Bilateral exudative retinal detachment in HELLP syndrome, first description in North Africa: a case report

¹Department of Ophthalmology, Military Hospital of Tunis, Mont Fleury-1008, Tunisia, ²Tunis El Manar University, Tunis, Tunisia

^&Corresponding author
Racem Choura, Department of Ophthalmology, Military Hospital of Tunis, Mont Fleury-1008, Tunisia

Introduction    

HELLP syndrome is a pregnancy disorder defined by hemolysis, elevated liver enzymes and low platelets. It was first described by Weinstein in 1982 [1]. Patients suffering from HELLP syndrome are typically multiparous young white women with a mean age of 25 years. Clinical symptoms develop during the second or third trimester. It represents a severe form of preeclampsia associated with high maternal and fetal mortality (3% and 24% respectively) [2]. The visual system may be affected in 25% to 100% of patients with severe hypertensive disorders of pregnancy [3]. Blurred vision is the most common visual complaint and the most frequent ocular finding is arterial narrowing. Severe hypertensive disorders of pregnancy may result in an increasing impedance in choroidal blood flow. This dysregulation can lead to choroidal ischemia causing exudative retinal detachment [4]. Usually, it occurs concomitantly with hypertensive retinopathy [5]. Exudative retinal detachment has been reported in eclampsia [6]. However, Bilateral exudative retinal detachment associated with HELLP syndrome has rarely been reported. We report an interesting case of a 32-year-old patient, gravida III para III, with hypertensive HELLP syndrome who developed bilateral exudative bullous retinal detachments.

Patient and observation     

Patient information: a 32-year-old caucasian woman, gravida III para III, with unremarkable ophthalmic history, complained of bilateral blurred vision and metamorphopsia following caesarean delivery at 33 weeks. She had the caesarean section for preeclampsia, for which she had been treated during the previous two months. On the first day post-partum, she developed features consistent with HELLP syndrome. The patient was referred to the intensive care unit.

Clinical findings and diagnostic assessment: at ophthalmic examination on day 1 post-partum, the visual acuity was reduced to 20/400 (snellen imperial) in the right eye and counting fingers at 1 meter in the left eye. Anterior segment examination was unremarkable in both eyes and the intraocular pressure was normal. Dilated fundus examination with indirect ophthalmoscopy revealed in both eyes a clear vitreous without any cells indicating an inflammatory condition. We noticed marked sclerosis of the retinal arterioles, few cotton wool spots associated with bilateral papilledema defining grade IV hypertensive retinopathy (Figure 1 A, B). The retina disclosed bilateral bullous serous retinal detachments reaching the macular region (Figure 1 C, D).

Therapeutic intervention: the patient received 1.5 mg/kg body weight/day prednisolone for one week to reduce edema, inflammation and vascular permeability, and was positioned upright. Blood pressure was controlled. The ophthalmic follow-up examination on day seven showed a reapplication of the retina and a regression of hypertensive retinopathy signs in both eyes (Figure 1 A, B). Prednisolone was reduced every week by 20mg.

Follow-up and outcomes: follow-up within 15 days, 30 days and 2 months revealed an improvement of visual acuity on both eyes. At 2 months, visual acuity has fully recovered (20/20 in both eyes) and subretinal fluid was totally reabsorbed. However, a mild irregularity of the retinal pigment epithelium was still present in extramacular location.

Patient perspective: the patient was initially hopeless and thought that recovery from blindness is beyond reasonable expectation. She was astonished to fully recover from serious visual impairment.

Informed consent: informed consent was obtained from the patient prior to collecting data and issuing iconography.

Discussion     

Exudative retinal detachment is an unusual but well documented complication of hypertensive disorders in pregnancy affecting 0.2 to 2% of patients with severe preeclampsia and 0.9% of patients with HELLP syndrome [7]. However, the bilateral presentation of serous retinal detachment in HELLP syndrome is extremely rare. Only few cases have been reported in the peer-reviewed literature [8-16]. The precise pathogenesis of serous retinal detachments in preeclampsia is still unclear. Nevertheless, it is admitted that the choriocapillaris is damaged by ischemia or thrombosis due to disseminated intravascular coagulation [17,18]. Furthermore, additional ischemic damage to the retinal pigment epithelium (RPE) may lead to the impairment of the physiologic pump mechanism. Pumping and metabolic functions of the RPE play the most important role in keeping sensorial retina attached to RPE. Moreover, fluid flow dynamics and strong connections between RPE cells contribute to this regulation. Conditions like inflammation, infection, hypertension, hypoproteinaemic states, subretinal neovascularization, and uveal effusion syndrome can cause exudative retinal detachment by disturbing these mechanisms [19]. In most cases, the detachment occurs concomitantly with hypertensive retinopathy [5].

Exudative retinal detachment in preeclampsia and particularly in HELLP syndrome can occur both ante partum and post-partum (as in our case) causing blurred vision, metamorphopsia and significant reduction in visual acuity. We underline the fact that therapeutic strategies do not obey to a standard-arranged evidence-based treatment. In many cases, the detachment resolves with a return to normal visual function within the first few weeks post-partum. Yet, some patients may develop residual macular RPE changes, overlaying areas of choriocapillaris infarction (Elschnig´s spots) and resulting in permanent visual impairment. This pregnancy pigment epitheliopathy may masquerade as a heredomacular dystrophy [20]. In a case of bilateral exudative retinal detachment complicating HELLP syndrome, Tranos et al. reported spontaneous resorption of the subretinal fluid started promptly 2 days after the delivery and complete resolution of the retinal detachments within 2 weeks and 1 month post-partum in the right and the left eye respectively, resulting in recovery of the visual function to pre-detachment levels [9]. In a similar case described by Schönfeld, the therapeutic strategy included 1.5 mg/kg body weight/day prednisolone for 3 days, reduced every third day by 20mg [14]. In our case, we decided to administer 1.5mg/kg body weight/day prednisolone for one week in order to reduce edema, inflammation and vascular permeability. The patient was positioned upright and blood pressure was controlled. Full retinal reapplication was obtained on day seven and full visual recovery (20/20 in both eyes) was attained at 2 months.

Conclusion     

Serous retinal detachment should always be considered within the differential diagnosis of sudden loss of vision in cases complicated with HELLP syndrome and more generally toxemia of pregnancy. Ophthalmologists should be aware that retinal detachment may be present in the absence of other hypertensive retinal changes. The relatively good prognosis with visual recovery and settling of detachment should not hide the seriousness of this disease. Only a comprehensive management and a scheduled monitoring could save useful vision.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

All the authors have read and agreed to the final manuscript.

Figures     

Figure 1: fundus photography of both eyes showing; A,B) sclerosis of the retinal arterioles (black arrows), few cotton wool spots (white circles) associated with bilateral papilledema defining grade IV hypertensive retinopathy; C) inferior bullous retinal detachment in the right eye; D) superior bullous retinal detachment in the left eye

Figure 2: A,B) fundus photography of both eyes on day eight showing the reapplication of the retina and the regression of hypertensive retinopathy signs

References