Small Intestine Flashcards
Describe the management of enterocutaneous fistulae
S- Sepsis
S- Skin
N- Nutrition (and electrolytes!)
A- Anatomy of the fistula
P- Planning of corrective surgery
Features of Crohns
Macroscopic
Endoscopic
Microscopic
Macroscopic
- Skip lesions/Discontinuous
- Distribution anywhere in the GI tract
- Transmural- fat wrapping occurs
Endoscopic
- Linear ulcers- progress to additional transverse sinuses and subsequent cobblestone appearance
- Skip lesions
Microscopic
- Transmural lymphoid infiltrates
- Sarcoid-like non caseating granulomas
- 50%
- Paneth cell hyperplasia is also common
Crohns distribution
55% SB and colon
30% SB only
15% Colon only
Classification of EC fistulae
Category
Low<200mL
Intermediate 200-500mL
High>500mL
Anatomy
site of defect in viscus e.g Jejunal, gastric etc
Etiology
IBD
Radiation
Iatrogenic
Foreign body
Spontaneous EC Fistula resolution timing
90% that close will do by 1 month
10% in months 1-3
None over 3 months
What is blind loop syndrome
Rare disorder secondary to bacterial overgrowth in areas of SB stasis
diarrhoea
steatorrhoea
Megaloblastic anaemia
Abdominal pain
Fat soluble vitamin deficiencies
B12 supplementation and Abx can resolve this temporarily
Surgical correction needed long term
Complications of jejunal diverticulosis
Bleeding
Enterolith formation
Perforation
Blind loop syndrome/malabsorption
Chronic pain
Malignancies with a preponderence for small bowel mets
Intraabdominal/transcoelomic/Local invasion (most common cause of SB malignancy)
Ovarian
Gastric
Cervical
Renal
Pancreas
Colon
Extraabdominal (rare)
Melanoma (most common)
Breast
Lung
SB GIST
- Origin
- Cellular markers
- Mutation
- Growth and Metastasis
- Adjuvant treatment
Origin
- Mesenchymal
- interstitial cells of Cajal
Cellular markers
- CD117 (molecular component of c-kit)
- ~85%
- DOG1 (Discovered on GIST-1)
- ~100%
- PKC-theta (Protein Kinase C- theta)
- ~100%
Mutation
- c-kit (of which the CD117 molecule is a component) present in most (~85%)
- position of c-kit mutation is important
- exon 11, codon 557/558 due to high recurrence risk
- Of those tumours without c-kt mutation many will have a PDGFRA mutation
- position of c-kit mutation is important
- 10-15% wild type
- NF-1
- Carney complex and Carney-Stratakis syndrome
- Succinate dehydrogenase
Growth and metastasis
- Invade locally, spread haematogenous- liver, lung, bone
Adjuvant (or neoadjuvant) treatment
- c-kit is a receptor tyrosine kinase
- Tyrosine kinase inhibitors are the agent of choice
- 3 years of adjuvant Imatanib is standard of care for intermediate to high risk disease
- induces cellular quiescence rather than demise
- TKI’s also have demonstrable antitumour activity in most tumours with PDGFRA mutations
How are GISTs risk stratified
What is the risk of progressive disease
Crohns perianal manifestations
Fissures
Fistulas
Abscesses
Cholecystokinin
Secreted by I cells in the duo and jej
- Response to lipid, protein and carb in duo
Actions
- Pancreatic enzyme release
- Relaxation of SOD
- GB contraction
Secretin
Secreted by S cells in Duo
- Response to low pH
Actions
- Secretion of Bicarb and water from pancreas
GIP
Gastric inhibitory polypeptide
Secreted by K cells in duo and jej
Acts to
- reduce gastric acid production
- Increase insulin release
Intestinal Malrotation
Normal rotation is counter clockwie (as the surgeon looks down on the abdomen
- 270 degrees
Non rotation is more common but not usually symptomatic
Ix-
- UGI series- bird beak at D3 with displaced ligament of Trietz
- USS- Alteration of usual SMA/SMV relationship
Management:
- Ladds procedure
How are GI neuroendocrine neoplasms subclassed
What genetic markers is each associated with
NENs are subdivided into:
- neuroendocrine tumours (NET)
- referring to well differentiated tumours
- MEN1, DAXX, ATRX
- grade low, int, high subtypes
- referring to well differentiated tumours
- neuroendocrine carcinoma (NEC)
- referring to poorly differentiated tumours
- TP53, RB1
- Small cell, large cell subtypes
- referring to poorly differentiated tumours
- mixed adenoneuroendocrine carcinoma (MANEC)
- Features of neuroendocrine and epithelial derived malignancy
Carcinoid syndrome
Carcinoid syndrome is classically characterised by:
- flushing (70%)
- diarrhoea (50%)
- intermittent abdominal pain (40%)
Due to the secretion of bioactive amines, most commonly serotonin, directly into the systemic circulation from either metastatic neuroendocrine tumour to liver or less commonly retroperitoneum.
Carcinoid crisis
Characterised by:
- profound flushing
- bronchospasm
- tachycardia
- labile blood pressure
Caused by:
- release of high levels of serotonin or other bioactive amines, such as histamine.
Can be precipitated by:
- induction of anaesthesia
- intraoperative tumour handling
- invasive techniques such as tumour ablation.
- histamine releasing drugs (e.g morphine)
- sympthomimetic drugs (e.g adrenaline)
It is critical that patients with known carcinoid syndrome or at those at risk of carcinoid crisis are managed appropriately in the perioperative period.
- octreotide and its analogues
Carcinoid heart disease
Seen in 20% of patients with carcinoid syndrome
Fibrosis of the right-side heart valves caused by many years of high serotonin levels
Perioperative octreotide in GI NETs
Grading of GI NETs
Describe the surgical management of intestinal malrotation
Ladds procedure has 5 steps
- Counter clockwise detorsion
- Division of Ladds bands
- Elongation of the mesentary
- Appendicectomy
- Placement of bowel to right and colon to left
What are the tumour markers for NETs
Why are they elevated in NET
What factors might falsely elevate each
Chromogranin A
- granins are proteins involved in exocytosis of secretory vesicles
- found in both functional and non functional NETs
- higher with higher secretion (volume or function)
- also elevated by:
- PPI use
- CHF
- CRF
5-HIAA (5-hydroxyindoleacetic acid)
- degradation product of serotonin
- Direct assay of serotonin is difficult
- Serotonin produced by >70% of NETS
- also elevated by
- chocolate, caffeine, nuts, nicotine, banana