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Neuropediatrics 1995; 26(6): 306-309
DOI: 10.1055/s-2007-979779
DOI: 10.1055/s-2007-979779
Short communications
© Hippokrates Verlag GmbH Stuttgart
Progressive Facial Hemiatrophy with Multiple Benign Tumors and Hamartomas
Further Information
Publication History
Publication Date:
19 April 2007 (online)
Abstract
We report the unusual association of Progressive Facial Hemiatrophy (Parry-Romberg syndrome) with multiple benign tumors (orbital neurinoma, mandibular odontogenous fibroma) and hamartomas. The neurological clinical features were infantile hemiplegia, mild mental retardation and focal seizures. Brain CT-scan and MRI showed porencephaly and cerebral calcifications ipsilateral to hemifacial atrophy. Immunological investigations proved negative. The etiology of the disease and the bridging of this case to phakomatoses are discussed.
Key words
Progressive facial hemiatrophy - Parry-Romberg syndrome - Microphthalmia - Benign tumors -Hamartomas - Phakomatoses