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Liver hematoma and rupture in pregnancy
Liver Hematoma and Rupture in Pregnancy Steven J. Ralston and Steven D. Schwaitzberg
Liver hematoma and rupture is a rare but devastating complication of pregnancy. The majority of cases have been associated with severe preeclampsia, but unlike typical preeclampsia, it is a disease of older, multiparous patients. Although there are predictable findings on liver pathology, the underlying pathophysiology is poorly understood. Early recognition and prompt surgical intervention are crucial to reduce the high fetal and maternal mortality rate associated with thi~ disease. Copyright 9 1998 by W.B. Saunders Company
n 1844,James Abercrombie described a pregI nant woman with gastrodynia who treated her discomfort by tying a silk handkerchief around her abdomen, pulled tight by a servant. Less than an hour after a vaginal delivery, this patient collapsed and died within a day. The autopsy showed 2 pounds of blood in the abdomen and a mottled, lacerated liver. 1 Since this earliest report of a ruptured liver associated with pregnancy, the literature has been filled with over 100 case reports of this extremely morbid and often fatal complication. The timely and appropilate treatment of this clinical entity depends on the early suspicion and diagnosis by the practicing obstetrician. However, given its clinical rarity, most individual obstetricians have only limited first-hand knowledge of the disease. Because of this, obstetricians often rely on the experience of general and trauma surgeons for the surgical management of these cases.
abscesses, 7 acute fatty liver of pregnancy, a and trauma. Henny et al 9 compiled 75 case reports from the literature in 1983 and found that 82% of the affected women were multiparous with an average age of 31.7 years (range, 21 to 46), a profile quite different from the "typical" preeclarnptic patient who tends to be younger and nulliparous, perhaps implying a somewhat different etiology for this disease. Elsewhere, a similar 10:1 ratio of multiparas to primiparas has been reported) ~ The majority of liver hematomas occur in the third trimester, at term, or immediately postpartumg; rare cases have been reported in the first two trimesters, lla~ In their extensive review of 442 patients with HELLP syndrome, Sibai et al ~s report an incidence of liver rupture of 1%, and it is this group of women who appear to be at highest risk.
Pathophysiology Epidemiology Most of what we know about liver h e m a t o m a and rupture during pregnancy comes from case reports scattered throughout the medical literature over the past 150 years. The incidence has been estimated to be from 1 in 45,000 to 1 in 250,000 deliveries. 2.s The majority of reported cases have occurred in women with some form of pregnancy-induced hypertension including preeclampsia, eclampsia, and the HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome. In retrospect, many of the cases of liver rupture reported in the literature before Weinstein's 4 description of HELLP in 1982 probably were complicated by this preeclampsia variant. Other conditions and diseases that also have been associated with liver hematoma and rupture include cocaine use? liver neoplasms? liver
Unfortunately, the pathophysiology of this disease is not completely understood. Pathological specimens, often from autopsies, show periportal fibrin deposition, hepatocyte necrosis, and parenchymal hemorrhage, ~4 implicafng disseminated intravascular coagulation (DIC) as an underlying etiology. In 1943, Rademaker ~5 suggested a process that begins with local infarction caused by fibrin deposition followed by hypervascularization, vessel rupture, intrahepatic hemorrhage, tissue disruption, subcapsular hematoma formation, perforation of From the Departmentsof Obstetrics& Gynecologyand Surgery, New England Medical Center, Tufts UniversitySchool of Medicine, Boston, MA. Address reprintrequeststo StevenJ. Ralston, MD, 750 Washington St, Box 360, Boston, MA 02111. Copyright9 199803 W.B. Saunders Company O146.0005/98/2202-0007508.00/0
Seminars in Perinatology, Vol 22, No 2 (April), 1998: pp 141-148
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Glisson's capsule, hemoperitoneum, peritonitis, and eventually death. It is this intraparenchymal hemorrhage leading to the capsular rupture that distinguishes this disease from the trauma of a patient suffering from liver hemorrhage: in the trauma patient, the rupture precedes the hemorrhage. Others 14have suggested that vasospasm and the increased sensitivity of the vasculamre to endogenous and exogenous vasospressor agents associated with preeclampsia lead to the hepatocellular necrosis, endothelial damage, and parenchymal hemorrhage seen in these patients. What is clear, however, is that the lesions seen in patients with liver rupture are similar to those seen in patients with the most severe forms of preeclampsia and eclampsia. 16 Many investigators have speculated on the preponderance of multiparous patients in reviews of this disease. Some propose a "desensitization" of the reticuloendothelial system in the liver during prior pregnancies, which then leads to an accumulation of fibrin deposits in subsequent pregnancies followed by the blockage of the sinusoids, cellular necrosis, and eventual hemorrhage and rupture. 17'xs There also has been a suggestion in the literature that the same degenerative process that predispose older, multiparous women to uterine rupture also predisposes them to liver rupture; however, there is no convincing evidence that these two entities are related except in their similar presentations. The location of the liver rupture in pregnancy is typically at the inferior edge of the right lobe of the liver. Although evidence of intrahepatic hemorrhage can appear throughout the liver parenchyma, the majority (74%) o f h e m a t o m a s occur on the superior and anterior aspect of the right lobe of the liver. Only 11% of hematomas are confined to the left lobe. ~ Exogenous trauma, although implicated in the majority of liver ruptures outside of pregnancy, has not been documented frequently in pregnant women with liver rupture. Some investigators, however, suggest that even minor increases in intraabdominal pressure associated with emesis, parturition, and patient transport may be sufficient to cause rupture of a preexisting subcapsular hematoma. ~0"2~ Clinical Presentation Henny et al ~ describe the formation of a liver hematoma and rupture as a biphasic disease con-
Table 1. Differential Diagnosis of RUQ Pain in Pregnancy Obstetric Ruptured uterus Labor Abdominal pregnancy Fetal movements/rib pain Gynecologic Adnexal torsion Rupture of adnexal cyst Gastrointestinal Liver hematoma/rupture Cholelithiasis/cholecystitis/cholangitis Hepatitis Pancreatitis Gastritis Peptic ulcer Crohn's disease Cardiac Myocardial infarction Pericarditis Pulmonary Pulmonary embolus Pneumonia Genitourinary Pyelonephrifis Nephrolithiasis
sisting of a prodromal period that can last up to a month, followed by an acute phase when the actual rupture occurs, which quickly leads to maternal cardiovascular collapse and death. The prodrome is often accompanied by vague systemic complaints of headache and malaise or specific gastrointestinal symptoms including nausea, vomiting, and jaundice. Sometimes, but not predictably, there will be hypertension and proteinuria present at this stage of the illness. However, by the time a hematoma or rupture is diagnosed, 80% to 90% of patients manifest signs and symptoms of preeclampsia. Jaundice and transaminitis may be present but are not consistently seen before hematoma formation. Many investigators refer to a triad of preeclampsia, right upper quadrant (RUQ) pain, and the acute onset of profound hypotension and shock. 21 Nevertheless, the diagnosis often is made retrospectively at the time of laparotomy; until then, the differential diagnosis includes a myriad of etiologies including obstetric (ruptured uterus, adnexal torsion), gastrointestinal (appendicitis, cholecystitis, pancreatitis, perforated ulcer, or viscus), and cardiac (myocardial infarction, Table 1).
