Wm. Budde et al., LACRIMAL DUCT OBSTRUCTION AND ENDONASAL B ONE THICKENING IN TUBEROUS SCLEROSIS, Klinische Monatsblatter fur Augenheilkunde, 210(3), 1997, pp. 169-171
Background Patients with tuberous sclerosis (Morbus Bourneville-Pringl
e) may show hamartomatous tumors of different organ systems. 50% of pa
tients display astrocytic hamartomas of the retina. The skeletal syste
m 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 inv
olvement of the facial skeleton in the literature. Patient A 54-year-o
ld man presented with recurrent dacryocystitis and lacrimal duct obstr
uction. 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 rev
ealed 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 li
kely a manifestation of tuberous sclerosis of the facial skeleton. The
y may have favored the development of marked sinusitis and lacrimal du
ct obstruction.