From Bascom Palmer Eye Institute, Miami, Florida.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Hasan A. Aziz, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136.
Retinopathy of prematurity (ROP) has a broad classification system that was revised in 2005.1 ROP pathophysiology consists of two phases, the vessel loss phase and the vessel proliferation phase. In the first phase, the retina that normally matures in utero slows or ceases to grow, causing regression of retinal vasculature. In this phase, the levels of insulin-like growth factor 1 and vascular endothelial growth factor are low. Later, after 32 weeks’ post-menstruation age, a switch occurs to a hypoxia-induced proliferative phase with a marked increase in the levels of insulin-like growth factor 1 and vascular endothelial growth factor, eventually resulting in neovascularization.2 Early detection and competent screening programs have been implemented nationwide to systemically screen for and treat this potentially blinding disease.
Approximately 14,000 infants suffer from shaken baby syndrome per year in the United Sates. Mortality due to shaken baby syndrome is a staggering 30% of affected infants and most survivors endure severe permanent neurological disabilities.3,4 Approximately 83% of infants with shaken baby syndrome present with retinal hemorrhages. Extensive multilayered retinal hemorrhage, perimacular fold, and macular retinoschisis are all considered suggestive of shaken baby syndrome.5
Note that preretinal hemorrhages are seen in ROP and preretinal and intraretinal hemorrhages are common at birth.
A former 29-week premature, 935-g neonate with a history of stage 2 ROP who was lost to follow-up was admitted at a gestational age of 42 weeks to the Jackson Memorial Hospital/Bascom Palmer Eye Institute for evaluation and management of suspected child abuse and urinary tract infection. She was found to have multiple bruises over the buttocks area with edema and bruises over the upper and lower eyelids with subconjunctival hematoma on the left eye. The child underwent computed tomography of the brain without contrast, which revealed no evidence of intracranial bleed. The skeletal radiographic survey showed fractures of the right radius and ulna. A urinalysis and urine culture showed group B streptococcus and she was prescribed intravenous ceftriaxone antibiotic for urinary tract infection.
Ophthalmic examination revealed bilateral vitreous hemorrhages that were not previously observed in the neonatal intensive care unit. A B-scan noted a shallow elevated membrane with mild traction on the retina peripherally in both eyes. ROP staging was consistent with zone II stage 4 disease in both eyes, although the visualization of the macula was not clear. Extensive retinal hemorrhage was noted in all three retinal layers (preretinal, intraretinal, and subretinal) (Figure). The infant underwent laser treatment to the peripheral avascular retina to halt the progression of the ROP.
Figure. Stage 4 Retinopathy of Prematurity Showing Evidence of Blood in All Retinal Layers (preretinal, Intraretinal, and Subretinal).
The patient stabilized with clearing of the retinal and vitreous hemorrhages. Both eyes showed macular ectasia, with preretinal tractional alterations in the right eye. At 24 months of age, she was noted to have asymmetric myopia that was greater in the left eye than in the right eye and visual loss secondary to amblyopia in the right eye.
This unique case presents with the concomitant occurrence of ROP and shaken baby syndrome. Shaken baby syndrome is usually not observed with ROP because the multiple systemic medical illnesses of the neonate necessitate a prolonged admission, and the patient’s ROP is typically resolving by discharge. Trese et al. noted peripheral avascular retina in children with shaken baby syndrome without prior ROP.6 In our patient, laser therapy was certainly beneficial in stabilizing advanced ROP and may have been therapeutic for the retinal damage in shaken baby syndrome. Laser therapy has been shown to promote retinal reattachment in eyes with early stage 4 ROP, especially where the tractional component of the detachment is limited and exudation predominates. In addition to stabilizing retinal detachment in some eyes with stage 4 ROP, laser therapy could be performed at the bedside. More advanced stage 4 disease frequently requires vitreoretinal surgical intervention. In patients with severe systemic compromise who are not amenable to timely surgery, laser treatment can temporize the intraoperative surgical management and prevent progression to stage 5 in some cases.
It is important to note that retinal hemorrhages in neonates do not occur solely in ROP, and one should maintain an elevated suspicion for shaken baby syndrome. Moreover, in cases of child abuse, one should remember the following three tenets. The infant could present with no signs of external trauma and still could have been abused. Retinal hemorrhages are not always present and could have resolved between the time frame of the traumatic event and the ocular evaluation. The child could show other ophthalmic signs of abuse such as rhegmatogenous retinal detachments, perimacular folds, hemorrhagic macular retinoschisis, or vitreous base avulsion.7
A large-scale study at the University of Warwick involved almost 120,000 children to study the relationship between premature children and rates of abuse. The results of this study suggest that prematurity and decreased rates of fetal growth are associated with increased likelihood of child abuse independent of maternal age or socioeconomic status.8
Thus, ophthalmologists should remain vigilant in evaluating premature infants, taking ROP into consideration but not forgetting the wide array of shaken baby syndrome presentation and the potential association between prematurity and abuse. The current case illustrates the integral part that ophthalmologists play in recognizing shaken baby syndrome.
- The International Classification of Retinopathy of Prematurity Committee. The International Classification of Retinopathy of Prematurity revisited. Arch Ophthalmol. 2005;123:991–999.
- Chen J, Smith LE. Retinopathy of prematurity. Angiogenesis. 2007;10:133–140. doi:10.1007/s10456-007-9066-0 [CrossRef]
- Newton AW, Vandeven AM. Update on child maltreatment with a special focus on shaken baby syndrome. Curr Opin Pediatr. 2005;17:246–251. doi:10.1097/01.mop.0000158730.56669.b1 [CrossRef]
- Keenan HT, Runyan DK, Marshall SW, Nocera MA, Merten DF, Sinal SH. A population-based study of inflicted traumatic brain injury in young children. JAMA. 2003;290:621–626. doi:10.1001/jama.290.5.621 [CrossRef]
- Kivlin JD, Simons KB, Lazoritz S, Ruttum MS. Shaken baby syndrome. Ophthalmology. 2000;107:1246–1254. doi:10.1016/S0161-6420(00)00161-5 [CrossRef]
- Trese M, Capone A. Non-accidental trauma and peripheral retinal nonperfusion. Paper presented at: Association of Pediatric Retina Specialists meeting. ; 2009; Hawks Cay Key, FL. .
- Gonzales CA, Scott IU, Chaudry NA, Oster AS, Hess DJ, Murray TG. Bilateral rhegmatogenous retinal detachments with unilateral vitreous base avulsion as the presenting signs of child abuse. Am J Ophthalmol. 1999;127:475–477. doi:10.1016/S0002-9394(98)00419-X [CrossRef]
- Spencer N, Wallace A, Sundrum R, Bacchus C, Logan S. Child abuse registration, fetal growth, and preterm birth: a population based study. J Epidemiol Community Health. 2006;60:337–340. doi:10.1136/jech.2005.0420 doi:10.1136/jech.2005.042085 [CrossRef]