Twin pregnancy with HELLP syndrome complicated with acute renal failure for emergency cesarean section: An unusual case and its anesthetic management.

Acute renal failure is not common in pregnancy. However, the incidence rises when pregnancy is complicated with Hemolysis, Elevated Liver enzymes, Low Platelets (HELLP) syndrome, which itself is a rare occurrence. We had an unusual case of HELLP syndrome in twin pregnancy with deranged renal profile for emergency cesarean section. We report the case, its anesthetic management for emergency cesarean section, and perioperative supportive treatment for acute renal failure.

INTRODUCTION

Hemolysis, Elevated Liver enzymes, Low Platelets (HELLP), the acronym, was coined by Weinstein, who described 29 patients with HELLP and argued that this was a distinct entity.[1] The incidence of HELLP among patients with eclampsia ranges from 4 to 14%, and is generally associated with poor maternal and perinatal outcome.[2]

Acute renal failure (ARF) is a rare complication of preeclampsia–eclampsia, occurring in only 1-2% of the patients.[3] However, the incidence of ARF when pregnancies are complicated by the HELLP syndrome rises to about 7.4%.[4]

We hereby present a case and the anesthetic management of HELLP syndrome in an eclamptic primigravida with twin pregnancy complicated with deranged renal function for emergency cesarean section.

CASE REPORT

A 22-year-old primigravida with 36 weeks twin pregnancy came to our hospital with convulsions. There was a history of generalized tonic clonic convulsions, first episode at home, second during transport, and third in casualty. There was one episode of vomiting too (no hematemesis). She was in post-eclamptic phase when first attended. Her antenatal care checkup card from a private hospital at her native place showed normal progress of pregnancy and normal blood pressure recordings. When examined, she was drowsy but conscious, pale, mildly icteric, and had pitting pedal edema extending up to knees. Her pulse was 134/min, regular, BP 150/100 mm of Hg, and cardiovascular examination revealed normal heart sounds with a hemic murmur. Rest of the systemic examination was normal. She was administered 5 g magnesium sulfate (MgSO4) as intravenous bolus followed by 5 g in each buttock. Her significant lab findings were: Hb 7.3 g%, total leukocyte count 17,840/mm3, platelets 40,000/mm3, peripheral smear examination showed hypochromasia, and dimorphic cells, schistocytes, and tear drop cells, increased reticulocyte count, urea 90 mg%, creatinine 1.86 mg%, total bilirubin 1.83 mg%, direct bilirubin 1.06 mg%, prothrombin time 18.23 seconds international normalized ratio 1.41, serum aspartate transaminase 109 IU/L, alanine transaminase 110 IU/L, alkaline phosphatase 182 IU/L, lactate dehydrogenase (LDH) 1542 IU/L, total proteins 4.40 g%, albumin 2.23 g%, and normal electrolytes. Urine examination revealed proteinuria ++. She was HIV nonreactive, and hepatitis B and C negative. Abdominal sonography revealed normal liver and spleen, but kidneys showed increased echogenicity. Ocular fundoscopy showed normal disc. Obstetric examination revealed full-term twin pregnancy with normal fetal heart sounds.

Based on these investigations, she was diagnosed as having HELLP syndrome with deranged renal profile. She was promptly catheterized to monitor the urine output.

