In case reports and case series, hepatic rupture associated with HELLP syndrome is seen primarily in older multiparous women. Araujo et al11 in 2005 presented a case series of 10 women with hepatic rupture and the majority of them were older than age 35 years and multiparous, with a maximum of 15 pregnancies. In our centers, the majority of women were younger than age 30 years and mostly primigravid. The cases reported by Araujo occurred in Brazil, where the sociocultural environment is quite different from that of Canada, which might explain this difference.
The diagnosis of hepatic rupture is difficult to make because symptoms are common and nonspecific. After a detailed literature review, Rinehart et al10 documented the most frequent signs and symptoms of hepatic rupture and hemorrhage. Epigastric pain was the most frequent with 70%, followed by hypertension (66%), shock (56%), nausea and vomiting (25%), shoulder pain (21%), and headache (11%). These findings reflect what we have observed in our cases: six of nine (67%) patients had epigastric pain as the presenting symptom and five of nine (56%) had shoulder pain. These symptoms can be related to a vast number of other diagnoses, and that is why physicians should be more aware of this pathology. Abdominal ultrasonography and abdominal computed tomography scan remain the methods of choice in confirming this diagnosis if the patient is stable. Clinicians should choose the method of diagnosis that they can access most rapidly.9,12
For hepatic blunt trauma, it is known that early embolization can decrease the need for liver-related operations.13 A recent review noted that 86.3% of hepatic injuries are now managed without operative intervention.14 In 1999, Rinehart10 found that survival rate of hepatic rupture in HELLP syndrome was better with embolization. Only 6% of cases from the literature published after 2000 used embolization as part of their treatment, and always in combination with surgery. Seven of our patients were treated with embolization, and no deaths occurred. Even though our centers are tertiary in hepatic disease, our strategy of treatment infrequently includes surgery. Four women who were transferred to us with packing had surgery to unpack the liver, but only one woman had another type of surgery (hemihepatectomy) in our hospital. It is important to remember that embolization can have some severe complications such as hepatic or biliary tract necrosis, but we think that the treatment of hepatic rupture should be reviewed and that embolization should be used as first-line treatment if the woman is stable.
Hepatic rupture associated with HELLP syndrome is a rare but catastrophic complication of pregnancy. Even if the diagnosis is difficult to make because the symptoms are nonspecific and common, clinicians should always have a suspicion of hepatic rupture because of HELLP syndrome when a pregnant woman presents with epigastric or shoulder pain with hypertension or shock. The maternal and fetal mortality rates remain high in some reviews, but we believe that an early diagnosis and the rapid use of hepatic artery embolization could help to improve these statistics.
1. Abercrombie J. Case of haemorrhage of the liver. Lond Med Gaz 1844; 34: 792–4.
2. 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.
3. Guntupalli SR, Steingrub J. Hepatic disease and pregnancy: an overview of diagnosis and management. Crit Care Med 2005; 33: S332–9.
4. Sherbahn R. Spontaneous ruptured subcapsular liver hematoma associated with pregnancy. A case report. J Reprod Med 1996; 41: 125–8.
5. Ibrahim N, Payne E, Owen A. Spontaneous rupture of the liver in association with pregnancy. Case report. Br J Obstet Gynaecol 1985; 92: 539–40.
6. Nelson DB, Dearmon V, Nelson MD. Spontaneous rupture of the liver during pregnancy: a case report. J Obstet Gynecol Neonatal Nurs 1989; 18: 106–13.
7. Erhard J, Lange R, Niebel W, Scherer R, Breuer N, Eigler FW. Liver complications in HELLP syndrome [in German]. Z Gastroenterol 1994; 32: 16–20.
8. Marsh FA, Kaufmann SJ, Bhabra K. Surviving hepatic rupture in pregnancy–a literature review with an illustrative case report. J Obstet Gynaecol 2003; 23: 109–13.
9. Barton JR, Sibai BM. Hepatic imaging in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count). Am J Obstet Gynecol 1996; 174: 1820–5.
