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Saudi Journal of Ophthalmology (2014) xxx, xxx—xxx

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Case Report

Partial HELLP syndrome with unilateral exudative retinaldetachment treated conservatively

Peer review under responsibilityof Saudi Ophthalmological Society,King Saud University Production and hosting by Elsevier

Access this article onlinwww.saudiophthaljournwww.sciencedirect.com

Received 2 July 2013; received in revised form 10 February 2014; accepted 18 March 2014; available online xxxx.

a Department of Ophthalmology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences, Manjushree Nagar,Dharwad 580009, Karnataka, Indiab Department of Obstetrics and Gynaecology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences, Manjushree Nagar,Dharwad 580009, Karnataka, India

⇑ Corresponding author. Mobile: +91 9986684811.e-mail addresses: [email protected] (A.V. Pradeep), [email protected] (S. Rao), [email protected] (R. Ramesh Kumar).

Please cite this article in press as: Pradeep A.V., et al. Partial HELLP syndrome with unilateral exudative retinal detachment treated conservatively.Ophthalmol (2014), http://dx.doi.org/10.1016/j.sjopt.2014.03.011

A.V. Pradeep a; Sonali Rao a,⇑; R. Ramesh Kumar b

Abstract

Peripartum vision loss, an uncommon, often reversible complication of pregnancy usually occurs in the setting of pre-eclampsia oreclampsia. The HELLP syndrome is characterized by hypertension, elevated liver enzymes and low platelets. This is a rare case ofunilateral exudative retinal detachment associated with the Partial HELLP syndrome that occurred after delivery in a 23-year-oldIndian woman. The retinal detachment subsequently reattached with good visual improvement under conservative treatment. Thiscase highlights the importance of early intervention by the ophthalmologist when pregnant women complain about visualsymptoms.

Keywords: Pre-eclampsia, Exudative retinal detachment, Partial HELLP syndrome

� 2014 Production and hosting by Elsevier B.V. on behalf of Saudi Ophthalmological Society, King Saud University.http://dx.doi.org/10.1016/j.sjopt.2014.03.011

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Introduction

Pre-eclampsia is a multisystem disorder and HELLPsyndrome is one of its manifestations. HELLP syndrome isdefined as the presence of thrombocytopenia (<150,000platelets/microliter), hemolysis and hepatic dysfunction(elevated transaminases and lactate dehydrogenase).1 HELLPsyndrome was first described by Weinstein in 1982.2 PartialHELLP syndrome is characterized by one or two of theseabnormalities.3 Up to 25% of the patients with pre-eclampsiareport visual problems, most common being a decrease invisual acuity. An association between HELLP syndrome andvisual problems such as cortical blindness was first describedin 1987.4 HELLP syndrome associated case with ophthalmicsigns in the form of retinal detachment was first reportedby Burke et al.5 Around 18 cases of retinal detachment havebeen reported so far in the literature in patients with HELLP

syndrome.1,6–9 Most occur bilaterally during the third trimes-ter. Unilateral retinal detachments occurring in the postpar-tum period are unusual. Majority of the patients have acomplete recovery of vision with conservative managementand surgery is usually unnecessary.10,11

Case report

A 23 year old female with Gravida 2 Abortion1 with34 weeks of gestation came with a history of pain in the abdo-men, headache, low grade fever & high colored urine. Uponarrival, her BP was 170/80 mmHg, hemoglobin concentration12.7 mg/dl, blood urea 43 mg/dl (range 0–45), creatinine1.9 mg/dl (range 0.5–1.5), uric acid 12 mg/dl (range 2.4–5.7),total bilirubin 6.39 mg/dl (range 0–1.1), direct bilirubin3.73 mg/dl (range 0–0.30), indirect bilirubin 2.66 mg/dl (range0.2–0.8), serum aspartate amino transferase (AST) 139 IU/L

e:al.com

Sattur,

Sattur,

Saudi J

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Figure 3. Fundus photo of the same eye one week later. Fundus photo ofthe left eye one week later revealed resolving retinal detachment.

2 A.V. Pradeep et al.

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(range 0–46), serum alanine aminotransferase (ALT) 93 IU/L(range 0–49), lactate dehydrogenase 1308 IU/L (range 200–400), platelet count of 1.72 lakhs/cu mm (range 1.5–4) and ablood picture of normocytic normochromia with leukocytosis.She was diagnosed with Partial HELLP syndrome was startedon calcium blocker, tablet nifedipine 5 mg twice a day alongwith alfa blocker, tablet prazocin 5 mg twice a day and subse-quently underwent an emergency cesarean section the follow-ing day for non-reactive Non Stress Test. A single live healthyfemale baby weighing 2.7 kg was extracted. One month prior,her retinal examination was within normal limits. On postoperative day one, her BP was 160/100 mm Hg with totalbilirubin of 1.99 mg/dl, direct bilirubin 0.83 mg/dl, indirectbilirubin 1.16 mg/dl, AST 22 IU/L, ALT 26 IU/L, blood urea6.22 mg/dl, and serum creatinine 0.8 mg/dl. On the seventhpost-operative day, the patient complained of a sudden onsetof blurring of vision in the left eye. On examination her visualacuity in the right eye was 6/6 whereas in the left eye it was6/6 parts. Slit lamp examinations of anterior segment of botheyes were unremarkable with normal IOP. Both eyes’ vitreouswas clear. Fundoscopy of the left eye showed dumbbellshaped shallow serous retinal detachment in the inferonasalquadrant extending up to the macula as seen in Fig. 1. Fundus

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Figure 1. Fundus photo of the left eye. Fundus photo of the left eyeshowing dumbbell shaped shallow retinal detachment inferior to diskextending up to the macula.

