Wiederherstellungschirurgie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 26 Oct 2020 09:08 |
| Last Modified: | 26 Oct 2020 09:08 |
| URI: | https://pred.uni-regensburg.de/id/eprint/30325 |
Actions (login required)
 |
View Item |
