Intensive Care Management For Patients With The Syndrome Of Hemolysis Elevated Liver Enzymes And Low Platelets (HELLP Syndrome)
M Takrouri, M Seraj, J Baaj, M Jasser, R Satli, A Turkustani, S Gourab, A Al-Jabari, A AL-Meshari
anaesthesia, and management, complications, hellp syndrome, intensive care, obstetrical
M Takrouri, M Seraj, J Baaj, M Jasser, R Satli, A Turkustani, S Gourab, A Al-Jabari, A AL-Meshari. Intensive Care Management For Patients With The Syndrome Of Hemolysis Elevated Liver Enzymes And Low Platelets (HELLP Syndrome). The Internet Journal of Emergency and Intensive Care Medicine. 2003 Volume 7 Number 2.
Since the description of the syndrome of hemolysis, elevated liver enzymes and low platelets as clinical entity in 1952  and then coining the acronym (HELLP) in 1982 , many reports appeared in obstetrical medical literature describing and giving better understanding of its pathophysiology and management. Few reports appeared describing the intensive care unit (ICU) course of HELLP patients. Recently a report appeared describing 13 cases treated in ICU  and another report appeared evaluating the value of intensive care management of cases of HELLP syndrome. The objective of this paper is to present the local intensive care experience with patients admitted with the diagnosis of HELLP syndrome at King Khalid University Hospital in Riyadh KSA.
Patients and method
All pregnant patients admitted to ICU and considered having HELLP syndrome at the King Khalid University Hospital in Riyadh were included. The period of the study extended from June 1996- June 2001. The obstetrical patient was defined as any patient who was pregnant or up to one month postpartum. The diagnosis of HELLP syndrome required the presence of thrombocytopenia (perinatal platelet count 150,000/µL), evidence of hepatic dysfunction (increased aspartate aminotransferase level of 40 IU/L, increased alanine aminotransferase level of 40 IU/L, or both, with increased lactate dehydrogenase [LDH] level of 600 IU/L), and evidence of hemolysis (increased LDH level, hyperbilerubinemia, progressive anemia), usually in association with hypertension or proteinuria considered to represent preeclampsia or eclampsia. Local hospital laboratory identified the thresholds for LDH and transaminase level abnormalities as the upper limits of normal pregnancy. The records were reviewed retrospectively. The following data were obtained: maternal age, nationality, and presence of co-existing diseases. The clinical history, the intensive care indication and the diagnosis for admission were determined and were recorded. The investigations of blood platelets count, Liver function tests, the blood sugar and other routine investigation were reviewed. Total days in the intensive care unit were calculated.
The need of monitoring mechanical ventilation and invasive monitoring or dialysis was recorded. Morbidity and mortality were recorded.
Numerical data are presented in mean and standard deviation, with student
Epidemiological and clinical profile data
16 patients diagnosed with HELLP syndrome (Class II: Three criteria of diagnosis were present), based on the number of abnormalities that are present. All patients were admitted to ICU during the study period. All patients were Saudi (n=16 100%). Age (y) Mean (s.d.) 31(7) y Range (21-43) y. Gestation age (w) Mean (s.d.): 31(3) w. Range (24-37 w). Multipara were 15/16 (94%) two had had previous spontaneous abortions. One patient had previously delivered a stillborn at (28) wk gestation; ten of the multigravida patients had previously delivered a live born, term infant. Only one patient has less than 34 wk gestational age. One patient had experienced toxemia during a previous pregnancy. Patients who delivered through lower uterine segment caesarean section (LUSCS), under general anesthesia, were 13/16 (81%) the rest three patients had vaginal delivery. No patient received epidural anesthesia for LUSCS. No patients delivered with forceps. Abnormal bleeding was noticed in nine patients (56.25.%) undergoing LUSCS, wound haematoma developed in 2 (12.5%) of them. No patient had postpartum hemorrhage. In one patient a placental abruption and impending uterine rupture was seen at LUSCS.
In one patient an arterial line a central line had been inserted prior to induction of anesthesia due to antenatal hemorrhage and hypotension, to monitor fluid therapy. No other invasive monitors were used. Four patients (25%) received intravenous xylocaine to blunt the hypertensive response to tracheal intubation. All patients underwent rapid sequence induction of anesthesia, with cricoid's pressure, no intubation was deemed to be difficult and the tracheas of six patients were extubated in the operating room at the end of the surgery. There were no complications related to the anesthetic.