10. Rinehart BK, Terrone DA, Magann EF, Martin RW, May WL, Martin JN Jr. Preeclampsia-associated hepatic hemorrhage and rupture: mode of management related to maternal and perinatal outcome. Obstet Gynecol Surv 1999; 54: 196–202.
11. Araujo AC, Leao MD, Nobrega MH, Bezerra PF, Pereira FV, Dantas EM, et al.. Characteristics and treatment of hepatic rupture caused by HELLP syndrome. Am J Obstet Gynecol 2006; 195: 129–33.
12. Nunes JO, Turner MA, Fulcher AS. Abdominal imaging features of HELLP syndrome: a 10-year retrospective review. AJR Am J Roentgenol 2005; 185: 1205–10.
13. Kozar RA, Moore FA, Moore EE, West M, Cocanour CS, Davis J, et al.. Western Trauma Association critical decisions in trauma: nonoperative management of adult blunt hepatic trauma. J Trauma 2009; 67: 1144–8; discussion 1148–9.
14. Tinkoff G, Esposito TJ, Reed J, Kilgo P, Fildes J, Pasquale M, et al.. American Association for the Surgery of Trauma Organ Injury Scale I: spleen, liver, and kidney, validation based on the National Trauma Data Bank. J Am Coll Surg 2008; 207: 646–55.
15. Bis KA, Waxman B. Rupture of the liver associated with pregnancy: a review of the literature and report of 2 cases. Obstet Gynecol Surv 1976; 31: 763–73.
16. El Youssoufi S, Nsiri A, Salmi S, Miguil M. [Liver rupture in peripartum: about 8 cases]. J Gynecol Obstet Biol Reprod (Paris) 2007; 36: 57–61.
17. Kelly J, Ryan D, O'Brien N, Kirwan W. Second trimester hepatic rupture in a 35 year old nulliparous woman with HELLP syndrome: a case report. World J Emerg Surg 2009; 4: 23.
18. Pavlis T, Aloizos S, Aravosita P, Mystakelli C, Petrochilou D, Dimopoulos N, et al.. Diagnosis and surgical management of spontaneous hepatic rupture associated with HELLP syndrome. J Surg Educ 2009; 66: 163–7.
19. Miguelote RF, Costa V, Vivas J, Gonzaga L, Menezes CA. Postpartum spontaneous rupture of a liver hematoma associated with preeclampsia and HELLP syndrome. Arch Gynecol Obstet 2009; 279: 923–6.
20. Raga F, Sanz-Cortes M, Bonilla-Musoles F. Three-dimensional ultrasound diagnosis of ruptured subcapsular liver hematoma caused by HELLP syndrome. Ultrasound Obstet Gynecol 2008; 32: 838–9.
21. Dart BW 4th, Cockerham WT, Torres C, Kipikasa JH, Maxwell RA. A novel use of recombinant factor VIIa in HELLP syndrome associated with spontaneous hepatic rupture and abdominal compartment syndrome. J Trauma 2004; 57: 171–4.
22. Merchant SH, Mathew P, Vanderjagt TJ, Howdieshell TR, Crookston KP. Recombinant factor VIIa in management of spontaneous subcapsular liver hematoma associated with pregnancy. Obstet Gynecol 2004; 103: 1055–8.
23. Shrivastava VK, Imagawa D, Wing DA. Argon beam coagulator for treatment of hepatic rupture with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. Obstet Gynecol 2006; 107: 525–6.
24. Descheemaeker PN, Compagnon P, Lavoue V, Seguin P, Lechaux D, Renaud-Giono A, et al.. [Liver transplantation for subcapsular haematoma during HELLP Syndrome]. Ann Fr Anesth Reanim 2009; 28: 1020–2.
25. Gilboa Y, Bardin R, Feldberg D, Bachar GN. Postpartum hepatic rupture and retroperitoneal hematoma associated with HELLP syndrome. Isr Med Assoc J 2006; 8: 219–20.
