Malformations of the biliary tract. Portal hypertension syndrome. Bleeding from the gastrointestinal tract

Malformations of the biliary tract.
Portal hypertension syndrome.
Bleeding from the gastrointestinal
tract
Urgench branch of TMA Department
of Pediatric Surgery
Lecture for 5th year students
of the Faculty of General
Medicine

The purpose of the lesson.
differential diagnosis of malformations of the biliary tract,
portal hypertension syndrome, bleeding from the
gastrointestinal tract. To acquaint with the clinical picture,
complications of the disease and the principles of
providing primary medical care to patients.

Lesson objective:
To train a general pediatrician, the ability to establish a
diagnosis of a disease, to study the features of the
clinical course and to carry out medical measures
requiring treatment for this pathology.
To train students to perform practical skills in the
diagnosis and differential diagnosis of diseases with
obstructive jaundice, portal hypertension syndrome and
bleeding from the gastrointestinal tract.

Frequency of biliary atresia: 1: 13000
(USA) 1: 11000 (Russia) 1: 7000 -
10000 (Japan, Southeast Asia,
Central Asia)
Mortality without surgery with b / atresia 100%
within 7-15 months. (WHO Annual Bulletin
2015)

etiopathogenesis
 Etiology unknown
 "Biliary atresia is a static state of complete
obstruction, or underdevelopment of the bile
ducts.
 "Pathology is a dynamic process of progressive
obliteration and sclerosis
 Genesis - violations of intrauterine liver
development at 4-6 weeks of gestation
 The most likely hypothesis is a viral infection
(TORCH, CMV, HEPATITIS)

Classification of
biliary atresias

CLINIC
ACHOLIC CHAIR, DARK URINE from birth, more
often from 2 weeks of development
Hepatomegaly, after 1-1.5 months -
splenomegalyIncreasing jaundice (bilirubin up to
300-400 mmol / l)
- in the first 2-3 months. the direct fraction prevails,
- from 3-4 months the content of direct and indirect
fractions of bilirubin levels out, bilirubin intoxication
joins
- an unfavorable prognosis and evidence of the
formation of biliary cirrhosis

Diagnostics
Biochemical doctor:
Bilirubinemia due to bound fraction (from 3 months of age
alignment of fractions)
Lack of stercobilin in feces Lack of urobilinogen in urine Increase in
ALP, GGT From 2-2.5 months - increase in ALT and AST
Instrumental diagnostics:Ultrasound (Doppler)
Cholangiography (percutaneous, transhepatic, antegrade, retrograde,
intraoperative)
CT, MRI (densitometry, non-contrast cholangiography)
Puncture liver biopsy

Diagnostic standards
 Jaundice from 2-3 weeks of life, or the duration of "neonatal
jaundice" more than 2-3 weeks
 Hepatomegaly with an increase in its density, later
hepatosplenomegaly
 Hyperbilirubinemia due to direct (bound) fraction (over
the age of 2.5-3 months, the bilirubin fractions level out!)
Growth of SHF and GGT
 Acholic stool from birth, dark urine (absence of
urobilinogen, in the presence of bile pigments)Ultrasound -
information content 65-75% (fibrous triangle)
 Cholangiography (percutaneous-transhepatic, retrograde,
introoperative) informative value 100% with non-total
atresiaCT - information content 70-75% - with atresia

Atresia of the distal common bile duct

Atresia of the common bile duct,cystic duct
and gallbladder

Introperative cholecystocholangiography common
bile duct atresia

Atresia of the gallbladder, common gallbladderand
common hepatic duct

Intraoperative cholangiography- stenosis (atresia)
of the external bile ducts

Atresia of the external bile ductsand
gallbladder

Intrahepatic bile duct atresiain the presence of a
rudimentary gallbladder, containing white bile

Total external atresiaand intrahepatic bile ducts

Atresia of the intrahepatic bile ducts

Macrodrug for obstructive
cholestasis,
age 2.5 months.

Macrodrug for biliary cirrhosis
(age - 3.5 months)

Late cholestasis with impaired liver
beam structure Age - 4.5 months.

Sclerosing cholangitis,
age - 5.5 months.

