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Volume 115, Issue 5, Pages 893-897 (May 2008)


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Histopathologic and Trypsin Digestion Studies of the Retina in Incontinentia Pigmenti

W. Robert Bell, MD1, W. Richard Green, MD1, Morton F. Goldberg, MD2Corresponding Author Informationemail address

Received 18 May 2007; received in revised form 23 July 2007; accepted 14 August 2007. published online 01 November 2007.

Objective

To report the ocular histopathologic features of a 55-year-old patient with incontinentia pigmenti retinopathy.

Design

Observational case report.

Participant

A 55-year-old patient with incontinentia pigmenti retinopathy.

Methods

Examination of eyes by light microscopy and retinal trypsin digestion.

Main Outcome Measures

Clinical and histopathological findings.

Results

Histopathologic examination disclosed inner retinal ischemic atrophy, capillary beading, arteriolar–venous anastomoses, preretinal neovascularization, vasculopathy located at the junction of central vascular and peripheral avascular retina, retinal tears, and tractional retinoschisis.

Conclusions

Patients with retinal manifestations of incontinentia pigmenti may progress to proliferative vitreoretinopathy or retinal detachment and should be observed closely over the course of their lifetime.

Available online: November 5, 2007.

1 Eye Pathology Laboratory, Department of Pathology, Wilmer Institute, Johns Hopkins Medical Institutions, Baltimore, Maryland.

2 Retina Service, Wilmer Institute, Johns Hopkins Medical Institutions, Baltimore, Maryland.

Corresponding Author InformationCorrespondence and reprint requests to Morton F. Goldberg, MD, Wilmer Eye Institute, Woods 276, Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21287-9128.

 Manuscript no. 2007-665.

 Supported in part by Independent Order of Odd Fellows, Winston-Salem, North Carolina (WRG), and the Guerrieri Retinal Research Fund at the Wilmer Eye Institute (MFG).

 The authors have no financial or proprietary interests in the subject.

PII: S0161-6420(07)00930-X

doi:10.1016/j.ophtha.2007.08.027


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