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Late Onset HELLP-Eclampsia-PRES

Author Information
Asmita Patil*, Parulekar SV**, Samant PY***.
(*Senior Resident, ** Professor and Head of Department, *** Associate Professor. Department of Obstetrics and Gynecology, Seth G. S. Medical College & K.E.M. Hospital, Mumbai, India.)

Abstract

Among the complications of eclampsia, Hemolysis Elevated Liver enzymes Low Platelets (HELLP) syndrome figures prominently as a cause of aggregated morbidity and of mortality. It is an important complication of pregnancy, and it typically happens in women with known diagnosis of pre-eclampsia.[1] Neurological complications of eclampsia include mental confusion, seizures, cortical blindness, deficits in the visual fields. At neuroimaging, abnormalities includes cortical edema, intracerebral hemorrhage and Posterior Reversible Encephalopathy Syndrome(PRES).[2] Here we present a case of late onset HELLP syndrome with postpartum eclampsia complicated with PRES.

Introduction

Preeclampsia and eclampsia are two clinical situations that are exclusively associated with pregnancy. Symptoms typically begin after the 24th week of pregnancy. Pre-eclampsia and eclampsia figure among the three most important causes of death in pregnancy.[2]
Pre-eclampsia and eclampsia can have many serious complications; among them HELLP syndrome and PRES are two separate entities but very rarely they can present concurrently. We describe a case of association between HELLP syndrome and PRES in a patient with postpartum eclampsia. This association is poorly described. Very few cases have been reported till today.[3-6]

Case Report

 A 25 year old female G2P1L1 with 9 months amenorrhea presented with pain in abdomen since 5 to 6 hours and gestational hypertention first time diagnosed on admission. Her blood pressure was 140/90 mm Hg and proteinuria of 4+. She had no premonitory symptoms. Her deep tendon reflexes were normal. She was given Tab. Alfa methyldopa 250 mg 8 hourly and Cap.Nifedipine 5mg orally as required. She was in active labor, and delivered a live1.7 kg female baby vaginally within 30 minutes. Two hours postpartum her B.P. was 130/90 mm Hg and proteinuria reduced to 2+. Her renal chemistry and liver function tests reports were within normal limits. After 12 hours patient was found slightly icteric and pale. Her blood pressure was 160/100 mm of Hg, urine albumin 2+ and deep tendon reflexes were normal, she had no premonitory symptoms; hence the dose of antihypertensives was increased, Tab. Alpha methyldopa 6 hrly and C. Nifedepin 10 mg 6hrly. Her PIH profile was sent again. Which came as, Hb 13.1gm% , WBC: 10000/cmm, platelets 56000/cmm, INR:1.25, P.T :16.5sec, control :13.5sec, total bilirubin 3.4mg/dL, direct bilirubin 1.5mg/dL, SGOT:520U/L, SGPT :362U/L, lactic dehydrogenase240U/L. Renal function test, serum electrolytes, plasma proteins were within normal limits. Viral markers for hepatitis A,C,B,E were negative. A diagnosis of HELLP syndrome was made; physician reference was done, they advised Injection Cefotaxime , Injection Metronidazole  and I.V. fluids to be given. 24 Hrs later icterus was profound, blood pressure was in the range of 150/90 mm Hg and hematuria was present. Her repeat investigations showed deranged coagulation; PT : 10.8 sec.,  control: 8.9 sec,  INR :1.21, PTT  Test:31.0 sec,  control: 29 sec, thrombin  time: 15, fibrinogen: 334, FDP:>320 (N-<5), D-Dimer: >10(N-<0.3 mg/L). As per Haemotology reference 4 units platelet transfusion was given, 35 hrs later patient had gereralized  tonic clonic seizures. Blood pressure: 150/100 mm Hg, Proteinuria- 2+, Deep tendon reflexes were normal. Inj.MGSO4 by ZUSPAN regime was started and antihypertensives were continued. Physician reference taken again advised Inj. Phenytoin sodium 100 mg stat and then twice a day , 4 units of platelets transfusion & transfer to Medical Intensive Care Unit(ICU) .Computed Tomography brain done, showed posterior reversible encephalopathy syndrome. IV antibiotics, Inj.mannitol and antihypertensives given in Medical ICU; patient recovered completely over a period of 5 days.

Discussion

HELLP syndrome is rare before the 20th week of pregnancy. In 1/3rd of the cases it happens in the immediate post-natal phase. Hematologic (DIC) and cardiopulmonary events (pulmonary edema, acute cardiac insufficiency) are commonly observed. Neurological alterations (cerebral bleeding, hypoxic-ischemic encephalopathy) are seen in only 4.5% of patients.[7]
Feske et al. showed that endothelial dysfunction is likely important cause in the genesis of the syndrome. The endothelium would be directly damaged by factors such as vasospasm, ischemia, platelet activation, increase in the levels of vasoactive molecules such as serotonin and thromboxane A-2, activation of the coagulation system, and impairment in the action of the normal placentally mediated adaptations to prostacyclin , nitrous oxide, as well other endothelial mediators of the vascular tone and of vascular permeability. Hinchey etal. also has described  that, the vasoactive mechanism, with damage and loss of the cerebral vascular auto regulation, is the proposed basic mechanism for PRES.[8] The clinical observation that the vascular system in the anterior regions of the brain have more dense sympathetic innervations, as compared to the posterior regions, which may explain the predilection for edema in the posterior regions of the brain, especially in the parieto- occipital regions.[9-11]
In this article we have reported a rare association between HELLP syndrome , PRES and eclampsia of late onset. It seems that endothelial vasogenic mechanisms led to the neurologic manifestations which occurred in our case. The effective and fast correction of the blood pressure levels, associated to the quick recognition and treatment of the  HELLP syndrome and PRESS resulted into good outcome.

References

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Citation

Patil A, Parulekar SV, Samant PY. Late Onset HELLP-Eclampsia-PRES. JPGO Volume 1 Issue 4, April 2014, available at: http://www.jpgo.org/2014/04/late-onset-hellp-eclampsia-pres.html