Progressive facial hemiatrophy in a child with disorders associated with Arnold-Chiari malformation
The article presents a case of the development of Parry-Romberg syndrome (facial hemiatrophy) against the background of neurological disorders associated with Arnold-Chiari malformation type I in an 8-year-old boy. The first symptom of disease was weakness of the muscles of the left angle of the mouth, and 6 months afterwards there appeared asymmetry, facial atrophy and hypoplasia of the left lower jaw. Later on, skin hyperpigmentation on the affected side of the face and neck was increasing. The morphological signs of a limited sclerodermic process of the atrophodermia type were found in skin bioptate of the affected side. The examination found that a child has no evidence of systemic scleroderma, and also diagnosed malformation of the cervicomedullary junction type I, which apparently caused the development of unilateral facial atrophoderma. During treatment (glucocorticosteroids- parenterally and locally, cytostatics, vascular, immunosupressive and detoxification drugs, multivitamins with trace elements, topical anti-inflammatory drugs) and after its termination a positive dynamics was noted in skin lesion foci: decreased severity of atrophy, recovery of tissue turgor, increased range of motion of facial muscles, improvement of the patient's appearance, reduction of the cosmetic defect. Parry-Romberg syndrome necessitates a multidisciplinary medical approach, since early diagnosis and adequate treatment in childhood might improve the quality of life of the patient and minimize the outcomes of atrophic process.