Liver transplantation in a child with liver cirrhosis caused by langerhans cell histiocytosis: a case report

Background Langerhans cell histiocytosis (LCH) is a rare condition that has a variety of clinical manifestations. But LCH in children localized only in the hepatobiliary system is unusual. Case presentation. Here we reported a rare case of a 2-year-old boy who was serendipitously found to have elevated liver enzymes while undergoing treatment of a perianal abscess. After a period of earlier conservative treatment in another hospital, the perianal abscess had resolved but the levels of liver enzymes were still rising slowly. The child was then referred to our institution for a definitive diagnosis. After laboratory tests, imaging and pathological examinations, a diagnosis of liver cirrhosis and sclerosing cholangitis was established, although the cause was unclear. Subsequently, living-donor liver transplantation was performed due to deterioration in liver function. Following successful liver transplantation, a diagnosis of LCH localized only within the hepatobiliary system was finally confirmed, based on additional pathological and imaging investigation. Additionally, the BRAF V600E mutation in this patient was also confirmed. The child has now recovered without evidence of LCH recurrence. Conclusions LCH localized only within the hepatobiliary system is unusual. The presence of unexplainable sclerosing cholangitis and liver cirrhosis in any child should raise the suspicion of LCH.

Multiple Secondary Healthcare-Associated Infections Due to Carbapenem-Resistant Organisms in a Critically Ill COVID-19 Patient on Extensively Prolonged Venovenous Extracorporeal Membrane Oxygenation Support—A Case Report

Patients with severe Coronavirus disease 2019 (COVID-19) are at high risk for secondary infection with multidrug-resistant organisms (MDROs). Secondary infections contribute to a more severe clinical course and longer intensive care unit (ICU) stays in patients with COVID-19. A man in his 60s was admitted to the ICU at a university hospital for severe COVID-19 pneumonia requiring mechanical ventilation. His respiratory condition worsened further due to persistent bacteremia caused by imipenem-non-susceptible Klebsiella aerogenes and he required VV-ECMO. Subsequently, he developed a catheter-related bloodstream infection (CRBSI) due to Candida albicans, ventilator-associated pneumonia (VAP) due to multidrug-resistant Pseudomonas aeruginosa (MDRP), and a perianal abscess due to carbapenem-resistant K. aerogenes despite infection control procedures that maximized contact precautions and the absence of MDRO contamination in the patient’s room environment. He was decannulated from VV-ECMO after a total of 72 days of ECMO support, and was eventually weaned off ventilator support and discharged from the ICU on day 138. This case highlights the challenges of preventing, diagnosing, and treating multidrug-resistant organisms and healthcare-associated infections (HAIs) in the critical care management of severe COVID-19. In addition to the stringent implementation of infection prevention measures, a high index of suspicion and a careful evaluation of HAIs are required in such patients.

Atypical Presentation of Adenosine Deaminase Deficiency ADA2 Deficiency

Here we report on a challenging diagnosis of VIAHS in a patient who presented at a very early age with a perianal abscess with fistula formation, fever, aphthous ulcers, bicytopenia, and hematochezia. Marked phenotypic variability can occur, and screening for families should be initiated in those with ADA2 mutation.

A Case Report: Diabetic Ketoacidosis in Patient with type 1 Diabetes Mellitus with Complication Septic Shock and AKI Stage III on HD Triggered by Perianal Abscess

Introduction. Diabetic ketoacidosis (DKA) is an acute, life-threatening complication in diabetes mellitus. Infection is a common precipitating cause of diabetic ketoacidosis (DKA) in known diabetic patient, and diabetic ketoacidosis (DKA) often presents as the first symptom of an undiagnosed diabetes. diabetic ketoacidosis (DKA) is diagnosed with combination of hyperglicaemia, acidosis metabolic and ketonuria. Case Presentation. A 27 years old male patient, admitted to Intensive Care Unit with decrease level of consciousness (GCS 3), he was intubated and present with respiratory distress, metabolic acidosis, high glucose level, ketonuria with renal failure as a target organ. Patient known has perianal abscess as a triggered of diabetic ketoacidosis (DKA), turn into septic shock and underwent debridement surgery to source control the infection. The patient was treated in intensive care unit for 9 days, and sent to ward with GCS 15 an no sequelae of organ failure. The treatment of diabetic ketoacidosis (DKA) should include correcting the often substantial hypovolemia, the hyperglycemia, electrolyte imbalance and the triggering factor of diabetic ketoacidosis (DKA). Conclusion. Prompt surgical intervention, antibacterial therapy, rapid restoration of glycemic control are crucial to prevent mortality in diabetes mellitus patients complicated with abscess.