Liver Hematoma and Rupture in Pregnancy
Patients may present at any time during the pregnancy or puerperium, but the majority are in the third trimester at the time of diagnosis. O f the 75 patients reviewed by H e n n y et al9 in 1983, 82% were in the third trimester. Up to 15%, however, have been reported to occur in the immediate postpartum period, usually within 24 hours of delivery. 22 T h e r e may be no antecedent abdominal trauma before rupture of a preexisting subcapsular hematoma, thus, the frequently used appellation spontaneous is given to these ruptures. It may be that the trauma that induces rupture in these patients often goes unrecognized as such. Nevertheless, once rupture of Glisson's capsule occurs, clinical deterioration usually is rapid, and often fatal. Patients complain of severe pain, present with signs and symptoms of shock, and there is usually evidence of fetal distress, either from p r o f o u n d maternal hypotension or concomitant abruption. A progressive fall in hematocrit level combined with abdominal distension will then lead to a laparotomy, which confirms the diagnosis. A subcapsular h e m a t o m a with its attendant symptom of R U Q pain, perhaps caused by capsular stretching, may develop in some patients, but they do not then progress to liver rupture. It is not clear how these patients differ from the ones who experience rupture and its devastating consequences. They may represent a unique group o f patients with spontaneous liver h e m a t o m a who can be treated more conservatively.
Diagnosis As stated earlier, the diagnosis of liver h e m a t o m a and rupture is often made at the time o f laparotomy for an acute a b d o m e n in a patient who has presented with R U Q pain and preeclampsia, in whom then p r o f o u n d hypotension, shock, and evidence o f intraabdominal h e m o r r h a g e develops acutely. Sometimes, a cesarean section is being p e r f o r m e d for fetal distress with an incidental finding of h e m o p e r i t o n e u m that is not originating from the uterus. In these two situations the clinical scenario will dictate the necessity to proceed to the operating r o o m with little n e e d for definitive diagnostic tests. However, an early suspicion of liver h e m a t o m a may allow for diagnosis before a catastrophic rupture. There-
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fore, multiple diagnostic modalities are available. Unfortunately, patient history, physical examination, and simple laboratory tests are insufficient to confirm a diagnosis of liver h e m a t o m a or rupture. Elevated liver function tests and thrombocytopenia tell more about the severity of the preeclampsia and, perhaps, show an increased risk for liver hematoma, but are not definitive. 14 Computerized tomography (CT) scanning provides the highest degree of diagnostic accuracy in assessing a patient for a subcapsular hematoma. With contrast, very small amounts of intraparenchymal and subcapsular h e m a t o m a can be recognized. T h e drawbacks of CT scanning are obvious: it is costly and may be impractical for a patient who requires intensive monitoring. For these reasons, many investigators ~ r e c o m m e n d ultrasonography (US) as a frst-line imaging study. T h e advantages of US over CT include its ease o f performance (a scan can be obtained at the bedside) and lower price compared with CT, while sacrificing little accuracy in detecting subcapsular bleeding and intraperitoneal blood. Acute subcapsular bleeding will appear as sonolucency overlying the liver parenchyma. However, as Goodlin et al ~ point out in their review, US may not detect subcapsular bleeding until liquefaction of the blood clot has occurred and the h e m a t o m a is greater than 2 cm in diameter. Manas et al24 c o n c u r r e d with this view in 1985, finding in their "experience, ultrasound examinations were unreliable." In their study, two of seven patients had normal US findings despite abnormal CT scanning. T h e lesson to be drawn here is that an US finding o f a liver h e m a t o m a is specific, but that the negative predictive value of the test may be slightly worse than that o f CT. Others have used hepatic angiography, ~5'26 paracentesis, is and radionuclide liver scans to make a diagnosis o f liver h e m a t o m a or rupture. Paracentesis, or peritoneal lavage, can certainly help to distinguish ascitic fluid from blood and has a low (0.5%) complication rate cited in the surgical literature. However, diagnostic lavage is invasive and perhaps contraindicated in anteparturn patients, so some investigators prefer CT to make this distinction if possible74 Liver scanning with 99mTechnicium can visualize small hematomas, is safe in pregnancy, and is cheaper than CT, but has the disadvantage of not being able
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to identify a cause for the h e m a t o m a or to accurately tell if the capsule is intact. 27 Hepatic artery angiography has the advantage of providing a means of embolotherapy in a stable patient with an expanding h e m a t o m a or, paradoxically, in a patient too ill to go to surgery who continues to bleed from a liver rupture. T h e disadvantages of angiography include the high radiation dose m limiting its use to postpartum p a t i e n t s - - a n d its inability to adequately treat venous hemorrhage. We believe that both CT scanning and US will continue to play the major role in the diagnosis of the stable patient with a suspected subcapsular liver hematoma. However, these "stable" patients are the exceptions rather than the rule; the majority of patients will be sick e n o u g h to necessitate surgical exploration, obviating the n e e d for any radiological intervention. As stated by Sherbahn 2s in 1996, " . . . because of the rarity of the condition, the varied presentation and the often rapid progression, the diagnosis will continue to be made at laparotomy in many cases." If the clinical suspicion of rupture is high, one should not hesitate to proceed directly to surgery, because a delay in surgery has been implicated in the extremely high mortality rate of this condition. 14'29
Treatment and Management Options The care of patients with liver rupture in pregnancy can be quite challenging and should be approached through a multidisciplinary effort o f surgeons, perinatologists, and intensivists. Initial treatment of patients with presumed liver rupture should be volume resuscitation en route to the operating room; multiple large bore peripheral intravenous lines remain the mainstay of resuscitation access. The surgical exploration should be carried out through a midline abdominal incision, often extending from the xiphoid to the symphysis pubis to allow sufficient access to the liver and uterus. Thoracoabdominal extension may be required to achieve adequate mobilization of the liver; this is particularly true for right-sided injuries. Often, the intrinsic liver disease in these patients can h a m p e r the surgeons' exposure efforts. If the patient is pregnant at the time o f laparotomy, a cesarean section may be p e r f o r m e d first, if there is fetal distress, followed by exploration of the u p p e r abdomen. If there is no evi-
dence of liver rupture and no visible hematoma, the a b d o m e n can be closed, and the patient observed. Some investigators 2 r e c o m m e n d observation of small intact hematomas discovered at laparotomy, but suggest evacuation o f larger hematomas to control bleeding sites and prevent catastrophic rupture. Because there are no data from randomized trials to show an advantage to either protocol, both are acceptable d e p e n d i n g on surgeon preference. A variety o f surgical techniques have been described in the literature to treat h e m o r r h a g e from a r u p t u r e d liver. 2'26'3~Rapid control of hemostasis is of utmost importance and can be accomplished initially through direct pressure on the rupture site with sponges or with temporary compression o f the porta hepatis (Pringle maneuver31). This initial packing maneuver is critical if the discovery of hepatic h e m o r r h a g e is made by a surgeon who is not experienced in the m a n a g e m e n t of major hepatic injury. This is also true in the event of hemodynamic instability in which tight packing of the RUQ allows the anesthesiologist the opportunity to completely resuscitate the patient. Patients are often coagulopathic and hypothermic because o f the massive blood loss and hepatic dysfunction, so care must be taken to replace clotting factors and platelets while treating the blood loss with volume expansion and transfusions of packed red blood cells. No attempt should be made to repair the injury until resuscitation is complete if hemostasis can be temporarily achieved through manual compression. T h e Pringle maneuver is accomplished by placing a noncrushing vascular clamp across the porta hepatis (Fig 1). This provides temporary occlusion of the hepatic artery and portal vein. Hepatic blood flow is reduced to about 10% of normal values, but bleeding from the hepatic veins is not controlled by this action. 32 T h e clamp can be left in place for as long as 1 hour. It should not be placed during the period of manual compression because total hepatic inflow occlusion for an extended period may result in increased mortality. 3a With adequate exposure and visualization, specific bleeding sites can be controlled with sutures (2-0 chromic, 0 chromic, or 2-0 silk on a blunt needle), electrocautery, the argon laser, and a n u m b e r of topical agents used to p r o m o t e hemostasis such as Gelfoam, Avitene, or Surgi-
Liver Hematoma and Rupture in Pregnancy
i C._'-/=::~.....