An emergency cesarean section was planned for this patient in view of eclampsia complicated with HELLP with twin pregnancy. Preoperatively she received aspiration prophylaxis and was administered intramuscular MgSO4 and oral nifedipine. Standard monitoring with cardioscope, non-invasive blood pressure, pulse oximetry, capnometry, and respiratory gases monitoring was carried out. In addition, peripheral nerve stimulator (PNS) and urine output monitoring were also included. We also placed a central line via right cephalic vein to monitor central venous pressure (CVP). Internal jugular vein was not opted for CVP monitoring due to deranged coagulation profile. Baseline ECG showed sinus tachycardia with heart rate 120/min and BP was 140/90 mm of Hg pre-induction. Difficult intubation cart and appropriate drugs like MgSO4 and phenytoin were kept ready. Patient was induced with rapid sequence induction using IV thiopentone and succinylcholine and was successfully intubated with size 7 cuffed endotracheal tube. Anesthesia was maintained with nitrous oxide-oxygen–isoflurane mixture, and neuromuscular blockade was monitored with PNS. Both the babies were successfully delivered and had favorable APGAR scores. Midazolam 1 mg and fentanyl 60 mcg were administered after baby delivery. CVP was maintained around 6-7 cm of H2O throughout the surgery. The patient did not require any additional muscle relaxation; however, atracurium was kept ready. Anesthesia was tapered at completion of surgery, and extubation was done after patient regained consciousness, and had spontaneous breathing and adequate tone as evidenced by PNS. The total blood loss was 1.5 L. She received 1 whole blood and two crystalloids intraoperatively; urine output was 50 mL. She was shifted postoperatively to intensive care unit for observation and monitoring.

Postoperatively, nifedipine 10 mg BD and MgSO4 in a dose of 5 g intramuscularly were continued. Her urine examination showed borderline oliguria (urine output 450 mL over 24 h) and proteinuria ++ Blood urea nitrogen and creatinine values were also on the higher side for the first 2 days postoperatively (BUN 105 mg%, creatinine 2.01 mg%). She was referred to nephrologist with a presumptive diagnosis of ARF, who advised dialysis after confirming rising trends of creatinine. Diuretics, the first line of treatment for oliguric ARF, were withheld owing to the already contracted blood volume in a patient with preeclampsia–eclampsia. However, patient showed improvement with intravenous fluid challenge, and on third postoperative day, the renal parameters were approaching normal values (BUN 58 mg%, creatinine 1.6 mg%). On the fourth day, her Hb was 10.2 g%, platelets 80,000/mm3, with normal proteins and liver and renal profile. She had no convulsion in the perioperative period or during her stay in the ICU. MgSO4 was omitted from the fourth day; however, nifedipine was continued. Dialysis was withheld by the nephrologist due to her favorable renal profile. She was shifted to ward and later on discharged after complete recovery.

DISCUSSION

Our patient satisfied all the criteria of HELLP syndrome. She had hemolysis [as evidenced by anemia, increased reticulocyte count, presence of schistocytes], raised bilirubin and LDH, elevated liver enzymes, and low platelets.[15] She also had ARF which was more likely to be of pre-renal/renal origin. ARF is not a component of HELLP, but is a well-recognized complication. The incidence of ARF increases when pregnancies are complicated by HELLP syndrome. Obstetric causes of pregnancy-related ARF are septic abortion, abruptio placentae, and prolonged intrauterine fetal death, while non-obstetric causes are snake bite, enterocolitis, pancreatitis, sepsis, etc., More than 50% of cases are due to obstetric cause, most of them occurring during their third trimester as a consequence of abruptio placentae or HELLP syndrome.[6] We ascribe our case to the latter since she had all the components of HELLP. Sibai and Ramadan reported highest incidence of ARF when pregnancies are complicated by HELLP (7.4%).[34] In their series, 31 out of 32 patients needed blood transfusion and 18 needed platelet transfusion. Our patient received blood transfusion, but there was no indication for platelet transfusion. Weinstein reported one case of renal cortical necrosis (RCN) among 300 patients with HELLP syndrome. RCN is diagnosed on the basis of anuria, calcification of kidney tissue, necrosis of glomeruli on renal biopsy, and non-recovery of renal function even post dialysis. The prognosis is poor in pre-renal ARF due to RCN.[7] Renal biopsy was neither performed nor advised in our patient as she showed gradual recovery of renal functions.

Management of eclamptic patient is itself a challenge for the anesthesiologist, and when eclampsia is complicated with HELLP with further complication of ARF, the challenge increases several fold. In HELLP syndrome, the anesthesiologist has not only to manage and prevent convulsions, but also to concentrate on each and every component of HELLP and its subsequent implications.[8] These are anemia which means low oxygen-carrying capacity in an already insulted nervous system prone to hypoxia, requirement and availability of blood, deranged liver functions affecting coagulation and metabolism of most of the anesthetic drugs, and low platelets implying more bleeding and relatively contraindicating central neuraxial block in such patients.[9] When such pregnant patients are complicated by ARF, although rarely, they may land up into end-stage renal disease if not diagnosed or treated promptly. Our patient is the only reported case of preeclampsia–eclampsia complicated with HELLP and ARF in twin pregnancy.