There were six cases of pre-eclampsia eclampsia: 6/16 (36.7%) representing 27/100000 or 0.0276% of obstetrical patients. There were total of 21665 deliveries at KKUH during the study period. This indicates the incidence of HELLP syndrome needed ICU admission mounting to 74/100000. This represented 0.0738% of the total obstetric deliveries during the period. During that period, the incidence of hypertensive disease of pregnancy-needed admission to ICU was 107/100000. There were three cases of stillbirth: 3/16 (19%). One case of twin delivery: 1/16 (6%).
The signs, symptoms at the time of admission are summarized in (Table 1).
These are related to clinical identity HELLP syndrome including clinical picture and positive liver enzymes laboratory tests (Fig 1).
Blood pressures were highly variable and did not show tendency to be high. 11 patients were within normal range of blood pressure, one patient was hypotensive because of bleeding and four patients had elevated either the systolic, the diastolic blood pressure or both. (Fig 2), the degree of thrombocytopaenia.(Fig 3 . Epigastric and right upper quadrant (RUQ) pain were prominent presenting complaints and in 11 patients (68.75%).
P=0.0001 statistically significant difference.
The diagnosis of pre-eclampsia complicated by HELLP syndrome was delayed in six patients (37%). The patients were diagnosed initially a nonspecific gastrointestinal pain were five patients (31.25%). Two patients (12.5%) had convulsions before delivery and one patient complained of visual impairment and was found to have retinal detachments due to sub retinal bleeding which followed up for one year in the ophthalmic clinic.
Coagulation abnormalities on admission to intensive care
Low platelets. Deranged liver function and fibrin degradation product were evident in all patients. (FDP) were noted in the serum of all the patients before and during delivery.
Hematological operative and intensive care management
One patient was transfused with platelets among blood transfusion of 16 packed cells units) platelets count was 38 X 100 L-1. In other patients there was either no increase or a decrease in subsequent platelet count (PCT) and in the other patient there was an increase in PCT averaging 44 X 109 L-1. Two patients (12.5%) received transfusions of packed red cells peri-operatively, and three patients (18.75%) in the postpartum period for low hemoglobin concentration at range of (66-96 g L-1), one during surgery and one in the recovery room because of intraoperative blood loss. (Two to four) units were transfused to each patient, with the mean transfusion requirement being three units. One patient was given transfusion of six units of FFP intra-operatively because of persistent oozing.
17 neonates were delivered. There was (3) stillbirth at 36 wk gestation (a recognized intrauterine death) and (one) patient delivered twins at (37) wk gestation. Three infants, at (27, 28 and 34) wk gestational ages, were transferred to neonatal intensive care facility for specialized care. There were no neonatal death and neonatal morbidity was related primarily to prematurity rather than to the maternal condition at the time of birth.
Intensive care postpartum course
After delivery, blood pressure returned to normal, in most patients, by the third or fourth postpartum day. One patient was discharged on anti-hypertensive therapy. The platelet count continued to decrease for few hours after delivery and then began to increase to normal over few days. The time required for the platelet count to return to levels above 100 X 109 W L-1 ranged from (2 to 7) days, with the majority of patients having achieved such levels by the third to fourth day. Two patients developed postpartum DIC, were treated with blood component infusions and the coagulopathy resolved. The elevated liver enzyme concentrations began to decrease within (12) hr of delivery and had returned to normal in most patients before discharge from the intensive care unit.
There were no maternal deaths attributable to HELLP syndrome.
HELLP syndrome occurs in approximately 0.2 to 0.6 percent of all pregnancies. In comparison, pre-eclampsia occurs in 5 to 7 percent of pregnancies. Superimposed HELLP syndrome develops in 4 to 12 percent of women with pre-eclampsia or eclampsia. When pre-eclampsia is not present, diagnosis of the syndrome is often delayed.
Maternal mortality was as high as 66% in early reports  In a report on 112 patients with HELLP syndrome; two deaths were recorded (1.8% maternal mortality). There was no maternal death in our series. Reported perinatal mortality rates have been high as 367 per 1000 deliveries with 22 stillbirths and 16 neonatal deaths. Although our series number is modest there were only three stillbirths, and no neonatal deaths, our corrected perinatal mortality was 29 per 1000 deliveries. In other series 41% percent of the infants were born at <30 wk gestational age compared with three (18.75)% of the infants in our group and this probably accounts for no mortality in our cases.
