Retinal hemorrhages associated with meningitis in a child with a congenital disorder of glycosylation

A 9-month old infant presented in a state of shock to a district hospital. She was subsequently referred to the regional tertiary hospital. On admission, bruises were noted on the vertex of the skull. Retinal hemorrhages were present on ophthalmological examination. CT scan of the brain showed poor grey-white matter differentiation with apparent frontoparietal fractures of the skull. Her case was subsequently referred to the relevant authorities as it was suspicious for nonaccident injury (NAI). Her condition deteriorated and she died the next day. Postmortem examination showed that the bruises on the vertex were caused by rapid widening of the sutures of the skull, caused by rising intracranial pressure. There was no skull fracture or evidence of trauma. Histological examination of the brain showed meningitis which had extended to the optic nerve sheath. Hemorrhages were noted in the retinas as well as the optic nerve sheath. An incidental congenital disorder of glycosylation (CDG) was diagnosed on brain histology and confirmed by metabolic tests. Retinal hemorrhages are known to occur in head injuries especially in association with NAI. In this case, suspicion of NAI was further augmented by the presence of apparent bruises on the head. The full postmortem examination showed no evidence of injuries and instead showed that the child was suffering from meningitis. Blood culture grew Group A Streptococcus pyogenes. The underlying mechanisms for such a presentation and the association with CDG are discussed.