Figure 1. The Pringle maneuver: compression of the hepatoduodenal ligament (porta hepatis). Reprinted with permission.5~
cel. Direct suturing is often difficult because of the consistency of the liver tissue, which has been disrupted by hemorrhage and hepatocyte necrosis. Recently, fibrin glue has been described as a useful hemostatic adjunct. ~ Although more commonly used in the repair of splenic injuries, absorbable polygalactin mesh has been placed perihepatically to control hemorrhage in trauma patients. ~5 The extent of the laceration, the n u m b e r of bleeding sites, and the accompanying coagulopathy may make absolute hemostasis difficult to achieve. The choices facing the surgeon at this point include temporary packing with sponges to be removed in 48 to 72 hours when the patient is more stable, packing with o m e n t u m or rectus muscle, hepatic artery ligation or embolization, and hepatic lobectomy. None of these options is ideal, and the surgeon's choice will depend on the clinical scenario. Hepatic artery ligation, a6 although reasonable in a trauma patient, s7 cannot be recommended except as a last resort because this procedure seems to be poorly tolerated in the pregnant woman who may have withstood significant insult to the liver. 1~ Moreover, the liver changes in preeclampsia are reversible, thus hepatic resection should be avoided if possible. 2 Finally, although Hunter et al aa described a successful liver transplant in a patient who required a total hepatectomy after a spontaneous liver rupture, this heroic intervention should be reserved for patients with truly extensive liver damage whose only hope for survival is to be rendered anhepatic and await an allograft. Adequate drainage is crucial in preventing
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the formation of infected hematomas and abscesses as well as in assessing the extent of ongoing hemorrhage. Most investigators recommend the use of closed drainage systems with bores large enough to avoid blockage and allow egress of clots. Intensive care unit observation will likely be necessary because these patients often remain quite unstable postoperatively. They are at risk for the many complications of massive blood loss and volume replacement including adult respiratory distress syndrome, infection, and multiorgan failure. In addition, patients who sustain major liver injury are at risk for profound hypoglycemia, which can be avoided by providing 10% dextrose solutions perioperatively. The postoperative nutritional needs of these patients also require particular attention because of the hepatic injury. Fever is c o m m o n for the first several postoperative days even in the absence of infection; it is thought that inflammatory mediators are responsible, s9 Finally, hypothermia may occur intraoperatively and postoperatively and must be corrected. At temperatures below 35~ patients have been shown to experience a reversible platelet dysfunction manifested by increased bleeding times. 4~ In Hibbard's series of eight patients, the mean volume of transfused packed red blood cells was 4,500 mL. s In 1995, Stevenson and Graham 26 reported a case that required 22 units of packed cells, 13 units of cryoprecipitate, 31 units of fresh frozen plasma, and 30 units of platelets. That same year, at our own institution, we had a padent who received 116 units of packed cells, 103 units of plasma, 14 units of cryoprecipitate, and 26 units of platelets. Such enormous volumes of transfused blood products can challenge the resources of any hospital's blood bank. Until recently, a diagnosis in pregnancy of a subcapsular liver hematoma without rupture mandated surgical exploration and evacuation. In part, this was based on early reports of extremely high (96%) mortality in women not undergoing laparotomy. 9 In 1971, Utley41 wrote, "It cannot be determined if there exists a mild, but not fatal form of hepatic rupture during pregnancy that does not require operation." Moreover, outside of pregnancy, a liver hematoma implied a significant, traumatic rupture of Glisson's capsule necessitating surgical intervention. However, because the mechanism of hematoma formation is not the rupture itself, and because
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Table 2. Liver Rupture in Pregnancy: Maternal and Fetal Mortality
Study, Year
No. of Cases
Maternal Mortality (%)
Abercrombie, 1 1844 Rademaker, 15 1943 Kramish et al, 51 1954 Pavlic and Townsend, ~2 1962 Hakim-Elahi, s~ 1965 Castafieda et al, ~3 1970 Utley, 41 1971 Bis and W a x m a n , 22 1976 Henny e t al, 9 1983
1 2 14 17 61 51 31 91 75
100 50 64 71 80 71 61 59 56-75
the early reviews were likely biased against those patients who did not have surgery (perhaps because they were too sick to withstand exploration), conservative m a n a g e m e n t ~4'27 of unruptured h e m a t o m a s was r e p o r t e d a n d accepted in the early 1980s. Similarly, there has b e e n greater attention recently in the surgical literature to nonsurgical m a n a g e m e n t of o t h e r types o f liver injuries. 42~4 Conservative m a n a g e m e n t should be reserved for those patients who are: (1) Clinically stable, (2) Postpartum, and (3) Do not have a diagnosis o f preeclampsia. In the setting o f preeclampsia, the risk to b o t h the fetus and m o t h e r is too high to warrant conservative m a n a g e m e n t before delivery. Thus, i f a preeclamptic patient is p r e g n a n t at the time of diagnosis of a subcapsular hematoma, this should p r o m p t an immediate cesarean section t h r o u g h a midline incision with visual inspection of the liver to rule out rupture. O n e m i g h t argue for a delay in therapy at extremely early gestational ages to allow for the administration o f corticosteroids to prevent fetal p u l m o n a r y and neurological morbidity. However, given the e x t r e m e morbidity and mortality of an actual rupture, this c a n n o t be recomm e n d e d . I m m e d i a t e exploration and delivery by cesarean section should remain the standard of care. It may be that p r e g n a n t patients without preeclampsia in w h o m liver h e m a t o m a s develop, eg, a t r a u m a victim, may be treated m o r e conservatively if hemodynamically stable; however, little data exist in the literature to support or refute this stance.
