Bilateral retinal detachment: a clue to diagnosis of HELLP syndrome

Abstract

HELLP syndrome is characterized by hypertension, elevated liver enzymes, and low platelets, and is a life-threatening complication during pregnancy, both for mother and fetus. Bilateral, serous, nonrhegmatogenous retinal detachment (RD) is a rare complication of pre-eclampsia/eclampsia in pregnancy. We report on the clinical course and outcome in a patient in whom sudden loss of vision from bilateral exudative RD was the presenting feature of HELLP syndrome. A 20-year-old primigravida presented to us with painless, rapid loss of vision of 3 days’ duration, associated with headache and vomiting at 28 weeks of gestation. There was no history of any loss of consciousness, seizures, or weakness of limbs. Her antenatal visits at a local nursing home had been irregular, and there were no prior records of blood pressure examination. Ophthalmologic evaluation revealed a visual acuity of only perception of light in both eyes. Funduscopy showed exudative RD in both eyes (Fig. 1A, 1B). Optical coherence tomography of the retinal layers revealed neurosensory detachment from underlying retinal pigment epithelium and intraretinal cystic changes characteristic of RD in both eyes (Fig. 2A, 2B).Fig. 2A and B, Optical coherence tomography (OCT) images at presentation showing detachment of neurosensory layer from the underlying retinal pigment epithelium (white arrow) and intraretinal cystoid changes (red arrow). C and D, OCT images 4 weeks later showing spontaneous resolution of the retinal detachment with minimal residual subretinal fluid (red arrowhead).View Large Image Figure ViewerDownload (PPT) On physical examination, she was conscious and well oriented in time, place, and person. Her pulse was 98, blood pressure was 140/100 mm Hg, and respiratory and cardiovascular systems were normal. On obstetrical examination, uterus was enlarged to 26 weeks’ size, relaxed and fetal heart was regular. Ultrasonography showed single live growth restricted fetus with severe oligohydramnios, no congenital malformation, and estimated fetal weight of 700 g. Investigations revealed hemoglobin 8.6 g%, platelet count 28,000/mm3, total leucocyte count 12,000/mm3, blood urea 64 mg%, serum creatinine 1.2 mg%, total bilirubin 0.8 mg%, aspartate aminotransferase 53 units/L, alanine aminotransferase 51 units/L, alkaline phosphatase 239 units/L, serum lactate dehydrogenase 2186 IU, and albuminuria 3+. Coagulation profile was normal. A diagnosis of HELLP syndrome with severe Intrauterine growth retardation (IUGR) with bilateral exudative RD complicating severe pre-eclampsia was made. The patient was started on oral labetalol to control blood pressure along with magnesium sulphate infusion (Zuspan regimen) as anticonvulsant. She could receive only a single injection of betamethasone (12 mg) for fetal lung maturity as decision to immediately terminate the pregnancy in view of HELLP syndrome and severe IUGR was taken. Labour was induced as the patient’s relatives refused caesarean section in view of the poor fetal prognosis. The ophthalmologists were consulted before allowing a trial of labour in view of bilateral RD. The patient delivered a stillborn 767-g female fetus. She was transfused 5 units of platelets because of the low platelet count. Postdelivery, her blood pressure gradually settled and the biochemistry returned to normal within 5 days. There was significant improvement in visual acuity 2 days postdelivery. One month postdelivery, her visual acuity was 6/6 and 6/18. Funduscopy was normal except for a small, solitary intraretinal hemorrhage in the right eye. Resolution of detachment was seen both clinically and on optical coherence tomographic imaging (Fig. 1C, 1D). The latter, however, demonstrated minimal residual subretinal fluid limited to subfoveal region in both eyes (Fig. 2C, 2D). Pre-eclampsia is a hypertensive disorder of pregnancy with multiorgan involvement and in which visual system is affected in 30% to 100% cases.1Sibai B.M. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing?.Am J Obstet Gynecol. 