Ciancia Syndrome with Pulled-in-two Syndrome: A Case Report

Abstract

Introduction: Cross-fixation with large-angle esotropia, known as Ciancia syndrome. This occurs during the first six months of life and associated with tight medial rectus muscle, and constant inward eye turn.

Case: We report a case of pulled-in-two syndrome without the known predisposing factors effecting muscle tensile strength include age, previous surgery, trauma, or infiltrative disease. This is a 15-year-old girl with convergence squint in both eyes (OU) since the age of 9 months old. There was no ophthalmology examination until patient at age of 13. Examination revealed cross fixation OU with good visual acuity, low refractive error, absent of stereopsis and large angle esotropia with 60∆ base out.

Patient was plan for OU medial rectus (MR) recession. Pre-operative force-duction test revealed OU very tight abduction. Intra-operatively, a sudden give away was felt while securing the right MR muscle. Pulled-in-two syndrome happened and distal MR was not found. Minimal proximal right MR fibers harvested and sent for histopathology examination (HPE). Uneventful left MR recession proceeded with extra gentle manipulation.

At one month post-operation, orthoptic assessment remained large-angle esotropia. Patient underwent second surgery with left lateral rectus muscle (LR) resection and a sample of left LR fibers was taken for HPE.

HPE reported right MR mild fibrosis and normal left LR muscle.

Conclusion: Prolonged cross-fixation with large-angle esotropia and very tight force-duction test alarm the surgeon for the risk of fibrotic rectus muscles that requires extra gentle manipulation of the rectus muscle while performing squint surgery to prevent pulled-in-two syndrome.