M Haustein1, N Terai, J Pablik, L E Pillunat, F Sommer. 1. Klinik und Poliklinik für Augenheilkunde, Universitätsaugenklinik der medizinischen Fakultät Carl Gustav Carus, TU Dresden, Fetscher Str. 74, 01307, Dresden, Deutschland, michael.haustein@uniklinikum-dresden.de.
Abstract
HISTORY: Swelling of the upper eyelid in childhood is caused by a variety of diseases and is very often generated by inflammation but orbital tumors should always be considered in the differential diagnostics. METHODS: We report about a 4-year-old girl with a drug-resistant swelling of the upper eyelid and ptosis of the right eye. This case report demonstrates the route from initial clinical examination to diagnosis and additionally reviews the current status of therapeutic options. RESULTS: After magnetic resonance imaging (MRI) and diagnostic excision, Langerhans cell histiocytosis (LCH) could be histologically proven. Visual acuity and levator muscle function improved from 0.5 to 1.25 and from 2 mm to 12 mm, respectively, by amblyopic prophylaxis and immunosuppressive therapy. CONCLUSIONS: Persistent and drug-resistant swelling of the upper eyelid in childhood is also strongly suspicious for tumors. The suspicion of rare orbital tumors in children can be frequently substantiated by MRI. Biopsy and histological diagnosis are essential to plan adequate treatment and to estimate the prognosis. Particularly in Langerhans cell histiocytosis the methods of choice for over 10 years are specific immunochemical procedures (detection of protein s100 and CD1a).
HISTORY: Swelling of the upper eyelid in childhood is caused by a variety of diseases and is very often generated by inflammation but orbital tumors should always be considered in the differential diagnostics. METHODS: We report about a 4-year-old girl with a drug-resistant swelling of the upper eyelid and ptosis of the right eye. This case report demonstrates the route from initial clinical examination to diagnosis and additionally reviews the current status of therapeutic options. RESULTS: After magnetic resonance imaging (MRI) and diagnostic excision, Langerhans cell histiocytosis (LCH) could be histologically proven. Visual acuity and levator muscle function improved from 0.5 to 1.25 and from 2 mm to 12 mm, respectively, by amblyopic prophylaxis and immunosuppressive therapy. CONCLUSIONS: Persistent and drug-resistant swelling of the upper eyelid in childhood is also strongly suspicious for tumors. The suspicion of rare orbital tumors in children can be frequently substantiated by MRI. Biopsy and histological diagnosis are essential to plan adequate treatment and to estimate the prognosis. Particularly in Langerhans cell histiocytosis the methods of choice for over 10 years are specific immunochemical procedures (detection of protein s100 and CD1a).
Authors: Jean Donadieu; C Piguet; F Bernard; M Barkaoui; M Ouache; Y Bertrand; H Ibrahim; J F Emile; O Hermine; A Tazi; T Genereau; C Thomas Journal: Pediatr Blood Cancer Date: 2004-12 Impact factor: 3.167
Authors: B E Favara; A C Feller; M Pauli; E S Jaffe; L M Weiss; M Arico; P Bucsky; R M Egeler; G Elinder; H Gadner; M Gresik; J I Henter; S Imashuku; G Janka-Schaub; R Jaffe; S Ladisch; C Nezelof; J Pritchard Journal: Med Pediatr Oncol Date: 1997-09