542 Ceftriaxone-induced Kounis syndrome in the time of COVID-19

A 53 years old male subject with diabetes mellitus, hypertension, dyslipidaemia, obesity, and history of perianal abscess was admitted to the local hospital for generalized maculopapular rash on his trunk and limbs, which was accompanied by intense itching, sweating, hypotension, and severe chest pain. The rash and the accompanying signs/symptoms appeared 10 min after the administration of ceftriaxone (2 g) as antibiotic therapy for the perianal abscess. The patient had no clinical history for any type of allergy. At the first medical contact, an urgent electrocardiogram was taken showing ST-segment elevation in the anterior–lateral leads. The patient was still then treated with methylprednisolone and adrenalin i.v. as an anaphylactic shock was suspected. Afterwards, the patient was admitted in the emergency department, where he showed flu-like symptoms, chills, and fever. An echo-fast showed left ventricular wall motion abnormalities with hypokinesia of the anterior and posterior wall and moderate mitral regurgitation with normal EF. Laboratory tests showed increased levels of high-sensitivity cTnT (32.8 ng/l; NV < 14), white blood cells (13.74 × 103/μl; NV 5.2–12.4 × 103), IL-6 (10.54 pg/ml; NV < 7), C-reactive protein (PCR) (29.3 mg/l; NV 0–3). As for the cutaneous manifestations, flu-like symptoms, and blood test results (elevation of IL-6 and PCR despite an increase of white cell count) a SARS COV-2 swab was done. As recently noted in several preliminary studies, COVID-19 patients indeed show erythematous rash, and localized or widespread urticaria as initial manifestations in acute severe cases along with the humoural acute-phase response. The latter made it complicated to distinguish viral infection vs. drug administration as the underlying cause of the event. In the meantime, the patient started the treatment for an acute coronary syndrome and acetylsalicylic acid 100 mg, clopidogrel 300 mg orally, and enoxaparin dose subcutaneously were administered. Chest pain disappeared 30 min later and the ECG returned to normal 40 min after drug administration. Subsequently, the swab test result turned to be negative for SARS-CoV-2 and the patient was transferred to our centre for an emergency coronary angiography that revealed proximal subocclusive thrombotic stenosis and middle 70–80% thrombotic stenosis of the left anterior descending (LAD) coronary artery and a 80% thrombotic stenosis of the distal portion of the circumflex. Both vessels’ respective stenoses were treated with PCIs. When considering all together the anamnestic, laboratory, and instrumental/invasive findings, a case of Kounis Syndrome (KS) was suspected. Kounis syndrome (KS) has been indeed defined as cardiovascular symptoms that occur secondary to allergic or hypersensitivity insults mainly elicited by specific medications in male patients. KS involves the following three recognized variants: Type 1: the acute coronary event is secondary to spasm; Type 2: coronary thrombosis is the main culprit, and Type 3: the coronary event occurs secondary to drug-eluting stent thrombosis. Therefore, the patient was finally discharged with the diagnosis of ST-elevated MI likely secondary to a type II KS.

Efficacy and Safety of Thalidomide in Crohn’s Disease Patients with Perianal Fistula and Abscess

Background Medical therapies of parianal Crohn’s disease (CD) are limited. Thalidomide is an effective medical therapy to alleviate disease activity of CD. However, the effects and safety of thalidomide in the treatment of perianal fistula and abscess was not evaluated. Methods This retrospective cohort study was performed at a tertiary referral centre and recruited 73 patients with perianal CD who received thalidomide (50–100 mg) daily for 1 year. Data collected included demographics, medications, and disease behaviour. Clinical assessment of CD was conducted using the Crohn’s Disease Activity Index (CDAI), and perianal lesions were evaluated using the Fistula Drainage Assessment index and Perianal Disease Activity Index (PDAI). At the same time, the occurrence of adverse effects was recorded during treatment. Wilcoxon's signed-rank test and Student’s t-test were used to analyse the data. Results The CDAI score and laboratory indices were significantly lower after thalidomide treatment than at baseline (all P < 0.01). The value of PDAI was significantly lower in patients with symptomatic perianal abscess after thalidomide treatment than at baseline (10 [6.25, 10] versus 2.5 [1, 3.75]; P = 0.05). PDAI was also significantly reduced in all patients treated with thalidomide whether with or without perianal abscess drainage (all P < 0.05). The rates of responsive patients were similar between the thalidomide group and thalidomide combined with azathioprine group (72.73% [8/11] and 84% [21/25], respectively; P = 0.65). In total, 31% (24/77) of patients experienced adverse events, and interventions were required in 15 patients to reduce or eliminate discomfort from adverse events. Four patients discontinued thalidomide due to adverse effects. Side effects (rash, diarrhoea, peripheral neuropathy, somnolence, constipation, and numbness) were mild and mostly transient. Conclusions Thalidomide is effective in inducing clinical remission and response in CD patients with perianal fistula and abscess with or without abscess drainage. Thalidomide in combination with azathioprine is also effective in these patients. Low-dose thalidomide is proven to be effective and safe in treating perianal CD patients.

