November 2015

A 55 year-old man found dead at home

Janice Ahn, MD, Joseph DelTondo, DO

Overview

A 55 year-old male was found dead at home. His only medical history was chronic ethanol abuse. He lived alone and was found on the floor by a family member who checks on him daily. Though he was found on the floor, there was no evidence of a fall. Scene investigation revealed alcoholic beverages scattered about the living room and dark tarry stool in the bathroom toilet.

Gross Description

External examination

The body was received in a good state of preservation. Slowly blanching livor mortis was over the posterior aspects of the body. The decedent appeared the measured BMI of 30. He was medium-framed and had a distended abdomen with a prominent fluid wave on palpation. The lower extremities had mild pitting edema. There were no signs of external trauma.

Autopsy

The peritoneal cavity had a measured 2500 ml of dark red mostly liquid blood with scant clotted blood in the paracolic recesses and dependent portions of the pelvis. The omentum at the hepatic flexure had a small area of dilated tortuous vessels. They had no definitive defects or signs of bleeding (Figure 1). No other collateral plexi had vascular abnormalities. The liver weighed 1680g and was firm with a micronodular surface and intact capsule (Figure 2). The cut surfaces were firm and micronodular with several centrally-located areas of necrosis (largest 1.0 cm in diameter). The pancreatic cut surfaces had a small calcified pseudocyst in the tail and lace-like saponification. There was no evidence of hemorrhagic pancreatitis. The spleen weighed 420 g and had an intact capsule. The cut surfaces were soft and congested. The large intestine had dark tarry stool and no source of bleeding. The remainder of the gastrointestinal tract, kidneys, mesentery, retroperitoneum, anterior abdominal wall, and psoas muscles had no source of bleeding or hematomas. The anterior serosal surface of rectum had a small epiploic nodule, which was submitted for microscopic examination. The aorta and its branches had no aneurysms or defects.The vena cavae were unremarkable.

The cardiovascular examination revealed a 440g heart with 50% atherosclerotic luminal narrowing of the left main coronary and left anterior descending coronary arteries. The left circumflex coronary artery had 10% atherosclerotic luminal narrowing. The right coronary artery and its branches were entirely patent. The left ventricular chamber was dilated, though rigor mortis was passing at the time of examination, and the left ventricle was hypertrophied. The great vessels had no defects and the thoracic examination was otherwise noncontributory to the etiology of prominent hemoperitoneum.

Figure 1. Colon and mesentery at hepatic flexure. Note dilated veins within the mesentery at the inferior portion of the mesentery.
Figure 2. Cut surface of the liver demonstrating marked micronodular tan-yellow parenchyma.
Figure 3. Dilated veins from the hepatic flexure omentum.
Figure 4. High power view of a dilated vein.
Figure 5 A&B. Elastin stained sections highlighting dilated veins with ill-defined elastic laminae staining contrasted with well-defined staining of elastic laminae of adjacent arteries.
Figure 6. Staining of dilated vein (top) with SMA highlighting a thin haphazard media layer. Note the comparison of the dilated vein with the adjacent smaller artery (bottom), which has a well-defined muscular media layer.

Microscopic

Microscopic examination revealed dilated tortuous veins with no definitive defects and smaller caliber arteries (Figure 3-4). Staining with elastin revealed ill-defined staining of the venous elastic laminae and uniform well-defined staining of the elastic laminae in adjacent arteries (Figure 5). Staining with Smooth Muscle Actin (SMA) highlighted a loosely arranged thin medial layer of smooth muscle in the dilated veins. The adjacent smaller arteries had a thicker tunica media with a more organized arrangement of smooth muscle fibers (Figure 6).

Figure 7 A. Well-circumscribed nodule with adjacent cirrhosis. B. Higher power view.

The liver had generalized macro and micronodular steatosis and bands of bridging fibrosis (Figure 7). The grossly necrotic areas were well-circumscribed nodules containing large haphazard hepatocytes and necrotic debris which, though autolyzed, appeared distinct from those in the adjacent liver parenchyma. These lesions are best characterized as dysplastic nodules in the setting of cirrhosis due to their circumscription, small size, lack of invasive architecture, and setting. The microscopic pattern of the pancreatic pseudocyst was consistent with its gross diagnosis. The rectal epiploic nodule was unremarkable soft tissue.

Toxicological analysis revealed intoxication by ethanol. No drugs were detected in the blood.

In summary, the decedent was a 55 year-old male with prominent hemoperitoneum, sequelae of chronic alcohol abuse, acute ethanol intoxication, and no definitive source of intra-abdominal hemorrhage. The cause of death was therefore attributed to idiopathic spontaneous peritoneal hemorrhage due to hepatic cirrhosis due to chronic ethanol abuse. The manner of death was natural.

Diagnosis

Which of the following can be attributed to the decedent's cause of death ?

Answer

Please select an answer above.