Figure 2. Fluorescein angiogram of the left eye. Left eye fluoresceinangiogram in the late venous phase showing confluent patches indicatingchoroidal vasculature hyper permeability and pooling of dye, compatiblewith the diagnosis of exudative retinal detachment.

Please cite this article in press as: Pradeep A.V., et al. Partial HELLP syndromeOphthalmol (2014), http://dx.doi.org/10.1016/j.sjopt.2014.03.011

fluorescein angiogram in the early phase showed an area ofdelayed choroidal filling. In the midphase, the retinal vascula-ture was relatively spared without any evident signs of vascularocclusion. In the late venous phase, confluent patches wereseen indicating choroidal hyper permeability and pooling ofthe dye, which was compatible with the diagnosis of exudativeretinal detachment seen in Fig. 2. The patient was started onan antioxidant tablet, once a day and Nepafenac 0.1% eyedrops four times per day along with bed rest and head endelevation. A diagnosis of severe preeclampsia with PartialHELLP syndrome complicated with a unilateral serous retinaldetachment was made. A week later fundoscopy of the lefteye revealed resolving serous retinal detachment as seen inFig. 3. One month post treatment the patient showed com-plete resolution of the detachment of retina with regainingof visual acuity.

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Discussion

Retinal detachment is an unusual but well documentedphenomenon in hypertensive disorders of pregnancy affect-ing 0.9% of patients with HELLP syndrome.12 Retinal detach-ment is seven times more common in women with pre-eclampsia or eclampsia with HELLP syndrome than thosewithout HELLP syndrome.1 Hutchings et al. suggested thatretinal detachment is a consequence of choroidal vasculardamage. Choroidal ischemia is secondary to arteriolar vaso-spasm which affects the retinal pigment epithelium andbreaks down the blood retinal barrier. HELLP syndromecauses hemolysis of red blood cells leading to focal ischemiadue to capillary obstruction.13 Retinal detachment in HELLPsyndrome is commonly seen in the last trimester of preg-nancy, but seldom within the first week postpartum. In thepostpartum period, subretinal fluid is absorbed by retinalpigment epithelium and the visual acuity generally improveswithin weeks.6 In our case, the patient with Partial HELLP syn-drome had only unilateral serous retinal detachment as com-pared to the bilateral detachments reported in the literature.Immediate management of HELLP syndrome and delivery ofthe fetus along with timely intervention by the ophthalmolo-gist are advisable to reverse any ocular manifestations andavoid long term visual sequelae.7

with unilateral exudative retinal detachment treated conservatively. Saudi J

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Partial HELLP syndrome with unilateral exudative retinal detachmentQ1 3

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Conclusion

The case demonstrates that timely intervention and regu-lar follow up visits are essential in pregnant women with pre-eclampsia or eclampsia with HELLP syndrome to avoid visualproblems. Hence, all patients with gestational hypertensionshould undergo a thorough retinal examination.

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Conflict of interest

The authors declared that there is no conflict of interest.

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References

1. Vigil-De Gracia P, Ortega-Paz L. Retinal detachment in associationwith pre-eclampsia, eclampsia and HELLP syndrome. Int J GynecolObstet 2011;114:223–5.

2. Burke JP, Whyte I, MacEwen CJ. Bilateral serous retinal detachmentin HELLP syndrome. Acta Ophthalmol 1989;67(3):322–4.

3. Ian Donald, Hypertensive disorders in pregnancy. In: Renu Misra,editors. Ian Donald’s Practical Obstetric Problems, New Delhi: BIpublications pvt ltd; 2008, p. 294.

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Please cite this article in press as: Pradeep A.V., et al. Partial HELLP syndromeOphthalmol (2014), http://dx.doi.org/10.1016/j.sjopt.2014.03.011

4. Levavi H, Neri A, Zoldan J, Segal J, Ovadia J. Pre-eclampsia, ‘‘HELLP’’syndrome and postictal cortical blindness. Acta Obstet GynecolScand 1987;66:91–2.

5. Jaffe G, Schatz H. Ocular manifestations of preeclampsia. Am JOphthalmol 1987;103:309–15.

6. Gundlach E, Junker B, Gross N, Hansen LL, Pielen A. Bilateral serousretinal detachment. Br J Ophthalmol 2013;97(7):939–40.

7. Schönfeld C-L. Bilateral Exudative Retinal Detachment in HELLPSyndrome. Case Rep Ophthalmol 2012;3(1):35–7.

8. Lin P, Hahn P, Fekrat S. Peripheral retinal vascular leakagedemonstrated by ultra-widefield fluorescein angiography inpreeclampsia with HELLP syndrome. Retina 2012;32(8):1689–90.

9. Mayer WJ, Hakim I, Ulbig MW, Kernt M, Haritoglou C. Non-mydriaticwide field fundus photography in bilateral serous retinal detachmentdue to HELLP syndrome. Arch Gynecol Obstet 2012;286(3):819–20.

10. Ramaesh K, Nagendran S, Saunders DC. Choroidal ischaemia andserous retinal detachment in toxemia of pregnancy. Eye1999;13:795–6.

11. Sathish S, Arnold JJ. Bilateral choroidal ischaemia and serous retinaldetachment in pre-eclampsia. Clin Exp Ophthalmol 2000;28:387–90.

12. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA.Maternal morbidity and mortality in 442 pregnancies with hemolysis,elevated liver enzymes, and low platelets (HELLP syndrome). Am JObstet Gynecol 1993;169:1000–6.

13. Hutchings K, Sangalli M, Halliwell T, Tuohy J. Bilateral retinaldetachment in pregnancy. Aust NZ J Obstet Gynaecol2002;42:409–11.

with unilateral exudative retinal detachment treated conservatively. Saudi J