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.0000000000000997DOI Listing

Publication Analysis

Top Keywords

angioid streaks
12
vaginal delivery
8
patients
8
patients pseudoxanthoma
8
pseudoxanthoma elasticum
8
patients deliveries
8
three patients
8
choroidal neovascularization
8
delivery
5
delivery harmful
4

Similar Publications

The abnormal growth of irregular new blood vessels into the subretinal or intraretinal space is known as macular neovascularization (MNV). People over 50 are often affected by this disorder, which is typically brought on by age-related macular degeneration. In addition, MNV can be found in people under 50 years of age, who may present primary ophthalmic diseases such as pathological myopia, angioid streaks, traumatic choroidal rupture, or suspected ocular histoplasmosis syndrome.

View Article and Find Full Text PDF

Background: Pseudoxanthoma elasticum (PXE) is characterized by aberrant calcification of elastic tissues throughout the body causing varying degrees of skin, cardiac, and ocular disease. Although PXE is classically regarded as an autosomal recessive disease, recent reports have demonstrated a haploinsufficiency phenotype, in which carriers of monoallelic ATP-binding cassette transporter () gene mutations demonstrate mild manifestations of PXE. In this case report, we describe a patient with a monoallelic mutation and atypical angioid streaks.

View Article and Find Full Text PDF

A 61-year-old woman had worsening vision for 12 years associated with progressive patchy macular chorioretinal atrophy. There was latent tuberculosis and angioid streaks. Testing revealed a pathogenic variant of PRPH2 and a heterozygous ABCC6 variant.

View Article and Find Full Text PDF
Article Synopsis
  • - Abetalipoproteinemia (ABL) is a rare genetic disorder characterized by the absence of certain lipoproteins due to a defective triglyceride transfer protein, impacting the retina and leading to vision problems, including conditions resembling retinitis pigmentosa.
  • - Multimodal retinal imaging was conducted on two ABL patients, revealing significant eye changes like choroidal neovascularization and deposits in the retina, alongside a range of vision loss.
  • - The study suggests that mutations in the MTTP gene within the retina may contribute to ABL-related eye diseases, highlighting the importance of ongoing eye care for affected individuals, particularly to manage treatable conditions like choroidal neovascularization.
View Article and Find Full Text PDF

Generalized Arterial Calcification of Infancy (GACI).

Arch Pediatr

September 2024

Pediatric Endocrinology, Diabetology, Gynecology Department, Necker-Enfants malades University Hospital, AP-HP Centre, Paris 75015, France.

Article Synopsis
  • Generalized arterial calcification of infancy (GACI) is a rare genetic disorder caused by mutations in the ENPP1 and ABCC6 genes, leading to abnormal mineralization in arteries and soft tissues, resulting in serious cardiovascular issues.
  • Nearly 50% of affected infants do not survive past six months due to these complications, highlighting the severe prognosis linked to early diagnosis.
  • Current treatment focuses on symptom management with options like bisphosphonates and requires ongoing support from specialized medical teams, while research aims to develop targeted therapies to correct the underlying causes of the disease.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!