1/11. 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 = malformation (Clic here for more details about this article) |
2/11. Neurocutaneous melanosis in association with the Dandy-Walker complex, complicated by melanoma: report of a case and literature review.Neurocutaneous melanosis is a rare congenital neurocutaneous syndrome in which benign and malignant melanocytic tumors of the leptomeninges with large or numerous congenital melanocytic nevi develop. The Dandy-Walker malformation occurs as a broad posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle communicating with the posterior fossa. association of these entities is very unusual and only 10 previous reports were found in the literature. Our patient had multiple, medium-size to small melanocytic nevi present since birth. At 5 years of age the patient has intracranial pressure secondary to hydrocephalus. A diagnosis of Dandy-Walker malformation and suspected neurocutaneous melanosis was established after a skull computed tomography (CT) scan. Three months later the patient developed a right frontal tumor shown on the CT scan. The histologic finding was nevomelanocytic infiltration with strong pleomorphism. The tumor grew rapidly, producing neurogenic shock and death. The postmortem report indicated malignant melanoma.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
3/11. Giant congenital melanocytic nevi in a patient with brain structural malformations and multiple lipomatosis.We present a 9-year-old boy diagnosed from birth with giant congenital melanocytic nevi. He had central structural brain malformations of hemimegalencephaly of the right frontotemporal lobe and left occipitoparietal lobe, choroid plexus hypertrophy, and a Dandy-Walker variant. In addition, he developed multiple lipomatoses. These lesions were cutaneous except for two at the cerebellopontine angles, which were present from birth. This patient represents a rarely documented example of two histopathologies resulting in serious complications. The diagnostic issues and histopathologic process are discussed.- - - - - - - - - - ranking = 0.83333333333333keywords = malformation (Clic here for more details about this article) |
4/11. Treatment of phacomatosis pigmentovascularis: a combined multiple laser approach.BACKGROUND: Phacomatosis pigmentovascularis (PPV) consists of a capillary malformation with a variety of melanocytic lesions, which involve various regions of the body and are difficult to treat with conventional therapeutic tools. OBJECTIVE: We described two cases with PPV (type IIa and IIb) that were successfully treated with different lasers. methods: The areas involved by both melanocytic lesions and port-wine stains were treated using the Q-switched ruby laser, the Q-switched Alexandrite laser, and the flashlamp pumped pulsed-dye laser. RESULTS: Removal of a good portion of cutaneous and vascular lesions using combined multiple laser approach was achieved after 6 sessions in the first case under general anesthesia and after 31 sessions under local anesthesia in the second case. CONCLUSION: PPV type II can be treated successfully by laser treatment. We prefer to start combined multiple laser treatment of PPV in childhood period under general anesthesia because it will reduce the number of treatment, improve the patient's quality of life, and increase the cost-effectiveness of the treatment.- - - - - - - - - - ranking = 0.16666666666667keywords = malformation (Clic here for more details about this article) |
5/11. 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 = 0.5keywords = malformation (Clic here for more details about this article) |
6/11. Coexisting intracranial meningeal melanocytoma, dermoid tumor, and Dandy-Walker cyst in a patient with neurocutaneous melanosis. Case report.Neurocutaneous melanosis (NCM) associated with Dandy-Walker malformation is a very rare congenital neurodysplasia with the same origin. Primary intracranial melanocytic and dermoid tumors are also benign congenital lesions that usually arise from the leptomeninges and are formed by the inclusion of cutaneous ectoderm at the time of neural tube closure. The authors describe a patient with coexisting intracranial meningeal melanocytoma, NCM with Dandy-Walker malformation, and intraventricular dermoid tumor.- - - - - - - - - - ranking = 0.33333333333333keywords = malformation (Clic here for more details about this article) |
7/11. Parenchymal neurocutaneous melanosis in association with intraventricular dermoid and Dandy-Walker variant: a case report.Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.- - - - - - - - - - ranking = 0.16666666666667keywords = malformation (Clic here for more details about this article) |
8/11. Congenital melanocytosis with myelomeningocele and hydrocephalus.We report an infant who had diffuse blue hyperpigmentation at birth, which rapidly progressed to gray, over the back, buttocks, and posterior aspect of the legs, and an associated congenital anomaly. The entire thickness of the dermis contained numerous very prominent melanocytic cells which, especially in the superficial dermis, were markedly dendritic, rather than merely fusiform. An epidermal melanocyte proliferation was also present. Histologically, the lesion thus differed markedly from a mongolian spot. We believe that diffuse hyperpigmentation in the neonate should call attention to the possibility of an associated neuroectodermal malformation.- - - - - - - - - - ranking = 0.16666666666667keywords = malformation (Clic here for more details about this article) |
9/11. Congenital arrector pili hamartoma. A case report and review of the spectrum of Becker's melanosis and pilar smooth-muscle hamartoma.Congenital pigmented arrector pili hamartomas are unique malformations of epidermis and pilar apparatus usually appearing as localized, lightly pigmented, hairy plaques. Characteristic microscopic features include smooth-muscle proliferation similar to irregularly disposed arrectores pilorum, and slight elongation of epidermal rete with hypermelanosis of the basal unit. An otherwise normally developed child who had this hamartoma at birth is described in an attempt to clarify the relationship between pilar smooth-muscle hamartomas and Becker's melanosis. We propose that these two entities belong at different poles of the same developmental spectrum of hamartomatous change.- - - - - - - - - - ranking = 0.16666666666667keywords = malformation (Clic here for more details about this article) |
10/11. Gastric triplication and peritoneal melanosis.Duplications of the intestinal tract are rare malformations, and triplications are even less frequent; only two cases are found in the literature. Peritoneal melanosis, a diffuse, black pigmentation of the peritoneum is also rare; only six cases have been reported; five in young women and one in a 2-year-old girl. The authors report on a 6-month-old boy with a gastric triplication and peritoneal melanosis. The diagnostic criteria and the possible embryological origin are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = malformation (Clic here for more details about this article) |
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