1990; 162: 311-316Abstract Full Text PDF PubMed Scopus (521) Google Scholar, 2Ober R.R. Pregnancy-induced hypertension (pre-eclampsiaeclampsia)..in: Retina. Vol. 2. CV Mosby, St. Louis, Mo1994: 1393-1403Google Scholar In patients with acute loss of vision, the causes may include such retinal vascular changes as retinal arteriolar spasm, retinal hemorrhage, serous RD, and cortical lesions.3Tung C.F. Peng Y.C. Chen G.H. Chow W.K. Yang D.Y. Hu W.H. Hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome with acute cortical blindness..Chin Med J (Taipei). 2001; 64: 482-485Google Scholar, 4Cunningham F.G. Fernandez C.O. Hernandez C. Blindness associated with preeclampsia and eclampsia.Am J Obstet Gynecol. 1995; 172: 1291-1298Abstract Full Text PDF PubMed Scopus (147) Google Scholar, 5Sreckovic S.B. Janicijevic M.A. Stefanovic I.B. et al.Bilateral retinal detachment in a case of preeclampsia.Bosn J Basic Med Sci. 2011; 11: 129-131PubMed Google Scholar Exudative RD is observed in 1% to 2% patients with severe pre-eclampsia and 10% of patients with eclampsia.6Vigil-De Gracia P. Ortega-Paz L. Retinal detachment in association with preeclampsia, eclampsia and HELLP syndrome.Int J Gynaecol Obstet. 2011; 114: 223-225Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar De Gracia and Ortega-Paz6Vigil-De Gracia P. Ortega-Paz L. Retinal detachment in association with preeclampsia, eclampsia and HELLP syndrome.Int J Gynaecol Obstet. 2011; 114: 223-225Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar have reported 7 times greater risk for development of RD in women with HELLP syndrome as compared with healthy pregnant females.7Mohan A.K. Al-Senawi R.N. Mathew M. Ganesh A. Al-Mujaini A. Exudative retinal detachment in eclampsia. A management dilemma.Saudi Med J. 2009; 30: 302-303PubMed Google Scholar RD is usually seen in cases of pre-eclampsia with severe hypertension, although it has also been reported in mild hypertension. Although the pathophysiology of RD is not clear, it has been proposed that terminal arteriolar spasm leads to choroidal ischemia and ischemic injury to the retinal pigment epithelium, thus breaking the blood–retinal barrier.8Sánchez Vicente J.L. Ruiz Aragón J. Nanwani K. et al.Retinal detachment in preeclampsia and HELLP syndrome.Arch Soc Esp Oftalmol. 2003; 78: 335-338Crossref PubMed Google Scholar Other proposed mechanisms include disseminated intravascular coagulation due to secretory products from placenta and combination of severe hypertension, microangiopathic hemolysis, and hypoalbuminemia in choroidal vessels that results in focal ischemia leading to RD.7Mohan A.K. Al-Senawi R.N. Mathew M. Ganesh A. Al-Mujaini A. Exudative retinal detachment in eclampsia. A management dilemma.Saudi Med J. 2009; 30: 302-303PubMed Google Scholar Management of exudative RD involves treatment of the underlying cause, which in our patient was control of hypertension and immediate delivery. Prophylactic anticonvulsants were administered, and immediate delivery was induced in view of severe pre-eclampsia. No guidelines are available in the literature regarding the preferred mode of delivery in pre-eclampsia complicated by RD. However, various series indicate higher use of caesarean deliveries as compared with vaginal deliveries.7Mohan A.K. Al-Senawi R.N. Mathew M. Ganesh A. Al-Mujaini A. Exudative retinal detachment in eclampsia. A management dilemma.Saudi Med J. 2009; 30: 302-303PubMed Google Scholar This may be because of the need for immediate termination of pregnancy in view of severe pre-eclampsia/eclampsia. Severe pre-eclampsia may be associated with fetal growth restriction caused by placental insufficiency and may result in asphyxia and intrauterine death. In our patient, the relatively poor prognosis of the very small fetus and the requirement of intensive neonatal care postdelivery were explained to the patient’s relatives. They refused caesarean section and intrauterine death resulted because of severe fetal hypoxia during the course of vaginal delivery. Most women with RD show complete recovery within 2 to 12 weeks from delivery and surgical intervention is generally not required in these cases.8Sánchez Vicente J.L. Ruiz Aragón J. Nanwani K. et al.Retinal detachment in preeclampsia and HELLP syndrome.Arch Soc Esp Oftalmol. 2003; 78: 335-338Crossref PubMed Google Scholar Our patient recovered to a major degree spontaneously within a week. In conclusion, RD complicating severe pre-eclampsia and HELLP syndrome is a rare complication and requires prompt management for survival of both mother and child, and also to prevent permanent visual disability.