*Sławomir Glinkowski, Daria Marcinkowska
A patient with Crohn’s disease – from diagnosis to therapy onset. Therapeutic standards vs. reality – a case report
Pacjent z chorobą Leśniowskiego-Crohna – od rozpoznania do wdrożenia terapii. Standardy postępowania a rzeczywistość – opis przypadku
Department of General and Oncologic Surgery, Health Centre in Tomaszów Mazowiecki
Streszczenie
Choroba Leśniowskiego-Crohna należy do nieswoistych chorób zapalnych jelit. Pierwsze objawy bywają niespecyficzne, co może powodować trudności diagnostyczne. Cechą charakterystyczną choroby jest pełnościenne zapalenie ściany jelita, które może objąć każdy odcinek przewodu pokarmowego. Najbardziej charakterystyczną lokalizacją jest końcowy odcinek jelita cienkiego – ileum terminale, która wystąpiła także w opisywanym przypadku. Leczenie chirurgiczne powinno być jak najmniej inwazyjne ze względu na duże prawdopodobieństwo kolejnej interwencji chirurgicznej.
Autorzy prezentują przypadek pacjenta przyjętego na oddział po kilku incydentach ambulatoryjnego leczenia ropnia odbytu w USA. Na oddział chirurgiczny trafił z powodu rozwijającej się ropowicy krocza w przebiegu nawrotowego ropnia odbytu. Został zakwalifikowany do nacięcia ropnia. W trakcie pobytu na oddziale u pacjenta wystąpiły objawy krwawienia do przewodu pokarmowego. Wykonana diagnostyka endoskopowa jelita grubego ujawniła makroskopowe zmiany o typie choroby Leśniowskiego-Crohna. Wielokrotne próby przeniesienia pacjenta do jednostki zajmującej się leczeniem chorób zapalnych przewodu pokarmowego zakończyły się niepowodzeniem. Ze względu na pogarszający się stan ogólny, postępującą anemizację, chorego zakwalifikowano do operacji. Wykonano resekcję końcowego odcinka jelita krętego z ileostomią końcową. Po ustabilizowaniu stanu ogólnego, pacjenta wypisano z zaleceniem dalszego leczenia w poradni gastroenterologicznej, w ośrodku zajmującym się leczeniem nieswoistych chorób zapalnych jelit.
Summary
Crohn’s disease is a nonspecific inflammatory bowel disease. The first symptoms tend to be non-specific, which can cause diagnostic difficulties. Full-thickness inflammation of the bowel wall, which may involve any part of the gastrointestinal tract, is a landmark of this disorder. Terminal ileum is the most commonly affected area, as also described in the presented case. Surgical treatment should be as least invasive as possible due to the high probability of another surgical intervention.
We present a case of a patient previously repeatedly treated for anal abscess in an outpatient setting in the USA. The patient was admitted to the surgical ward due to perineal phlegmon in the course of recurrent anal abscess. He was qualified for abscess incision. During hospital stay, the patient developed symptoms of gastrointestinal bleeding. Diagnostic endoscopy of the large bowel revealed macroscopic lesions typical of Crohn’s disease. Repeated attempts to transfer the patient to a unit specialised in the treatment of inflammatory bowel diseases (IBDs) have been unsuccessful. Due to the deteriorating general condition and progressive anaemisation, the patient was qualified for surgery. The end section of the ileum was resected and an end ileostomy was created. After stabilisation of the general condition, the patient was discharged home having been recommended to continue treatment in a gastroenterology clinic, in a centre specialised in the treatment of inflammatory bowel diseases.
Introduction
The constant search for new therapeutic options and the possible adverse effects of long-term use of some therapies in Crohn’s disease make it an issue that is still eagerly discussed among gastroenterologists. The role of surgery in treating this disorder is also constantly discussed. Is there still a place for the surgical approach in the era of biological treatment? Is it reserved for abscesses, fistulas and strictures? Is CD always surgically treated in designated accreditation units?
In this paper, we present a case of a patient admitted to a district hospital at a time point when it was too late to initiate biological treatment without prior surgical intervention. We also discuss the key therapeutic principles, emphasising that it is not always possible to follow generally accepted standards.
