Commentary: Anomalous extraocular muscles in Crouzon syndrome with V-pattern exotropia

Abstract

Crouzon syndrome is a complex craniosynostoses with hypertelorism, shallow orbits, and prominent globes.[1 2] Premature closure of the sutures (coronal, sagittal, and lambdoid) results in deformation of the head and orbit. This is often accompanied with anomalies of extraocular muscles (including hypoplasia, aplasia, etc) leading to a complicated strabismus.[3 4 5] In this regard, a detailed analysis of EOM anatomy in MRI is helpful and should be considered in all cases. Each surgical plan is dependent on the individual patient's extraocular muscle anatomy and is highly unpredictable. Despite the best efforts in localizing the muscle anatomy on neuroimaging it is still possible to have surgical surprise.[6] The surgeon should be prepared in all such cases to handle the intraoperative challenges in the form of muscle transplantation and resurgery options. A detailed consent should be obtained from the patient and all the surgical options discussed with either the patient or his/her parents (in case of minors). The surgical results may not always match up to the expectations; however, they do improve the quality of life for both children and their families by improving the functional fields of gaze.