Bilateral vision loss after delivery in two cases: severe preeclampsia and HELLP syndrome.
Preeclampsia, the most common medical complication of pregnancy, is characterized by elevated systemic blood pressure ([greater than or equal to] 140/100 mmHg), proteinuria ([greater than or equal to] 300 mg/24 hours) and generalized edema, and typically appears after the 20th week of pregnancy. Its occurs in 5-10% of all pregnancies. (1) Severe preeclampsia presents with systemic blood pressure over 160/110 mmHg, proteinuria [greater than or equal to] 2 gr/24 hours, serum creatinine >2 mg/dl, oliguria, thrombocytopenia, epigastric pain, cerebral and visual disruptions, headache, pulmonary edema, and elevated liver enzymes. Convulsions before or after birth in addition to the symptoms of preeclampsia indicates progression to eclampsia. HELLP (Hemolysis-Elevated Liver enzymes-Low Platelets) syndrome, which is a more severe form that develops in approximately 4-20% of preeclamptic patients, is characterized by hemolysis, elevated liver enzymes and low thrombocyte numbers, and is associated with high maternal and perinatal morbidity and mortality. HELLP syndrome was first mentioned in 1954 by Prichard and was described clinically in detail in 1982 by Weinstein. (2) It generally occurs in the third trimester, but more rarely can develop in earlier weeks of pregnancy or in the postpartum period, and may appear without hypertension.
Preeclampsia or HELLP syndrome patients may develop retinal and choroidal circulation dysfunction, and various fundoscopic findings and subsequent vision loss may occur as a result. These patients may have severe hypertensive retinopathy findings such as retinal hemorrhage, subretinal serous fluid accumulation, papilledema, and Elschnig spots. (3)
In this study we present the concurrent fundus findings of one case of severe preeclampsia and one case of HELLP syndrome.
A 37-year-old patient in the 32nd week of her second pregnancy presented to our hospital with headache and blurred vision. At initial examination, systemic blood pressure was 220/110 mmHg and bilateral (+ + + +) pretibial edema was observed during physical examination. Laboratory tests revealed thrombocytopenia (72 x [10.sup.3]/[mm.sup.3]) and liver function tests were elevated: alanine aminotransferase (ALT), 104 IU/L; aspartate aminotransferase (AST), 129 IU/L; and lactate dehydrogenase (LDH), 603 U/L. Urine analysis showed (+++) proteinuria. On obstetric ultrasonography the fetus showed symmetrical developmental delay consistent with 29 weeks. The patient was diagnosed with HELLP syndrome and an emergency cesarean section was performed. On ophthalmologic examination conducted in the intensive care unit on postpartum day 2, the patient's visual acuity was counting fingers at 5 meters. Bilateral light reflexes were natural, anterior segment examination revealed no irregularities in either eye, and intraocular pressure was within normal range. Retinal hemorrhage and bilateral macular hemorrhage with cotton wool spots were observed during fundus examination. Systemic blood pressure regulation and follow-up were recommended. In postpartum week 1 the patient's best corrected visual acuity (BCVA) was 2/10 in the right and 5/10 in the left eye. At 1-month follow-up examination the hemorrhages had completely resolved, BCVA was 8/10 in the right and 10/10 in the left eye, and optical coherence tomography (OCT) was normal. At 1-year followup, both eyes had perfect visual acuity, examination revealed no pathology, and OCT was normal (Figure 1).
A 20-year-old patient in her first, unfollowed pregnancy presented to our hospital with headache and blurred vision. Initial systemic blood pressure was 180/100 mmHg and bilateral (++) pretibial edema was observed on physical examination. Obstetric ultrasonography showed the fetus was consistent with 39 weeks gestation. Thrombocyte count was 176 x [10.sup.3]/ [mm.sup.3], ALT was 11 IU/L, AST was 28 IU/L, and urine analysis showed (+++) proteinuria. The patient was admitted to the hospital with a diagnosis of severe preeclampsia and cesarean section was performed. The patient had a BCVA of 2/10 in both eyes on ophthalmologic examination on postpartum day 1. Anterior segment examination findings were normal in both eyes and intraocular pressure was within normal range. Fundus examination revealed bilateral macular serous retinal detachment (SRD) and areas of serous elevation in the peripheral retina. On postpartum day 3, the patient's vision had decreased to counting fingers at 1 meter and fundus examination revealed widespread SRD at the macula and peripheral retina. OCT done at the same time showed elevation of the inner and outer retinal layers associated with severe neurosensorial retinal detachment as well as intraretinal cysts. Due to these findings, systemic blood pressure control and follow-up were advised. At 1-month followup the patient's vision had fully recovered. At final 1-year followup, vision was normal and no irregularities were observed during examination. OCT showed complete regression of the macular SRD and normal foveal contours (Figure 2).
