ABSTRACT
Cyclic strabismus is a rare entity and is unique in that patients follow variable but reliable time cycles where they alternate between orthotropia or "straight" eyes and strabismus, most commonly in the form of esotropia. Despite many theories on the underlying etiology and unique features of this diagnosis, none have been proven and its pathophysiology remains unknown. Four cases of cyclic esotropia diagnosed by pediatric ophthalmologists have been included in this report. The ages of the patients ranged from 10 months to eight years. The time duration from the onset of deviation to presentation to an ophthalmologist ranged from 1-52 weeks with three of the four patients presenting in the cyclic phase and the fourth presenting with a constant esotropia after a clear history and photographically documented cyclic esotropia for the preceding two months. All four patients were followed for periods ranging from one to four months to confirm their diagnosis or obtain multiple readings of the maximal deviation on the strabismic days before any intervention. The angle of esotropia when present ranged from 25 to 35 prism diopters and the cycle duration was 48 hours for all four cases (24 hours of esotropia followed by 24 hours of orthotropia). All patients were treated with botulinum toxin A injections to both medial recti, which resulted in an end to their cyclic deviation with excellent alignment obtained during follow-up periods ranging from 12-36 months for all cases. Cyclic esotropia is an elusive diagnosis that can be easily overlooked. When identified, classical treatment is usually extraocular muscle surgery targeting the largest angle of deviation. Many non-surgical treatments have been tried to no avail. However, in recent times, botulinum toxin A has been seen as a viable alternative.
ABSTRACT
A baby girl who underwent cesarean section delivery and had a complicated postnatal course requiring neonatal intensive care unit (NICU) is followed in the pediatrics clinic for several months. At five months old, the baby girl was referred to an ophthalmology clinic with brain stem and cerebellum malformation consistent with the molar tooth sign (MTS) on magnetic resonance imaging (MRI) of the brain, hypotonia, and developmental delay. She has the classic features of Joubert Syndrome (JS). Other findings not typically associated with the clinical picture of the syndrome were observed in this patient, specifically skin capillary hemangioma of the forehead. Cutaneous capillary hemangioma was an incidental finding in this JS patient and responded favorably to medical treatment with propranolol where a significant reduction in the size of the mass was observed. This incidental finding can be seen as a potential addition to the spectrum of associated findings in JS.
