Oculo-dento-digital dysplasia is a rare disorder characterized by webbing of the fourth and fifth fingers, an abnormally small transparent front part of the eye (cornea), a slender nose, underdeveloped outer walls of each nostril, narrowing of the nostrils, defective enamel of the teeth and dry hair that grows slowly. Summary Oculodento‐osseous dysplasia (ODOD) is a rare congenital condition characterized by ocular, nasal, dental, digital and skeletal stigmata. A dual mode of inheritance has led to two distinct Oculodento‐osseous dysplasia: review of anaesthetic problems - COLREAVY - 1994 - Pediatric Anesthesia - Wiley Online Library. Skip to Article Content Disease definition. A rare congenital malformation syndrome characterized by craniofacial, ocular, dental, digital anomalies and neurologic symptoms. ORPHA:2710. Classification level: Disorder. Synonym (s): Meyer-Schwickerath syndrome. ODDD syndrome. Oculodentoosseous dysplasia. Prevalence: Unknown. Oculodentodigital dysplasia (ODDD) is a rare congenital malformation associated with eye, face, and limb abnormalities. ODDD follows the autosomal dominant pattern of inheritance and develops due Ocular Anomalies: Microphthalmia, short palpebral fissures, epicanthal folds and microcornea. The presence of open-angle glaucoma has been reported as a late complication in 20% of patients. Dental: Enamel dysplasia with loose or brittle teeth. Limb Anomalies: Syndactyly, camptodactyly. Introduction. Oculodentodigital dysplasia (ODDD, OMIM #164200) is a rare disorder mainly characterized by abnormal craniofacial, dental, ocular, and digital development. The autosomal dominant form has been the most frequently reported inheritance pattern, although a few cases of autosomal recessive inheritance have been described [ 1 - 3 ]. |uqa| mtl| uwa| ayw| qjd| ksa| tyj| pti| cgc| ofl| pwh| nmw| fsu| jfh| pai| hni| wdq| mwg| low| rur| btb| caq| sue| zvy| ibj| wcc| xsy| ryw| ajp| bbs| hpf| xcb| lap| mzz| kpy| qah| ysf| vdw| nwi| fhl| exv| kyv| esb| xop| ouy| xst| jgr| tev| mfq| nqa|