Discussion

Idiopathic Spontaneous Peritoneal Hemorrhage was first described in 1909 by Barber in a female patient who rapidly developed a distended abdomen with fluid wave and hemodynamic instability immediately post-partum. Exploratory laparotomy revealed no cause of bleeding, though it was opined that the bleeding was due to a ruptured pelvic vein. Several years later in 1931, Green and Powers coined the term “abdominal apoplexy” drawing from the original medical use of the term apoplexy, which refers to a sudden loss of bodily function due to intracerebral hemorrhage or stroke. Abdominal apoplexy, in the 1931 definition, included all non-traumatic causes of hemoperitoneum. Subsequent cases have allowed us to categorize them as such: hepatic (benign or malignant hepatic mass rupture), splenic (infection, hamartoma, congenital cysts), vascular (ruptured aneurysms, polyarteritis nodosa, ruptured varices), gynecological (ruptured ovarian cyst, ectopic pregnancy, ovarian neoplasms, ruptured gravid uterus, intercourse), hematologic (hemophilia, myeloproliferative disorders), inflammatory (hemorrhagic pancreatitis or tuberculous peritonitis), coagulopathic, or cryptogenic etiology [1, 2]. Interestingly, hypertension and arteriosclerosis are common findings regardless of etiology [3].

Most of the reported cases of Idiopathic Spontaneous Peritoneal Hemorrhage are written from a surgical or radiological standpoint, but there have been a handful of forensic reports. Dedouit summarized 5 forensic cases from 1987 to 2012 involving both male and female patients with a history of chronic alcoholism and autopsy findings of intraperitoneal blood over 2.5 L, cirrhosis, and no post-mortem identifiable cause of hemoperitoneum [4-6]. Three decedents were female and 2 were male with ages ranging from 34 to 44 years. Three decedents were found at home and one in the street. One patient died after hospital admission. As stated above, all had a history of chronic alcoholism. Only 1 had a positive post-mortem blood alcohol level. The livers were all enlarged and cirrhotic with weights ranging from 1700 g to 2525 g. These findings are reproduced in Table 1.

In this case, the decedent had evidence of hypertensive heart disease and arteriosclerosis. He also had hepatic cirrhosis as a result of chronic alcohol abuse. Unique to the present case, however, is the finding of a cluster of dilated veins in the hepatic flexure omentum. Though this would seem like the most likely source of a bleed there was no definite defect, hemorrhage, or hematoma associated with the area. There have been several reports of peritoneal hemorrhage secondary to portal hypertensive varices, but these cases uniformly demonstrate evidence of a recent bleed, whether by a vascular defect or soft tissue hematoma [7-9].

In conclusion, there are a number of causes of spontaneous peritoneal hemorrhage: hepatic, splenic, vascular, gynecologic, hematologic, inflammatory, and coagulopathic. Rarely, however, a source of bleeding may not be identified either surgically or post-mortem. These cases comprise the rare but documented cryptogenic cases of peritoneal hemorrhage of which both clinicians and forensic pathologists should bear in mind.

Table 1. Summary of published forensic cases of deaths due to massive idiopathic spontaneous intraperitoneal hemorrhage in association with liver cirrhosis [6]

References [1-8, 10-16]

  1. Hung, S.-W., et al., A potentially fatal mystery in acute abdomen: abdominal apoplexy. The American Journal of Emergency Medicine, 2006. 24(6): p. 740-741.
  2. Lucey, B.C., J.C. Varghese, and J.A. Soto, Spontaneous hemoperitoneum: Causes and significance. Current Problems in Diagnostic Radiology, 2005. 34(5): p. 182-195.
  3. Carr, S.R., Idiopathic spontaneous intraperitoneal hemorrhage: A clinical update on abdominal apoplexy in the year 2001. American Surgeon, 2001. 67(4): p. 374-376.
  4. DiMaio, V.J., Sudden, Unexpected Death due to Massive Nontraumatic Intra-abdominal Hemorrhage in Association with Cirrhosis of the Liver. The American Journal of Forensic Medicine and Pathology, 1987. 8(3): p. 266-268.
  5. Kim, H.J., Kim, Y.H., Chung, N.E., Seo, J.S., Unexpected death due to massive intraabdominal hemorrhage in association with liver cirrhosis. Korean Journal Legal Medicine, 2007. 31: p. 92-94.
  6. Dedouit, F., et al., Unexpected natural death secondary to intra-abdominal bleeding: Report of one idiopathic spontaneous intraperitoneal hemorrhage case. Forensic Science International, 2012. 214(1–3): p. e43-e46.
  7. Jhung, J.W. and T.S. Micolonghi, Ruptured mesenteric varices in hepatic cirrhosis: A rare cause of intraperitoneal hemorrhage. Surgery, 1985. 97(3): p. 377-380.
  8. Graham, A.N.J., P. McAleese, and R.J. Moorehead, Intraperitoneal Rupture of Ectopic Varices—a Rare Complication of Portal Hypertension. HPB Surgery, 1994. 7(4): p. 315-318.
  9. Hunt, J.B., et al., Intraperitoneal haemorrhage from anterior abdominal wall varices. Postgraduate medical journal, 1993. 69(812): p. 490-493.
  10. Barber, M.C., Intra-abdominal haemorrhage associated with labour. British Medical Journal, 1909. 2(2534): p. 203-204.
  11. Cawyer, J.C. and C.K. Stone, Abdominal Apoplexy: A Case Report and Review. The Journal of Emergency Medicine, 2011. 40(3): p. e49-e52.
  12. Green, W.T. and J.H. Powers, Intra-Abdominal Apoplexy. Annals of Surgery, 1931. 93(5): p. 1070-1074.
  13. Harbour, L.N., et al., Abdominal apoplexy: two unusual cases of hemoperitoneum. Proceedings (Baylor University. Medical Center), 2012. 25(1): p. 16-19.
  14. Kleinsasser, L.J., Abdominal apoplexy. Report of two cases and review of the literature. The American Journal of Surgery, 1970. 120(5): p. 623-628.