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Information

Definition

Form of abusive head trauma characterized by intracranial hemorrhage, brain injury, multifocal fractures, and/or retinal hemorrhages due to repeated acceleration–deceleration forces with or without blunt head impact. External signs of trauma are often absent.

Symptoms

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 often inconsistent with history.

Signs

Critical

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.

Other

Subretinal and VH less common. 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.

Differential Diagnosis

  • 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 shaken baby syndrome 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.
  • 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 is helpful in ascertaining causality.

Workup

  1. 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.
  2. Complete ophthalmic examination, including pupils (for afferent pupillary defect) and dilated fundus examination.
  3. Laboratory: CBC with platelet count, PT/INR, and PTT. Consider additional evaluation based on initial screening results.
  4. Imaging: CT or MRI; skeletal survey. Consider bone scan.
  5. Admit patient to hospital if shaken baby syndrome is suspected. Requires coordinated care by neurosurgery, pediatrics, ophthalmology, and social services.
NOTE:

Careful documentation is an integral part of the evaluation, as the medical record may be used as a legal document. Ocular photography is not the gold standard for documenting retinal hemorrhages but may be useful if available. A thorough detailed description is essential with or without a drawing, including type, number, and distribution of hemorrhages and presence/absence of retinoschisis/folds.

Treatment

Predominantly supportive. Focus is on systemic complications. Ocular manifestations are usually observed. In cases of nonabsorbing dense VH, vitrectomy may be considered due to the risk of amblyopia.

NOTE:

All physicians are legally mandated to report suspected child abuse. There is legal precedence for prosecution of nonreporters.

Follow Up

Prognosis is variable and unpredictable. Survivors can suffer from significant cognitive disabilities, and severe visual loss occurs in 20% of children, usually from optic atrophy or brain injury. Even if no retinal hemorrhages exist, ophthalmologic follow up is recommended for children with brain injury.