OBJECTIVE To describe our results using augmented temporal superior rectus transposition (SRT) with adjustable medial rectus muscle recession (MRc) for treatment of Duane syndrome and sixth nerve palsy. METHODS Retrospective surgical case review of patients undergoing SRT. Preoperative and postoperative orthoptic measurements were recorded. Minimum follow-up was 6 weeks. Main outcome measures...

BACKGROUND/AIMS Surgical correction of ocular alignment in patients with third cranial nerve paralysis is challenging, as the unopposed lateral rectus muscle often pulls the eye back to exotropia following surgery. The authors present a simple surgical approach to overcome this difficulty. This approach is also applicable to removal of unwanted overactivity of the lateral rectus in Duane syndro...

PURPOSE To describe phenotyping and linkage analysis results for available members from a consanguineous nuclear family with hereditary congenital strabismus. METHODS Both parents and all 12 children underwent clinical examination. Available affected and several unaffected family members had venous blood sampling for DNA extraction and 10K single nucleotide polymorphism (SNP) genotyping (Affy...

Carboxypeptidase A6 (CPA6) is an extracellular protease that cleaves carboxy-terminal hydrophobic amino acids and has been implicated in the defective innervation of the lateral rectus muscle by the VIth cranial nerve in Duane syndrome. In order to investigate the role of CPA6 in development, in particular its potential role in axon guidance, the zebrafish ortholog was identified and cloned. Ze...

PURPOSE To evaluate the surgical results of asymmetric bilateral lateral rectus recession in exotropic Duane retraction syndrome with abnormal face turn toward the opposite side according to secondary deviation measurements. METHODS Retrospective chart review. RESULTS Seven cases of unilateral exotropic Duane retraction syndrome were reviewed. All cases had globe retraction on adduction and...

We report a case of a four year old Nigerian girl who presented to the paediatric unit of our eye clinic with complaints of a squint on looking to the right side and reduction in the size of the right eye when looking to the left. On examination, she had right exotropia in the primary position of gaze. There was limitation of abduction and widening of the palpebral fissure of the right eye on r...

Acrofacial dysostosis of Nager is a little known hereditary syndrome in which the findings of mandibulofacial dysostosis are associated with defects of the limbs. The present case showed other abnormalities including the Stilling-Turk-Duane syndrome, conductive deafness and ptosis of the transverse colon.