Miscellaneous Disorders

of Small Intestine
Dr. Ali A. Ramadhan
M.B.Ch.B., FIBMS, FKBMS (G & H)

1
 Learning objectives
• To explore the rare SI disorders & tumors
• To recognize the clinical features & identify
the lines of diagnosis and management of
lactose intolerance and food allergies.

2
 Topics
• Chronic intestinal pseudo-obstruction (CIPO)
• Protein-losing enteropathy
• Ulceration of the small intestine
• NSAID-associated small intestinal toxicity
• Eosinophilic gastroenteritis
• Lactose intolerance
• Food allergy
• Abdominal tuberculosis
• Tumors of the small intestine

3
Chronic intestinal pseudo-obstruction (CIPO)
• Causes of CIPO:
A. Primary or idiopathic: familial visceral myopathies or
neuropathies, congenital aganglionosis
B. Secondary
– Drugs: opiates, tricyclic antidepressants, phenothiazines
– Smooth muscle disorders: scleroderma, amyloidosis,
– Myenteric plexus disorders, e.g. paraneoplastic syndrome
– CNS disorders: Parkinson’s dis. autonomic neuropathy
– Endocrine & metabolic disorders: hypothyroidism,
phaeochromocytoma, acute intermittent porphyria,

4
Chronic intestinal pseudo-obstruction (CIPO)
• C/F: recurrent episodes of intestinal obstruction, weight loss
from malabsorption, symptoms of dysmotility elsewhere eg
dysphagia, bladder dysfunction
• Investigations: Plain X-rays show intestinal obstruction, but
barium studies demonstrate no mechanical obstruction.
• Laparotomy is sometimes required to exclude obstruction
and to obtain full-thickness biopsies of the intestine.
• Treatment is often difficult.
– Treatment of underlying cause. Avoid further surgery
– Metoclopramide or domperidone may enhance motility
– Antibiotics for bacterial overgrowth
– Nutritional and psychological support
5
 Protein-losing enteropathy
• Excessive loss of protein into the gut lumen, sufficient to
cause hypoproteinaemia.
• Features: peripheral edema and hypoproteinaemia with
normal liver function and no proteinuria.
• The diagnosis can be confirmed by measurement of
fecal clearance of α1-antitrypsin or 51Cr-labelled albumin
after intravenous injection.
• Treatment is of the underlying cause, with nutritional
support and measures to control peripheral edema

6
7
 Ulceration of the small intestine
• Ulcers are more common in the ileum
• Cause bleeding, perforation, stricture or obstruction.
• Barium studies and enteroscopy confirm the diagnosis

8
NSAID-associated small intestinal toxicity
• Manifestations range from erosions and ulcers to
mucosal webs, strictures and, rarely ‘diaphragm
disease’, in which intense submucosal fibrosis results in
circumferential stricturing.
• C/F: pain, obstruction, bleeding or anemia.
• DDx: Crohn’s disease, carcinoma or lymphoma.
• Enteroscopy or capsule endoscopy can reveal the
diagnosis but sometimes it is only discovered at
laparotomy

9
10
 Eosinophilic gastroenteritis
• It is of unknown etiology (no parasitic infection or
eosinophilia of other tissues).
• It can affect any part of GIT
• Clinical features
– Features of obstruction and inflammation, such as colicky
pain, nausea & vomiting, diarrhea, weight loss and
protein-losing enteropathy
– Up to 50% have a history of other allergic disorders
– Serosal involvement may produce eosinophilic ascites

11
 Eosinophilic gastroenteritis
• The diagnosis is made by biopsies.
• Investigations are done to exclude parasitic infection &
other causes of eosinophilia.
• Peripheral blood eosinophilia is present in 80%
• Serum IgE concentration is often raised.
• Dietary manipulations are rarely effective, although
elimination diets, especially of milk, may benefit a few.
• Severe symptoms are treated with prednisolone and/or
sodium cromoglicate.
• The prognosis is good in the majority

12
All of the followings cause protein losing
enteropathy through mucosal ulceration or
erosions EXCEPT
A. Crohn’s disease
B. Gastric cancer
C. Radiation enterocolitis
D. Lymphoma
E. Celiac disease

