Diagnostic Algorithm in the Management of Acute Febrile Abdomen in Patients with Autosomal Dominant Polycystic Kidney Disease

Abstract

BackgroundAcute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Although criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments. Furthermore, distinguishing cystic from non-cystic complications remains problematic.DesignADPKD patients presenting with abdominal pain and/or fever between 01/2005 and 06/2015 were retrospectively identified in a systematic computerized billing database. CyH was defined as spontaneous intracystic density above 50 Hounsfield units on computed tomography (CT). CyI was definite if confirmed by cyst puncture, and probable if 4 criteria were met: 3-day fever, loin/liver tenderness, C-reactive protein (CRP) plasma levels >50mg/L and no CT evidence for CyH. Other episodes were grouped as inflammation of unknown origin (IUO).ResultsAmong a cohort of 173 ADPKD patients, 101 presented with 205 episodes of abdominal pain (n = 172) and/or fever (n = 33). 20 patients experienced 30 CyH, whereas 16 presented 23 episodes of definite (n = 11) or probable (n = 12) CyI. 35 IUO were observed in 31 patients. Clinically, fever was observed in 7% vs. 100% vs. 66% of CyH, CyI and IUO, respectively. Biologically, CRP cut-off at 70 mg/dl showed 92% sensitivity and 81% specificity in CyI diagnosis. Urine or blood cultures remained sterile in >90% of CyH, but were contributive in 53.4% of CyI and IUO, with a 74.2% prevalence for E. coli. Radiologically, ultrasounds, CT and magnetic resonance diagnosed CyI in 2.6%, 20% and 16.7% of cases, respectively. 18F-FDG positron-emission tomography (PET)/CT was done within a median period of 7 days post antibiotics, and significantly changed patient management in 71.4%.ConclusionsThis retrospective single-center series underscores the usefulness of clinical–fever–and biological–CRP–parameters, but emphasizes the limitations of bacteriological and radiological investigations in cases of acute febrile abdomen in ADPKD patients. 18F-FDG-PET/CT imaging may be helpful in such condition.

International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people

“…Cyst infection is a very rare complication of childhood ADPKD. If suspected clinically or on ultrasonography imaging, experience in adults suggests that 18 F-FDG-PET/computed tomography (CT) is superior to contrast CT or MRI to confirm the diagnosis and localize the infected cyst, but this approach can produce false-negative results 143,144 . Treatment of cyst infection has a high failure rate and requires long-term use of antibiotics 145 .…”

Liver cyst infection in kidney transplant patient with autosomal dominant polycystic kidney disease: Interest of PET/CT in diagnosis and treatment

“…Lantiga et al recently summarized diagnostic criteria for liver cyst infection: infection is « de inite » if con irmed by cyst content analysis showing bacteria or neutrophils, infection is « probable » if the four following criteria are concomitantly met: 3-day fever >38°C, liver tenderness, CRP plasma levels >50 mg/L and no CT evidence for cystic haemorrhage. Additional criteria are weight loss, recent instrumentation of biliary tract and increased white blood cell count [6].…”

18F-FDG PET/CT in Autosomal Dominant Polycystic Kidney Disease Patients with Suspected Cyst Infection

“…Given the lack of cyst aspiration and subsequent microbiologic testing for previously mentioned reasons, a composite reference standard based on the criteria of Sallée et al (5,(10)(11)(12)(13) was used for cyst infection. According to these criteria (5,10-13), hepatic or renal cyst infection was (likely) considered to be present when a patient met all 5 of the following criteria: fever exceeding 38.5°C for more than Other clinical criteria and follow-up were used as reference standard for diagnosing cyst infection on 18 F-FDG PET/CT.…”

“…Previous studies reported 18 F-FDG PET/CT to be useful in ADPKD patients (Table 1) (5,(10)(11)(12)(13)(14)(15). However, because of few included patients or unclear reporting of diagnostic power in these studies, there is still no consensus on the widespread use of 18 F-FDG PET/CT in this setting.…”