Delayed or missed diagnosis has been a common occurrence in reports of HELLP syndrome. This has been attributed to the unusual features of presentation, with impressive abdominal pain, nausea and vomiting. As well, the classical signs of pre-eclampsia (hypertension, proteinuria and oedema) may be unimpressive. In our series, ten patients (62.5%) that presented with epigastric or RUQ pain were initially diagnosed as having a condition other than pre-eclampsia with HELLP syndrome (missed diagnosis). This has obvious implications for the anesthetist engaged in obstetrical anesthesia practice in that patients may present for anesthetic care without a correct diagnosis having been made. One patient in our series diagnosed on table as impending uterine rupture and HELLP syndrome.
The incidence of DIC of 38%; 21% of patients were reported. [7,8,9,10]. and demonstrated prolonged prothrombin and partial thromboplastin times compared with 9% and 12%, respectively, the reported patients had placental abruption and 19% had in utero fetal death and the high incidence of coagulopathy may have been influenced by these abnormalities. There was three in utero death (18.75%) in our series and one case of placental abruption. The infusion of platelet concentrates into thrombocytopenic patients without clinical evidence of bleeding is controversial . The pathophysiological process in HELLP syndrome is one of accelerated platelet consumption and the transfused platelets are likely to be consumed as quickly as native platelets, providing no therapeutic advantage. Also, there is some concern that the consumptive process may be accelerated and DIC may result [11,12,13]. In one patient who transfused with platelet concentrates preoperatively, the platelet count was 38 X 100 L-1. There appears to be little reason to delay operation to provide platelet transfusions in patients with HELLP syndrome in the absence of clinical bleeding. However, because of the high, although variable, reported incidence of DIC, the availability of cross-matched blood, platelet concentrates and plasma should be assured before taking the patient to the operating room.
Invasive monitoring was utilized in the management of one patient in our series. HELLP Syndrome, by itself, is not an indication for invasive monitoring and it is the severity of the coexisting state that will determine the need. An arterial line is useful in the parturient that need repeated blood sampling, which is receiving potent vasodilators for control of blood pressure or in those patients whose blood pressure is controlled poorly. Pre-eclamptic women have smaller plasma volumes than normal pregnant women but this appears to be especially important in women with severe disease or with growth retarded fetuses (IUGR). Use of invasive central monitoring should reflect this and so, in woman with mild to moderate pre-eclampsia and normally growing fetus, there is little indication for invasive central monitoring. However, in severe pre-eclampsia, and especially if complicated by oliguria, pulmonary edema or underlying cardiac pathology, a role for central venous monitoring has been established. The pulmonary artery catheter should be used to guide therapy of women with severe unresponsive hypertension, oliguria resistant to fluid therapy with appropriate CVP or signs and symptoms of pulmonary edema. The role of the Intensivist is to follow up the management of the obstetrician and anesthesiologist dealing with the case. For patients with known or suspected HELLP syndrome presenting for Caesarean section, preoperative assessment must include a recent complete blood count with platelet count, liver function tests, a prothrombin and partial thromboplastin time and fibrin degradation products. Blood components including cross-matched red cells, platelet concentrates and plasma should be immediately available.
Premature delivery is usual in HELLP syndrome. The pregnancies are complicated by intrauterine growth, retardation and placental abruption. The presence of a neonatalogist or a pediatrician waiting, in order to care for the neonate, and to prepare equipment and drugs to resuscitate the neonate are mandatory.
Finally, the patient should be monitored to detect the development of postpartum hemorrhagic complications, DIC and eclampsia. In most patients, platelet count, blood pressure and liver function should return to normal by the fourth postoperative day. [Fig 6]
Provision must be made, immediately after establishing the diagnosis, for both the care of the mother and a premature, often growth-retarded, infant. Maternal and perinatal morbidity is likely to be higher than normal and the challenge to the perinatal team (obstetrician, anesthetist, intensivist and pediatrician) is to cooperate in order to optimize care and reduce both mortality and morbidity.
M. S. M. Takrouri, Department of Anaesthesia, College of Medicine, King Saud University, P. O. Box. 2925. Riyadh, 11461 Kingdom of Saudi Arabia. Tel.: 009661- 4671595. Fax. 009661-4679364. E-mail: firstname.lastname@example.org & email@example.com