INIENCEPHALIES
\ɪnˈi͡ənsɪfˌaliz], \ɪnˈiənsɪfˌaliz], \ɪ_n_ˈiə_n_s_ɪ_f_ˌa_l_i_z]\
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Congenital malformations of the central nervous system and adjacent structures related to defective neural tube closure during the first trimester of pregnancy generally occurring between days 18-29 of gestation. Ectodermal and mesodermal malformations (mainly involving the skull and vertebrae) may occur as a result of defects of neural tube closure. (From Joynt, Clinical Neurology, 1992, Ch55, pp31-41)
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Sporadic Retinoblastoma
- A malignant arising nuclear layer retina that is most primary eye in children. The tumor tends to occur early childhood or infancy present at birth. majority are sporadic, but condition may be transmitted as autosomal dominant trait. Histologic features include dense cellularity, small round polygonal cells, areas of calcification and necrosis. An abnormal pupil reflex (leukokoria); NYSTAGMUS; STRABISMUS; visual loss represent common clinical characteristics this condition. (From DeVita et al., Cancer: Principles Practice Oncology, 5th ed, p2104)