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Case report

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

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

Asma Khallouli1,2, Racem Choura1,2,&, Rahma Saidane1,2, Dhouha Gouider1,2, Afef Maalej1,2, Riadh Rannen1,2

 

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

 

 

Abstract

HELLP syndrome (hemolysis, elevated liver enzymes, low platelets) is a pregnancy disorder representing a severe form of preeclampsia and associated with high maternal and fetal mortality. Serous retinal detachment has been reported in eclampsia. However, bilateral exudative retinal detachment associated with HELLP syndrome has rarely been described. We report a case of a 32-year-old patient, gravida III para III, with hypertensive HELLP syndrome who developed bilateral exudative bullous retinal detachment following caesarean delivery at 33 weeks. A comprehensive management and a scheduled monitoring of this potentially sight-threatening eye disease led to saving useful vision.

 

 

Introduction    Down

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 Up    Down

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 Up    Down

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 Up    Down

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 Up    Down

The authors declare no competing interests.

 

 

Authors' contributions Up    Down

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

 

 

Figures Up    Down

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 Up    Down

  1. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol. 1982;142(2):159-67. PubMed | Google Scholar

  2. Sibai BM, Taslimi MM, El-Nazer A, Amon E, Mabie BC, Ryan GM. Maternal-perinatal outcome associated with the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia eclampsia. Am J Obstet Gynecol. 1986;155(3):501-9. PubMed | Google Scholar

  3. Ober RR. Pregnancy-induced hypertension (preeclampsia-eclampsia). In: Ryan SJ, editor Retina Vol 2 2nd ed St Louis: Mosby. 1994;1405-11.

  4. Sunness JS. Pregnancy and retinal disease. In: Ryan SJ, editor Retina St Louis: Mosby. 1994;1393-403.

  5. McEvoy M, Runciman J, Edmonds DK, Kerin JF. Bilateral retinal detachment in association with preeclampsia. Aust N Z J Obstet Gynaecol. 1981;21(4):246-7. PubMed | Google Scholar

  6. Fry WE. Extensive bilateral retinal detachment in eclampsia with complete reattachment: report of two cases. Arch Ophthalmol. 1929;1(5):609-14. Google Scholar

  7. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal morbidity and mortality in 442 pregnancies with haemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol. 1993;169(4):1000-6. PubMed | Google Scholar

  8. Burke JP, Whyte I, MacEwen CJ. Bilateral serous retinal detachments in the HELLP syndrome. Acta Ophthalmol Scand. 1989;67(3):322-4. PubMed | Google Scholar

  9. Tranos PG, Wickremasinghe SS, Hundal KS, Foster PJ, Jagger J. Bilateral serous retinal detachment as a complication of HELLP syndrome. Eye. 2002;16(4):491-2. PubMed | Google Scholar

  10. Gupta LY, Mansour SE. Bilateral bullous retinal detachment as a complication of the HELLP syndrome. Can J Ophthalmol. 1994;29(5):242-5. PubMed | Google Scholar

  11. Karaguzel H, Guven S, Karalezli A, Erdol H. Bilateral serous retinal detachment in a woman with HELLP syndrome. J Obstet Gynaecol. 2009;29(3):246-8. PubMed | Google Scholar

  12. Mendez-Figueroa H, Davidson C. Bilateral retinal detachments and preeclampsia: thrombotic thrombocytopenic purpura or syndrome of haemolysis, elevated liver enzymes, low platelets. J Matern Fetal Neonatal Med. 2010;23(10):1268-70. PubMed | Google Scholar

  13. Kovács EM, Molvarec A, Rigó J Jr, Szabó A. Bilateral serous retinal detachment as a complication of acquired peripartum thrombotic thrombocytopenic purpura bout. J Obstet Gynaecol Res. 2011;37(10):1506-9. Google Scholar

  14. Schönfeld CL. Bilateral exudative retinal detachment in HELLP Syndrome. Case Rep Ophthalmol. 2012;3(1):35-7. Google Scholar

  15. Mayer WJ, Hakim I, Ulbig MW, Kernt M, Haritoglou C. Non-mydriatic wide field fundus photography in bilateral serous retinal detachment due to HELLP syndrome. Arch Gynecol Obstet. 2012;286(3):819-20. Google Scholar

  16. Singh A, Yadav I, Deshmukh S, Maurya RP, Pandey S. Rare case of exudative retinal detachment in Normotensive HELLP syndrome: a case report. Ind J Clin Exp Ophthalmol. 2015;1(3):187-8. Google Scholar

  17. Valluri S, Adelberg DA, Curtis RS, Olk RJ. Diagnostic indocyanine green angiography in preeclampsia. Am J Ophthalmol. 1996;122(5):672-7. PubMed | Google Scholar

  18. Cogan DG. Ocular involvement in disseminated intravascular coagulopathy. Arch Ophthalmol. 1975;93(1):1-8. PubMed | Google Scholar

  19. Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-44. PubMed | Google Scholar

  20. Gass JDM, Pautler SE. Toxemia of pregnancy pigment epitheliopathy masquerading as a heredomacular dystrophy. Trans Am Ophthalmol Soc. 1985;83:114-30. PubMed | Google Scholar