American Journal of Forensic Medicine & Pathology:
Peliosis Hepatis Presenting as Liver Rupture in a Vulnerable Adult: A Case Report
Buelow, Ben MD, PhD*; Otjen, Jeffrey MD†; Sabath, Annette P. MD*; Harruff, Richard C. MD, PhD‡
From the Departments of *Pathology and †Radiology, University of Washington School of Medicine, and Harborview Medical Center; and ‡King County Medical Examiner’s Office, Seattle, WA.
Manuscript received July 1, 2011; accepted September 22, 2011.
The authors report no conflicts of interest.
Reprints: Richard C. Harruff, MD, PhD, King County Medical Examiner’s Office, 325 Ninth Ave, HMC Box 359792, Seattle, WA 98104. E-mail: email@example.com.
Abstract: Liver rupture is a serious, life-threatening event that is commonly due to blunt abdominal trauma, which should be suspected in a patient who is unconscious or unable to communicate. We report an autopsy case of a 28-year-old woman with severe developmental delay who presented to the emergency department with hemoperitoneum due to massive liver rupture and subsequently died without a diagnosis. An autopsy performed by the hospital pathology department confirmed hemoperitoneum due to hepatic rupture. The case was then referred to the medical examiner to exclude a traumatic etiology. After review of the clinical data, radiological images, and gross and microscopic pathological features, a diagnosis of peliosis hepatis was established. This rare entity has been reported previously as a cause of spontaneous, nontraumatic liver rupture and is reported here to demonstrate its characteristic features and potential to present as fatal hepatic rupture in circumstances in which occult injury must be excluded.
The liver is a the most commonly injured organ in blunt abdominal trauma, notably in cases of abuse.1–5 Therefore, hepatic injury without a clear etiology should raise the question of nonaccidental trauma, especially in vulnerable persons. In such cases, gross and microscopic pathology has significant medicolegal relevance, and a firm understanding of traumatic liver injury is crucial. This is particularly true in cases of hepatic rupture, which is associated with a high mortality secondary to intraperitoneal hemorrhage.1 In addition to traumatic etiologies, nontraumatic hepatic rupture has occurred secondary to hemangiomatosis, neoplasm, and the rare malformation, peliosis hepatis (PH).6 Thus, these entities should always be considered in a case of hepatic rupture without obvious trauma. The present report describes a case of fatal spontaneous hepatic rupture in a vulnerable adult, highlighting the need for an appropriate degree of suspicion and a comprehensive differential diagnosis when evaluating such lesions.
A 28-year-old woman with severe developmental delay, cerebral palsy, cortical blindness, and a remote history of seizure disorder, who was fully dependent on her caregivers, was transported by medics to the hospital after her mother found her groaning and unable to stand or sit. Seizures were noted during transport and upon admission to the emergency department. Initial blood pressure was 110/73 mm Hg and shortly thereafter dropped to 76/52 mm Hg. Hematocrit dropped from 22 to 9. Abdominal computed tomography (CT) scan showed multiloculated, low-density collections in the right lobe of the liver communicating with a capsular rupture and hemoperitoneum (Fig. 1, A–C); traumatic versus spontaneous rupture of a hepatic neoplasm was suspected. Transfusions and fluid therapy stabilized systolic blood pressures in the 70s to 80s range, and emergent embolization of the middle and right hepatic arteries was performed (Fig. 1D). A liver biopsy was performed and interpreted as showing focal, nonspecific hepatocellular necrosis with no evidence of neoplasm or other significant lesion (Fig. 2, A–C). Despite supportive therapy, respiratory and oliguric renal failure developed, and the patient died under comfort care on hospital day 4.
Consent for autopsy was obtained, and the postmortem examination was performed by the hospital pathology department. The autopsy confirmed hemoperitoneum with 2 L in the abdominal cavity. The liver weighed 2100 g. The right lobe of the liver was covered with a subcapsular hematoma 20 cm in diameter up to 5 cm thick that was in continuity with the hemoperitoneum through a distinct rupture site in the capsule (Fig. 3A). Sectioning revealed innumerable, irregular cavities filled with clotted blood occupying most of the right lobe and communicating with the subcapsular hematoma (Fig. 3, B and C). Microscopically, the lesion consisted of cystlike cavities without endothelial lining or fibrous walls, containing partially organized blood clot; the intervening parenchyma showed focal necrosis and sinusoidal dilatation. The portal tract architecture was unremarkable, and no neoplasm was identified (Fig. 3, D and E). The case was referred to the King County Medical Examiner’s Office to exclude the possibility of trauma. Finding no such evidence, the diagnosis of PH was established. In addition to the liver findings, the autopsy revealed microcephaly with cystic encephalopathy and urinary cystitis. The decedent had no known predisposing conditions for PH, clinically or anatomically.