TA: E

13
 Lactose intolerance
• Lactose is normally digested to glucose and galactose
by the brush border enzyme lactase.
• Lactase is deficient in up to 90% of adult Africans,
Asians and South Americans, but only 5% of northern
Europeans.
• In genetically determined (primary) lactase deficiency,
jejunal morphology is normal. ‘Secondary’ lactase
deficiency occurs in disorders of jejunal mucosa
• Unhydrolysed lactose enters the colon, where bacterial
fermentation produces volatile short-chain fatty acids,
hydrogen and carbon dioxide
14
 Lactose intolerance
• C/F: In most people, it is asymptomatic. Some have
colicky pain, increased flatus, borborygmi and diarrhea
after ingesting milk or milk products.
• The correct diagnosis is suggested by clinical
improvement on lactose withdrawal.
• Lactose hydrogen breath test is a useful confirmatory
test
• Dietary exclusion of lactose is recommended, although
most sufferers are able to tolerate small amounts of milk
without symptoms.
• Commercial lactase preparations are effective but is
costly
15
 Food allergy
• Food allergies are most commonly due to type I HSR
with production of IgE Ab, although type IV delayed HSR
are also seen.
• Common culprits are peanuts, milk, eggs, soya, shellfish
• Manifestations occur immediately on exposure & range
from trivial to life-threatening or even fatal anaphylaxis
• Oral allergy syndrome from contact with benzoic acid in
fresh fruit juices, leads to urticaria & angioedema of lips
and oropharynx. This is not immune mediated reaction.
• ‘Gastrointestinal anaphylaxis’ consists of nausea,
vomiting, diarrhea and sometimes collapse

16
 Food allergy
• The diagnosis is difficult to prove or refute.
• Skin prick tests and IgE antibodies have limited
predictive value.
• Double-blind placebo-controlled food challenges are the
gold standard, but are not readily available.
• In many cases, clinical suspicion and trials of elimination
diets are used.
• Treatment consists of education, elimination of the
offending antigen, and, in some cases, antihistamines or
sodium cromoglicate.

17
 Abdominal tuberculosis
• Gut infection usually results from human M.
tuberculosis, which is swallowed after coughing.
• Many patients have no pulmonary symptoms and a
normal chest X-ray.
• TB most commonly affects the ileocaecal region.
• TB mimic Crohn’s disease but diarrhea is less common
in TB
• Low-grade fever is common but not invariable.
• Perianal disease with fistula is recognised.
• Peritoneal tuberculosis causes exudative ascites,
associated with abdominal pain and fever.
• Granulomatous hepatitis occurs. 18
 Abdominal tuberculosis
• Diagnosis:
– Elevated ESR, raised alkaline phosphatase suggests
hepatic involvement.
– Histological confirmation is by endoscopy,
laparoscopy or liver biopsy.
– Granulomas is not always seen and acid fast bacteria
are often scanty.
– Diagnosis is now possible on biopsy using PCR.
• Treatment:
– When the presentation is very suggestive of
abdominal tuberculosis, anti-TB should be
commenced, even if bacteriological or histological
proof is lacking. 19
 True regarding lactose intolerance
A. Lactase is deficient in 10% of Asians adults
B. Most patients have abdominal pain and
diarrhea
C. Lactose hydrogen breath test is mandatory
D. Most patients can tolerate small amounts of
milk
E. Lactase preparation are ineffective as they are
destroyed by gastric juice.
TA: D
20
 Tumors of the small intestine
• The small intestine is rarely affected by neoplasia.
• Fewer than 5% of all GI tumors occur at this site.
• Benign tumors: the most common are adenomas,
GIST, lipomas & hamartomas.
• Malignant tumors: adenocarcinoma, carcinoid tumor,
malignant GIST and lymphoma. Majority occur in
middle age or later

21
 Neuro-endocrine tumors (NETs)
• Also known as carcinoid tumors.
• Derived from entero-chromaffin cells & are less
aggressive than carcinomas with slow growth rate
• Lesions > 2 cm have more potential to spread locally &
metastasize to the liver.
• Those in the appendix are usually benign.
• Some are “functioning” producing hormones or
vasoactive peptides which are usually metabolized in
the liver and do not reach the systemic circulation.
• The diagnosis is made by detecting increased 5-HT
metabolite, 5-HIAA, in a 24-hr urine collection and by
raised serum chromogranin A levels
22
 Neuro-endocrine tumors (NETs)
• Symptoms depend on the tumor location and function:
– S.I. obstruction from tumor mass
– ‘Carcinoid syndrome’:
• Flushing, wheezing, diarrhea
• Seen only with hepatic metastasis (secretory products
can reach the systemic circulation).
– Hepatic metastases cause pain, hepatomegaly & jaundice
– Cardiac involvement (tricuspid regurgitation, pulmonary
stenosis) leading to heart failure
• The treatment is surgical resection.
• For carcinoid syndrome: surgery, hepatic artery embolization,
octreotide & chemotherapy may have a role.
23
 Immuno-proliferative small
intestinal disease (IPSID)
• Also known as alpha heavy chain disease
• It is a variant of B-cell lymphoma of MALT type
• It often associated with Campylobacter jejuni
• Enlarged mesenteric lymph nodes are common.
• Most patients are young adults
• C/F: malabsorption, anorexia and fever.
• Serum electrophoresis shows alpha heavy chains (from
the Fc portion of IgA).
• Prolonged remissions by long-term antibiotic therapy
• Chemotherapy for those who fail to respond or who
have aggressive disease 24