Abstract
BackgroundMonocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS). Patients often adopt a significant anomalous head posture (AHP) towards the fixing eye in order to dampen the nystagmus. This clinical entity has also been reported as unilateral Ciancia syndrome. The aim of the study was to ascertain the clinical features and surgical outcome of patients with FMNS with infantile unilateral visual loss.MethodsIn this retrospective case series, nine consecutive patients with FMNS with infantile unilateral visual loss underwent strabismus surgery to correct an AHP and/or improve ocular alignment. Outcome measures included amount of AHP and deviation at last follow-up.ResultsEye muscle surgery according to the principles of Kestenbaum resulted in a marked reduction or elimination of the AHP. On average, a reduction of AHP of 1.3°/mm was achieved by predominantly performing combined horizontal recess-resect surgery in the intact eye. In cases of existing esotropia (ET) this procedure also markedly reduced the angle of deviation. A dosage calculation of 3 prism diopters/mm was established.ConclusionsWe advocate a tailored surgical approach in FMNS with infantile unilateral visual loss. In typical patients who adopt a significant AHP accompanied by a large ET, we suggest an initial combined recess-resect surgery in the intact eye. This procedure regularly led to a marked reduction of the head turn and ET. In patients without significant strabismus, a full Kestenbaum procedure was successful, while ET in a patient with a minor AHP was corrected by performing a bimedial recession.
Highlights
Monocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS)
Four patients demonstrated a rather simple anomalous head posture (AHP) consisting of a head turn only, and the remaining five patients instead exhibited a more complex AHP
In patients adopting a significant head turn accompanied by a large esotropia we suggest an initial combined recessresect surgery in the intact eye, which regularly led to a marked reduction of the head turn and the ET
Summary
Monocular infantile blindness may be associated with bilateral horizontal nystagmus, a subtype of fusion maldevelopment nystagmus syndrome (FMNS). Patients often adopt a significant anomalous head posture (AHP) towards the fixing eye in order to dampen the nystagmus This clinical entity has been reported as unilateral Ciancia syndrome. It has been hypothesized that severe monocular visual impairment are in the position of least ocular instability and maximal visual acuity, is similar to the common null zone scenario in INS This preferred eye position of minimal nystagmus intensity is quite typical, the underlying cause remains unclear. Patients often adopt a significant head turn towards the fixing eye in order to dampen the nystagmus, which typically increases on abduction and decreases on adduction This clinical entity has been reported in the literature as unilateral Ciancia syndrome [3,4,5,6]. The ET increases as the nystagmus dampens, and decreases with increasing nystagmus [7]
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