Prognosis Unfortunately, liver h e m a t o m a a n d rupture during pregnancy is associated with a high maternal and fetal mortality rate (Table 2). H e n n y et al 9
Fetal Mortality (%)
Not Not Not Not
0 50 Reported Reported Reported Reported 71 62 62-77
r e p o r t e d a fetal mortality rate of 62% to 77% and a maternal mortality rate o f 56% to 75%. T h e majority o f maternal deaths are caused by complications of massive h e m o r r h a g e a n d coagulopathy. T h e fetal deaths are caused by a combination o f maternal hypotension, abruption, and prematurity. Advances in intensive care, radiology, blood p r o d u c t administration, and h e m o static agents have led to better outcomes over the past few decades, but the case fatality rate and associated morbidity remain high. T h e r e have b e e n a few reports in the literature of future p r e g n a n c y o u t c o m e in patients who have had a history of liver rupture. Several have r e p o r t e d successful p r e g n a n c y outcomes in these women. 4~47 However, Greenstein et a148 in 1994 r e p o r t e d a case of r e c u r r e n t intrahepatic h e m o r r h a g e in a patient whose first pregnancy was unremarkable; her second pregnancy was complicated by H E L L P syndrome with a subcapsular h e m a t o m a diagnosed by CT a n d m a n a g e d conservatively. In her third pregnancy, she develo p e d r e c u r r e n t preeclampsia and a p o s t p a r t u m h e m a t o m a that required surgical intervention despite early diagnosis with US and CT and a trial of embolization. Given that this is a disease of older multiparas who may have significant morbidity associated with a h e m a t o m a a n d rupture, it is not surprising that there are so few r e p o r t e d cases o f subsequent pregnancies in the literature. Because we know that severe preeclampsia, a n d in particular the H E L L P syndrome, often recur in subsequent pregnancies, 49 w o m e n with a history of liver h e m a t o m a a n d rupture should a p p r o a c h the question o f future pregnancies with great caution.
Summary A flow chart for the evaluation a n d t r e a t m e n t o f a patient with a suspected liver h e m a t o m a is
Liver Hematoma and Rupture in Pregnancy
[SUSPECTEDLIVERR ~
EHgMATOMAINTACT OR NOT S E E N
RUFTIJRED HEMATOMA
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BLOOD I ] PRODUCTS ] ,
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,
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ANGIOGP.~blI I EMBOLIT~TIONI
s h o w n i n Fig 2. This is a rare diagnosis that s h o u l d b e c o n s i d e r e d i n a n y p a t i e n t with R U Q p a i n i n p r e g n a n c y , especially those with severe p r e e c l a m p s i a o r the H E L L P s y n d r o m e . I n the stable p a t i e n t , C T s c a n n i n g o r US will o f t e n confirm t h e diagnosis. However, a n y p a t i e n t with e v i d e n c e o f i n t r a a b d o m i n a l h e m o r r h a g e o r fetal distress d e m a n d s i m m e d i a t e surgical exploration. C o n s u l t a t i o n with a g e n e r a l s u r g e o n well versed i n h e p a t i c a n a t o m y a n d t r a u m a m a n a g e m e n t is i n t e g r a l to the i n t r a o p e r a t i v e care o f these patients, a n d t h e i r postoperative care o f t e n r e q u i r e s intensive care a n d a m u l t i d i s c i p l i n a r y approach.
References 1. AbercrombieJ: Case of hemorrhage of the liver. London Med Gaz 34:792, 1844 2. Smith LG, Moise KJ, Dildy GA, et al: Spontaneous rupture of liver during pregnancy: Current therapy. Obstet Gynecol 77:171-175, 1991 3. Hibbard LT: Spontaneous rupture of the liver in pregnancy: A report of eight cases. Am J Obstet Gynecoi 126:334-338, 1976 4. Weinstein L: Syndrome of hemolysis, elevated liver enzymes, and low platelet count: A severe consequence of hypertension in pregnancy. Am J Obstet Gynecoi 142:159-167, 1982 5. Moen MD, Calinedo MJ, Marshall W, et al: Hepatic rupture in pregnancy associated with cocaine use. Obstet Gynecol 82:687-689, 1993 6. Baird JN, Hawley RG: Spontaneous rupture of the liver during pregnancy. J Repro Med 6:93-95, 1971
Figure 2. Management of a suspected liver hemat o m a / r u p t u r e . Adapted and reprinted with permission2.
7. Yen SS: Spontaneous rupture of liver during pregnancy. Obstet Gynecol 23:783, 1964 8. Minuk GY, Lui RC, KellyJFa Rupture of the liver associated with acute fatty liver of pregnancy. Am J Gastroenteroi 82:457-460, 1987 9. Henny P, Lim AE, Brummelkamp WH, et al: A review of the importance of acute multidisciplinary treatment following spontaneous rupture of the liver capsule during pregnancy. Surg Gynecol Obstet 156:593-596, 1983 10. AzizS, Merrell PC, CollinsJA: Spontaneous hepatic hemorrhage during pregnancy. AmJ Surg 146:680-682, 1983 11. Links H: Spontaneous rupture of the liver complicating pregnancy. Br MedJ 1:275-276, 1946 12. Cerone DM, Catalino G: Spontaneous rupture of the liver during pregnancy. Obstet Gynecol 12:459-461, 1958 13. Sibai BM, Ramadan MK, Usat I, et al: Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes and low platelets (HELLP syndrome). AmJ Obstet Gynecoi 169:1000-1006, 1993 14. Rolfes DB, Ishak KM: Liver disease in toxemia of pregnancy. AmJ Gastroenterol 81:1138-1144, 1986 15. Rademaker L: Spontaneous rupture of liver complicating pregnancy. Ann Surg 118:396-401, 1943 16. Antia FP: Liver in normal pregnancy, pre-eclampsia, and eclampsia. Lancet 2:776-779, 1958 17. Westergaard L: Spontaneous rupture of the liver in pregnancy. Acta Obstet Gynecol Scand 59:559-561, 1980 18. Mokotoff R, Weiss I_S, Branson LH, et al: Liver rupture complicating toxemia of pregnancy. Arch Intern Med 119:375-580, 1967 19. Speert H, Tillman AJB: Rupture of the liver in pregnancy: A rare complication of eclampsia. Am J Obstet Gynecol 63:1127-1132, 1952 20. Barry AP, Meagher DJ: Rupture of the liver complicating pregnancy. Obstet Gynecol 22:381-385, 1964 21. Stalter KD, Sterling WA: Hepatic subcapsular hemorrhage associated with pregnancy. Surgery 98:112-114, 1985
