Abstract
The purpose of this study was to explore the patient whose diffuse parotid gland enlargement propable related with glycogen storage disease III(Cori-Forbes Disease).
Study Design and Setting: A 13 year-old child who has a glycogen storage disease(GSD) III was referred to our department due to bilateral parotid gland swelling. We performed an open incisional biopsy of the parotid gland with continuous monitoring of the facial nerve.
Results: Periodic Acid Shift (PAS) painting was studied to specimen and light microscopy of prepared was examined. The histological result was correlated well with parotid gland glycogenosis.
Conclusion: Polysaccharide deposits in glycogenosis III are not restricted to skeletal muscle and liver, and to considering that polysaccharide accumulates to salivatory glands such as parotid gland and open incisional biopsy of the parotid gland can provide an accurate diagnosis, and can avoid the need for superficial parotidectomy for nonneoplastic or some disease assosiated with storage disorder This case is reported
for salywatory, and especially parotid, gland glycogenosis have never been demonstrated before.