Abstract:We describe two cases of congenital cranial dysinnervation disorders(CCDD) who were seen at our centre.The first patient was a 7-year-old Chinese girl with no medical illness,who was noted by her parents to have a habit of tilting her head to one side and "lazy eyes" since she was a toddler.Best-corrected visual acuity(BCVA) in both eyes were 6/6 on Snellen chart and her head was tilted to the right.There was limitation of abduction in both eyes on horizontal gaze.Enophthalmos was seen associated with down shooting on attempted adduction.The second case was a 10-year-old Malay boy with no medical illness,who was noted by his parents to have "poor eye movements" since birth.BCVA in both eyes were 6/6 on Snellen chart with a left face turn and bilateral ptosis.Eye movements in all gazes were restricted and force duction test was positive.Both cases were diagnosed as congenital fibrosis of extraocular muscle(CFEOM) initially,but the diagnosis of the first case was later revised as bilateral Duane syndrome.Although both Duane syndrome and CFEOM are included under CCDD,both entities have distinct pathology.The presentation of CCDD is extremely variable.Nonetheless,the treatment is often limited and surgical outcome is often unpredictable.Hence,much research is still needed to be carried out for more in depth understanding of CCDD,so as to improve the management and outcome of the disease.