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22. Bis KA, Waxman B: Rupture of the liver associated with pregnancy: A review of the literature and report of 2 cases. Obstet Gynecol Surv 31:763-773, 1976 23. Goodlin RC, Anderson JC, Hodgson PE: Conservative treatment of liver hematoma in the postpartum period. J Repro Med 30:368-370, 1985 24. Manas KJ, Welsh JD, Rankin RA, et al: Hepatic hemorrhage without rupture in preeclampsia. N Engl J Med 312:424-426, 1985 25. Sommer DG, Greenway GD, Bookstein JJ, et al: Hepatic rupture with toxemia of pregnancy: Angiographic diagnosis. Am J Radiol 132:455-456, 1979 26. StevensonJT, Graham DJ: Hepatic hemorrhage and the HELLP syndrome: A surgeon's perspective. Am Surg 61:756-760, 1995 27. CheathamJE, Smith EI, Tunell WP, et al: Nonoperative management of subcapsular hematomas of the liver. Am J Surg 140:852-857, 1980 28. Sherbahn R: Spontaneous ruptured subcapsular liver hematoma associated with pregnancy.J Repro Med 41:125128, 1996 29. Stalter KD, Sterling WA: Hepatic subcapsular hemorrhage associated with pregnancy. Surgery 98:112-114, 1985 30. Hakim-Elahi E: Spontaneous rupture of the liver in pregnancy. Obstet Gynecol 26:435-440, 1965 31. Pringle JH: Notes on the arrest of hepatic hemorrhage due to trauma. Ann Surg 48:541-548, 1908 32. Kim YI, Ishii T, Aramaki M, et al: The Pringle maneuver induces only partial ischemia of the liver. Hepatogastroenterology 42:169-171, 1995 33. Tatsuma T, Kim YI, Kai T, et al: hnportmace of hepatovenous back-perfusion for maintenance of liver viability during the Pringle manoeuvre. B r J Surg 82:1071-1075, 1995 34. Uranus S, Mischinger HJ, Pfeifer J, et al: Hemostatic methods for the management of spleen and liver injuries. WorldJ Surg 20:1107-1111, 1996 35. Brunet C, Sielezneff I, Thomas P, et al: Treatment of hepatic trauma with perihepadc mesh: 35 cases. J Trauma 37:200-204, 1994 36. Mays ET, Cond S, Fallahzadeh H, et al: Hepatic artery ligation. Surgery 86:536-543, 1979 37. Gore RM, Nahrwold DL: Hepatic trauma and surgery, in Gore RM, Levine MS, Laufer I (eds): Textbook of Gastrointestinal Radiology, Philadelphia, PA, Saunders, 1994, pp 2051-2064
38. Hunter SK, Martin M, BendaJA, et al: Liver transplant after massive spontaneous hepatic rupture in pregnancy complicated by preeclampsia. Obstet Gynecol 85:819822, 1995 39. Cogbill TH, Moore EE, Feliciano DV, et al: Hepatic enzyme response and hyperpyrexia after sever liver injury. Am Surg 58:395-399, 1992 40. Valeri CR, Khabbaz K, Khuri SF: Effect of skin temperature on platelet function in patients undergoing extracorporeal bypass. J Thorac Cardiovasc Surg 104:108-116, 1992 41. UtleyJP~ Spontaneous rupture of the liver during pregnancy. Surg Gynecol Obstet 133:250-252, 1971 42. Pachter HL, Feliciano DV: Complex hepatic injuries. Surg Clin North Am: 76:763-782, 1996 43. Pachter HL, Knudson MM, Esrig B, et al: Status of nonoperative management of blunt hepatic injuries in 1995: A multicenter experience with 404 patients. J Trauma 40:31-38, 1996 44. Boone DC, Federle M, Billiar TR, et al: Evolution of management of major hepatic trauma: Identification of patterns of injury. J Traum 39:344-350, 1995 45. Alleman JS, Delarue MW, Hasaart TH: Successful delivery after hepatic rupture in previous pre-eclamptic pregnancy. E u J Obstet Gynecol 47:76-79, 1992 46. PortnuffJ, Ballon S: Hepatic rupture in pregnancy. Am J Obstet Gynecol 114:1102, 1972 47. Sakala EP, Moore WD: Successful term delivery after previous pregnancy with ruptured liver. Obstet Gynecol 68:124, 1986 48. Greenstein D, Henderson LM, Boyer TD: Liver hemorrhage: Recurrent episodes during pregnancy" complicated by preeclampsia. Gastroent 106:1668-1671, 1994 49. Sullivan CA, Magann EF, Perry KG, et al: Ther recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and tow platelets (HELLP) in subsequent gestations. A m J Obstet Gynecol 171:940-943, 1994 50. Feliciano DV, Pachter HL: Hepatic trauma revisited. Curr Prob Surg 26:453-524, 1989 51. Kramish D, Auer ES, Reckler SM: Spontaneous rupture of the liver during pregnancy. Obstet Gynecol 4:21-28, 1954 52. Pavlic RS, Townsend DE: Spontaneous rupture of the liver in pregnancy. Am J Obstet Gynecol 83:1373-1376, 1962 53. Castafieda H, Garcia-Romero H, Canto M: Hepatic hemorrhage in toxemia of pregnancy. A m J Obstet Gynecol 107:579-584, 1970
Liver hematoma and rupture is a rare but devastating complication of pregnancy. The majority of cases have been associated with severe preeclampsia, but unlike typical preeclampsia, it is a disease of older, multiparous patients. Although there are predictable findings on liver pathology, the underlying pathophysiology is poorly understood. Early recognition and prompt surgical intervention are crucial to reduce the high fetal and maternal mortality rate associated with thi~ disease. Copyright 9 1998 by W.B. Saunders Company
n 1844,James Abercrombie described a pregI nant woman with gastrodynia who treated her discomfort by tying a silk handkerchief around her abdomen, pulled tight by a servant. Less than an hour after a vaginal delivery, this patient collapsed and died within a day. The autopsy showed 2 pounds of blood in the abdomen and a mottled, lacerated liver. 1 Since this earliest report of a ruptured liver associated with pregnancy, the literature has been filled with over 100 case reports of this extremely morbid and often fatal complication. The timely and appropilate treatment of this clinical entity depends on the early suspicion and diagnosis by the practicing obstetrician. However, given its clinical rarity, most individual obstetricians have only limited first-hand knowledge of the disease. Because of this, obstetricians often rely on the experience of general and trauma surgeons for the surgical management of these cases.
abscesses, 7 acute fatty liver of pregnancy, a and trauma. Henny et al 9 compiled 75 case reports from the literature in 1983 and found that 82% of the affected women were multiparous with an average age of 31.7 years (range, 21 to 46), a profile quite different from the "typical" preeclarnptic patient who tends to be younger and nulliparous, perhaps implying a somewhat different etiology for this disease. Elsewhere, a similar 10:1 ratio of multiparas to primiparas has been reported) ~ The majority of liver hematomas occur in the third trimester, at term, or immediately postpartumg; rare cases have been reported in the first two trimesters, lla~ In their extensive review of 442 patients with HELLP syndrome, Sibai et al ~s report an incidence of liver rupture of 1%, and it is this group of women who appear to be at highest risk.