26. Mascarenhas R, Mathias J, Varadarajan R, Geoghegan J, Traynor O. Spontaneous hepatic rupture: a report of five cases. HPB (Oxford) 2002; 4: 167–70.
27. Srivastava G, Vine SJ, Asaad KA, Wolfe J. Successful outcome after hepatic rupture in previous eclamptic pregnancy. Arch Gynecol Obstet 2007; 276: 73–5.
28. Dessole S, Capobianco G, Virdis P, Rubattu G, Cosmi E, Porcu A. Hepatic rupture after cesarean section in a patient with HELLP syndrome: a case report and review of the literature. Arch Gynecol Obstet 2007; 276: 189–92.
29. Wicke C, Pereira PL, Neeser E, Flesch I, Rodegerdts EA, Becker HD. Subcapsular liver hematoma in HELLP syndrome: Evaluation of diagnostic and therapeutic options–a unicenter study. Am J Obstet Gynecol 2004; 190: 106–12.
30. Sutton BC, Dunn ST, Landrum J, Mielke G. Fatal postpartum spontaneous liver rupture: case report and literature review. J Forensic Sci 2008; 53: 472–5.
31. Chou PY, Yu CH, Chen CC, Chen WT. Spontaneously ruptured subcapsular liver hematoma associated with hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. Taiwan J Obstet Gynecol 2010; 49: 214–7.
32. Shaw C, Fattah N, Lynch D, Stokes M. Spontaneous rupture of the liver following a normal pregnancy and delivery. Ir Med J 2005; 98: 27–8.
33. Yagmurdur MC, Agalar F, Daphan CE. Spontaneous hepatic rupture in pregnancy. Eur J Emerg Med 2000; 7: 75–6.
34. Harris BM, Kuczkowski KM. Diagnostic dilemma: hepatic rupture due to HELLP syndrome vs. trauma. Arch Gynecol Obstet 2005; 272: 176–8.
35. Wust MD, Bolte AC, de Vries JI, Dekker GA, Cuesta MA, van Geijn HP. Pregnancy outcome after previous pregnancy complicated by hepatic rupture. Hypertens Pregnancy 2004; 23: 29–35.
36. Gutierrez-Cafranga E, Garcia-Molina FJ, Leon-del-Pino R, Montes-Posada E, Franco-Osorio JD, Mateo-Vallejo FJ. Hepatic rupture and hemoperitoneum in a pregnant woman with HELLP syndrome. Rev Esp Enferm Dig 2010; 102: 453–4.
37. Mikou MM, Sefrioui F, Harrandou M, Khatouf M, Kenjaa N. [Surgical management of rupture of a subcapsular hepatic haematoma at 39 weeks' gestation]. Ann Fr Anesth Reanim 2008; 27: 271–2.
38. Reck T, Bussenius-Kammerer M, Ott R, Muller V, Beinder E, Hohenberger W. Surgical treatment of HELLP syndrome-associated liver rupture – an update. Eur J Obstet Gynecol Reprod Biol 2001; 99: 57–65.
39. Varotti G, Andorno E, Valente U. Liver transplantation for spontaneous hepatic rupture associated with HELLP syndrome. Int J Gynaecol Obstet 2010; 111: 84–5.
40. Strate T, Broering DC, Bloechle C, Henschen S, Pothmann W, Hoffmann S, et al.. Orthotopic liver transplantation for complicated HELLP syndrome. Case report and review of the literature. Arch Gynecol Obstet 2000; 264: 108–11.
41. Stella CL, Malik KM, Sibai BM. HELLP syndrome: an atypical presentation. Am J Obstet Gynecol 2008; 198: e6–8.
42. Seren G, Morel J, Jospe R, Mahul P, Dumont A, Cuileron M, et al.. [HELLP syndrome and ruptured subcapsular hepatic haematoma. Case report and therapeutic options]. Ann Fr Anesth Reanim 2006; 25: 1067–9.
