Session: Poster session C
Monocular infantile blindness with nystagmus : oculomotor features and surgical outcome
Introduction : Oculomotor status in monocular infantile blindness (MIB) may be considered as a subtype of infantile esotropia. Patients frequently present with nystagmus increasing in abduction and usually adopt anomalous head posture (AHP) with fixation in adduction in order to decrease the amplitude of nystagmus.
Methods: We studied a series of 27 cases of MIB with nystagmus resulting from strictly unilateral organic ocular damage. Their clinical history with, medical and surgical treatments were recorded.
Results: 27 patients (13 women) aged 4 to 46 years were included (median 8.9 years). The etiology found was papillary atrophy or hypoplasia in 12 cases, colobomatous damage in 4 cases, congenital cataract in 8 cases, consequences of forceps trauma in 2 cases and retinal hamartoma in one case. The visual acuity of the healthy eye was on average 0.06 log Mar. 92% of patients presented AHP, the sound eye fixating in adduction. A torsional component was also observed in half of the cases. 75% of patients presented esotropia. 20 patients received strabismus surgery in order to treat esotropia and improve a disabling torticollis. In 15 cases, surgery was indicated to treat torticollis and thus performed on the healthy eye. In 12 cases, a faden-operation of medial rectus associated or not with recession was performed to treat fixation in adduction, and 2 cases were treated for torsional torticollis. AHP improved in all cases. In 5 cases, esotropia was the main reason for surgery and it was performed on the amblyopic eye, with good results.
Conclusion: MIB with nystagmus is a rare situation that can lead to disabling head posture requiring appropriate oculomotor care. In this case, surgery of the sound eye is mandatory, and fadenoperation seems convenient to treat fixation in adduction.
Methods: We studied a series of 27 cases of MIB with nystagmus resulting from strictly unilateral organic ocular damage. Their clinical history with, medical and surgical treatments were recorded.
Results: 27 patients (13 women) aged 4 to 46 years were included (median 8.9 years). The etiology found was papillary atrophy or hypoplasia in 12 cases, colobomatous damage in 4 cases, congenital cataract in 8 cases, consequences of forceps trauma in 2 cases and retinal hamartoma in one case. The visual acuity of the healthy eye was on average 0.06 log Mar. 92% of patients presented AHP, the sound eye fixating in adduction. A torsional component was also observed in half of the cases. 75% of patients presented esotropia. 20 patients received strabismus surgery in order to treat esotropia and improve a disabling torticollis. In 15 cases, surgery was indicated to treat torticollis and thus performed on the healthy eye. In 12 cases, a faden-operation of medial rectus associated or not with recession was performed to treat fixation in adduction, and 2 cases were treated for torsional torticollis. AHP improved in all cases. In 5 cases, esotropia was the main reason for surgery and it was performed on the amblyopic eye, with good results.
Conclusion: MIB with nystagmus is a rare situation that can lead to disabling head posture requiring appropriate oculomotor care. In this case, surgery of the sound eye is mandatory, and fadenoperation seems convenient to treat fixation in adduction.