Retinal hemorrhages have been reported in 50% to 100% of infants diagnosed as having shaken baby syndrome (SBS).1 Retinoschisis and circular perimacular retinal folds are associated with poor prognosis in SBS.2 Although these ophthalmologic findings have been well documented in the literature, macular holes have not been described. We present 5 cases of children who developed macular holes as a sequela to SBS.
Five patients were diagnosed as having SBS based on systemic, intracranial, and ophthalmologic findings. The median age of trauma was 9 months (range, 6-10 months), and the median age of macular hole diagnosis was 10 months (range, 8-12 months) (Table 1). All macular holes were unilateral, despite severe bilateral retinal disease. Four patients had severe vitreous hemorrhage and intraretinal hemorrhage, and 1 patient had diffuse retinal hemorrhage affecting all retinal layers (Table 2). The median size of the macular hole was 700 μm (range, 500-1500 μm). Three macular holes were centrally located; 2 macular holes were ectopically located (juxtafoveal). The diagnosis of macular hole was made during initial funduscopic examination in 2 patients, during vitrectomy in 2 patients, and after clearing of the vitreous hemorrhage, initially obscuring the visual axis, in the final patient.
Surgical intervention was performed in 4 cases to clear the visual axis of vitreous and subhyaloidal hemorrhage. Surgery included vitrectomy, internal limiting membrane peel, and tamponade (no tamponade in patient 1, silicone oil in patient 2, perfluoropropane 12% tamponade in patient 3, and air in patient 4). The median age at the time of vitrectomy was 11.5 months. Three out of 4 eyes had successful hole closure following surgery (that in patient 1 remained open). The median follow-up period was 12 months (mean, 12.4 months; range, 6-24 months). In all 5 cases, blood was seen at the base of the hole, occasionally pluming outward.
Visual acuity outcomes were poor: 1 patient was able to track objects with the affected eye, 1 patient had aphakia and amblyopia, and 3 patients could not be assessed owing to severe intracranial damage. There were no mortalities.
Case 1. A 7-month-old boy (patient 4; Table 1) was diagnosed as having SBS. A computed tomographic scan showed intracranial hemorrhage resulting in hemiplegia. Initial fundus examination at the time of injury showed massive preretinal, intraretinal, subretinal, and vitreous hemorrhage in both eyes. Medical instability precluded further ophthalmologic evaluation for 4 months. His hemorrhages did not resolve, and he underwent lensectomy combined with vitrectomy in the left eye. One month later a lensectomy and vitrectomy were performed in his right eye, during which an ectopic macular hole with fluid cuff was noted inferotemporal to the fovea. The posterior hyaloid had spontaneously separated. An epiretinal membrane was seen and peeled with partial removal of the internal limiting membrane. Laser photocoagulation was subsequently placed around the edge of the hole (Figure). The hole remained visible but closed postoperatively. Postoperatively the patient was aphakic, wore a contact lens, and underwent patching for amblyopia.
RetCam (Clarity Medical Systems, Pleasanton, Calif) photograph of the right fundus following pars plicata vitrectomy, lensectomy, membrane peel, barrier laser photocoagulation, and air tamponade. The retinal pigment epithelium surrounding the visible but closed macular hole is hyperpigmented in response to laser photocoagulation (arrowheads).
Case 2. Lens-sparing vitrectomy was performed in the left eye of an 8-month-old boy (Table 1; patient 1) with SBS. The macula was detached with organized blood; a thick plume of old blood spouted through the fovea via a 700-μm hole. A skirt of retinal pigment epithelial atrophy, almost extending to the arcades, surrounded the submacular blood. Postoperatively the hole was closed. Fluorescein angiography demonstrated disruption of the retinal pigment epithelium with numerous window defects.
The macular holes seen in our patients with SBS represent a previously unreported finding. In all 5 cases of patients with SBS with macular holes, blood emanated from the base of the holes. We believe that the underlying retinal hemorrhage caused mechanical pressure leading to full-thickness macular hole formation in a structurally weakened retina. The causes of a weakened retina include necrosis due to underlying hemorrhage (demonstrated in pathologic specimens in a rat model by Lincoff et al)3 and mechanical forces of shaking (a break in the internal limiting membrane over an area of retinal hemorrhage has been reported).4
Attempts to preserve vision and prevent amblyopia by macular hole repair are important. However, successful macular hole repair did not lead to good visual outcomes. In one case (patient 1), no tamponade was used because the surgeon believed that functional vision was not salvageable; the hole remained open in that eye, and visual acuity is poor. Limited visual outcome in these patients can be attributed to severe intracranial damage (Table 2),5 severe retinal hemorrhages (Table 2), and aphakia with amblyopia.
The presence of a macular hole in SBS is a poor prognostic finding. Affected patients usually have severe visual acuity deficits, even with successful anatomical repair. Additionally, macular hole may be a predictor of severe neurologic injury in SBS, as was the case in 3 of the 5 patients we described.
Correspondence: Dr Moshfeghi, Department of Ophthalmology, Stanford University School of Medicine, 1225 Crane St, Menlo Park, CA 94025 (firstname.lastname@example.org).
Financial Disclosure: None.
Ou JI, Moshfeghi DM, Tawansy K, Sears JE. Macular Hole in the Shaken Baby Syndrome. Arch Ophthalmol. 2006;124(6):913-915. doi:10.1001/archopht.124.6.913