43. Shames BD, Fernandez LA, Sollinger HW, Chin LT, D'Alessandro AM, Knechtle SJ, et al.. Liver transplantation for HELLP syndrome. Liver Transpl 2005; 11: 224–8.
44. Carlson KL, Bader CL. Ruptured subcapsular liver hematoma in pregnancy: a case report of nonsurgical management. Am J Obstet Gynecol 2004; 190: 558–60.
45. Maoz D, Sharon E, Chen Y, Grief F. Spontaneous hepatic rupture: 13-year experience of a single center. Eur J Gastroenterol Hepatol 2010; 22: 997–1000.
46. Zeirideen R, Kadir RA. Spontaneous postpartum hepatic rupture. J Obstet Gynaecol 2009; 29: 155.
47. Aldemir M, Bac B, Tacyildiz I, Yagmur Y, Keles C. Spontaneous liver hematoma and a hepatic rupture in HELLP syndrome: report of two cases. Surg Today 2002; 32: 450–3.
48. Das CJ, Srivastava DN, Debnath J, Ramchandran V, Pal S, Sahni P. Endovascular management of hepatic hemorrhage and subcapsular hematoma in HELLP syndrome. Indian J Gastroenterol 2007; 26: 244–5.
49. Connor K, Rubin RA, Shrestha R, Johnson M, Sellers M, Butler B. ABO incompatible liver transplantation as a bridge to treat HELLP syndrome. Gastroenterol Res Pract 2009; 2009: 713937.
50. Hafeez M, Hameed S. Hellp syndrome and subcapsular liver haematoma. J Coll Physicians Surg Pak 2005; 15: 733–5.
51. Singh Y, Kochar SPS, Biswas M, Singh KJ. Hepatic rupture complicating HELLP syndrome in pregnancy. MJAFI 2009; 65: 89–90.
52. Fat BC, Terzibachian JJ, Grisey A, Houze JP, Faller JP, Leung F, et al.. Spontaneous hepatic rupture during an uncomplicated twin pregnancy [in French]. Gynecol Obstet Fertil 2011; 39: e7–10.
53. Zarrinpar A, Farmer DG, Ghobrial RM, Lipshutz GS, Gu Y, Hiatt JR, et al.. Liver transplantation for HELLP syndrome. Am Surg 2007; 73: 1013–6.
54. Suner DB, Salais SF, Garcia CD, Almela VD, Marin AP. Hepatic rupture associated with pre-eclampsia and HELLP syndrome, with catastrophic results. Prog Obstet Ginecol 2009; 52: 402–6.
55. Martinez A, Arvizu R, Camps L, Raga F, Sabater L, Juste J. Spontaneous hepatic rupture in HELLP syndrome: treatment through hepatic packing. Prog Obstet Ginecol 2010; 53: 194–97.
56. Ouvina Millan O, Alonso Vaquero MJ, Vidal Hernandez R, De Juan Barquin A. Hepatic subcapsular hematoma in HELLP syndrome. Prog Obstet Ginecol 2010; 53: 373–6.
57. Pliego Perez AR, Zavala Soto JO, Rodriguez Ballesteros R, Martinez Herrera FJ, Porras Jimenez A. Spontaneous hepatic rupture of pregnancy. A report of four cases and medical literature review. Ginecol Obstet Mexico 2006; 74: 224–31.
58. Boormans EMA, Bekedam DJ, Lenters E, Schoonderbeek FJ, Tilanus HW. Spontaneous liver haemorrhage during pregnancy: A rare and life-threatening situation. Nederlands Tijdschrift voor Geneeskunde 2007; 151: 1157–62.
59. Juarez-Azpilcueta A, Motta-Martinez E, Montano-Uzcanga A. Hepatic rupture as a complication of hypertensive disease of pregnancy maternal and the HELLP syndrome. Gaceta Med Mexico 2003; 139: 276–80.