Pathophysiology Epidemiology Most of what we know about liver h e m a t o m a and rupture during pregnancy comes from case reports scattered throughout the medical literature over the past 150 years. The incidence has been estimated to be from 1 in 45,000 to 1 in 250,000 deliveries. 2.s The majority of reported cases have occurred in women with some form of pregnancy-induced hypertension including preeclampsia, eclampsia, and the HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome. In retrospect, many of the cases of liver rupture reported in the literature before Weinstein's 4 description of HELLP in 1982 probably were complicated by this preeclampsia variant. Other conditions and diseases that also have been associated with liver hematoma and rupture include cocaine use? liver neoplasms? liver
Unfortunately, the pathophysiology of this disease is not completely understood. Pathological specimens, often from autopsies, show periportal fibrin deposition, hepatocyte necrosis, and parenchymal hemorrhage, ~4 implicafng disseminated intravascular coagulation (DIC) as an underlying etiology. In 1943, Rademaker ~5 suggested a process that begins with local infarction caused by fibrin deposition followed by hypervascularization, vessel rupture, intrahepatic hemorrhage, tissue disruption, subcapsular hematoma formation, perforation of From the Departmentsof Obstetrics& Gynecologyand Surgery, New England Medical Center, Tufts UniversitySchool of Medicine, Boston, MA. Address reprintrequeststo StevenJ. Ralston, MD, 750 Washington St, Box 360, Boston, MA 02111. Copyright9 199803 W.B. Saunders Company O146.0005/98/2202-0007508.00/0
Seminars in Perinatology, Vol 22, No 2 (April), 1998: pp 141-148
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Glisson's capsule, hemoperitoneum, peritonitis, and eventually death. It is this intraparenchymal hemorrhage leading to the capsular rupture that distinguishes this disease from the trauma of a patient suffering from liver hemorrhage: in the trauma patient, the rupture precedes the hemorrhage. Others 14have suggested that vasospasm and the increased sensitivity of the vasculamre to endogenous and exogenous vasospressor agents associated with preeclampsia lead to the hepatocellular necrosis, endothelial damage, and parenchymal hemorrhage seen in these patients. What is clear, however, is that the lesions seen in patients with liver rupture are similar to those seen in patients with the most severe forms of preeclampsia and eclampsia. 16 Many investigators have speculated on the preponderance of multiparous patients in reviews of this disease. Some propose a "desensitization" of the reticuloendothelial system in the liver during prior pregnancies, which then leads to an accumulation of fibrin deposits in subsequent pregnancies followed by the blockage of the sinusoids, cellular necrosis, and eventual hemorrhage and rupture. 17'xs There also has been a suggestion in the literature that the same degenerative process that predispose older, multiparous women to uterine rupture also predisposes them to liver rupture; however, there is no convincing evidence that these two entities are related except in their similar presentations. The location of the liver rupture in pregnancy is typically at the inferior edge of the right lobe of the liver. Although evidence of intrahepatic hemorrhage can appear throughout the liver parenchyma, the majority (74%) o f h e m a t o m a s occur on the superior and anterior aspect of the right lobe of the liver. Only 11% of hematomas are confined to the left lobe. ~ Exogenous trauma, although implicated in the majority of liver ruptures outside of pregnancy, has not been documented frequently in pregnant women with liver rupture. Some investigators, however, suggest that even minor increases in intraabdominal pressure associated with emesis, parturition, and patient transport may be sufficient to cause rupture of a preexisting subcapsular hematoma. ~0"2~ Clinical Presentation Henny et al ~ describe the formation of a liver hematoma and rupture as a biphasic disease con-
Table 1. Differential Diagnosis of RUQ Pain in Pregnancy Obstetric Ruptured uterus Labor Abdominal pregnancy Fetal movements/rib pain Gynecologic Adnexal torsion Rupture of adnexal cyst Gastrointestinal Liver hematoma/rupture Cholelithiasis/cholecystitis/cholangitis Hepatitis Pancreatitis Gastritis Peptic ulcer Crohn's disease Cardiac Myocardial infarction Pericarditis Pulmonary Pulmonary embolus Pneumonia Genitourinary Pyelonephrifis Nephrolithiasis
sisting of a prodromal period that can last up to a month, followed by an acute phase when the actual rupture occurs, which quickly leads to maternal cardiovascular collapse and death. The prodrome is often accompanied by vague systemic complaints of headache and malaise or specific gastrointestinal symptoms including nausea, vomiting, and jaundice. Sometimes, but not predictably, there will be hypertension and proteinuria present at this stage of the illness. However, by the time a hematoma or rupture is diagnosed, 80% to 90% of patients manifest signs and symptoms of preeclampsia. Jaundice and transaminitis may be present but are not consistently seen before hematoma formation. Many investigators refer to a triad of preeclampsia, right upper quadrant (RUQ) pain, and the acute onset of profound hypotension and shock. 21 Nevertheless, the diagnosis often is made retrospectively at the time of laparotomy; until then, the differential diagnosis includes a myriad of etiologies including obstetric (ruptured uterus, adnexal torsion), gastrointestinal (appendicitis, cholecystitis, pancreatitis, perforated ulcer, or viscus), and cardiac (myocardial infarction, Table 1).
Liver Hematoma and Rupture in Pregnancy
Patients may present at any time during the pregnancy or puerperium, but the majority are in the third trimester at the time of diagnosis. O f the 75 patients reviewed by H e n n y et al9 in 1983, 82% were in the third trimester. Up to 15%, however, have been reported to occur in the immediate postpartum period, usually within 24 hours of delivery. 22 T h e r e may be no antecedent abdominal trauma before rupture of a preexisting subcapsular hematoma, thus, the frequently used appellation spontaneous is given to these ruptures. It may be that the trauma that induces rupture in these patients often goes unrecognized as such. Nevertheless, once rupture of Glisson's capsule occurs, clinical deterioration usually is rapid, and often fatal. Patients complain of severe pain, present with signs and symptoms of shock, and there is usually evidence of fetal distress, either from p r o f o u n d maternal hypotension or concomitant abruption. A progressive fall in hematocrit level combined with abdominal distension will then lead to a laparotomy, which confirms the diagnosis. A subcapsular h e m a t o m a with its attendant symptom of R U Q pain, perhaps caused by capsular stretching, may develop in some patients, but they do not then progress to liver rupture. It is not clear how these patients differ from the ones who experience rupture and its devastating consequences. They may represent a unique group o f patients with spontaneous liver h e m a t o m a who can be treated more conservatively.
Diagnosis As stated earlier, the diagnosis of liver h e m a t o m a and rupture is often made at the time o f laparotomy for an acute a b d o m e n in a patient who has presented with R U Q pain and preeclampsia, in whom then p r o f o u n d hypotension, shock, and evidence o f intraabdominal h e m o r r h a g e develops acutely. Sometimes, a cesarean section is being p e r f o r m e d for fetal distress with an incidental finding of h e m o p e r i t o n e u m that is not originating from the uterus. In these two situations the clinical scenario will dictate the necessity to proceed to the operating r o o m with little n e e d for definitive diagnostic tests. However, an early suspicion of liver h e m a t o m a may allow for diagnosis before a catastrophic rupture. There-