SRD occurs in approximately 1% of preeclampsia and HELLP syndrome cases. SRD may be unilateral or bilateral and may appear before birth or in the postpartum period. (4,5) Other findings such as central retinal vein occlussion, vitreous hemorrhage and cortical blindness have also been reported in HELLP syndrome. (6,7,8) Different theories are proposed for the mechanism of SRD development in disease. One theory is that SRD results from choroidal ischemia; another is that it develops due to subretinal fluid accumulation secondary to increased vascular permeability. SRD in preeclampsia is attributed to damage to choroidal vessels and small-to-medium diameter vessels in the choriocapillaris resulting from severe hypertension. (9,10) Another study from Turkey reported fundus fluorescein angiography findings consistent with choroidal ischemia in a preeclamptic patient with bilateral SRD. (11) Lin et al. (12) performed wide-field angiography in a preeclamptic patient with HELLP syndrome and bilateral serous macular detachment and found peripheral retinal leakage, which they attributed to choroidal ischemia as well as blood-retina barrier dysfunction. In our preeclamptic patient, we also observed bilateral serous macular detachment and retinal SRD, especially in the inferior region. Furthermore, widespread ischemic lesions consistent with hypertensive retinopathy at the choroid level were observed on color fundus photographs. However, the patient's fundus findings completely resolved within 1 month. Similarly, Atis et al. (13) reported bilateral SRD in a patient with severe preeclampsia, and the patient's vision completely recovered after 6 weeks.
In the literature, various findings have been identified in the ocular involvement of HELLP syndrome, especially the development of retinal hemorrhage due to lowered thrombocyte count has been reported. In a Turkish study, a case of bilateral concommitant macular hemorrhage was presented. (14) One of our cases also had bilateral macular hemorrhage accompanied by areas of retinal hemorrhage and hypertensive retinopathy findings. A study of ocular findings in HELLP syndrome patients reported hypertensive changes in 16%, SRD in 3.7% and cortical blindness in 2.7% of cases. (15) As stated in the literature, we also believe the retinal hemorrhage in our patient was associated with vascular changes arising in the disease due to other causes. In addition to the vasospasm commonly seen with HELLP, endothelial dysfunction, inflammation and altered chorioretinal circulation result in ischemia, which plays a role in the development of disease signs. (9)
In conclusion, various ocular findings may occur in preeclampsia and HELLP syndrome; in most cases, these findings spontaneously resolve with proper management of the disease. Visual symptoms in these patients should definitely be regarded as serious, and the patients should be referred to an ophthalmologist in a timely manner.
Informed Consent: It was taken, Concept: Gokhan Qelik, Design: Gokhan Qelik, Data Collection or Processing: Ahmet Eser, Gokhan Qelik, Analysis or Interpretation: Gokhan Qelik, Murat Giinay, Nursal Melda Yenerel, Literature Search: Gokhan Qelik, Murat Giinay, Writing: Gokhan Qelik, Peer-review: External peer-reviewed, Conflict of Interest: No conflict of interest was declared by the authors, Financial Disclosure: The authors declared that this study received no financial support.
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Gokhan Celik *, Ahmet Eser **, Murat Gunay *, Nursal Melda Yenerel ***
* Zeynep Kamil Women and Children's Diseases Education and Research Hospital, Clinic of Ophthalmology, Istanbul, Turkey
** Zeynep Kamil Women and Children's Diseases Education and Research Hospital, Clinic of Obstetrics and Gynecology, Istanbul, Turkey
*** Haydarpasa Numune Education and Research Hospital, Clinic of Ophthalmology, Istanbul, Turkey
Address for Correspondence: Gokhan Qelik MD, Zeynep Kamil Women and Children's Diseases Education and Research Hospital, Clinic of Ophthalmology, Istanbul, Turkey
Tel.: +90 505 646 06 96 E-mail: firstname.lastname@example.org Received: 26.02.2014 Accepted: 01.09.2014
This article is also published in Turkish under doi:10.4274/tjo.45722 pages 2015; 45:271-273
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|Title Annotation:||Case Report|
|Author:||Celik, Gokhan; Eser, Ahmet; Gunay, Murat; Yenerel, Nursal Melda|
|Publication:||Turkish Journal of Ophthalmology|
|Date:||Nov 1, 2015|
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