This case report of fatal spontaneous hepatic rupture secondary to PH demonstrates an appropriate concern for occult injury and potential abuse in a vulnerable patient presenting with hepatic rupture, while simultaneously illustrating the need for a differential diagnosis including spontaneous rupture secondary to neoplasm, hemangiomatosis, or PH. These considerations are particularly relevant for the forensic pathologist, who is likely to become involved in such cases when there are suspicions of abuse. Pathological examination was crucial in the diagnosis of PH in this case. Although the history and physical examination gave no indication of abuse, occult injury could not be excluded by the clinical evidence alone. Furthermore, although the CT studies identified an intraparenchymal hepatic lesion as the source of the hemoperitoneum, the radiographic differential did not include PH and could not be narrowed beyond abscess, hemangioma, embryonal sarcoma, and mesenchymal hamartoma, nor could a traumatic contribution be excluded. The limitations of radiography in differentiating among diffuse hepatic parenchymal diseases have been described previously; even CT scan and angiography can miss the lesion entirely and may be no better than ultrasound in identifying PH.7–10 Gross and microscopic examinations were necessary to establish the diagnosis of PH and to exclude alternative diagnoses. The central role of pathological findings in establishing the diagnosis of PH and excluding injury in this case demonstrates that familiarity with PH is important for the practicing pathologist.
The gross and microscopic features of PH have been well characterized.11–13 Grossly, PH consists of numerous irregular, blood-filled cystlike spaces within the liver parenchyma 0.1 to 5 cm in diameter. As noted previously, PH most commonly involves the right lobe (as in this case), but cases involving both lobes have been reported.10,14 Histologically, 2 patterns of PH have been described. The “parenchymal” type consists of irregular, blood-filled, cystic spaces within the hepatic architecture, which may have scattered foci of necrosis but is otherwise normal. In this type, the cystic spaces are unlined by endothelial cells or fibrosis and do not communicate with the central vein or compress adjacent parenchyma. The “phlebectatic” type differs by having regular, centrilobular, spherical spaces lined by fibrosis or endothelial cells that communicate with sinusoids and compress the surrounding parenchyma.15–17 It has been suggested that the 2 types are part of a single disease spectrum, with fibrosis and endothelial lining of spaces (phlebectatic) downstream of dilatation and necrosis (parenchymal).18–20 Although the lesion is quite distinct histologically, the present report demonstrates that the lesion may be missed by biopsy. Furthermore, an important caveat in pursuing biopsy in suspected PH is that, even under ultrasound guidance, puncture of the lesion may lead to fatal hemorrhage21; accordingly, arterial embolization should be readily available during the procedure.
Peliosis hepatis is associated with chronic illnesses such as AIDS and tuberculosis,11,15,22 as well as chronic anabolic-, sex-, or corticosteroid treatment,10,23 but the underlying etiology is unknown. Toxic or viral insult has been postulated to induce hepatocyte necrosis with subsequent dilatation of the sinusoids and/or the space of Disse,10,15,20 although a mechanistic explanation remains elusive. The decedent described in the present report had none of the risk factors described above, although it is possible that one or more of her comorbidities represent an as yet unidentified risk factor for PH. In this regard, it is useful to point out that several reports have shown PH to develop without identifiable risk factors, and as illustrated by this case, a high index of suspicion is required to correctly diagnose PH.
In conclusion, this case illustrates that PH must be considered as a possible contributing factor in hepatic rupture, even in patients without identifiable risk factors. Furthermore, it highlights that careful gross and histological examination is required for an accurate diagnosis of the cause of hepatic rupture. This is particularly relevant for the forensic pathologist when there is suspicion of physical abuse. Finally, this case demonstrates the need for an appropriate level of concern for occult injury in a vulnerable individual and the benefits of close collaboration between hospital and forensic pathologists.
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peliosis hepatis; nontraumatic liver rupture; fatality; forensic pathology; autopsy
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