Shaken Baby Syndrome

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Shaken Baby Syndrome or Non Accidental Trauma

Overview:

Child abuse is a significant social problem which is associated with a high morbidity and mortality. “Shaken Baby Syndrome” (SBS) is one form of physical child abuse, a non-accidental traumatic (NAT) brain injury. In 6% of reported cases of child abuse, an ophthalmologist is responsible for initially recognizing the abuse[1]. Shaken Baby Syndrome (SBS) affects an estimated 1400 children/year in the United States [2] and it is thought an astounding 2 million children are abused each year in the US alone. Retinal findings may be the only manifestation of this abuse. It is a diagnosis that has important medical-legal implications and one that cannot be overlooked as a child’s safety may very well be at stake.

Detailed Description:

The ocular manifestations of child abuse are numerous. There may be signs of periorbital trauma (i.e. ecchymosis, lid edema, orbital fractures), anterior segment trauma (i.e. hyphema, iris prolapse, corneal laceration, cataract), or posterior segment trauma (i.e. vitreous hemorrhage, retinal detachment, optic nerve avulsion)[3]. There may be associated brain injury resulting in nystagmus, cortical blindness, encephalopathy, or cranial nerve palsies[3]. Intracranial bleeding may be associated with SBS[3] - most commonly subdural hemorrhage.
Retinal hemorrhages are the cardinal manifestation of SBS. The incidence of retinal hemorrhages in SBS is approximately 85%[4]. Classically, children with SBS have retinal hemorrhages which are multilayered – preretinal, intraretinal, and subretinal. They are usually too numerous to count and extend out to the retinal periphery (i.e. not just confined to the posterior pole). Macular retinoschisis (splitting of the retinal layers) may also be associated with SBS[5]. As retinal hemorrhages may subside over time, prompt retinal photography is recommended at the time of discovery of retinal hemorrhages on dilated funduscopic exam to document findings as a baseline for future comparison. If possible, extended ophthalmoscopy with examination of the entire retina with scleral depression is recommended to also evaluate for peripheral nonperfusion (from retinal vascular disruptions) and neovascularization as well as for peripheral retinal tears, hemorrhages, and other pathology. If possible, wide sweeping photographs and fluorescein angiography are recommended as soon as the discovery of retinal hemorrhages from SBS / NAT are discovered. Examination under anesthesia may be required to obtain imaging as well as administer treatment.

Shaken Baby2.png SBS1.jpg

Epidemiology/Risk Factors:

Perhaps the greatest diagnostic clue is a detailed history that is incompatible with the extent and severity of the injuries found on dilated fundus exam. Suspected abusers may confess to investigators in up to 47% of cases[6]. A child with classic evidence supporting physical abuse (i.e. old fractures, bruises of varying ages, signs of neglect, new sleep or behavioral issues) should alert the clinician to request a dilated fundus exam looking for SBS.

SBS2.png

Etiology/Pathophysiology:

Several mechanisms for the retinal hemorrhages have been postulated and recently been the subject of some debate (mostly in the courts). One likely hypothesis implicates repetitive acceleration-deceleration forces which cause damage via vitreomacular traction[7]. Other possible mechanisms include blunt head impact, increased intracranial pressure, increased intrathoracic pressure, hypoxia, sodium imbalance, or coagulopathies. Vascular disruption may also lead to peripheral nonperfusion which subsequently may result in neovascularization, vitreous hemorrhage, and tractional or combined tractional / rhegmatogenous retinal detachment.

Histopathology:

Pathology may reveal findings detailed above (e.g. retinal hemorrhages at multiple levels, nerve avulsion). Perimacular folds and hemorrhagic macular retinoschisis may be found on histopathologic exam and may not be well appreciated on dilated fundus exam if the view is obstructed by vitreous hemorrhage.

Differential Diagnosis:

  • Accidental Head Trauma
  • Purtscher’s retinopathy: associated with blunt thoracic trauma
  • Terson Syndrome: intraocular hemorrhage associated with intracranial hemorrhage
  • Normal Birth
  • Anemia
  • Blunt ocular trauma
  • Coagulopathy

Management/Treatment:

Overall, prevention is the best therapy. Many hospitals and healthcare centers offer classes and education on how to cope with the stresses of parenthood. Unfortunately, some children will require treatment for amblyopia or strabismus. Patching therapy and glasses may be needed to treat the amblyopia induced by the ocular trauma.

Surgical vitrectomy may rarely be needed for nonclearing vitreous hemorrhage, macular hole, or retinal detachment. Often, the prognosis can vary significantly for victims of SBS depending on the severity of the trauma. Careful physician documentation of findings and fundus photography (if available) can prove very helpful especially in a court of law. Child protective services and local law enforcement should immediately be alerted in all cases of abuse or suspected abuse.

Retina photographs with wide sweeping fluorescein angiography is recommended to document baseline intraretinal hemorrhages and other pathologic changes as described above as well as to evaluate for peripheral nonperfusion and possible neovascularization development. Peripheral nonperfusion may lead to development of neovascular tissue that subsequently may contribute to vitreous hemorrhage and tractional or complex tractional / rhegmatogenous retinal detachment. If significant peripheral nonperfusion is detected, peripheral scatter laser to all regions of nonperfusion may be considered to reduce the risk of neovascularization and subsequent retinal detachment.

Prognosis:

Poor. Cortical blindness occurs in up to 15%. Victims suffer from high incidences of behavioral, social, motor, and visual problems.

References:

  1. Friendly DS. Ocular manifestations of physical child abuse. Trans Am Acad Ophthalmol Otolaryngol 1971; 75: 318-332.
  2. Newton AW, Vandeven AM. Update on child maltreatment with a special focus on shaken baby syndrome. Curr Opin Pediatr 2005; 17: 246-251.
  3. 3.0 3.1 3.2 Sternberg P Jr. Trauma: principles and techniques of treatment. In: Ryan SJ, ed. Retina, 2ndedn, vol. 3. St. Louis: Mosby; 1994: 2351-2378.
  4. Kivlin J, Simons K, Lazoritz S, Ruttum M. Shaken baby syndrome. Ophthalmology. 2000; 107(7): 1245-1254.
  5. Greenwald, Weiss, Oestrerle, Friendly. Traumatic retinoschisis in battered babies. Ophthalmology. 1986; 93(5): 618-625.
  6. Jenny, Hymel, Ritzen, et al. Analysis of missed cases of abusive head trauma. JAMA 1999; 281: 621-626.
  7. Levin, Alex. Retinal Hemorrhage in Abusive Head Trauma. Pediatrics 2010; 126; 961.
  1. Caffey J. On the theory and practice of shaking infants: its potential residual effects of permanent brain damage and mental retardation. Am J Dis Child 1972; 124: 161-169.