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*Reader (Department of Anaesthesiology & Critical Care), +Reader (Department of Obstetrics & Gynaecology), AFMC, Pune-40.Received : 29.11.2004Accepted : 18.05.2006CaseReportIntroductionThe acronym HELLP was coined in 1982 to describea syndrome consisting of Haemolysis Elevated Liverenzyme levels and Low Platelet count [1]. The syndromeconsidered a variant of preeclampsia, can occur on itsownorinassociationwithpreeclampsia.Pregnancyinduced hypertension (PIH), preeclampsia and HELLPare related and overlap in their presentation. MaternalandfoetalmorbidityandmortalityaresignificantinHELLPsyndrome[2].Variouslifethreateningcomplications such as placental abruption, pulmonaryoedema, cerebral haemorrhage, hepatorenal failure anddisseminated intravascular coagulation (DIC) can occurinthesepatients.WepresenttwocasesofHELLPsyndrome with vague presenting complaints. First patientdevelopedHELLPinassociationwithseverepreeclampsia and in the second patient HELLP led tofoetaldeath.Wediscussthesurgicalandanaestheticimplications during peri-operative period.Case Report-1A 21 year old primigravida at 35 weeks of gestation wasadmittedwithlabourpain,headache,epigastricpainandblurringofvision.Onexaminationtherewasalteredconsciousness, pulse 86 per minute, blood pressure 170/110mm Hg, breath rate 24 per minute, and brisk tendon jerks.Based on obstetrical examination delivery by vaginal routewas planned. Baseline investigations showed haemoglobin11.9gm%, platelets 1,60,000/mm3, blood urea 26mg%, serumcreatinine0.9mg%,serumbilirubin0.9mg%,alanineaminotransferase(ALT)40unitsperlitre,aspartateaminotransferase(AST)28unitsperlitre.Totreathypertension oral nifedipine 10mg and magnesium sulphateby Pritchards regime was started. After 4 hrs of admission,repeat examination revealed pulse rate of 84 per minute, bloodpressure of 160/100 mm Hg and urinary output was 50 ml.Because of falling urinary output magnesium sulphate waswithheld and oral nifedipine continued. After 2 hrs patienthad an episode of generalised tonic clonic seizures, for whichdiazepam 10 mg intravenously was administered. It was thendecidedthatthepregnancybeterminatedbyemergencycaesareansection.Generalanaesthetictechniquewithacidaspirationprophylaxis was selected. Pre-induction fentanyl 20 g andesmolol 10 mg administered to decrease the pressure responsetolaryngoscopyandendotrachealintubation.Afterpreoxygenationarapidsequenceinduction-intubationtechniquewasusedtofacilitateendotrachealintubation.Anaesthesiawasmaintainedwithgas,oxygen,isofluraneandatracurium. Additionaldosesoffentanylandesmololwere given to keep haemodynamic parameters within 20% ofpreoperative values. A live male baby was delivered. Due toweak cry, newborn was given 300 g of naloxone. Neostigmineand glycopyrrolate were given for reversal of muscle relaxant.Followingextubationpatientwasdrowsyanddisorientedhence observed in intensive care unit. Overthe next 12 hourspatienthadfourgeneralisedseizuresdespitecontinuingintramuscular magnesium sulphate. Blood pressure rangedfrom 160 to 200 mm Hg systolic and 110 to 130 mm Hg diastolicwith heart rate between 120 to 130 per minute. At this timeoliguria and tender hepatomegaly was noticed. Because ofpersistent seizures and hypertension a continuous infusionof magnesium sulphate 1 gm/hour and labetalol 20 mg/hourwasstartedandmonitoredbykneejerkresponse.Repeatinvestigations after 24 hours revealed haemoglobin 6.8 gm%,platelets count 86,000/mm3, prothrombin time (PT) 07 secondsand partial thromboplastin time (PTT) was 08 seconds morethancontrolvalues.Thebloodureawas60mg%,serumcreatinine 1.9 mg %, serum bilirubin 3.4 mg %, ALT 1040 unitsper litre, AST 646 units per litre, lactate dehydrogenase (LDH)658 units per litre and peripheral smear showed evidence ofhaemolysis. Antiphospholipid antibodies were negative. Bothinfusions were continued for 36 hours after last seizure. Atotal of 260 mg of labetalol was used. Blood and fresh frozenplasma was transfused appropriately. Over the next two dayspatient improved clinically, however laboratory parameterstook10daystoreturntopreoperativevalues.On12thdaypatient was discharged home uneventfully.Case Report-2A 26 year old lady with twin pregnancy presented at 33weeks of gestation, with intra-uterine death of one foetus atHELLP Syndrome : Report of Two CasesWg Cdr RM Sharma*, Wg Cdr GS Sandhu+, VSMMJAFI 2006; 62 : 373-374Key Words: HELLP syndrome; PreeclampsiaMJAFI, Vol. 62, No. 4, 2006374 SharmaandSandhuthetimeofadmissionandwastakenupforemergencycaesareanundersubarachnoidblock.Onexaminationshewas drowsy, pulse 120 per minute, blood pressure 100/56 mmHg, had pedal and sacral oedema . Preoperative investigationsshowed