Introduction
Dysgenetic polycystic disease (DPD) is one of the rarest cystic conditions affecting the salivary glands. It is considered a developmental malformation, during which the normal parenchyma of the involved gland is replaced by multiple cystic spaces of various sizes.
The lesions are predominantly seen in the parotid glands, with only a few documented cases affecting the submandibular and minor salivary glands1,2,3. An association with polycystic disease affecting other solid viscera, such as the liver, pancreas and kidneys has been suggested4,5.
A review of the literature revealed 19 reported cases of DPD, only two of which involved minor salivary glands. Radiographic findings of the disease were rarely described, as reports mainly focused on clinical and histopathological features2,3.
Case Report
We report a case of a 70-year-old male who turned to the oral medicine clinic in Rambam Health Care Campus complaining of painful labial mucosal swelling. His medical history revealed kidney transplantation due to polycystic kidney disease, and a complete surgical excision of the right parotid gland for unknown reasons.
Physical examination showed swollen minor salivary glands all over the labial mucosa some of which secreting yellow mucous.
MRI of the head and neck demonstrated multiple cystic lesions in numerous salivary glands. The lesions affected minor salivary glands in the hard and soft palate and in the sublingual, labial and buccal mucosa bilaterally. The left parotid gland was less severely involved. There was no contrast enhancement, and no solid masses were identified.
A non-contrast CT of the upper abdomen, essential for estimation of renal, hepatic or pancreatic involvement, showed innumerable renal cysts, compatible with known polycystic kidney disease.
Initial biopsy of the oral mucosa was inconclusive. A second excisional biopsy confirmed a diagnosis of DPD.
Conclusion
Only 19 cases of DPD are reported in the literature, with limited portrayal of radiographic findings. We present Head and Neck MRI and Abdominal CT findings in a patient with systemic involvement of DPD. MRI findings include multiple cysts in salivary glands, with no mass lesions and no contrast enhancement. Abdominal imaging demonstrated polycystic kidneys.