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fore, multiple diagnostic modalities are available. Unfortunately, patient history, physical examination, and simple laboratory tests are insufficient to confirm a diagnosis of liver h e m a t o m a or rupture. Elevated liver function tests and thrombocytopenia tell more about the severity of the preeclampsia and, perhaps, show an increased risk for liver hematoma, but are not definitive. 14 Computerized tomography (CT) scanning provides the highest degree of diagnostic accuracy in assessing a patient for a subcapsular hematoma. With contrast, very small amounts of intraparenchymal and subcapsular h e m a t o m a can be recognized. T h e drawbacks of CT scanning are obvious: it is costly and may be impractical for a patient who requires intensive monitoring. For these reasons, many investigators ~ r e c o m m e n d ultrasonography (US) as a frst-line imaging study. T h e advantages of US over CT include its ease o f performance (a scan can be obtained at the bedside) and lower price compared with CT, while sacrificing little accuracy in detecting subcapsular bleeding and intraperitoneal blood. Acute subcapsular bleeding will appear as sonolucency overlying the liver parenchyma. However, as Goodlin et al ~ point out in their review, US may not detect subcapsular bleeding until liquefaction of the blood clot has occurred and the h e m a t o m a is greater than 2 cm in diameter. Manas et al24 c o n c u r r e d with this view in 1985, finding in their "experience, ultrasound examinations were unreliable." In their study, two of seven patients had normal US findings despite abnormal CT scanning. T h e lesson to be drawn here is that an US finding o f a liver h e m a t o m a is specific, but that the negative predictive value of the test may be slightly worse than that o f CT. Others have used hepatic angiography, ~5'26 paracentesis, is and radionuclide liver scans to make a diagnosis o f liver h e m a t o m a or rupture. Paracentesis, or peritoneal lavage, can certainly help to distinguish ascitic fluid from blood and has a low (0.5%) complication rate cited in the surgical literature. However, diagnostic lavage is invasive and perhaps contraindicated in anteparturn patients, so some investigators prefer CT to make this distinction if possible74 Liver scanning with 99mTechnicium can visualize small hematomas, is safe in pregnancy, and is cheaper than CT, but has the disadvantage of not being able
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to identify a cause for the h e m a t o m a or to accurately tell if the capsule is intact. 27 Hepatic artery angiography has the advantage of providing a means of embolotherapy in a stable patient with an expanding h e m a t o m a or, paradoxically, in a patient too ill to go to surgery who continues to bleed from a liver rupture. T h e disadvantages of angiography include the high radiation dose m limiting its use to postpartum p a t i e n t s - - a n d its inability to adequately treat venous hemorrhage. We believe that both CT scanning and US will continue to play the major role in the diagnosis of the stable patient with a suspected subcapsular liver hematoma. However, these "stable" patients are the exceptions rather than the rule; the majority of patients will be sick e n o u g h to necessitate surgical exploration, obviating the n e e d for any radiological intervention. As stated by Sherbahn 2s in 1996, " . . . because of the rarity of the condition, the varied presentation and the often rapid progression, the diagnosis will continue to be made at laparotomy in many cases." If the clinical suspicion of rupture is high, one should not hesitate to proceed directly to surgery, because a delay in surgery has been implicated in the extremely high mortality rate of this condition. 14'29
Treatment and Management Options The care of patients with liver rupture in pregnancy can be quite challenging and should be approached through a multidisciplinary effort o f surgeons, perinatologists, and intensivists. Initial treatment of patients with presumed liver rupture should be volume resuscitation en route to the operating room; multiple large bore peripheral intravenous lines remain the mainstay of resuscitation access. The surgical exploration should be carried out through a midline abdominal incision, often extending from the xiphoid to the symphysis pubis to allow sufficient access to the liver and uterus. Thoracoabdominal extension may be required to achieve adequate mobilization of the liver; this is particularly true for right-sided injuries. Often, the intrinsic liver disease in these patients can h a m p e r the surgeons' exposure efforts. If the patient is pregnant at the time o f laparotomy, a cesarean section may be p e r f o r m e d first, if there is fetal distress, followed by exploration of the u p p e r abdomen. If there is no evi-
dence of liver rupture and no visible hematoma, the a b d o m e n can be closed, and the patient observed. Some investigators 2 r e c o m m e n d observation of small intact hematomas discovered at laparotomy, but suggest evacuation o f larger hematomas to control bleeding sites and prevent catastrophic rupture. Because there are no data from randomized trials to show an advantage to either protocol, both are acceptable d e p e n d i n g on surgeon preference. A variety o f surgical techniques have been described in the literature to treat h e m o r r h a g e from a r u p t u r e d liver. 2'26'3~Rapid control of hemostasis is of utmost importance and can be accomplished initially through direct pressure on the rupture site with sponges or with temporary compression o f the porta hepatis (Pringle maneuver31). This initial packing maneuver is critical if the discovery of hepatic h e m o r r h a g e is made by a surgeon who is not experienced in the m a n a g e m e n t of major hepatic injury. This is also true in the event of hemodynamic instability in which tight packing of the RUQ allows the anesthesiologist the opportunity to completely resuscitate the patient. Patients are often coagulopathic and hypothermic because o f the massive blood loss and hepatic dysfunction, so care must be taken to replace clotting factors and platelets while treating the blood loss with volume expansion and transfusions of packed red blood cells. No attempt should be made to repair the injury until resuscitation is complete if hemostasis can be temporarily achieved through manual compression. T h e Pringle maneuver is accomplished by placing a noncrushing vascular clamp across the porta hepatis (Fig 1). This provides temporary occlusion of the hepatic artery and portal vein. Hepatic blood flow is reduced to about 10% of normal values, but bleeding from the hepatic veins is not controlled by this action. 32 T h e clamp can be left in place for as long as 1 hour. It should not be placed during the period of manual compression because total hepatic inflow occlusion for an extended period may result in increased mortality. 3a With adequate exposure and visualization, specific bleeding sites can be controlled with sutures (2-0 chromic, 0 chromic, or 2-0 silk on a blunt needle), electrocautery, the argon laser, and a n u m b e r of topical agents used to p r o m o t e hemostasis such as Gelfoam, Avitene, or Surgi-
Liver Hematoma and Rupture in Pregnancy
i C._'-/=::~.....
Figure 1. The Pringle maneuver: compression of the hepatoduodenal ligament (porta hepatis). Reprinted with permission.5~
cel. Direct suturing is often difficult because of the consistency of the liver tissue, which has been disrupted by hemorrhage and hepatocyte necrosis. Recently, fibrin glue has been described as a useful hemostatic adjunct. ~ Although more commonly used in the repair of splenic injuries, absorbable polygalactin mesh has been placed perihepatically to control hemorrhage in trauma patients. ~5 The extent of the laceration, the n u m b e r of bleeding sites, and the accompanying coagulopathy may make absolute hemostasis difficult to achieve. The choices facing the surgeon at this point include temporary packing with sponges to be removed in 48 to 72 hours when the patient is more stable, packing with o m e n t u m or rectus muscle, hepatic artery ligation or embolization, and hepatic lobectomy. None of these options is ideal, and the surgeon's choice will depend on the clinical scenario. Hepatic artery ligation, a6 although reasonable in a trauma patient, s7 cannot be recommended except as a last resort because this procedure seems to be poorly tolerated in the pregnant woman who may have withstood significant insult to the liver. 1~ Moreover, the liver changes in preeclampsia are reversible, thus hepatic resection should be avoided if possible. 2 Finally, although Hunter et al aa described a successful liver transplant in a patient who required a total hepatectomy after a spontaneous liver rupture, this heroic intervention should be reserved for patients with truly extensive liver damage whose only hope for survival is to be rendered anhepatic and await an allograft. Adequate drainage is crucial in preventing
