Results of lacrimal assessment in patients with congenital clinical anophthalmos or blind microphthalmos

Aim: To report clinical findings relating to the lacrimal system in congenital clinical anophthalmos and severe blind microphthalmos. Methods: A retrospective (up to 2003) and prospective (2004 onwards) study of the notes of 60 consecutive patients treated surgically with highly hydrophilic self-inflating expanders for congenital anophthalmos or severe blind microphthalmos between 1997 and 2006. The lacrimal drainage system was always probed and irrigated under general anaesthesia before any other procedure was started. Results: Nine patients were excluded due to possible misdiagnosis because of previous lid or orbit surgery elsewhere or due to missing data. The analysis therefore included 23 girls and 28 boys aged between 1 and 90 months (median age: 4 months). Twenty-three patients presented with unilateral and 18 with bilateral anophthalmos, and 10 had unilateral microphthalmos; consequently, 102 orbits (of which, 69 were with probable pathology) were available for assessment. In unilateral cases, the lacrimal system on the normal side was never affected. On the anophthalmic or microphthalmic side, the lacrimal system was normal in 17 orbits only (24.6%). The most frequent finding was canalicular stenosis (40 orbits; 58%). Common canaliculus stenosis was observed in 5 orbits (7.3%) and nasolacrimal duct obstruction in 7 orbits (10.1%). There were no cases of punctal anomaly. Conclusions: In congenital clinical anophthalmos the lacrimal system is affected in up to 78% of cases, mostly due to canalicular stenosis. Even if there is no clear evidence of an embryological connection, this association is certainly not a random finding.