Form of nonaccidental trauma in young children characterized by intracranial hemorrhage, brain injury, multifocal fractures, bruising, and/or retinal hemorrhages due to repeated acceleration–deceleration and/or rotational forces with or without blunt head impact. External signs of head trauma are often absent.
Change in mental status, new-onset seizures, poor feeding, and irritability. Child is usually <1 year of age but rarely >3 years of age. Symptoms and signs are often inconsistent with history, including caregiver reports of falls from low heights (e.g., fall from a bed).
Retinal hemorrhages are present in ∼85% of cases. Two-thirds are too numerous to count and multilayered (pre-, intra-, and subretinal), extending throughout the retina to the ora serrata. Markedly asymmetric hemorrhages in up to 20% of cases, unilateral in ∼2%. Macular retinoschisis (hemorrhagic macular cysts, most often subinternal limiting membrane) may be seen with or without surrounding paramacular retinal folds. Most commonly associated brain lesions are subarachnoid and subdural hemorrhages. Characteristic fractures include the ribs and/or long bone epiphyses. Cerebral edema and death occur in ∼20% to 30% of cases.
Subretinal and VH may also occur. Retinal detachment, papilledema, late optic atrophy, and optic nerve avulsion are infrequent. Postmortem findings include orbital, optic nerve sheath, optic nerve sheath intradural, and posterior intrascleral hemorrhage.
Severe accidental injury: Accompanied by other external injuries consistent with the history. Even in the most severe accidental injuries (e.g., motor vehicle accidents [MVA]), retinal hemorrhages are uncommon. In the usual trauma of childhood, retinal hemorrhages are typically mild and do not extend to the ora serrata. Severe retinal hemorrhages similar to abusive head trauma have only been reported in fatal head crush, fatal MVA, and an 11-m fall onto concrete.
Birth trauma: Retinal hemorrhages can be extensive, but nerve fiber layer hemorrhages are gone by 2 weeks, and dot/blot hemorrhages typically disappear by 4 to 6 weeks. Foveal, preretinal, and VH may persist longer. No retinoschisis or retinal folds. Clinical history must be consistent. Most common cause of retinal hemorrhage in neonates.
Coagulopathies, leukemia, and other blood dyscrasias. Rare, but should be ruled out. Other than leukemia, in which infiltrates are usually present, these entities do not cause extensive retinal hemorrhages. Retinal hemorrhages associated with severe leukemias are generally few, limited to the posterior pole, and not multilayered.
Hyperacute elevation of intracranial pressure (e.g., ruptured aneurysm) may cause extensive retinal hemorrhage. Easily differentiated by neuroimaging. Otherwise, increased intracranial pressure in children does NOT result in extensive retinal hemorrhage beyond the peripapillary area.
Hypoxia, immunizations, cardiopulmonary resuscitation (CPR), meningitis, sepsis, and cortical vein thrombosis are often offered as alternate explanations in the courtroom for retinal hemorrhages, but these are not usually supported by available clinical and research evidence. Type, distribution, and number of hemorrhages are helpful in ascertaining causality.
History from caregiver(s) is best obtained by a child abuse pediatrician or a representative team. Be alert for history incompatible with injuries or changing versions of history.
Complete ophthalmic examination, including pupils (for afferent pupillary defect) and dilated fundus examination.
Laboratory: CBC with platelet count, PT/INR, and PTT. Consider additional evaluation based on initial screening results.
Admit patient to hospital if shaken baby syndrome is suspected. Requires coordinated care by neurosurgery, pediatrics, ophthalmology, and social services.
Predominantly supportive. Focus is on systemic complications. Ocular manifestations are usually observed. In cases of nonabsorbing or dense VH, vitrectomy may be considered due to the risk of amblyopia.
 NOTEAll physicians are legally mandated to report suspected child abuse. There is legal precedence for prosecution of nonreporters. |
Prognosis is variable and unpredictable. Survivors can suffer from significant cognitive disabilities; severe visual loss occurs in 20% of children, usually from optic atrophy or cortical visual impairment. Even if no retinal hemorrhages exist, ophthalmologic follow-up is recommended for children with brain injury.