CASE 15664 Published on 04.05.2018

Teaching Case

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Tonolini Massimo, MD.

Luigi Sacco University Hospital,
Radiology Department,
via G.B. Grassi 74
20157 Milan, Italy.
Email:mtonolini@sirm.org

Patient

74 years, female

Categories

Area of Interest Spleen ; Imaging Technique CT ;

Procedure Diagnostic procedure ; Special Focus Metastases, Haemorrhage ;

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Clinical History

Woman with history (15 months earlier) of resected mesenterial pleomorphic sarcoma, followed (10 weeks later) by reoperation to relieve bowel obstruction from neoplastic implant on the ileal peritoneum. After completing adjuvant chemotherapy, she suddenly experienced left flank pain radiating to shoulder, syncope and hypotension. Laboratory revealed decreased haemoglobin (9 g/dl).

Differential Diagnosis List

Spontaneous (atraumatic) splenic rupture from solitary metastasis

Rupture secondary to systemic infection e.g. malaria

Mononucleosis

Endocarditis

Rupture secondary to splenic leukemia / lymphoma involvement

Idiopathic spontaneous splenic rupture

Spontaneous liver bleeding e.g. ruptured hepatocellular adenoma or carcinoma

Spontaneous renal bleeding e.g. from angiomyolipoma or cancer

Ruptured visceral aneurysm

Imaging Findings

Previously, oncologic follow-up CT (Fig. 1) had detected a solitary liver metastasis, which regressed after carboplatin+taxole chemotherapy (Fig. 2); meanwhile a 2.5-cm minimally exophytic splenic metastasis developed.
Currently, a month after treatment completion emergency CT (Fig. 3) showed haemoperitoneum (previously suggested at bedside ultrasound) and a sizeable, acute splenic haematoma with prominent contrast medium extravasation (isoattenuating with enhanced blood vessels) indicating non-contained active bleeding.
Emergency laparotomic surgery confirmed splenic rupture, and histology of splenectomy specimen and metastatic implant was consistent with the previously treated tumour.
Unfortunately, shortly after surgery postoperative CT (Fig. 4) showed the appearance of a lobulated subphrenic hypodense mass consistent with tumour recurrence in the surgical bed, which rapidly grew at subsequent follow-up (not shown).

Discussion

In patients with acute non-traumatic abdominal symptoms, the uncommon spontaneous splenic rupture (SSR) is seldom suspected by clinicians. This life-threatening condition (12-15% mortality) may develop secondary to a variety of causes, which encompass infections (particularly malaria, mononucleosis and endocarditis), malignancies (most usually haematologic and myeloproliferative disorders), coagulation disorders, haemodialysis, therapeutic anticoagulation, amyloidosis, acute pancreatitis and pregnancy in descending order of frequency. In nearly half of the patients, SSR represents the first manifestation of a previously unknown disease [1-3].
Due to its sporadic occurrence and heterogeneity of causes, the incidence, mechanism and treatment of SSR are poorly defined. Occurring in a wide age range, SSR generally manifests with hypotension and upper or left-sided abdominal pain, that may progress to generalised peritonism and haemodynamic shock. Radiologists should suggest SSR when faced with intra-abdominal haemorrhage, even in the absence of known diseases involving the spleen. Ultrasound is helpful as it rapidly detects haemoperitoneum, but has moderate sensitivity and specificity for the diagnosis of SSR [4]. In the vast majority of situations, multidetector CT represents the ideal modality to assess patients presenting with acute abdomen and haemodynamic instability. CT detects intra-abdominal haematomas and higher-than-water attenuation (30-45 HU) peritoneal effusion, reliably identifies the spleen as the injured organ, and detects active haemorrhage as serpiginous or jet-like non-contained contrast medium extravasation [5-7].
Different from liver and kidney haemorrhages, pathologic splenic bleeding is generally associated with splenomegaly and leukemic or lymphomatous parenchymal infiltration, rather than with the presence of a solitary tumour. As in the hereby reported patient, occasional SSR cases secondary to either primary splenic angiosarcoma [8-10] or metastases particularly from melanoma [11-13] have been described. Therefore, radiologists should search previous studies and current emergency CT studies for single or multifocal solid masses. Diagnosing malignant SSR is important since in these occurrences splenectomy remains the treatment of choice. Conversely, in non-malignant cases and in young patients there is an increasing trend towards non-operative management to preserve splenic function, including transcatheter arterial embolisation as necessary. Furthermore, along with splenomegaly and advanced age the presence of a neoplastic disorder represents a risk factor associated with increased SSR-related mortality [2, 7, 14].

References

[1] Aubrey-Bassler FK, Sowers N (2012) 613 cases of splenic rupture without risk factors or previously diagnosed disease: a systematic review. BME Emerg Med 12:11. (PMID: 22889306)

[2] Renzulli P, Hostettler A, Schoepfer AM, et al (2009) Systematic review of atraumatic splenic rupture. Br J Surg 96:1114-1121 (PMID: 19787754)

[3] Debnath D, Valerio D (2002) Atraumatic rupture of the spleen in adults. J R Coll Surg Edinb 47:437-445 (PMID: 11874265)

[4] Gorg C, Colle J, Gorg K, et al (2003) Spontaneous rupture of the spleen: ultrasound patterns, diagnosis and follow-up. Br J Radiol 76:704-711 (PMID: 14512330)

[5] Furlan A, Fakhran S, Federle MP (2009) Spontaneous abdominal hemorrhage: causes, CT findings, and clinical implications. AJR Am J Roentgenol 193:1077-1087. (PMID: 19770332)

[6] Lubner M, Menias C, Rucker C, et al (2007) Blood in the belly: CT findings of hemoperitoneum. Radiographics 27:109-125. (PMID: 17235002)

[7] Tonolini M, Ierardi AM, Carrafiello G (2016) Atraumatic splenic rupture, an underrated cause of acute abdomen. Insights Imaging 7:641-646 (PMID: 27193528)

[8] Alexandrino H, Juliao MJ, Tralhao JG, et al (2013) Rupture of splenic angiosarcoma: a rare cause of spontaneous haemoperitoneum. BMJ Case Rep 2013 May 24;2013. doi: 10.1136/bcr-2013-009748. (PMID: 23709556)

[9] Badiani R, Schaller G, Jain K, et al (2013) Angiosarcoma of the spleen presenting as spontaneous splenic rupture: A rare case report and review of the literature. Int J Surg Case Rep 4:765-767. (PMID: 23856255)

[10] Duan YF, Jiang Y, Wu CX, et al (2013) Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review.. World J Surg Oncol 11:53. (PMID: 23497454)

[11] Buzbee TM, Legha SS (1992) Spontaneous rupture of spleen in a patient with splenic metastasis of melanoma. A case report.. Tumori 78:47-48 (PMID: 1609460)

[12] Mirfazaelian H, Oryan A, Davari A, et al (2014) Spontaneous splenic rupture in melanoma.. Case Rep Pathol 2014:865453 (PMID: 24795827)

[13] Sajid MS, Howell P, Leaver C, et al (2012) Spontaneous splenic rupture secondary to metastatic malignant spindle cell tumour. Singapore Med J 53:e208-210 (PMID: 23112031)

[14] Hadary A, Dashkovsky I, Rapaport A, et a (2008) Non-traumatic rupture of spleen: can splenectomy be applied selectively?. Isr Med Assoc J 10:889-891 (PMID: 19160949)

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