Diffuse choroidal hemangioma in pregnancy: Symptomatic diffuse choroidal hemangioma spontaneously resolving postpartum

Abstract

We present the case of a pregnant young woman with symptomatic diffuse choroidal hemangioma (CH), diagnosed with Sturge–Weber syndrome (SWS). Three months postpartum, there was full spontaneous resolution of the subretinal fluid and improvement in the visual acuity (VA). A 29-year-old, 31-week pregnant female with a coagulation disorder and enoxaparin (clexane) treatment, complained of a left visual disturbance of 2 weeks duration. On examination, the right eye was normal; left eye VA was 6/12 (20/40) with a left eyelid port-wine stain and on funduscopic examination there was a serous retinal detachment. On ultrasound, there was diffuse thickening of the choroid with a focus superior and nasal to the disc with active vascularization – a diagnosis of diffuse CH was made. Magnetic resonance imaging (MRI) and MR venography studies of the brain and orbit revealed engorged orbital vasculature, and the diagnosis of SWS was made. Three months after Cesarean section with birth of a healthy baby boy, VA returned to 6/9 with full resorption of subretinal fluid. On fundoscopic exam, there were no retinal or vein occlutions but there were tortuous blood vessels with A-V crossing. This is a rare case of newly diagnosed SWS with symptomatic diffuse CH in pregnancy. Postpartum, there was full resolution of the subretinal fluid. A watchful waiting approach can be considered in such cases.

Introduction

Choroidal hemangiomas (CHs) are generally described as reddish orange lesions of varying thickness. They can affect visual acuity (VA) either by direct involvement of the lesion in the macular area or by subretinal fluid that accumulates causing a decrease in VA. We would like to present a case of a pregnant young woman with symptomatic diffuse CH, diagnosed with Sturge–Weber syndrome (SWS). Three months postpartum, there was full spontaneous resolution of the subretinal fluid and improvement in the VA.

Case Report

A 29-year-old woman presented in her 31 week of pregnancy to the Ocular Oncology Department at Sheba Hospital complaining of a 2-week decrease in the vision in her left eye. This patient had a positive medical history of a homozygous mutation of the prothrombin gene and was treated with enoxaparin (clexane). On examination, VA in the right eye was 6/6 (20/20), and complete ophthalmic examination was normal. On the left side, a prominent port-wine stain was seen involving, left cheek and left eyelid [Figure 1]. VA was 6/12 (20/40) with prominent episcleral vessels; on dilated funduscopic examination, tortuous blood vessels with A-V crossing and serous retinal detachment were seen with no tear or hole seen [Figure 2]. As a workup, B-scan ultrasound was performed; there was diffuse choroidal thickening with the thickest area superior nasal to the disk. There was diffuse vascularization seen in the thickest area [Figure 3]. On optical coherence tomography, there was a mass superior nasal to the disk with subretinal fluid in the macular area [Figure 4]. Due to the findings, a suspected diagnosis of SWS was assumed. A magnetic resonance imaging (MRI) and MR venography of the brain and orbits were performed revealing engorged orbital vasculature and confirming the diagnosis of SWS. The revision of an old MRI study from 2014 [Figures 5 and 6] depicted choroidal thickening back then. We debated treatment versus watchful waiting, as well as method of birthing; vaginal versus cesarean-section (CS). After a multidisciplinary consultaion with the hematology, gynecology and ocular oncology departments, a watchful waiting approach was decided upon.

Figure 1.

Clinical photo: Port-wine stain

Figure 2.

Fundus photo: Serous retinal detachment

Figure 3.

Ultrasound: Diffuse choroidal thickening (yellow arrow) with the thickest area superior nasal to the disk

Figure 4.

Optical coherence tomography: Subretinal fluid in the macular area

Figure 5.

Magnetic resonance imaging/magnetic resonance venography of brain and orbits coronal view: Engorged orbital vasculature

Figure 6.

Magnetic resonance imaging/magnetic resonance choroidal thickening ad lesion (red arrow) venography of brain and orbits, axial view: Engorged orbital vasculature

The multidisciplinary consultation recommended a C- Section delivery delivery to avoid Valsalva during labor and risk of worsening the exudation or induction of vascular occlusion. The patient had a planned CS, and 3-months postpartum, there was visual improvement to 6/9 with full resorption of the subretinal fluid; return of the proper macular contour. There was no retinal or vein occlusions were seen, yet the tortuous blood vessels with A-V crossing remain unchanged [Figure 7].

Figure 7.

Optical coherence tomography: Full resorption of the subretinal fluid in the macula

Discussion

CHs are generally described as reddish orange lesions of varying thickness. They can affect VA either by direct involvement of the lesion in the macular area or by subretinal fluid that accumulates causing a decrease in VA.[1] CH can appear as an isolated circumscribed lesion or as a part of a rare congenital sporadic syndrome called SWS, also known as encephalofacial cavernous hemangiomatosis which can also have dermatological and neurological manifestations.[2] According to the literature, generally, CHs are isolated.[1] CHs often are diffuse when associated with SWS, therefore can sometimes be overlooked in the funduscopic examination. The syndrome is generally characterized by a congenital unilateral port-wine stain – A vascular malformation of the skin, located on the eyelid and forehead in the distribution of the cranial nerve V1 and V2. This raises the probability of ocular manifestations on that side, including glaucoma. In severe cases, there can be brain and/or meninges involvement with developmental delay and seizures.

In addition to antiseizure medication and support for the developmental delay, there are laser multiple treatment options for the vascular malformation of the skin.

Circumscribe or diffuse can be asymptomatic, when symptomatic, CH typically causes exudative retinal detachement symptomatic CH typically causing exudative retinal detachment. The treatment options include photodynamic treatment, plaque radiation, cryopexy, and laser photocoagulation.

There are few reports in the literature of symptomatic CH during pregnancy.[3,4,5] Nagaradh et al. described a similar case to ours with newly diagnosed SWS and symptomatic diffuse CH with spontaneous improvement postpartum.[3] Cohen et al. described two cases of circumscribed CH, one delivered with CS and treated with twin-to-twin transfusion postpartum and another with spontaneous resolution.[4] In our previous report, we described a case with a symptomatic circumscribed CH during pregnancy that spontaneously resolved after delivery.[6] Our case showed spontaneous resolution postpartum with a watchful waiting approach during pregnancy.

During pregnancy, there are significant hemodynamic changes including engorgement of the vascular networks within the hemangioma, leading to fluid leakage in the subretinal space, and postpartum, there is a decrease in the venous resistance. In addition, the hormonal changes during pregnancy, such as an increase in catecholamine, cause an increase in permeability in the blood retinal barrier, increasing the risk of subretinal fluid formation.

Unlike previously described cases, this patient also had a homozygous mutation of the prothrombin gene and was treated with clexane. She also presented with tortuous blood vessels with A-V crossing, which posed a dilemma as per the mode of delivery. After a multidisciplinary meeting, a CS was chosen to avoid Valsalva and risk of vascular occlusion.

Conclusion

We describe a symptomatic diffuse CH in a pregnant patient with newly diagnosed SWS. In this case, the subretinal fluid resolved postpartum with no additional ocular treatment.

Patient consent

The patient consented to the publication of the case in orally.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Summary statement

To present a pregnant young woman with symptomatic diffuse choroidal hemangioma (CH), diagnosed with Sturge–Weber syndrome (SWS). Three months postpartum, there was full spontaneous resolution of the subretinal fluid and improvement in the VA.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.