Cholestatic form of congenital liver vibrosis

A characteristic view of the gate of
the liver with biliary atresia

Operation Kasai
(formation of portojunostomy)

Survival after Kasai surgery
when performing an intervention:
at the age of 2 months. - 65-75%
(Karlsen T.H. et al, 2010, Maggiore G., 2011,
Futagava J., 2015)

Treatment protocols
Before surgery:
Hepatoprotectors (ursosan, heptral)
Steroids (prednisolone 4-5mg / kg) from 1 month. age
Hepatic hydrolysates (vitohepat, folic acid)
Surgical intervention at the age of 1.5 - 2 months. -
biliodigestive anastomoses, Kasai's operation
After operation:
Steroids (prednisone 4-5mg / kg) 3-6 months
Hepatoprotectors (ursosan, heptral) in tech. 6 months
Hepatic hydrolysates (vitohepat, folic acid)
Selective antispasmodics (duspatalin)
Enzymes (creon), eubiotics

is rare, accounting for 3.2% of all
surgical pathology of the gastrointestinal
tract.
The ratio of girls: boys - 3: 1 (sometimes
4: 1)
More than 50% of children under 10
years old
Late diagnosis leads to serious
complications.

Classification (Alonso-Lej, 1959)
1. Cystic enlargement of the common bile duct
2. Diverticulum of the common bile duct
3. Choledochocele
4. Intra- and extrahepatic cysts of the common
bile duct
5. Isolated cystic degeneration of the intrahepatic
biliary tract (Caroli's disease)

35
The main clinical signs are paroxysmal abdominal pain, jaundice,
the presence of a palpable tumor-like formation in the abdominal
cavity. Various combinations of the above symptoms or in the form
of a "classic triad".
CLINICAL PICTURE

Complications
Pancreatitis,
Cholangitis
ZhKBCPU,
PGCyst rupture with biliary peritonitis

Ultrasound picture of the cyst of the
common bile duct

38
Киста желчного протока
Желчный пузырь
Ultrasound using a cholereticbreakfast
is used for differentialspecial diagnostics
with solitaryand parasitic cysts of the liver,
pancreas. AtKTVZHP reduction
Occursgallbladder and / or cysts,
manifested in changetheir sizes
(or one ofthem) and shape, which
increasesspecificity
characteristicfor КТВЖП.
Functional echolecystocholangiography
Bile duct cyst
Gall bladder

Computed tomography (MSCT and MRCP) with CTED
With ultrasound, it is possible to obtain a clear image of the gallbladder and
fragments of the dilated bile ducts. However, cystic enlargement can be
localized in any part of the biliary tract in the form of a total expansion or its
individual sections.
The advantage of MSCT is that the method allows you to show the expansion
along the entire length, the connection of the cyst with the bile ducts, the
state of the pancreas and the Wirsung duct.
Bile duct cyst
Gall bladder

41
Computed tomography for vein dysplasia
Bile duct cyst
Gall bladder

42
3D reconstruction of CTE and GB with MSCT and MRCP
a) fusiform expansion of the common bile duct with distal stenosis
b) cylindrical expansion of the common bile duct without distal stenosis
c) cystic enlargement of the bile ducts with stenosis of the distal common bile duct

Treatment
External drainage - cystostomy:Absolute indications for
external cyst drainage:
- newborns and children under 6 months. with obstructive
cholangiopathies, symptoms of hepatic failure;
- with severe obstructive jaundice with severe blood
clotting disorders in children aged 11 months. up to 8.5
years;
- cyst rupture, complicated by diffuse biliary peritonitis,
mainly among children under the age of 3 months.

Radical treatment -
CYSTECTOMY,
HEPATICOENTEROSTOMY
according to Roux

Hydroelectric power station
Across Ru
Withoutantireflux
mechanism
Hydroelectric power
station across Ru
Withinvaginatingantireflux
mechanism
Hydroelectric power
station across Ru
withcuffantireflux
mechanism
Length of entero-enteroanastomosis from the site of applicationhepaticoenterostomyis
selected depending on the age of the children:
up to 5 years old - 20-25 cm,
up to 10 years old - 25-30 cm,
over 10 years old - 30-35 cm.
CYSTECTOMY, HEPATICOENTEROSTOMY according to Roux

The cyst is fully exposed on all sides to prepare for excision.
The most important thing in isolating a cyst is its release from
blood vessels.

