Biomaterial Database

Congenital melanocytosis with myelomeningocele and hydrocephalus. 1986

R A Schwartz, and N Cohen-Addad, and M W Lambert, and W C Lambert

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.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008544	Melanocytes	Mammalian pigment cells that produce MELANINS, pigments found mainly in the EPIDERMIS, but also in the eyes and the hair, by a process called melanogenesis. Coloration can be altered by the number of melanocytes or the amount of pigment produced and stored in the organelles called MELANOSOMES. The large non-mammalian melanin-containing cells are called MELANOPHORES.	Melanocyte
D008548	Melanosis	Disorders of increased melanin pigmentation that develop without preceding inflammatory disease.	Chloasma,Freckles,Melanism,Melasma,Chloasmas,Freckle,Melanoses,Melasmas
D008591	Meningomyelocele	Congenital, or rarely acquired, herniation of meningeal and spinal cord tissue through a bony defect in the vertebral column. The majority of these defects occur in the lumbosacral region. Clinical features include PARAPLEGIA, loss of sensation in the lower body, and incontinence. This condition may be associated with the ARNOLD-CHIARI MALFORMATION and HYDROCEPHALUS. (From Joynt, Clinical Neurology, 1992, Ch55, pp35-6)	Myelocele,Myelomeningocele,Acquired Meningomyelocele,Myelomeningocele, Acquired,Acquired Meningomyeloceles,Acquired Myelomeningocele,Acquired Myelomeningoceles,Meningomyelocele, Acquired,Meningomyeloceles,Meningomyeloceles, Acquired,Myeloceles,Myelomeningoceles,Myelomeningoceles, Acquired
D009432	Neural Crest	The two longitudinal ridges along the PRIMITIVE STREAK appearing near the end of GASTRULATION during development of nervous system (NEURULATION). The ridges are formed by folding of NEURAL PLATE. Between the ridges is a neural groove which deepens as the fold become elevated. When the folds meet at midline, the groove becomes a closed tube, the NEURAL TUBE.	Neural Crest Cells,Neural Fold,Neural Groove,Cell, Neural Crest,Cells, Neural Crest,Crest, Neural,Crests, Neural,Fold, Neural,Folds, Neural,Groove, Neural,Grooves, Neural,Neural Crest Cell,Neural Crests,Neural Folds,Neural Grooves
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006849	Hydrocephalus	Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, INTRACRANIAL HYPERTENSION; HEADACHE; lethargy; URINARY INCONTINENCE; and ATAXIA.	Communicating Hydrocephalus,Congenital Hydrocephalus,Obstructive Hydrocephalus,Post-Traumatic Hydrocephalus,Aqueductal Stenosis,Cerebral Ventriculomegaly,Fetal Cerebral Ventriculomegaly,Hydrocephalus Ex-Vacuo,Hydrocephaly,Aqueductal Stenoses,Cerebral Ventriculomegalies,Cerebral Ventriculomegalies, Fetal,Cerebral Ventriculomegaly, Fetal,Fetal Cerebral Ventriculomegalies,Hydrocephalus Ex Vacuo,Hydrocephalus Ex-Vacuos,Hydrocephalus, Communicating,Hydrocephalus, Congenital,Hydrocephalus, Obstructive,Hydrocephalus, Post-Traumatic,Post Traumatic Hydrocephalus,Stenoses, Aqueductal,Stenosis, Aqueductal,Ventriculomegalies, Cerebral,Ventriculomegalies, Fetal Cerebral,Ventriculomegaly, Cerebral,Ventriculomegaly, Fetal Cerebral