Crohn’s disease (CD), along with ulcerative colitis (UC), belongs to the group of inflammatory bowel diseases (IBDs). IBDs are the second most common group of autoimmune disorders after rheumatoid arthritis (RA) (1). Although the pathogenesis of CD is still under investigation, it is currently assumed that multiple genetic factors, as well as environmental and individual aspects are involved. CD is a full-thickness inflammation of the bowel wall that can involve any part of the gastrointestinal (GI) tract, from the vestibule to the anus. Terminal ileum is the most commonly affected area, as also described in the presented case. Inflammatory lesions are described as granulomatous and non-caerating. These lesions are not continuous as in the case of ulcerative colitis (UC), but form microscopically inflamed sections separated by macroscopically normal appearing mucosa (skip areas). Endoscopically, a ‘cobblestone’ appearance of the mucosal surface is usually described. The most common clinical manifestations include abdominal pain (up to 90% of patients) and diarrhea (bloody or not). Treatment delay may result in malnutrition, hypoalbuminaemia, oedema, and complications involving other organs. Despite diagnostic advances and the increasing awareness among doctors, hospitalisation rates due to complications of the underlying disease, such as (sub)obstruction, external or internal fistulas, and abscesses, are still high. The case we describe shows that there is still too little vigilance among doctors for IBDs in diagnostically and therapeutically challenging patients. Thorough diagnosis and treatment will be discussed in more detail later in this article.
Case report
A 30-year-old male patient living in the USA was admitted to hospital after almost 4 months of treatment for perineal and perianal phlegmon in the USA. According to patient-reported medical history (unavailable medical records and data on previous treatment), the man was treated by a primary care physician due to a perianal lesion, possibly an abscess, by repeated thick-needle punctures and evacuation of abscess contents. This, however, brought only minimal improvement. He did not receive any medications and no diagnostic workup was performed. There was only minor and temporary improvement in the local condition. The patient was admitted to the hospital in a moderate general condition, due to the symptoms of massive perineal and perianal phlegmon. Rectal examination on admission, which was very painful for the patient, revealed a large, boggy mass in the anterior rectal wall. Bilateral perianal pus-discharging wounds after previous incisions were found. The patient was put on empiric intravenous antibiotic therapy (Metronidazole 500 mg every 8 hours, Biotrakson 1 g every 12 hours) and scheduled for surgery. The ischiorectal abscess was incised and drained to evacuate the purulent contents. The abscess cavity was thoroughly rinsed. The drain was maintained. Due to the lack of expected improvement after 3 days, the patient was qualified for a revision of the site after the incision of perineal phlegmon in an operating setting. The applied anesthesia allowed for a thorough examination of the patient, during which, three fistulous tracts were revealed by methylene blue solution administered into the incisions. The local and general condition as well as the treatment course raised the suspicion of inflammatory bowel disease. Azathioprine, mesalazine and hydrocortisone were included. Due to anaemisation up to 6.7 g/dL (starting Hb 10.0 g/dL), 3 units of packed red blood cells (PRBCs) were ordered for the patient. On the day of surgery, 1 unit of PRBC was transfused. In the following days, 2 more units were administered (the method of PRBC administration was due to the deficit of the preparation at the blood donation station). Haemoglobin levels increased to 9.7 g/dL. The patient was qualified for colonoscopy. During prep, another drop in haemoglobin (6.6 gL/dL) occurred. Therefore, it was decided to administer another 2 units of PRBC.
A colonoscope was inserted into the final segment of the small intestine, showing the appendix opening and the Bauhin’s valve. Distinct inflammatory lesions with flat ulcers and pseudopolyps were found in the terminal ileum. Samples were collected for histopathology. Bloody content in the small intestine was also detected. Multiple ulcers with islands of normal appearing intestinal mucosa in between (“cobblestone appearance”) were found in the large bowel. Histopathological samples were also collected from ulcers. After PRBC transfusion, the haemoglobin level was 8.5 g/dL. After endoscopic confirmation of the diagnosis of Crohn’s disease, numerous attempts were made to transfer the patient to a unit with a higher reference level. This, however, was unsuccessful due to the Christmas and New Year period. Further deterioration of the patient’s general condition was observed, with progressing anaemisation (haemoglobin decreased to 6.4 g/dL). Due to the deteriorating general condition, the inability to perform further transfusions and control the GI bleeding, as well as impossible transfer to a reference centre, the patient was qualified and agreed for surgery. Intraoperatively, a small amount of peritoneal fluid was found, the small intestine from the Treitz ligament appeared macroscopically normal, with dark, possibly bloody content in some of its sections. The final section, approx. 10 cm before the Bauhin valve, had a thickened, bloodshot, inflamed wall, with multiple hard adhesions, e.g. with the sigmoid colon, forming a lumpy conglomerate over the length of approx. 30 cm. Just at the Bauhin valve, the final section of 5-7 cm appeared macroscopically normal. A decision was made to resect the final, inflamed segment of the ileum (approx. 40 cm of the intestine) by performing Hartmann’s procedure and preserving the cecum. During isolation of adhesions and separation of the small intestinal conglomerate from the large bowel, a small fistula to the sigmoid colon was found. The fistulous opening of about 4 mm in the sigmoid was closed with two levels of sutures.
