Revascularization After Intravitreal Bevacizumab and Laser Therapy of Bilateral Retinal Vascular Occlusions in Incontinentia Pigmenti (Bloch-Sulzberger Syndrome).

No consensus exists for the treatment of retinopathy in incontinentia pigmenti (IP). Vascular ischemia leads to tractional retinal detachments if untreated. Ultra-widefield fluorescein angiography (FA) is used to follow the vascular status of the retina. A 13-week-old female with IP presented with bilateral retinal vascular occlusions in both eyes. Ultra-widefield FA showed reperfusion after treatment with intravitreal bevacizumab (IVB) and angiography-guided laser to the avascular retina. Anti-vascular endothelial growth factor treatment reduces neovascularization and allows for growth of retinal vessels. IVB and FA-guided laser to the avascular retina is an option in IP. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e33-e37.].

[1]  A. Young,et al.  SUCCESSFUL TREATMENT OF REFRACTORY PROLIFERATIVE RETINOPATHY OF INCONTINENTIA PIGMENTI BY INTRAVITREAL RANIBIZUMAB AS ADJUNCT THERAPY IN A 4-YEAR-OLD CHILD , 2016, Retinal cases & brief reports.

[2]  Marielle P. Young,et al.  Structural Abnormalities of the Inner Macula in Incontinentia Pigmenti. , 2015, JAMA ophthalmology.

[3]  Dennis P. Han,et al.  Incontinentia Pigmenti: A Comprehensive Review and Update. , 2015, Ophthalmic surgery, lasers & imaging retina.

[4]  V. Narendran,et al.  Intravitreal bevacizumab for incontinentia pigmenti. , 2013, Journal of pediatric ophthalmology and strabismus.

[5]  M. D’Elios,et al.  Arterial occlusion mimicking vasculitis in a patient with incontinentia pigmenti , 2013, Autoimmunity Highlights.

[6]  A. Smahi,et al.  Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-κB essential modulator gene mutations. , 2011, Journal of the American Academy of Dermatology.

[7]  A. Hart,et al.  ‘Destructive encephalopathy in incontinentia pigmenti' , 2009, Developmental Medicine & Child Neurology.

[8]  T. Hirose,et al.  Prospects for Treatment of Pediatric Vitreoretinal Diseases with Vascular Endothelial Growth Factor Inhibition , 2009, Seminars in ophthalmology.

[9]  M. Lequin,et al.  Extensive cerebral infarction in the newborn due to incontinentia pigmenti. , 2008, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.

[10]  W. Green,et al.  Histopathologic and trypsin digestion studies of the retina in incontinentia pigmenti. , 2008, Ophthalmology.

[11]  C. Shields,et al.  MULTIFOCAL HYPOPIGMENTED RETINAL PIGMENT EPITHELIAL LESIONS IN INCONTINENTIA PIGMENTI , 2006, Retina.

[12]  S. Hayreh,et al.  Central retinal artery occlusion: visual outcome. , 2005, American journal of ophthalmology.

[13]  M. Goldberg,et al.  Retinal tears occurring at the border of vascular and avascular retina in adult patients with incontinentia pigmenti. , 2003, Retina.

[14]  J. Nguyen,et al.  Laser photocoagulation in preproliferative retinopathy of incontinentia pigmenti. , 2001, Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus.

[15]  M. Goldberg,et al.  Macular vasculopathy and its evolution in incontinentia pigmenti. , 1998, Transactions of the American Ophthalmological Society.

[16]  R. Bryan,et al.  Intracranial assessment of incontinentia pigmenti using magnetic resonance imaging, angiography, and spectroscopic imaging. , 1995, Archives of pediatrics & adolescent medicine.

[17]  T. Hirose,et al.  Retinal detachments in incontinentia pigmenti. , 1993, Archives of ophthalmology.

[18]  L. Wolansky,et al.  Characteristic MR findings in a neonate with incontinentia pigmenti. , 1993, AJR. American journal of roentgenology.

[19]  R. Bryan,et al.  Destructive encephalopathy in incontinentia pigmenti: a primary disorder? , 1990, Pediatric neurology.

[20]  S. Hayreh,et al.  Experimental occlusion of the central artery of the retina. I. Ophthalmoscopic and fluorescein fundus angiographic studies. , 1980, The British journal of ophthalmology.

[21]  Natalie T. Huang,et al.  Management of Retinopathy in Incontinentia Pigmenti: A Systematic Review and Update , 2018 .

[22]  D. Parke,et al.  Use of fluorescein angiography in incontinentia pigmenti: a case report. , 2013, Ophthalmic surgery, lasers & imaging retina.

[23]  M. Goldberg The blinding mechanisms of incontinentia pigmenti. , 1994, Ophthalmic genetics.