Document Detail


Pre-term diagnosis of choristoma and choroidal coloboma in Goldenhar's syndrome.
MedLine Citation:
PMID:  21345085     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Abstract objectives: Besides general pathological findings characteristic for Goldenhar's syndrome, we report ocular findings in a 22-week-old fetus with hemifacial microsomia endorsing this diagnosis. methods: After abortion the fetus was examined by a standard paidopathological autopsy including ophthalmopathologic macroscopic and microscopic examination of both eyes.principal results: Post mortem findings included left hemifacial microsomia with ipsilateral microtia, atresia of the acoustic meatus, microphthalmia, a ventricular septal defect and abnormalities of the ribs. Ophthalmopathological examination of the affected microphthalmic eye revealed a scleral choristoma (cartilage), choroidal/RPE coloboma and staphyloma.conclusion: General pathology findings plus the ocular findings (including a cartilaginous choristoma previously not reported in association with this syndrome) allowed the diagnosis of Goldenhar's syndrome. A discussion of differential diagnoses is provided thus confirming that the ophthalmopathological investigation of fetal eyes can be of great value for classifying syndromes associated with microphthalmia.
Authors:
Martina C Herwig; Ulrich Gembruch; Mark Born; Karin U Loeffler; Annette M Müller
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-2-23
Journal Detail:
Title:  Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society     Volume:  -     ISSN:  1093-5266     ISO Abbreviation:  -     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-2-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9809673     Medline TA:  Pediatr Dev Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
1 University of Bonn.
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