98%
921
2 minutes
20
Background/purpose: To investigate the effect of a vaginal delivery (VD) on retinal pathology in patients with pseudoxanthoma elasticum.
Methods: Retrospective case series. All 14 consecutive women with pseudoxanthoma elasticum who visited the ophthalmology department during pregnancy and after delivery between 2010 and 2018 were included. Prepartum and postpartum imaging consisted of color imaging, near-infrared imaging, and optical coherence tomography and was assessed on occurrence of (sub)retinal hemorrhages and change in angioid streaks.
Results: Fourteen patients (15 deliveries) were included, of whom 11 patients (79%) had a VD and three patients (21%) a secondary caesarian section. Data of three patients with VD (four deliveries) could not be assessed for (sub)retinal hemorrhage within 10 weeks postpartum. The median age at delivery was 31 years (IQR 29-37). One patient with VD (9%) had a choroidal neovascularization and was treated with anti-VEGF injections before assisted delivery. All patients had angioid streaks in the central 5,500 µ m of the posterior pole of both eyes. After delivery, no patient in the VD or caesarian section group presented with progression of angioid streaks or (sub)retinal hemorrhage.
Conclusion: Pushing during the expulsion phase of VD seems safe in pseudoxanthoma elasticum patients without active choroidal neovascularization, and the presence of angioid streaks alone should not be an indication for elective caesarian section.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1097/ICB.0000000000000997 | DOI Listing |
Cureus
August 2025
Ophthalmology, Burjeel Medical City, Abu Dhabi, ARE.
We present a case of a 23-year-old female with characteristic skin papules and angioid streaks characteristic of pseudoxanthoma elasticum (PXE), an autosomal recessive disorder of elastic fiber mineralization. Genomic sequencing revealed a heterozygous variant in the ABCC6 gene. Despite the absence of biallelic mutations, the clinical phenotype aligns with PXE.
View Article and Find Full Text PDFCureus
July 2025
Medical Education, OhioHealth Riverside Methodist Hospital, Columbus, USA.
Pseudoxanthoma elasticum (PXE) is a rare systemic disorder of elastic fibers. The most common manifestations include characteristic papules and ocular angioid streaks. Cardiovascular complications include peripheral arterial disease, hypertension, premature coronary artery disease, and cerebrovascular disease.
View Article and Find Full Text PDFDoc Ophthalmol
July 2025
Department of Ophthalmology, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-ku, Tokyo, 105-8461, Japan.
Purpose: To report a case of hereditary spherocytosis (HS) carrying a pathogenic variant in the SPTB gene, presenting with angioid streaks (ASs) and central retinal dysfunction.
Methods: A 63-year-old female patient diagnosed with HS was evaluated using multimodal imaging, full-field and multifocal electroretinograms (ffERGs and MfERGs), and genetic testing with an HS gene panel.
Results: Her best-corrected visual acuity was Snellen equivalent 20/22 in the right eye and 20/20 in the left eye.
Cureus
May 2025
Department of Ophthalmology, Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia, Kuala Lumpur, MYS.
A 58-year-old Malay male with underlying beta thalassemia presented with distorted vision in the right eye for two weeks. On presentation, his visual acuity (VA) was 6/18 OD (right eye) and 6/9 OS (left eye). Fundus examination of the right eye revealed a pink optic disc with peripapillary atrophy and numerous narrow, irregular streaks radiating in a circumferential pattern, along with macular scarring and pigment deposition.
View Article and Find Full Text PDFJ Invest Dermatol
June 2025
Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands. Electronic address: