Wunderlich’s syndrome associated with anticoa­ gulant treatment, heart failure, and post-radiation vasculopathy (RCD code: I-O)

Abstract

Background Definition: Wunderlich syndrome (WS) is defined as spontaneous, nontraumatic haemorrhage into the renal subcapsular, and retroperitoneal spaces. It was described first by Carl Reinhold August Wunderlich in 1858 in "Grundriss der speciellen Pathologie und Therapie"[1]. Epidemiology: Rare disease, a total of 267 cases were reported from 1985 to 2016 [2], including 102 cases described since 2000 [3]. Aetiology and pathogenesis: Causes of WS can be divided into neoplastic, which are more frequent, and non-neoplastic [2]. Neoplastic causes: 1. Renal angiomyolipoma (AML)-a benign tumour of the kidney, is the most common cause of WS, found in 57-73% of cases of WS [4,5]. Frequency of AML in the general population is estimated to be between 0.2% and 0.6%, with a higher prevalence among women. In 20% of cases, AML coexists with tuberous sclerosis complex or pulmonary lymphangioleiomyomatosis. Usually, AML is an asymptomatic, incidental finding [6]. 2. Malignant primary renal tumours e.g. renal cell carcinoma [2]. 3. Metastases of malignant tumours to the kidney e.g. choriocarcinoma, gastric sarcoma [7]. Other causes:

Wielonarządowe powikłania odległe po radioterapii i chemioterapii chłoniaka Hodgkina

“…Arteries, especially of the smallest radius, exposed to radiation develop stenosis, occlusion and wall thickening [2], which can partially explain our patient's pulmonary hypertension, which also left heart disease contributed. It is worth mentioning radiotherapy is also one of the risk factors for vessel fracture [3]. Coronary artery disease associated with radiotherapy (the amount of radiation is the risk factor) is frequently dispersed and affects mainly arteries' initial parts [4], probably caused by endothelial damage and cytokines stimulating inflammation and fibrosis [2].…”

Wunderlich’s Syndrome in Covid-19 Patient

“…However, the use of heparin and other anticoagulant drugs is associated with the bleeding trend and occasionally major complications and death outcomes (4). The proportion of bleeding complications varies from 4.8% to 8% (5) and one of the rarest conditions from this spectrum is Wunderlich's syndrome (WS) which is defined as spontaneous, nontraumatic hemorrhage into the perirenal and retroperitoneal spaces (6). The etiology of this syndrome includes neoplastic and non-neoplastic causes such as vasculopathy, kidney diseases, and anticoagulant drugs (6)(7)(8).…”

“…The proportion of bleeding complications varies from 4.8% to 8% (5) and one of the rarest conditions from this spectrum is Wunderlich's syndrome (WS) which is defined as spontaneous, nontraumatic hemorrhage into the perirenal and retroperitoneal spaces (6). The etiology of this syndrome includes neoplastic and non-neoplastic causes such as vasculopathy, kidney diseases, and anticoagulant drugs (6)(7)(8). The golden standard for diagnosis of Wunderlich's syndrome is MDCT of abdomen and pelvis, together with the correlating clinical picture which includes Lenk's triade: an acute flank pain, flank mass, and hypovolemic shock (6,9,10).…”

“…The etiology of this syndrome includes neoplastic and non-neoplastic causes such as vasculopathy, kidney diseases, and anticoagulant drugs (6)(7)(8). The golden standard for diagnosis of Wunderlich's syndrome is MDCT of abdomen and pelvis, together with the correlating clinical picture which includes Lenk's triade: an acute flank pain, flank mass, and hypovolemic shock (6,9,10). The syndrome is rare, with only 102 cases described since 2000 (6,11,12).…”

“…The golden standard for diagnosis of Wunderlich's syndrome is MDCT of abdomen and pelvis, together with the correlating clinical picture which includes Lenk's triade: an acute flank pain, flank mass, and hypovolemic shock (6,9,10). The syndrome is rare, with only 102 cases described since 2000 (6,11,12).…”