1/5. Neurocutaneous melanosis associated with Dandy-Walker malformation. case report and review of the literature.Neurocutaneous melanosis is a rare dysmorphogenesis associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the leptomeninges anywhere in the central nervous system (CNS). It is interesting that almost 8-10% of patients had associated Dandy-Walker malformation in the literature, suggesting a common origin of the developmental abnormalities. In this article, we present a 2-year-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. We reviewed the literature and discuss the pathogenesis based on the preferred hypotheses so far.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/5. PHACE syndrome: report of one case.PHACE syndrome indicates a neurocutaneous syndrome including Posterior fossa malformation, large facial hemangioma, Arterial malformation, Coarctation of the aorta and/or other Cardiac anomalies, and eye abnormalities. We here report a female infant who presented a giant facial hemangioma. Dandy-Walker malformations with hydrocephalus was found subsequently. We treated the hemangioma with prednisolone, methylprednisolone, interferon-alpha, and local injections of corticosteroid. encephalomalacia was found later and it was attributed to carotid artery anomalies. brain image studies, thorough ophthalmologic and cardiac examinations are strongly recommended in infants with large, plaquelike facial hemangiomas. Carotid angiography should also be considered if carotid artery anomalies are suspected.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/5. Neurocutaneous melanosis associated with Dandy-Walker malformation and a meningohydroencephalocele. Case report.Neurocutaneous melanosis and Dandy-Walker malformation are both forms of rare congenital neurodysplasia. Interestingly, 8 to 10% of patients with neurocutaneous melanosis also harbor an associated Dandy-Walker malformation, indicating that these developmental abnormalities share a common origin. The authors describe a case of neurocutaneous melanosis associated with Dandy-Walker malformation and an occipital meningohydroencephalocele with a giant melanotic nevus. Multiple congenital liver masses were also observed in the infant. The occipital nevus was totally excised, and ventriculoperitoneal and cyst-peritoneal shunts were created to prevent subsequent hydrocephalus. Findings in this case support the possibility that excessive melanocytes hinder normal mesenchymal development, causing Dandy-Walker malformation and an occipital meningocele.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/5. Neurocutaneous melanosis in association with the Dandy-Walker complex.An infant had a giant congenital nevus, neurocutaneous melanosis (NCM), and a Dandy-Walker malformation of the brain. The diagnosis of NCM was suspected at 6 weeks of age when macrocephaly was noted, resulting in the discovery of hydrocephalus and a Dandy-Walker malformation. Serial magnetic resonance imaging scans demonstrated so-called T1 shortening in the pia or subarachnoid spaces surrounding the cerebellar vermis and in the temporal lobes anterior to the temporal horns. Eventually, a biopsy-proved melanoma developed in the anterior temporal lobe, in an area previously noted to have T1 shortening. Since meningeal cells have been shown experimentally to play a critical role in cerebellar development, we hypothesize that the association of NCM with a Dandy-Walker malformation may be due to meningeal melanosis disrupting the normal development of the cerebellum and fourth ventricle.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/5. dandy-walker syndrome forming a giant occipital meningocele--case report.A boy was born with dandy-walker syndrome associated with a giant occipital meningocele, cleft lip, and cleft palate. The meningocele was actually a component of the giant posterior fossa cyst which communicated with the fourth ventricle. A cyst-peritoneal shunt achieved a considerable decrease in the size of the meningocele, but decubital ulcers developed due to restricted head movement caused by the occipital lesion. Cranioplasty removed a wide area of the inferior occipital bone, and the boundary between the superior occipital and parietal bones was thinned to allow free bending of the bone flap. The meningocele was removed totally in the third operation, but infection of the wound and pneumonia developed, causing death. The coexistence of dandy-walker syndrome and occipital meningocele, together with midline facial anomalies, may suggest a later pathogenesis of dandy-walker syndrome than previously believed.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |