Spontaneous Liver Hematoma and a Hepatic Rupture in HELLP ...

Surg Today (2002) 32:450–453

Spontaneous Liver Hematoma and a Hepatic Rupture in HELLP Syndrome: Report of Two Cases . Mustafa Aldemir, Bilsel Baç, I brahim Taçyıldız, Yusuf Yag˘mur, and Celalettin Keles¸ Department of General Surgery, Faculty of Medicine, Dicle University, Diyarbakır, Turkey

Abstract Subcapsular liver hematomas and ruptures are unusual fatal complications of HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome (HS). We present two cases of a spontaneous rupture of subcapsular liver hematoma occurring in HS and review the literature on this subjects. One case demonstrated a secondary rupture of a subcapsulary liver hematoma due to HS in one patient and HS associated with preeclampsia in another. The defects were on the medial and lateral sectors of the left lobe in one patient and on the medial sector of the right lobe in the other patient. In case 1 deep mattress sutures and omentoplasty were performed, and in the other case a defective area was closed with an absorbable gelatin sponge with a hemostatic effect. In addition, the liver was compressed by abdominal towels. A high index of suspicion and immediate recognition are keys to proper diagnosis and management of affected patients. The multidisciplinary approach to the management of these patients led to a remarkable decrease in the mortality rates. Less aggressive treatment is preferable to aggressive intervention such as a hepatic resection in such patients with coagulopathy.

In addition to more common gestotic symptoms, such as edema, proteinuria, and hypertension, the clinical picture is characterized by microangiopathic hemolysis, thrombocytopenia and, especially, impaired hepatic function. Within this clinical picture severe complications can occur, such as eclamptic attacks, renal dysfunction, intracranial hemorrhage, intrahepatic hemorrhage, and coagulopathy. The HELLP syndrome (HS) may cause subcapsular liver hematomas. When a hepatic rupture occurs, the mortality of mother and unborn is high. A spontaneous rupture of a subcapsular liver hematoma in pregnancy is a rare and potentially life-threatening complication of preeclampsia. The incidence is approximately 1 in 45 000 live births. A liver hematoma is often not suspected until it ruptures.2–6 We present our experience with two cases of a spontaneous rupture of subcapsular liver hematoma occurring in association with the HS and review the literature on this subject.

Key words HELLP syndrome · Subcapsular liver hematoma · Rupture · Pregnancy

A 35-year-old multigravida was admitted in the 35th week of gestation because of HS. She complained of severe left upper quadrant and left shoulder pain with no previous trauma history. During the initial examination, the patient had hypertension (150/110 mmHg) and pleural effusion. Laboratory evaluations showed proteinuria (2⫹), hypoalbuminemia, hematuria, anemia, and low platelet count (48 000/µl), high serum alanine aminotransferase (ALT ⫽ 486 IU/l), high aspartate aminotransferase (AST ⫽ 309 IU/l), high lactic dehydrogenase (LDH ⫽ 595 IU/l), and high unconjugated bilirubin. Ultrasonography showed a large liver with structural irregularities and free fluid in the abdominal cavity (Fig. 1). The abdomen was mildly tender without

Introduction HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count) was first referred to by Weinstein in 1982 as an extremely progressive form of gestosis.1 Reprint requests to: M. Aldemir, Dicle Universitesi, Tıp . Fakültesi, Ilk ve Acıl Yardim Abd., 21280 Diyarbakır, Turkey Received: May 8, 2001 / Accepted: November 20, 2001

Case Reports Case 1

M. Aldemir et al.: Hepatic Rupture in HELLP Syndrome

451

put had decreased. The abdomen was diffusely tender with right upper quadrant rebound. Coagulation studies were normal. The hematocrit fell from 35% to 24%. Blood transfusions were started and the patient was taken immediately to the operating room. An exploratory laparatomy revealed hemoperitoneum (1000 cc bleeding) and a large subcapsular hematoma (about 10 ⫻ 8 cm) on the diaphragmatic surface of the right liver combined with a 7-cm laceration. The defect of liver capsule was closed with an absorbable gelatin sponge with a hemostatic effect. In addition, the liver was compressed by abdominal towels which were removed 3 days later. A cesarean section and bilateral tubal ligation were also performed by gynecologists, and the baby was safely delivered without any complications postoperatively. The postoperative course was uneventful and the patient was discharged on the tenth day. Fig. 1. Ultrasonographic view of the subcapsular liver hematoma of case 1 (arrows)

rebound. On the fifth day of admission, tachycardia and hypotension appeared and the patient was immediately taken to the operating room. A Phannenstiel incision was performed for a cesarean section and a large amount of blood (about 750 cc) was noticed in the abdomen. The baby was successfully delivered and had no problems. A midline laparatomy was immediately performed. On the diaphragmatic surface of the medial and lateral sector of the left lobe of the liver, a subcapsular hematoma (10 ⫻ 10 cm) combined with an 8-cm laceration was found. Hepatorrhaphy was done with deep mattress sutures and omentoplasty. Two suction drains were placed in the left subdiaphragmatic area and removed on the third postoperative day. One unit of fresh frozen plasma and 3 units of whole blood were transfused during operation. Postoperatively, the patient was administered parenteral and enteral nutrition, and the serum albumin level increased. Apart from mild pleural effusion no complication was noticed, and the patient was discharged on the ninth postoperative day. Case 2 A 32-year-old multigravida was admitted in the 34th week of gestation because of preeclempsia and HS. The patient had hypertension (200/120 mmHg), proteinuria, hypoalbuminemia, hemolytic anemia, and low platelet count (55 000/µl), high serum ALT (612 IU/l) and AST (623 IU/l). The patient suddenly suffered from severe epigastric pain, nausea, and vomiting while taking magnesium sulfate and methyldopa medication for preeclempsia. At this time her blood pressure had fallen to 100/70 mmHg, heart rate was 120 bpm and urine out-

Discussion The median gestational age for HS is 32–34 weeks; however, HS may also develop during the early postpartum period.7,8 A spontaneous hepatic rupture in pregnancy is rare, and is often associated with significant maternal morbidity and mortality.9 The clinical cardinal symptom of the disease is right upper quadrant or epigastric pain accompanied with nausea, vomiting, and sudden hypotension without any obvious external blood loss. However, shoulder pain should also be regarded with suspicion in patients with preeclampsia.5,7,8,10 One of our patients was in her 34th and the other was at her 35th gestational week. One patient had sudden pain at the left upper quadrant and the shoulder without any trauma, and the other had epigastric pain and vomiting. A subcapsular hematoma was initially suspected in case 1 since she had sudden abdominal pain with defecation 3 days prior to her admission to the hospital. The secondary clinical symptoms were thought to be due to the Glisson sheath laceration. Early recognition and prompt surgical intervention are crucial to reduce the high fetal and maternal mortality rate associated with this disease.11 There has been, to date, neither a reliable early recognition nor effective prevention of HS.12 Pain in the right upper abdomen and signs of abdominal mass bleeding occurring within HS are important evidence for hepatic rupture. An immediate ultrasonic examination may verify the tentative diagnosis.13,14 Various modalities including a liver scan, computed tomography, ultrasonography, peritoneal tab, and arteriography have been used to confirm suspicions of subcapsular hematomas. However, in a typical patient the diagnosis can usually be made clinically and these confirmatory tests may delay an urgent laparatomy. The early recognition of hemolysis is most

452

sensitively managed by the determination of the serum haptoglobin. The increase in the AST and ALT levels often precedes a decrease in the platelet level. In 20% of the cases with HS there is no hypertension and 5%– 15% of the pregnant patients present a low level of proteinuria or none at all.7,8 While the compensated form of activation of intravascular coagulation, which is diagnosed by a decrease in antithrombin III and an increase in thrombinantithrombin III complex (TAT) and the appearance of fibrin, monomers, and D-dimers, is found in almost all cases of HS, the decompensated form of intravascular coagulation with prolonged bleeding time (prothrombin time, partial thromboplastin time) and drop in fibrinogen is found only in the most severe forms.15 The first case was hospitalized for an investigation of her symptoms and hematuria. She was diagnosed to have HS due to an elevation of the liver function tests, hemolytic anemia and thrombocytopenia being detected. At an ultrasonographic examination after the acute symptoms had resolved, massive fluid and constitutional irregularity of the medial and lateral sectors of left lobe were detected in the liver. In the second case, a diagnosis of HS was associated with preeclempsia. She underwent an urgent surgical operation as massive hemoperitoneum was shown on ultrasonography. In both patients liver function tests were abnormal, but coagulation studies were normal. The best prophylaxis against the development of life-threatening complications is an early diagnosis and termination of pregnancy after a stabilization of the maternal condition, consisting of magnesium sulfate infusion and antihypertensive treatment with dihydralazine or calcium antagonists, steroids, etc.15,16 Various new therapies which have been proposed to permit the prolongation of preterm pregnancy and fetal maturation include vasodilatation therapy using fluids and antihypertensives, and intensive corticosteroid therapy using intravenously administered high-dose dexamethasone, usually at a dose of 10 mg every 12 h.17 Our patients were not treated with steroids since fetal maturation had occurred, but magnesium sulfate and methyldopa were given to case 2 to treat preeclampsia. As a result of endothelial dysfunction, activation of intravascular coagulation occurs with fibrin deposition in the capillaries and consecutive microcirculation disorders.8 In the liver, sinusoidal obstruction causes vascular congestion, leading to an increase in the intrahepatic pressure, a dilatation of Glisson’s capsule, and the development of subcapsular hepatic hematomas and hepatic rupture.6 However, an imbalance between oxidative stress and maternal detoxification or antioxidant capacity may explain the symptoms of preeclampsia and HS.12 A rapid termination of pregnancy, either by vaginal delivery or by a cesarean section, is generally

M. Aldemir et al.: Hepatic Rupture in HELLP Syndrome

recommended when the fetal lung maturity is sufficient or when maternal complications appear. Conversely, conservative management is acceptable before 32 weeks. Corticosteroid therapy or hemodynamic therapy could allow the fetus to obtain lung maturity.8,18,19 The diagnosis of a subcapsular liver hematoma must lead to an urgent delivery through a cesarean section. Subcapsular liver hematomas do not, however, require operative treatment, unless they have ruptured. Packing of the ruptured liver with gauze and later removal of the gauze is preferable to lobectomy. In addition, collagen patches coated with fibrin glue components, abdominal packs, electrocoagulation, use of methacrylate, gel foam, surgical, and omental patches, as well as suture ligation of hepatic parenchyma may be applied in liver hemorrhage and rupture.9,14,20–22 In a recent report, Yotsumoto et al.23 treated a case of spontaneous subcapsular hepatic hemorrhage by transcatheter arterial embolization. In case 1, hepatorrhaphy with deep mattress sutures plus omental patches were applied to control the hemorrhage. In case 2, packing with an absorbable gelatin sponge and towels was performed. The pregnancies of both patients were terminated by cesarean section. The course of HS is incalculable. The maternal mortality reported from the international literature is 3.3%, and the perinatal mortality 22.6%.7,8 In hepatic hematomas, mortality is around 35%.6 Both the mothers and babies survived in our cases. HS may cause subcapsular liver hematomas. When a hepatic rupture occurs the mortality of the mother and unborn child is high. A liver rupture and hemorrhaging are the most unusual and serious complications of HS. A high index of suspicion and prompt recognition are keys to the proper diagnosis and management of affected patients. The management should be aggressive by treating coagulopathy and favoring a prompt delivery. A rupture remains a surgical emergency with a control of bleeding necessary, based on trauma principles. Major surgical interventions such as resections have to be withheld in patients with HS since their coagulometric tests and liver functions are impaired. An aggressive multidisciplinary approach to the management of these patients has led to a remarkable decrease in the mortality rates.

