Long-term outcome and morphology/function correlates in cases of shaken baby syndrome

PURPOSE: Haken Baby Syndrome (SBS) presents with acute encephalopathy, subdural and retinal haemorrhages, with inconsistent history, in < 2 years old. Long-term sequelae are still under study. Aim is to present the long-term outcome in a case series of SBS followed-up by a multidisciplinary team. METHODS: Case series of 14 children (9M,%F) with confirmed SBS has been examined and followed-up. Patients underwent funds evaluation (indirect ophtalmoscopy) and wide-field digital ophthalmic camera (RetCam II) until bleeding reabsorption. Assessment was repeated at follow-up combined with ocular motility evaluation, visual field (BEFIE test), visual acuity (Teller acuity cards), refractometry, cognitive-behavioural evaluations (Griffiths scales, Child Behaviour Check List), and family stress measurement (Parenting Stress Index), segmentation analysis of MRI. RESULTS: Mean age at acute episode of SBS: 6.5 months (range 2-20), 9/14 in the first 6 months of life. Mean age at last follow-up evaluation: 32 months (range 8-65). 4 cases were lost to follow-up. At last follow-up evaluation: 4 out of 10 had a decreased visual acuity (cortical visual impairment), 4 out of 10 had visual field deficits, 3 out of 10 had strabismus. None of the cases showed significant refractive errors. Cognitive/behavioural assessment demonstrated global delay and impairments in neuromotor ability, speech/language development and attention problems. MRI analysis showed atrophic alterations in several brain areas. CONCLUSION:Multidisciplinary assessment including ophthalmologic examination and neurobehavioral evaluation provides crucial prognostic information in cases of suspected SBS. Functional outcome is largely dependent on brain injury and atrophy.