Transcript Management of complicated IBD: Medicine or Surgery?

Patient 1 – acute abdomen
• 19 year old male – no prior medical history
• Just back from a trip to Israel
• Two days of “writhing in pain”, fever to 102.5
– Similar episode about a week ago in Israel
resolved
• Pediatrician – acute abdomen, wbc 30,000
• CT – pelvic abscess, inflamed small and large
bowel, inflamed Ileum
Questions
• In an acute presentation, how does one
differentiate between:
– Perforated appendix
– Newly presenting Crohn’s with perforation
– Other causes of pelvic abscess
• How do you determine which patients warrant
emergency surgery, vs. which should be “cooled
off” medically?
• What is your protocol for medical management
prior to surgery?
Management
1. Abscess drained by IR
2. One week in ICU
3. IV antibiotics
(Piperacillin/ Tazobactam)
3. NG suction to decompress
4. IV hydration
5. Pain medication
6. Parenteral nutrition
7. Re-imaging after 10 days
Questions
• What additional evaluation would you perform at
this time to establish a Crohn’s diagnosis?
• Would you consider treating this patient
medically?
– Short term vs. long term?
– With what agent?
• If you choose surgery, what is the appropriate
time to intervene?
– Laparoscopic vs. open?
“Abdominal phlegmons in Crohn's
disease: outcomes following antitumor
necrosis factor therapy”
• Thirteen patients with abdominal phlegmon treated
with antibiotics and anti-TNF - 2004-2010
– 12 patients also had abscess
• Anti-TNF with antibiotics
– Two patients underwent surgery within a year
• “Penetrating CD complicated by phlegmon formation
may be safely and effectively managed with a
combination of antibiotics and anti-TNF therapy”
• Selection bias?
Cullen et al, IBD Journal 2013
Management and outcome
• Hospitalized with PN, NPO, decompression and
medical management for approximately 3 weeks.
• EGD/Colonoscopy – no gastric or colonic disease
• Laparoscopic ileocecectomy
– Fibrotic ileum and cecum
– Residual abdominal abscesses
– 30 cm of ileum plus cecum resected
• Uneventful recovery
Postoperative management
• How would you monitor patient postoperatively?
– Clinical follow-up and labs only?
– Small bowel imaging?
– Colonoscopy (timing)
• What (if any) postoperative medical therapy
would you utilize ?
– Aminosalicylate
– Thiopurine
– Infliximab
Postoperative management
and outcome
• Discussion with family and patient
• Opted for mercaptopurine
– Transaminitis developed
– Changed to low dose 6MP and allopurinol
• Surveillance 1 year after initial presentation
– Normal MRI, normal colonoscopy
• No clinical or laboratory recurrence 3 years
after his initial surgery.
– Follow up colonoscopy planned
Presenting history
• 7 year old female
• Presented with Serratia osteomyelitis at 3
months of age
• History of multiple GI issues in infancy
– Poor weight gain requiring NG tube
– Diarrhea
– Rectal bleeding as infant while breast-fed,
responsive to mother eliminating milk from diet.
• Diagnosis of CGD by dihydrorhodamine test
Chronic granulomatous disease
Winkelstein et al 2000; Medicine 79:155
• Primary immunodeficiency characterized by inability of cells to
kill bacteria and fungi.
– Staphylococcus, Aspergillus cause serious infection
– Catalase positive organisms
– Pneumonia most common infection (80%)
• 75% of patients present under age 5 years
• Autosomal recessive or X-linked
• GI manifestations
– Colitis present in approximately 20%
– Perianal abscess
– Gastric outlet obstruction
• Diagnosis – defective NADPH oxidase
– Dihydrorhodamine test
Dihydrorhodamine test
Children’s Hospital of Wisconsin
Neutrophils given DHR dye, activated
Normal neutrophils produce superoxides that oxidize DHR, resulting in fluorescence.
Chronic granulomatous disease
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Mimics Crohn disease on endoscopy and histology
Granulomas in only 34 % (Levine, Histopathology 2005)
Paucity of neutrophils compared to UC (Shappi JPGN 2003)
Decreased CD68+ macrophages (Liu et al, IBD Journal 2009)
Therapy – gamma-IFN, steroids, thalidomide, SCT
Clinical course
• Continued with diarrhea, anemia for over a year
despite therapy.
– Labial abscess grew E. coli
• By age 4:
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3 BM daily, no bleeding
Height at 25% ile, but falling off
Colonoscopy with ileal and colonic granulomas
Therapies included
• Alpha - Interferon
• Bactrim and fungal prophylaxis
Medical options for this colitis
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Aminosalicylates
Antibiotics
Probiotics
Corticosteroids
Azathioprine
GM-CSF
Anti-TNF agents
Medical options for this colitis
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Aminosalicylates - intolerant, got diarrhea
Antibiotics – vancomycin for C. difficile
Probiotics – VSL 3
Corticosteroids – recurrent courses
Azathioprine – tried for over 6 months
– unable to wean off steroids without worsening
• GM-CSF
• Anti-TNF agents?
Caution with anti-TNF in CGD!
• NIH published experience with 5 patients
– All with active colitis despite thiopurines
– Two deaths from opportunistic lung disease
• Infliximab effective for GI disease (including
fistulizing disease), but severe infectious
complications developed
– B. cepacia
– CMV colitis
– S. aureus liver abscess
Potential options
• GM-CSF
– Wang et al, J . Allergy Clinical Immunology 2005
• Anakinra
– Rationale – upregulation of IL-1 beta pathway
– van de Veerdonk et al, Netherland Journal of
Medicine 2011
• Stem cell transplant
– Controversial in CGD
– No matched siblings in this case.
Current outcome
• Anakinra 200 mg daily for 3 weeks
– Azathioprine stopped
• Diarrhea improving with 2 stools daily
• Prednisone weaned to 6 mg daily
• CRP improved from
– 2.2 mg/dL to 0.9 mg/dL
• Plan for 3-6 months of Anakinra, the reevaluate with colonoscopy
Fever in CD Patient on 6-MP
Presenting history
• 18 year old male with CD
• Maintained on 6-MP for four years with stable
remission and mucosal healing
• Headache, fever and malaise
• Leukopenia and elevated transaminases
• No lymphadenopathy or HSM
• EBV IgM+
• EBV PCR 291 copies/mcg DNA
Options
• Additional diagnostic tests?
• What to do with 6-MP?
• Follow-up?
Clinical course
• Initially improved
• Within a few weeks fevers & chills returned
• Became jaundiced
Options
• Additional diagnostic tests?
• Differential diagnosis?
Diagnostic Tests
• Hyponatremia, pancytopenia, elevated
bilirubin and transaminases
• Increased EBV PCR
• Elevated sIL2R, ferritin
• Abdominal ultrasound: massive HSM
• Diagnosis?
• Other tests?
Diagnostic Tests
• EGD/colon: multiple gastric polypoid lesions
with path consistent with EBV+ lymphoma
• Liver biopsy: EBV+ lymphoma infiltrate
• Bone marrow: hemophagocytosis
• PET-CT: widespread
Discussion
• What to do with 6MP with febrile
illness/mono?
• RISK of EBV associated HLH or lymphoma with
6-MP?
• Different for primary EBV infection versus
reactivation?
• Monitoring for this?