 After isolation of the
cyst of the common bile
duct, part of which is
located close to the liver,
it is necessary to dissect
and select, with the
calculation of the size,
the end of the free loop
of the small intestine
 The distal part of the
common bile duct, as
much as possible, is
allocated as much as
possible, then it is cut off
and sutured

 Before excising the cyst and
placing an anastomosis, it is
necessary to isolate a loop
of the jejunum, at the
beginning to impose an
anastomosis between the
end of the adductor and to
the side of the abducting
part of the small intestine
 After this, the isolated loop
of the small intestine is
drawn behind the large
intestine and then an
anastomosis is applied
between the end of the
jejunum and the rest of the
common bile duct.

 Anastomosis is usually
applied with two rows of
sutures and the 3rd row
of sutures is applied with
four sutures, departing
from the anastomosis line
by 1 cm between the
intestinal wall and the
Glisson capsule

 After the
complete
imposition of
these sutures, the
proximal part of
the common bile
duct invaginates
into the lumen of
the freely
adducted bowel
loop and,
thereby, creates
an obstacle for
reflux into the
bile ducts.

 Postoperative
ultrasound,
where the
permeability
of the
anastomosis
was good and
no clinical
manifestation
s of reflux
were noted.

Pre- and postoperative ultrasound
Cystic enlargement of the external
bile ducts
HEC, Roux hydroelectric power
station with antireflux mechanism
53
The accumulation of bile above the site of the
antireflux anastomosis is determined; the
intrahepatic bile ducts are not dilated.

54
Doppler ultrasound of the liver vessels before surgery
Doppler studies of hepatic blood flow:changes in the liver and in the portal system
associatedwith a belated diagnosis of the disease.
Doppler study of hepatic blood flow in the postoperative period:Violation of blood flow is not detected.
Doppler ultrasound of the liver vessels after surgery

55
Computed tomography (MSCT and MRCP) in the pre-
and postoperative period with KTZHP

56
Endoscopic picture after the operation of
cystoduodenoanastomosis with partial excision of the
cyst
FEGDS is a picture of putty bile (a) and the remaining
ligatures
(b) at the site of BDA.

PORTAL HYPERTENSION
SYNDROME - is a symptomatic
complex characterized by
increased pressure in the portal
vein basin, expansion of natural
portocaval anastomoses,
ascites, splenomegaly.

Normally, portal pressure ranges
from 7 to 12 mm Hg. (70-120
mm.w.c.), with SG it significantly
exceeds this level, reaching 400
mm.w.c. and higher

Portal (splenic) vein thrombosis
Contributing factors:
slow blood flowpressure
dropdiseases that
compress the portal
veinThrombophlebitis of
the portal vein and its
branches

Cavernous transformation of the portal
vein
a pathological condition in
which peculiar formations,
resembling cavities, appear
in the lumen of the portal
and splenic veins. It is
believed that these are
recanalized blood clots.

Congenital occlusionportal vein
This disorder should be
suspected if portal
hypertension develops
during childhood or
adolescence. Usually there
is no indication of
previous liver disease

Diseaseand Budd-Chiari
syndrome

•Budd-Chiari disease is understood as primary
obliterating endophlebitis of the hepatic veins
with thrombosis and subsequent occlusion. This
also includes anomalies in the development of
the hepatic veins, when the outflow of blood
from the liver is impaired.
•The Budd-Chiari syndrome is a secondary
violation of the outflow of venous blood from
the liver in patients with various diseases -
constrictive pericarditis, thrombosis or stenosis
of the inferior vena cava above the confluence
of the hepatic veins.

As the liver cells die, bridging
necrosis forms, dividing the
normal lobule into false ones.
The peculiarity of the false
lobules is that they do not have
normal triads - there are no
central veins. In the connective
tissue cords, vessels develop -
portal shunts. Veins are
partially compressed and
destroyed, sinusoidal blood flow
is disturbed.
The blood flow through the hepatic artery is reduced.
Blood is thrown through the splenic artery into the portal
vein - portal hypertension increases.
Intrahepatic PG morphology

with cirrhosis of the liver, the unity of the
simultaneously occurring three processes is
observed:
necrosis of hepatocytes;
1. regeneration of liver tissue with the
formation of pseudo-lobules, i.e.
2. perverted regeneration process, which
does not lead to the restoration of the
normal morphological structure of the
organ;
3. massive proliferation of connective tissue.

