Infrasakkale Tränenwegsstenose durch Knochenverdickungen des inneren Nasenskelettes bei tuberöser Sklerose

Summary

Background Patients with tuberous sclerosis (Morbus Bourneville-Pringle) may show hamartomatous tumors of different organ systems,50% of patients display astrocytic hamartomas of the retina. The skeletal system is affected in 40%:peri-, en- or exostoses, or cysts may occur. To the best of our knowledge, there are only three descriptions of an involvement of the facial skeleton in the literature.

Patient A 54-year-old man presented with recurrent dacryocystitis and lacrimal duct obstruction. Tuberous sclerosis and epilepsy were present since childhood. Besides typical skin lesions and intracranial calcified astrocytomas a retinal astrocytoma was detected in his left eye. Coronal CT scan revealed endonasal bone thickening involving the nasal floor, conchae and lateral walls. Evidence of maxillary and ethmoidal sinusitis was also present.

Conclusion The endonasal findings in our patient are most likely a manifestation of tuberous sclerosis of the facial skeleton. They may have favored the development of marked sinusitis and lacrimal duct obstruction.

Schlüsselwörter

Tränenwegsstenose - tuberöse Sklerose - Gesichtsschädel - koronares CT

Key words

Lacrimal duct obstruction - tuberous sclerosis - facial skeleton - coronal CT