haemoglobin of 11.2gm %, platelet count 2,10,000/mm3, blood urea 24 mg %, serum creatinine 0.8 mg %, serumbilirubin 3.0 mg%, ALT 184 units per litre, AST 158 units perlitre. After adequate preloading, 12 mg of bupivacaine wasinjectedintrathecally.Boththebabiesdeliveredwerestillbornandtheintraoperativecoursewasuneventful.Inthepostoperative period renal function deteriorated with bloodurea of 36 mg % and serum creatinine 1.8mg %. There wasevidence of haemolysis on peripheral blood smear and LDHwas raised to 1110 units perlitre. Coagulation profile wasderangedwithPT12secsandPTT23secsmorethanthecontrol values and platelets count fell to 1,95,000/mm3. Onfirst postoperative day, patient developed tachycardia (heartrate 130-140/min) and hypertension (170/100 mm Hg) whichwas treated with oral atenolol. With supportive care patientrecovered and was discharged after 2 weeks.DiscussionHELLPisamulti-systemdisease,resultingingeneralisedvasospasm,microthrombiformationandcoagulation defects [3]. The syndrome seems to be thefinal manifestation of insult that leads to micro vascularendothelialdamageandintravascularplateletaggregation.Significantsymptomsandsignsinanypatientwithpreeclampsiaincludeheadache,blurredvision, altered consciousness, clonus, increasing serumcreatininelevel,consumptivecoagulopathywiththrombocytopenia, and abnormal liver function tests [4].Both our patients had altered consciousness, possibilyan early but subtle sign of developing HELLP syndrome.The HELLP syndrome occurs in 4-18% of patientswith preeclampsia. Upto 30% patients develop HELLPsyndrome after parturition, typically appearing within 48hours. In fact, there may be no evidence of preeclampsiabeforeorduringlabourin20%ofcases.Theserumtransaminase levels may be elevated and platelet countscan drop to as low as 6000/mm3. Platelet count is thebestindicatorofHELLP.Progressiveisolatedthrombocytopenia may be one of the first clues to thediagnosis[5].Boththeabovementionedpatientsdeveloped renal dysfunction, consumptive coagulopathyand thrombocytopenia. Excessive fluid overload duringanaestheticmanagementcanresultincerebralorpulmonary oedema. A positive D-dimer test in the settingofpreeclampsiahasrecentlybeenreportedtobepredictive of patients who will develop HELLP syndrome[6].The laboratory abnormalities typically worsen afterdeliveryandpeakat24to48hourspostpartumandresolve by three to four days [7]. Patients with HELLPsyndromeshouldbetreatedprophylacticallywithmagnesiumsulphatetopreventseizures.InIntensiveCareUnit(ICU)settingmagnesiumsulphateandlabetalol can be used as an intravenous infusion for thetreatmentofseizuresandhypertension.Thisalsoreduces the risk of maternal cerebral haemorrhage. Themainstayoftherapyissupportivemanagementanddelivery is the definitive treatment. These patients maydevelopplacentalabruptionandrequireemergencycaesareansection.Althoughhypertensionisnotacontradiction to regional anaesthesia [8] but patients withaltered consciousness and platelet counts of less than70,000/mm3shouldnotbegivenspinalorepiduralanaesthesia.Ahighdegreeofsuspicionshouldbemaintained whenever patients with preeclampsia presentwithnonspecificfeatures.Thefirstpatientdevelopedeclampsiadespiteprophylacticmagnesiumsulphatetherapy.Thecauseforfoetaldeathinsecondpatientcould be severe coagulopathy and haemolysis. Womenwith a history of HELLP syndrome are considered tobeatincreasedriskforcomplicationsinfuturepregnancies.Conflicts of InterestNone identifiedReferences1. Weinstein L. Syndrome of haemolysis, elevated liver enzymesand low platelet count: a severe consequence of hypertensionin pregnancy. Am J Obstet Gynecol 1982; 142:159-67.2. Sibai BM. Treatment of hypertension in pregnant woman. NEng J Med 1996; 335:257-60.3. Sibai BM. The HELLP syndrome (haemolysis, elevated liverenzymes,andlowplatelets):muchadoaboutnothing. AmJObstet Gynecol 1990; 162:311-6.4. LapinskySE,KruczynskiK,SlutskyAS.Criticalcareinpregnant patient. Am J Respir Crit Care Med 1995; 152: 427-30.5. Magann EF, Perry KG, Meydrech EF, Harris RL, Chauhan SP,MartinJN.Postpartumcorticosteroids:acceleratedrecoveryfrom the syndrome of haemolysis, elevated liver enzymes, andlow platelets (HELLP). Am J Obstet Gynecol 1994; 171: 1154-8.6. Neiger R, Trofatter MO, Trofatter KF. D-dimer test for earlydetection of HELLP syndrome. South Med J 1995; 88: 416-9.7. Martin JN, Blake PG, Perry KG, McCaul JF, Hess LW, MartinRW.ThenaturalhistoryofHELLPsyndrome:patternsofdisease progression and regression. Am J Obstet Gynecol 1991;164: 1500-9.8. HoodDD,CurryR.Spinalversusepiduralanaesthesiaforcaesarean section in severely eclamptic patients: a retrospectivesurvey. Anaesthesiology 1999; 90: 1276-82.


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