PG Clinic (hepatosplenomegaly,
phlebectasia, ascites)

hapatospleomegaly
Typical for intra- and suprahepatic portal
hypertension
Isolated splenomegaly (with a decrease in
liver size, typical for subhepatic and mixed
forms of PG)

Phlebectasias of the
esophagus, stomach,
hemorrhoidal pool. Erosive
esophagitis, gastritis.

ascites
- Transient
- Diuretic labile
- Diuretic-resistant

Laboratory diagnostics
hypersplenism (cytopenia:
thrombocytopenia,
leukocytopenia)
With bleeding, pancytopeniaWith cirrhosis -
hypo-, dysproteinemia, decreased PTI,
increased enzymes
Moderate hyperbilirubinemia

The choice of the method of
instrumental research in case of
suspicion of portal hypertension
syndrome can be conditionally
divided into four groups, depending
on the stage of diagnosis.
Instrumental methods

Stage I - confirmation of the
diagnosis of portal hypertension
syndrome.
Research methods:
Abdominal ultrasound
Esophagogastroduodenoscopy

Stage II - establishing the level of the portal
block (intrahepatic, subhepatic,
suprahepatic).
Methods of angioraphy are used:
1. LNG
2. celliac, mesentericoportography
3. Retrograde venography
4. transhepatic portography

Stage III - Measurement of the pressure level in
the hepatic and portal veins:
1) determination of the "jammed" hepatic-
venous pressure (HVPP).
2) determination of portal pressure through the
umbilical vein.
3) determination of portal pressure by
puncture of the spleen.
4) measurement of portal pressure using
transhepatic puncture with a thin needle.
5) Intraoperative measurement of portal
pressure.

Intra-splenic
pressure (VVP),
free portal
pressure (SPP)
and wedged
hepatic venous
pressure
(HVPP) are
measured -

Norms:
VSD and SPD are equal to 10-15 cm of water.
Art.
ZPVD about 5.5 cm of water column.

Stage IV - establishing a nosological
diagnosis of the disease that led to
portal hypertension syndrome.
Applicable:
Ultrasound
hepatoscintigraphy liver biopsy.

Ultrasonic signs of PG are
1. Expansion and appearance of tortuosity of the
portal, splenic and superior mesenteric veins;
2. Varicose enlargement of the lumen of the upper
part of the stomach with thickening of its walls
3. Increase or decrease in the size of the liver,
splenomegaly (!)
4. The appearance of natural portocaval collaterals
5. Ascites
6. Slowdown of blood flow in the portal vein
according to the results of Doppler examination

The spleen is sharply enlarged, the left kidney is
pushed back and deformed.

expansion of the diameter of the portal vein

Cavernous transformation of the portal
vein

pronounced tortuosity of the splenic vein

Intraorgan expansion of the splenic vein

propulsive type of blood flow through
the splenic vein.

Phlebectasias
VRV of the esophagus and
stomach: I degree - the diameter
of the veins does not exceed 3
mm, elongated, located only in the
lower third of the esophagus; II
degree - VRV with a diameter of 3
to 5 mm, convoluted, spreading in
the middle third of the esophagus;
III degree - the size of the veins
is more than 5 mm, tense, with a
thin wall, located close to each
other, on the surface of the veins
"red markers".

esophagogastroduodenoscopy
VRVPiZH-I st

CT and NMR tomography allows to
obtain an image of the parenchymal
organs of the abdominal cavity, large
vessels, the blood flow in which can
act as a natural contrast.
(angiography)

Complications of GHG
1. Reduction of portohepatic circulation
2. Bleeding from phlebectasias of the
esophagus and stomach, rectum
3. Ascites
4. Hepatic encephalopathy

The most effective is the combined use of the
antialdosterone drug veroshpiron 150-200 mg / day daily
and one of the saluretics (furosemide - 40 mg / day, uregit
- 50 mg / day, hypothiazide - 100 mg / day) in courses of
3-4 days with a break of 2 days ... With a decrease in the
effectiveness of such treatment, it is advisable to prescribe
saluretics in the form of "cocktails" in a half dose
(furosemide-hypothiazide, triampur). In patients with
refractory to treatment ascites, ascites-leading surgeries
are recommended.
Ascites treatment

Surgical treatment of portal hypertension (aimed
mainly at preventionand treatment of its
complications)
Palliative methods:
Endovascular (splenic and hepatic artery embolization,
TIPS)
Disconnection operations with and without splenectomy
Ascitic diverting operations (peritoneoatrial and
peritoneovenous bypass grafting)
Radical methods:
Direct PCS
Partial PCB
Selective
PCH
Superselective PCS
Liver transplant

