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Research Letters |

Subinternal Limiting Membrane Hemorrhage With Perimacular Fold in Leukemia FREE

Ajay Bhatnagar, FRCS; Laura B. Wilkinson, BMedSci(Hons); Ajai K. Tyagi, FRCS; Harry E. Willshaw, FRCS
Arch Ophthalmol. 2009;127(11):1547-1553. doi:10.1001/archophthalmol.2009.284.
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We report a case that demonstrates the appearance of perimacular folds associated with extensive retinal hemorrhages occurring in the absence of trauma in a patient with acute myeloid leukemia. We discuss the clinical features, relevant literature, and suggested mechanisms for perimacular folds in such a situation.

A 14-year-old boy with acute myeloid leukemia visited the eye department with blurred vision of recent onset. Visual acuity was 6/48 OD and 6/18 OS. Examination revealed bilateral extensive retinal hemorrhages over the posterior pole involving all retinal layers (Figure 1). The patient's right eye had a dome-shaped cavity centered over the macula. This cavity was filled with fluid blood and surrounded by a raised ridge outlined by a shiny retinal reflex resembling a perimacular fold (Figure 1A). The patient had no history of head injury and no signs of intracranial hemorrhage. Hematological investigations showed a markedly reduced platelet count (18 × 103/μL; to convert to ×109 per liter, multiply by 1.0) and hemoglobin level (6.7 g/dL; to convert to grams per liter, multiply by 10.0); the prothrombin time was 13 seconds (reference range for this age group, 9-13 seconds) and the activated partial thromboplastin time was 32 seconds (reference range, 26-35 seconds).

Place holder to copy figure label and caption
Figure 1.

Fundus photographs showing extensive retinal hemorrhages over the posterior poles of both eyes. A, The right eye has a dome-shaped cavity centered over the macula. The cavity is filled with fluid blood and surrounded by a raised ridge outlined by a shiny retinal reflex resembling a perimacular fold (arrow). B, The left eye has superficial flame-shaped hemorrhages and a premacular hemorrhage. No perimacular fold is present.

Graphic Jump Location

Six weeks later, the premacular hemorrhage in the right eye persisted (Figure 2A). Nd:YAG laser membranotomy drained the blood into the vitreous cavity and his visual acuity improved to 6/6 (Figure 2B).

Place holder to copy figure label and caption
Figure 2.

Fundus photographs showing persistent premacular blood 6 weeks later (A) and showing the eye after Nd:YAG laser membranotomy to drain blood into the vitreous cavity (B). Note that the appearance of the perimacular fold (arrows) remained unchanged.

Graphic Jump Location

Traumatic retinoschisis with perimacular fold has been described following abusive head injury, particularly when characterized by the repetitive acceleration and deceleration forces seen in shaken baby syndrome.1,2 A similar ophthalmoscopic appearance was reported twice in fatal crush head injury of childhood3,4 and in adult Terson syndrome.5 In shaken baby syndrome, violent shaking is believed to generate shearing forces at the vitreoretinal interface, resulting in traction on the retina that causes traumatic retinoschisis and creates perimacular retinal folds.1 Retinoschisis may involve only separation of the internal limiting membrane (ILM) or a more sight-threatening split in deeper retinal layers. Elevated retinal blood vessels over the cavity aid in differentiation.

We believe the reduced platelet count in our patient caused extravasation of blood from retinal vessels, resulting in rapid accumulation of blood in the sub-ILM space in the right eye. This raised the ILM from the inner retina and created a perimacular fold at the edge where the ILM remained attached.

Retinal hemorrhages at any level are often seen in leukemia with thrombocytopenia.6 It is unlikely that the appearance of a perimacular fold is unique to this setting. It may be seen in eyes with rapid accumulation of large premacular sub-ILM hemorrhage due to any cause and may be more common than reported in the literature. Published photographs of a large premacular hemorrhage seen in a patient with lymphoma7 and in Valsalva retinopathy8 show what appears to be a perimacular retinal fold. The fact that this has not been commented on in these reports perhaps reflects the lack of familiarity with this sign among ophthalmologists not used to seeing patients with shaken baby syndrome.

This case highlights the facts that perimacular folds may be seen with large sub-ILM hemorrhage in a nonabusive setting and that hematological diseases should be excluded in all cases. However, the term retinoschisis resulting in perimacular fold may be reserved for traumatic settings where shearing forces at the vitreoretinal interface cause perimacular folds.