HELLP syndrome is a rapidly progressive disorder and its diagnosis calls for immediate delivery regardless of gestation if maternal and fetal deaths are to avoided.[10] Hence, an emergency cesarean section was planned for our patient. The upper airway and laryngeal edema is aggravated with preeclampsia–eclampsia and may pose problems during mask ventilation and intubation. Anesthesiologist must be prepared in all ways to manage difficult airway. The respiratory system is not much affected in preeclampsia–eclampsia, but may be compromised more in multiple gestations as in our patient. The hemolysis in HELLP results in increased carboxyhemoglobin, causing a leftward shift of oxygen hemoglobin dissociation curve. MgSO4 used to treat eclampsia and anesthetic drugs can cause respiratory depression.[9] All these factors make the patient more prone to hypoxia and hypercarbia, affecting not only the mother but also the fetus. Moreover, MgSO4 interacts with non-depolarizing muscle relaxants, and hence should be used cautiously. Neuromuscular monitoring is desirable in patients receiving MgSO4, who are administered muscle relaxants.

Hepatic involvement in HELLP syndrome is in the form of elevated liver enzymes. There may be periportal hemorrhages, ischemic lesions, and subcapsular hematoma in the liver.[11] Deranged liver functions and hypoproteinemia hamper drug metabolism and dose, respectively. Hence, all the drugs must be administered carefully. Moreover, hypoproteinemia makes the patient prone to cerebral and pulmonary edema.[34] Therefore, intravenous fluids should be given vigilantly. There may also be an increase in intracranial pressure in patients with eclampsia that may be exacerbated by hypercarbia, metabolic acidosis, and hypoxia. All these points must be taken into consideration and appropriate care should be taken to avoid the same. We used thiopentone and succinylcholine for intubation, which are the drugs of choice for eclamptic patient on MgSO4. Utmost care was taken and vigilant monitoring was done to avoid any kind of hypoxia and hypercarbia.

Renal function is adversely affected in preeclampsia–eclampsia. Renal blood flow decreases, proteinuria occurs, and blood uric acid level increases which generally correlates with the severity of the disease.[12] Significant reduction in renal perfusion may occur in HELLP due to vasospasm, microvascular injury, intravascular coagulation, or a combination of these factors.[34] When renal failure occurs in antepartum period with HELLP, rapid resolution generally follows expeditious delivery of fetus. However, this renal failure should be seriously dealt with as it can behave in extremes of acute tubular necrosis on one side and severe renal cortical necrosis on the other side, adversely affecting the outcome.[13] Patient needs to be judiciously hydrated in case of deranged renal function as oliguria and renal failure may occur in the absence of hypovolemia and excess hydration may lead to pulmonary edema. Hence, patients with renal failure who do not respond to modest fluid therapy should be further investigated for correct diagnosis and timely treatment. The use of diuretics is also discouraged as diuresis occurs at the expense of already contracted blood volume. Our patient had deranged renal profile which was probably due to acute tubular necrosis that recovered with hydration without any need for dialysis or diuretics.

Thus, our patient was adequately evaluated and diagnosed beforehand which led to a favorable outcome. By presenting this case we want to emphasize the aims of therapy in preeclampsia–eclampsia complicated with HELLP and ARF. These aims are to minimize vasospasm by avoiding hypoxia and hypercarbia, to improve circulation not only to uterus and placenta but also to kidneys, to improve intravascular volume; yet to avoid fluid overload, and finally to decrease both CNS and reflex hyperactivity. Our case highlights the importance of proper preoperative evaluation of preeclampsia–eclampsia patients to diagnose HELLP syndrome and identify ARF, and shows how these patients can be appropriately managed.