Powyżej zamieściliśmy fragment artykułu, do którego możesz uzyskać pełny dostęp.
Mam kod dostępu
- Aby uzyskać płatny dostęp do pełnej treści powyższego artykułu albo wszystkich artykułów (w zależności od wybranej opcji), należy wprowadzić kod.
- Wprowadzając kod, akceptują Państwo treść Regulaminu oraz potwierdzają zapoznanie się z nim.
- Aby kupić kod proszę skorzystać z jednej z poniższych opcji.
Opcja #1
29 zł
Wybieram
- dostęp do tego artykułu
- dostęp na 7 dni
uzyskany kod musi być wprowadzony na stronie artykułu, do którego został wykupiony
Opcja #2
69 zł
Wybieram
- dostęp do tego i pozostałych ponad 7000 artykułów
- dostęp na 30 dni
- najpopularniejsza opcja
Opcja #3
129 zł
Wybieram
- dostęp do tego i pozostałych ponad 7000 artykułów
- dostęp na 90 dni
- oszczędzasz 78 zł
Piśmiennictwo
1. Szmidt J, Gruca Z, Krawczyk M et al.: Podstawy chirurgii. Tom 2 – podręcznik dla specjalizujących się w chirurgii ogólnej. MP, Kraków 2013: 780-786.
2. Radwan P: Czy można przewidzieć przebieg choroby Leśniowskiego-Crohna? Jak reagować? Gastroenterologia Kliniczna 2018; 10(1): 32-38.
3. Hebzda A, Górecka M, Dembiński M, Kukla M: Choroba Leśniowskiego-Crohna – diagnostyka i możliwości terapeutyczne. Medycyna po Dyplomie 2020; 29(12): 23-25.
4. Lazarev M, Huang C, Bitton A et al.: Relationship between proximal Crohn’s disease location and disease behavior and surgery: A cross-sectional study of the IBD Genetics Consortium. Am J Gastroenterol 2013; 108(1): 106-112.
5. Haens G, Baert F, Assche G et al.: Early combined immunosuppresion or convenional management in patiens with newly diagnosed Crohn’s disease: an open randomised trial. Lancet 2008; 371(9613): 660-667.
6. Lowenberg M, Peppelenbosch M, Hommes D: Biological therapy in the management of recent – onset Crohn’s disease: why, when and how? Drugs 2006; 66(11): 1431-1439.
7. Jones J, Panaccione R: Biologic therapy in Crohn’s disease: state of art. Curr Opin Gastroenterol 2008; 24(4): 475-481.
8. Ramadas AV, Gunesh S, Thomas GAO et al.: Natural history of Crohn’s disease in a population – based cohort from Cardiff (1986-2003): a study of changes in medical treatment and surgical resection rates. Gut 2010; 59(9): 1200-1206.
9. Picco MF, Zubiaurre I, Adluni M et al.: Immunomodulators are associated with lower risk of first surgery among patients with non-penetrating non-stricturing Crohn’s disease. Am J Gastroenterol 2009; 104(11): 2754-2759.
10. Feagan BG, Panaccione R, Sandorn WJ et al.: Effects of adalimumab therapy on incidence of hospitalization and surgery in Crohn’s disease: results from the CHARM study. Gastroenterology 2008; 135(5): 1493-1499.
11. Lichtenstein GR, Yan S, Bala M et al.: Infliximab maintenance treatment reduces hospitalizations, surgeries, and procedures in fistulizing Crohn’s disease. Gastroenterology 2005; 128(4): 862-869.
12. Antunes O, Filippi J, Hebuterne X, Peyrin-Biroulet L: Treatment algorithms in Crohn’s – up, down or something else? Best Pract Res Clin Gastroentrol 2014; 28(3): 473-483.
13. Sandborn WJ, Hanauer S, Assche GV et al.: Treating beyond symptoms with a view to improving patient outcomes in inflammatory bowel diseases. J Crohns Colitis 2014; 8(9): 927-935.
14. Guizzetti L, Zou G, Khanna R et al.: Development of Clinical Predicition Models for Surgery and Complications in Crohn’s Disease. J Crohn Colitis 2018; 12(2): 167-177.
15. Skamnelos A, Lazaridis N, Vlachou E et al.: The role of small-bowel endoscopy in inflammatory bowel disease: an updated review on the state-of-the-art in 2021. Ann Gastroenterol 2021; 34(5): 599-611.
16. Tai FWD, Ellul P, Elosua A et al.: Penetric capsule endoscopy identifies proximal small bowel disease guiding upstaging and treatment intensification in Crohn’s disease: A European multicentre observational cohort study. UEG Gastroenterol J 2021; 9: 248-255.