Ocular defect in children with cerebral palsy and its correlation with the types of cerebral palsy

Background: Cerebral palsy (CP) describes a group of permanent disorder of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbance that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication and behavior, by epilepsy, and secondary musculoskeletal problem. However, no known study has been found on this aspect. Objective of this study was to evaluate the ocular defect in children with cerebral palsy and to correlate with the types of CP.Methods: Study was done in the Paediatric Neurology unit of BSMMU from January 2012 to July 2012. One hundred thirty children with cerebral palsy were studied. The patients were randomly selected who full filled the inclusion criteria and ophthalmological examination was done.Results: Sixty four (64%) of CP patients had pathological finding and 36% percent had normal finding. Most of the abnormalities were squint (strabismus) (40%) and refractive error (36.9%). Most of the ocular defects were found in spastic cerebral palsy, mainly in spastic quadriplegia (34.6%) and spastic diplegia (29.2%).Conclusions: Ocular defect like squint and refractive error common associations of cerebral palsy. Spastic quadriplegic and diplegic children had more ocular defects.

Hmx1 regulates urfh1 expression in the craniofacial region in zebrafish

H6 family homeobox 1 (HMX1) regulates multiple aspects of craniofacial development as it is widely expressed in the eye, peripheral ganglia and branchial arches. Mutations in HMX1 are linked to an ocular defect termed Oculo-auricular syndrome of Schorderet-Munier-Franceschetti (MIM #612109). We identified UHRF1 as a target of HMX1 during development. UHRF1 and its partner proteins actively regulate chromatin modifications and cellular proliferation. Luciferase assays and in situ hybridization analyses showed that HMX1 exerts a transcriptional inhibitory effect on UHRF1 and a modification of its expression pattern. Overexpression of hmx1 in hsp70-hmx1 zebrafish increased uhrf1 expression in the cranial region, while mutations in the hmx1 dimerization domains reduced uhrf1 expression. Moreover, the expression level of uhrf1 and its partner dnmt1 was increased in the eye field in response to hmx1 overexpression. These results indicate that hmx1 regulates uhrf1 expression and, potentially through regulating the expression of factors involved in DNA methylation, contribute to the development of the craniofacial region of zebrafish.

FABRICATION OF OCULAR PROSTHESIS - A COMBINATION OF A STOCK AND CUSTOM MADE PROSTHESIS- A CASE REPORT

An ocular defect may affect a patient psychologically and an ocular prosthesis is given to uplift the patient psychologically and improve the confidence. Defects of the eye may follow the removal of a part of or the entire orbit. This results in the patient becoming visually and psychologically handicapped. Restoring the defect with a prosthesis not only restores esthetics but also gives back the lost confidence to the patient. This is a case report of a patient with a prosthetic eye fabricated by the combination of a stock eye and a custom ocular prosthesis.

A Simplified Approach for Ocular Rehabilitation - A Case Report

Loss of an eye or any body part has an intimidating and crippling effect on the psychosocial well-being of the patient. Although the artificial prosthesis cannot restore the function, it can highly improve the patient’s esthetics and help them regain their psychological confidence. Literature has advocated various rehabilitation modalities including empirical use of stock shells, modifying stock eyes, custom-made ocular prostheses, ocular implants, etc. A custom-made ocular prosthesis, among all the techniques, shows improved adaptation to tissue bed, distributes uniform pressure, provides a more esthetic and precise result, and is relatively cost-effective. This case report explores a relatively comprehensive method of custom ocular prosthesis fabrication for an ocular defect with a satisfactory outcome.