Correspondence: Ms Wilkinson, Eye Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, West Midlands B4 6NH, England (laura.wilkinson@bch.nhs.uk).

Financial Disclosure: None reported.

Greenwald  MJWeiss  AOesterle  CSFriendly  DS Traumatic retinoschisis in battered babies. Ophthalmology 1986;93 (5) 618- 625
PubMed Link to Article
Levin  AV Retinal hemorrhages of crush head injury: learning from outliers. Arch Ophthalmol 2006;124 (12) 1773- 1774
PubMed Link to Article
Lantz  PESinal  SHStanton  CAWeaver  RG  Jr Perimacular retinal folds from childhood head trauma. BMJ 2004;328 (7442) 754- 756
PubMed Link to Article
Lueder  GTTurner  JWPaschall  R Perimacular retinal folds simulating nonaccidental injury in an infant. Arch Ophthalmol 2006;124 (12) 1782- 1783
PubMed Link to Article
Keithahn  MAZBennett  SRCameron  DMieler  WF Retinal folds in Terson syndrome. Ophthalmology 1993;100 (8) 1187- 1190
PubMed Link to Article
Sharma  TGrewal  JGupta  SMurray  PI Ophthalmic manifestations of acute leukemias: the ophthalmologist's role. Eye 2004;18 (7) 663- 672
PubMed Link to Article
Gelston  CDCiardella  AP Neodymium:YAG laser treatment for a premacular haemorrhage in a T cell lymphoblastic lymphoma patient. Br J Ophthalmol 2006;90 (10) 1329- 1330
PubMed Link to Article
Durukan  AHKerimoglu  HErdurman  CDemirel  AKaragul  S Long-term results of Nd:YAG laser treatment for premacular subhyaloid haemorrhage owing to Valsalva retinopathy. Eye 2008;22 (2) 214- 218
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure 2.

Fundus photographs showing persistent premacular blood 6 weeks later (A) and showing the eye after Nd:YAG laser membranotomy to drain blood into the vitreous cavity (B). Note that the appearance of the perimacular fold (arrows) remained unchanged.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 1.

Fundus photographs showing extensive retinal hemorrhages over the posterior poles of both eyes. A, The right eye has a dome-shaped cavity centered over the macula. The cavity is filled with fluid blood and surrounded by a raised ridge outlined by a shiny retinal reflex resembling a perimacular fold (arrow). B, The left eye has superficial flame-shaped hemorrhages and a premacular hemorrhage. No perimacular fold is present.

Graphic Jump Location

Tables

References

Greenwald  MJWeiss  AOesterle  CSFriendly  DS Traumatic retinoschisis in battered babies. Ophthalmology 1986;93 (5) 618- 625
PubMed Link to Article
Levin  AV Retinal hemorrhages of crush head injury: learning from outliers. Arch Ophthalmol 2006;124 (12) 1773- 1774
PubMed Link to Article
Lantz  PESinal  SHStanton  CAWeaver  RG  Jr Perimacular retinal folds from childhood head trauma. BMJ 2004;328 (7442) 754- 756
PubMed Link to Article
Lueder  GTTurner  JWPaschall  R Perimacular retinal folds simulating nonaccidental injury in an infant. Arch Ophthalmol 2006;124 (12) 1782- 1783
PubMed Link to Article
Keithahn  MAZBennett  SRCameron  DMieler  WF Retinal folds in Terson syndrome. Ophthalmology 1993;100 (8) 1187- 1190
PubMed Link to Article
Sharma  TGrewal  JGupta  SMurray  PI Ophthalmic manifestations of acute leukemias: the ophthalmologist's role. Eye 2004;18 (7) 663- 672
PubMed Link to Article
Gelston  CDCiardella  AP Neodymium:YAG laser treatment for a premacular haemorrhage in a T cell lymphoblastic lymphoma patient. Br J Ophthalmol 2006;90 (10) 1329- 1330
PubMed Link to Article
Durukan  AHKerimoglu  HErdurman  CDemirel  AKaragul  S Long-term results of Nd:YAG laser treatment for premacular subhyaloid haemorrhage owing to Valsalva retinopathy. Eye 2008;22 (2) 214- 218
PubMed Link to Article

Correspondence

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