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Giant retinal tears are defined as retinal tears extending 90 degrees or more around the circumference of the fundus.1 They most commonly occur spontaneously but are associated with direct trauma in 20–25% of cases.2,3 We report the case of a 360 degree giant retinal tear occurring in a baby girl as a result of presumed non-accidental injury (NAI).
A 4 month old girl presented with iris heterochromia, bruising over the left eye, a right relative afferent pupillary defect, and a right vitreous haemorrhage. She was born at 28 weeks gestation by normal vaginal delivery, screening for retinopathy of prematurity by the college recommendations detected no abnormality. She was bottle fed and gaining weight. There was no previous medical history and the child was not on any medication. The mother was an injecting drug user and the girl was on the child protection register, subject to an interim care order at the time of presentation.
A number of investigations were performed, including a clotting screen which was normal. An magnetic resonance image (MRI) revealed areas of cystic encephalomalacia associated with haemorrhagic elements and areas of cavitation parasagittally in the frontal and left parietal lobes consistent with multiple contusions.
On examination the child did not fix to light or a target with the right eye and there was no response to the optokinetic nystagmus drum. There was no red reflex visible in right eye and a right exotropia was present. Cycloplegic refraction revealed a refractive error of –0.50/+2.00 × 30 in the left eye. B-scan revealed a mass of tissue centrally with a corrugated membranous configuration and high reflectivity. Electrophysiological findings were consistent with severe retinal dysfunction with a flat ERG on the right. There was also evidence of occipital asymmetry suggestive of left hemisphere dysfunction. Occipital flash VEPs to independent testing of each eye were detectable but were broadened and degraded. There was consistent conspicuous asymmetry in the occipital distribution of the responses that was present for the majority of pattern and flash VEP recordings.
Examination under anaesthesia revealed eyes of normal axial lengths. An anterior lens opacity was present in the right eye with a 360 degree giant retinal tear resulting in a total retinal detachment. There was no anterior funnel configuration, the mass of retina posteriorly confirmed the previous ultrasound findings. A vitreoretinal opinion was sought but the retina was fibrosed and thickened and not considered viable for surgical repair. Examination of the left eye was entirely normal.
Giant retinal tears are commonly idiopathic (70%) but are associated with trauma in about 20% of cases.3 Myopia is a common finding with 40% of eyes having more than 8 dioptres of myopia.3 Non-traumatic giant retinal tears occur more frequently in males and tears occur in the fellow eye in about 10% of cases.2 Kanski reviewed 100 eyes with giant retinal tears and found that 71% of eyes with non-traumatic breaks were myopic and severe retinal pathological findings were present in 57% of fellow eyes.4 Idiopathic giant tears have been found in identical twins raising the question of genetic influences in the pathogenesis of this condition.5
Ocular injury is the presenting sign of physical abuse in 4–6% of cases although it may be evident in up to 40% of abused children.6
The commonest abnormality is retinal haemorrhage, which is the cardinal sign of shaken baby syndrome, occurring in some 80% of cases.7 The next most common finding is periorbital oedema with subconjunctival haemorrhage.7 Other manifestations reported include retinal detachment8 and retinoschisis.9
The collection of signs seen in this case, including retinal detachment, anterior lens opacity, bruising of the contralateral eye, MRI and electrophysiological findings are highly suggestive of NAI. The electrophysiological findings were suggestive of multiple contusions to the head and it is our belief that the giant retinal tear and subsequent retinal detachment occurred as a result of a direct blow to the eye rather than violent shaking. This is, to our knowledge, the first reported case of a 360 degree retinal tear associated with NAI. Practitioners should be alert to the possibility of NAI when faced with a giant retinal tear in a young child.