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the formation of infected hematomas and abscesses as well as in assessing the extent of ongoing hemorrhage. Most investigators recommend the use of closed drainage systems with bores large enough to avoid blockage and allow egress of clots. Intensive care unit observation will likely be necessary because these patients often remain quite unstable postoperatively. They are at risk for the many complications of massive blood loss and volume replacement including adult respiratory distress syndrome, infection, and multiorgan failure. In addition, patients who sustain major liver injury are at risk for profound hypoglycemia, which can be avoided by providing 10% dextrose solutions perioperatively. The postoperative nutritional needs of these patients also require particular attention because of the hepatic injury. Fever is c o m m o n for the first several postoperative days even in the absence of infection; it is thought that inflammatory mediators are responsible, s9 Finally, hypothermia may occur intraoperatively and postoperatively and must be corrected. At temperatures below 35~ patients have been shown to experience a reversible platelet dysfunction manifested by increased bleeding times. 4~ In Hibbard's series of eight patients, the mean volume of transfused packed red blood cells was 4,500 mL. s In 1995, Stevenson and Graham 26 reported a case that required 22 units of packed cells, 13 units of cryoprecipitate, 31 units of fresh frozen plasma, and 30 units of platelets. That same year, at our own institution, we had a padent who received 116 units of packed cells, 103 units of plasma, 14 units of cryoprecipitate, and 26 units of platelets. Such enormous volumes of transfused blood products can challenge the resources of any hospital's blood bank. Until recently, a diagnosis in pregnancy of a subcapsular liver hematoma without rupture mandated surgical exploration and evacuation. In part, this was based on early reports of extremely high (96%) mortality in women not undergoing laparotomy. 9 In 1971, Utley41 wrote, "It cannot be determined if there exists a mild, but not fatal form of hepatic rupture during pregnancy that does not require operation." Moreover, outside of pregnancy, a liver hematoma implied a significant, traumatic rupture of Glisson's capsule necessitating surgical intervention. However, because the mechanism of hematoma formation is not the rupture itself, and because
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Table 2. Liver Rupture in Pregnancy: Maternal and Fetal Mortality
Study, Year
No. of Cases
Maternal Mortality (%)
Abercrombie, 1 1844 Rademaker, 15 1943 Kramish et al, 51 1954 Pavlic and Townsend, ~2 1962 Hakim-Elahi, s~ 1965 Castafieda et al, ~3 1970 Utley, 41 1971 Bis and W a x m a n , 22 1976 Henny e t al, 9 1983
1 2 14 17 61 51 31 91 75
100 50 64 71 80 71 61 59 56-75
the early reviews were likely biased against those patients who did not have surgery (perhaps because they were too sick to withstand exploration), conservative m a n a g e m e n t ~4'27 of unruptured h e m a t o m a s was r e p o r t e d a n d accepted in the early 1980s. Similarly, there has b e e n greater attention recently in the surgical literature to nonsurgical m a n a g e m e n t of o t h e r types o f liver injuries. 42~4 Conservative m a n a g e m e n t should be reserved for those patients who are: (1) Clinically stable, (2) Postpartum, and (3) Do not have a diagnosis o f preeclampsia. In the setting o f preeclampsia, the risk to b o t h the fetus and m o t h e r is too high to warrant conservative m a n a g e m e n t before delivery. Thus, i f a preeclamptic patient is p r e g n a n t at the time of diagnosis of a subcapsular hematoma, this should p r o m p t an immediate cesarean section t h r o u g h a midline incision with visual inspection of the liver to rule out rupture. O n e m i g h t argue for a delay in therapy at extremely early gestational ages to allow for the administration o f corticosteroids to prevent fetal p u l m o n a r y and neurological morbidity. However, given the e x t r e m e morbidity and mortality of an actual rupture, this c a n n o t be recomm e n d e d . I m m e d i a t e exploration and delivery by cesarean section should remain the standard of care. It may be that p r e g n a n t patients without preeclampsia in w h o m liver h e m a t o m a s develop, eg, a t r a u m a victim, may be treated m o r e conservatively if hemodynamically stable; however, little data exist in the literature to support or refute this stance.
Prognosis Unfortunately, liver h e m a t o m a a n d rupture during pregnancy is associated with a high maternal and fetal mortality rate (Table 2). H e n n y et al 9
Fetal Mortality (%)
Not Not Not Not
0 50 Reported Reported Reported Reported 71 62 62-77
r e p o r t e d a fetal mortality rate of 62% to 77% and a maternal mortality rate o f 56% to 75%. T h e majority o f maternal deaths are caused by complications of massive h e m o r r h a g e a n d coagulopathy. T h e fetal deaths are caused by a combination o f maternal hypotension, abruption, and prematurity. Advances in intensive care, radiology, blood p r o d u c t administration, and h e m o static agents have led to better outcomes over the past few decades, but the case fatality rate and associated morbidity remain high. T h e r e have b e e n a few reports in the literature of future p r e g n a n c y o u t c o m e in patients who have had a history of liver rupture. Several have r e p o r t e d successful p r e g n a n c y outcomes in these women. 4~47 However, Greenstein et a148 in 1994 r e p o r t e d a case of r e c u r r e n t intrahepatic h e m o r r h a g e in a patient whose first pregnancy was unremarkable; her second pregnancy was complicated by H E L L P syndrome with a subcapsular h e m a t o m a diagnosed by CT a n d m a n a g e d conservatively. In her third pregnancy, she develo p e d r e c u r r e n t preeclampsia and a p o s t p a r t u m h e m a t o m a that required surgical intervention despite early diagnosis with US and CT and a trial of embolization. Given that this is a disease of older multiparas who may have significant morbidity associated with a h e m a t o m a a n d rupture, it is not surprising that there are so few r e p o r t e d cases o f subsequent pregnancies in the literature. Because we know that severe preeclampsia, a n d in particular the H E L L P syndrome, often recur in subsequent pregnancies, 49 w o m e n with a history of liver h e m a t o m a a n d rupture should a p p r o a c h the question o f future pregnancies with great caution.
Summary A flow chart for the evaluation a n d t r e a t m e n t o f a patient with a suspected liver h e m a t o m a is
Liver Hematoma and Rupture in Pregnancy
[SUSPECTEDLIVERR ~
EHgMATOMAINTACT OR NOT S E E N
RUFTIJRED HEMATOMA
147
]
BLOOD I ] PRODUCTS ] ,
,,
, - 7 o-,
,
.
I
I I
/
ANGIOGP.~blI I EMBOLIT~TIONI
s h o w n i n Fig 2. This is a rare diagnosis that s h o u l d b e c o n s i d e r e d i n a n y p a t i e n t with R U Q p a i n i n p r e g n a n c y , especially those with severe p r e e c l a m p s i a o r the H E L L P s y n d r o m e . I n the stable p a t i e n t , C T s c a n n i n g o r US will o f t e n confirm t h e diagnosis. However, a n y p a t i e n t with e v i d e n c e o f i n t r a a b d o m i n a l h e m o r r h a g e o r fetal distress d e m a n d s i m m e d i a t e surgical exploration. C o n s u l t a t i o n with a g e n e r a l s u r g e o n well versed i n h e p a t i c a n a t o m y a n d t r a u m a m a n a g e m e n t is i n t e g r a l to the i n t r a o p e r a t i v e care o f these patients, a n d t h e i r postoperative care o f t e n r e q u i r e s intensive care a n d a m u l t i d i s c i p l i n a r y approach.
References 1. AbercrombieJ: Case of hemorrhage of the liver. London Med Gaz 34:792, 1844 2. Smith LG, Moise KJ, Dildy GA, et al: Spontaneous rupture of liver during pregnancy: Current therapy. Obstet Gynecol 77:171-175, 1991 3. Hibbard LT: Spontaneous rupture of the liver in pregnancy: A report of eight cases. Am J Obstet Gynecoi 126:334-338, 1976 4. Weinstein L: Syndrome of hemolysis, elevated liver enzymes, and low platelet count: A severe consequence of hypertension in pregnancy. Am J Obstet Gynecoi 142:159-167, 1982 5. Moen MD, Calinedo MJ, Marshall W, et al: Hepatic rupture in pregnancy associated with cocaine use. Obstet Gynecol 82:687-689, 1993 6. Baird JN, Hawley RG: Spontaneous rupture of the liver during pregnancy. J Repro Med 6:93-95, 1971
Figure 2. Management of a suspected liver hemat o m a / r u p t u r e . Adapted and reprinted with permission2.
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