A Case of Papillophlebitis Caused by the Contraceptive Implant in a Healthy Young Woman with Angioid Streaks

Introduction: Papillophlebitis is a rare condition that has a high probability of misdiagnosis due to its puzzling resemblance to common conditions, especially Central Retinal Vein Occlusion (CRVO) and papillitis. Methods: We present an interesting case of a 30-year-old healthy woman with mild CRVO in the right eye associated with ocular pain on upgaze, visual field defect, dyschromatopsia, and desaturation of red color along with initially normal visual acuity, nondetectable relative afferent pupillary defect, and normal foveal reflex. She underwent a subdermal etonogestrel implant (progestin) by a gynecologist two months before presentation. Following the removal of the progestin implant by her gynecologist, we started the patient on intravenous methylprednisolone (500mg daily for 3 days). Results: This combination of incomplete features of CRVO and papillitis guide us to the diagnosis of atypical papillophlebitis. The fundi showed a typical appearance of angioid streaks. The association between papillophlebitis and angioid streaks was unclear. After a thorough review of the literature, no correlation was found, indicating that angioid streak is only an incidental finding. Conclusion: The patient’s condition improved with systemic steroid administration, and complete visual recovery was noted after one year, despite the presence of macular ischemia and nerve fiber layer atrophic changes on Optical Coherence Tomography (OCT) and OCT angiography.

Grönblad-Strandberg syndrome

The Grönblad-Strandberg syndrome is an inherited disease characterized by mineralization of elastic fibers in the skin, blood vessels, and the eye. Skin lesions are represented by flat xanthomatous ivory-white nodules. Eye lesions are divided into stages. In the first stage, there is angioid streak formation. In the second stage, subretinal neovascular membranes may form. If this occurs in the subfoveal area, the patient may lose central vision. The third stage is characterized by scarring.

Optical Coherence Tomography (OCT) and Angiography in Patients with Angioid Streaks

Purpose To investigate the retinal and choroidal features of angioid streak by fluorescein angiography (FA), indocyanine green anigography (ICG-A) and optical coherence tomography (OCT). Methods We analysed 46 eyes of 23 patients with angioid streaks examined at our retina section between March 1998 and June 2000. Their mean age was 42.8 years (26–61 years). After a routine ophthalmological examination, OCT and simultaneous fluorescein and indocyanine green angiography (FA/ICG-A) were done. Color fundus photographs were also taken. Results A peau d'orange appearance and angioid streaks were more numerous and apparent with ICG-A than FA. In two eyes (4.3%), some streaks that were well visualized on FA were not visible on ICG-A. Twenty eyes (43.5%) had macular complications and 26 (56.5%) were normal. Occult choroidal neovascularization (CNV) was diagnosed by ICG-A. Nine eyes had occult, and 11 had classic CNV. Ruptures of Bruch's membrane were hypofluorescent in 20 (43.5%) and hyperfluorescent in 26 eyes (56.5%). Optic disc drusen were detected in both eyes of one patient (4.3%). Localized hyperreflectivity was thought to be due to calcium deposits. Conclusions Angioid streaks were visualized more clearly and in larger numbers by ICG-A than FA. However, in some cases streaks that were funduscopically and fluorescein angiogrpahically visible could not be seen by ICG-A. Occult CNV was detected by ICG-A. Some mottled areas were seen and more clearly visualized by ICG-A. Calcium deposits were observed as localized areas of hyperreflectivity on OCT. These findings indicate that fluorescein angiography, ICG-A and optical coherence tomography all provide supportive information for each other and can be used for either diagnosis or follow-up of those patients.