References 1. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982;142:159–67. 2. Yagmurdur MC, Agalar F, Daphan CE. Spontaneous hepatic rupture in pregnancy. Eur J Emerg Med 2000;7:75–6. 3. Castriconi M, Aragiusto G, Ansalone M, Aloj G, Grasso A, Bartone G, et al. Liver rupture in HELLP syndrome. Report of a case. Minerva Chir 2000;55:167–71.

M. Aldemir et al.: Hepatic Rupture in HELLP Syndrome 4. Sheikh RA, Yasmeen S, Pauly MP, Riegler JL. Spontaneous intrahepatic hemorrhage and hepatic rupture in the HELLP syndrome: four cases and a review. J Clin Gastroenterol 1999; 28:323–8. 5. Sherbahn R. Spontaneous ruptured subcapsular liver hematoma associated with pregnancy. A case report. J Reprod Med 1996; 41:125–8. 6. Raumanns J, Behrendt W, Lehnen H. Spontaneous liver rupture as a rare complication of the HELLP syndrome. Anaesthesist 1992;41:386–90. 7. Rath W, Loos W, Kuhn W. HELLP syndrome. Zentralbl Gynakol 1994;116:195–201. 8. Rath W, Faridi A, Dudenhausen JW. HELLP syndrome. J Perinat Med 2000;28:249–60. 9. Wijesinghe PS, Gunasekera PC, Sirisena J. Spontaneous hepatic rupture in pregnancy. Ceylon Med J 1998;43:109–11. 10. Vigil-De Gracia P. Pregnancy complicated by pre-eclampsiaeclampsia with HELLP syndrome. Int J Gynaecol Obstet 2001;72: 17–23. 11. Ralston SJ, Schwaitzberg SD. Liver hematoma and rupture in pregnancy. Semin Perinatol 1998;22:141–8. 12. Roes EM, Raijmakers MT, Zusterzeel PL, Knapen MC, Peters WH, Steegers EA. Deficient detoxifying capacity in the pathophysiology of preeclampsia. Med Hypotheses 2000;55:415–8. 13. Volz J, Volz E, Stoz F, Keckstein J. Spontaneous rupture of the liver in HELLP syndrome. Geburtshilfe Frauenheilkd 1992;52: 152–6.

453 14. Weemhoff RA, van Loon AJ, Aarnoudse JG. Liver rupture in pregnancy: a life-threatening complication of the HELLP syndrome. Ned Tijdschr Geneeskd 1996;140:2140–2. 15. Schneider H. Liver pathology within the scope of HELLP syndrome. Arch Gynecol Obstet 1994;255(suppl)2:S245–54. 16. Schlembach D, Munz W, Fischer T. Effect of corticosteroids on HELLP syndrome: a case report. J Perinat Med 2000;28:502–5. 17. Magann EF, Martin JN. Critical care of HELLP syndrome with corticosteroids. Am J Perinatol 2000;17:417–22. 18. Barton JR, Sibai BM. Care of the pregnancy complicated by HELLP syndrome. Gastroenterol Clin North Am 1992;21:937– 50. 19. Dreyfus M, Tissier I, Baldauf JJ, Ritter J. HELLP syndrome. Review and update. J Gynecol Obstet Biol Reprod 1997;26:9– 15. 20. Risseeuw JJ, de Vries JE, van Eyck J, Arabin B. Liver rupture postpartum associated with preeclampsia and HELLP syndrome. J Matern Fetal Med 1999;8:32–5. 21. Cerwenka H, Bacher H, Werkgartner G, El-Shabrawi A, Mischinger HJ. Massive liver haemorrhage and rupture caused by HELLP-syndrome treated by collagen fleeces coated with fibrin glue. Eur J Surg 1998;164:709–11. 22. Hakim-Elahi E. Spontaneous rupture of the liver in pregnancy. Obstet Gynecol 1976;26:435–40. 23. Yotsumoto G, Tanaka K, Ishizaki N, Ikoma A, Kawashima S, Taira A. Spontaneous subcapsular hepatic hemorrhage associated with pregnancy: report of a case. Surg Today 1997;27:657–60.