Coinfection of Clostridium perfringens and Escherichia coli in gas-producing perianal abscess diagnosed by 16S rDNA sequencing: a case report

Background Gas-producing perianal abscess raises the possibility of clostridial infection, with Clostridium perfringens being the most common causative agent, which is highly lethal if untreated timely. As the treatment of clostridial infections often differs from that of non-clostridial infections, which they may closely resemble, the importance of accurate pathogenic organism identification cannot be overemphasized. The 16S rDNA of bacteria is highly conserved within a species and among species of the same genus but demonstrates substantial variation between different species, thus making it a suitable genomic candidate for bacterial detection and identification. Case presentation Here, we report the case of a 53-year-old patient who was admitted to the hospital for a gas-producing perianal abscess. The patient was managed with ceftizoxime and ornidazole and then received debridement and drainage at the lesion on the second day after admission. The bacterial cultures of the patient isolates from the debridement showed a coinfection of Escherichia coli and Enterococcus faecium. Although perianal redness and swelling subsided obviously after the surgery, the patient was febrile to 38.3℃ with his left upper thigh red and swollen, aggravated with tenderness and crepitus. Considering insufficient debridement and the risk of incorrect identification of pathogens, a second debridement and drainage were performed 4 days after the primary operation, and 16S rDNA sequencing of the isolates implicated Clostridium perfringens infection. Given the discrepancies in diagnostic results and the treatment outcomes, Enterococcus faecium was identified as sample contamination, and a diagnosis of coinfection of Clostridium perfringens and Escherichia coli in gas-producing perianal abscess was confirmed. The patient was then successfully treated with meropenem and vancomycin and was discharged at 27 days of admission. Conclusions This case represents the first report of coinfection of both clostridial and non-clostridial organisms in gas-producing perianal abscess and the first case reporting the use of 16S rDNA sequencing in the diagnosis of perianal abscess. Timely pathogen identification is critical for treating gas-producing perianal abscess and an antibiotic regimen covering both aerobic and anaerobic organisms is recommended before true pathogens are identified.

TP9.2.6An update of the surgical management of truncal cutaneous abscesses: A systematic scoping review of current practice in 2021

Introduction NHS England has shown efficiency in patient flow and reduction of length of stay resulting in cost savings. There’s considerable variability in local and national guidelines for Incision and drainage (I&D) of cutaneous truncal abscesses which can financially impact NHS expenditure. This Systematic Scoping Review (SSR) aims to explore current management recommendations for I&D of cutaneous truncal abscesses in both adult and paediatric populations; and assess the quality of this research. Method SSR search strategy employed the PICO framework. Cochrane Library, NICE Evidence, Pubmed, OVID medicine, CINAHL and EBSCO databases were searched using search syntax ((“abscess*”OR“perianal abscess*”) AND (“incision and drainage”OR“I&D”) AND (“Equipment*”OR”instrument*”OR“Kit*”OR“theatre staff*”OR“team*”)). Full texts were systematically assessed. Final review performed in accordance with Joanna-Briggs-Institute protocol. Results 131 articles were identified. No limit was placed on publication date. After removal of duplicates and abstract screening, 10 full text articles were assessed. Research suggests day-case emergency surgery pathways to manage I&D of abscesses are efficient and sustainable. Antibiotic use after I&D is weakly recommended and there is low quality evidence for using internal packing for healing perianal abscess cavities, and yet, this is established, common practice. Conclusion There’s variable quality evidence reviewing the surgical pathways for I&D and the post-operative management of cutaneous truncal abscesses. Overall, the findings are limited and further work is required to establish national guidelines.

EP.FRI.535 A clinical audit of post-operative antibiotics prescription in uncomplicated perianal abscesses

Aims Management of perianal abscesses continues to revolve around prompt surgical drainage. The Association of Coloproctology of Great Britain and Ireland (ACPGBI) guidelines state that all patients should have incision and drainage within 24 hours and antibiotics are not indicated in routine uncomplicated perianal abscesses. We aimed to study the antibiotics prescription after surgical drainage in a London university teaching hospital against the national standard. Methods A single-centred retrospective analysis of all emergency surgical admissions for incision and drainage of perianal abscess was carried out for a 6 month period. Patients’ demographics, Co-morbidities, local and systemic complications and readmissions were studied. Results A total of 36 patients, (mean age 43, 64% males) were included in this study, 21 received incision and drainage without antibiotics prescription, while 15 received empirical post-operative antibiotics. Indications for antibiotic therapy in this group included diabetes, immunocompromise, local complications (necrosis, cellulitis) and recurrence. There was no clear indication for antibiotics in 60% of patients who received them. 86% of patients had surgical drainage within 24 hours of presentation. One patient was readmitted for a second drainage 3 months later. Most common empirical agent used was co-amoxiclav (53%), followed by (33%) combination of co-amoxiclav and metronidazole. Conclusion Although surgical drainage was generally carried out in timely manner according to guidance, there was excessive post-operative antibiotic prescriptions. Increase awareness of guidelines is required to improve antibiotic stewardship in these surgical patients in order to avoid unnecessary drugs’ prescription.