Endosurgery in the treatment of
PH
Endovascular separation of low and high
pressure systems
Transjugular portosystemic shunting
(TIPS)
Percutaneous-transhepatic embolization of
the coronary vein of the stomach
Endoscopic sclerotherapy, ligation of
phlebectasias

Chronic splenic artery embolization

Results of PG endosurgery
Not always comforting
Recurrence of bleeding 22-58% in the first
5 years
Effectively 5-6 fold ligation or hardening
Children have a high risk of esophageal
perforation

MCA "H" -shaped with autograft

Dorsal scanning reveals the
anastomosis of the left renal
and splenic veins.
1 - splenic vein,
2 - renal vein
3 -anastomosis.

Cross scan
1 - aorta,
2 - superior mesenteric artery,
3 - splenic vein, 5 - left renal vein A significant increase in the
blood flow rate in the area of the anastomosis of the renal and
splenic veins is determined (long arrow).

Surgical results
HSV - direct PCB are effective (thrombosis
rate 5-9%, encephalopathy 1-3%)
Liver cirrhosis - selective shunting
(frequency of shunt thrombosis 3-8%,
encephalopathy 7-15%)
Congenital fibrosis - selective shunting
(thrombosis rate 4-7%, encephalopathy 3-
7%)

Bleeding from varicose
veins of the esophagus
with portal hypertension

Bleeding from dilated veins of the esophagus
with portal hypertension
It is the most severe and frequent complication of portal
hypertension syndromearise from varicose veins of the
esophagus and the cardiac part of the stomach as a
result of erosion of the esophageal mucosa with peptic
esophagitis or rupture of thinned vein walls.
The reasons are increased pressure in the portal vein
system, peptic factor, as well as disorders in the blood
coagulation system.
Bleeding from dilated veins of the esophagus may be the
first clinical manifestation of portal hypertension.

Clinical picture
The first indirect signs are the child's complaints of
weakness, malaise, nausea, lack of appetite, fever;
Profuse bloody vomiting suddenly appears;
Vomiting is repeated after a short period of time.
The child turns pale, complains of headache, dizziness,
becomes lethargic, drowsy.
A tarry, fetid stool appears.Blood pressure drops to 80 /
40-60/30 mm Hg. Art.Increasing anemia. BCC decreases
sharply.After 6-12 hours, the severity of the condition is
aggravated by intoxication as a result of absorption of
blood breakdown products from the gastrointestinal tract.

DIAGNOSTICS
EFGDS
Ultrasound of the liver and vessels of the
portal basin
Doppler ultrasonography of the
hepatolienal zone
Dynamics of hemogram indicators

Differential diagnosis
To clarify the cause of bleeding, anamnestic
data are of primary importance.
Children with bleeding from chronic gastric
ulcers usually have a characteristic and
prolonged "ulcerative" history. Profuse bleeding
occurs extremely rarely. Acute ulcer in children
who have received hormonal therapy for a long
time is also rarely complicated by bleeding
(perforation is more characteristic), but with an
appropriate history, diagnosis is usually not
difficult.

Differential diagnosis
In children with a hernia of the esophageal orifice,
intermittent bloody vomiting is not abundant, and the
presence of "black" stool is usually not always observed.
The general condition of the child deteriorates slowly
over many months. Children are usually admitted to the
hospital for mild anemia of unknown etiology. During
clinical and radiological examination, the presence of a
hernia of the esophageal opening of the diaphragm is
established.
The cause of bloody vomiting that occurs after
nosebleeds is clarified with a detailed anamnesis and
examination of the patient.

Treatment
In many cases of bleeding, therapeutic measures should
begin with complex therapy.
Conservative therapy:
Blood transfusion;
Hemostatic therapy: concentrated plasma, vicasol,
pituitrin; inside - e-aminocaproic acid, adroxone,
thrombin, hemostatic sponge.
On the epigastric region, an ice pack.
Elimination of enteral nutrition, parenteral administration
of fluids and vitamins (C and group B).Broad-spectrum
antibiotics; Patients with intrahepatic portal hypertension
1% glutamic acid solution.

Treatment
Attempts to stop bleeding mechanically:
Introduction into the esophagus of the Blackmore
obstructing probe.
If conservative measures are ineffective, urgent
surgical intervention:
Ligation of varicose veins of the esophagus or
cardiac part of the stomachan operation aimed at
reducing blood flow to the varicose veins of the
esophagus.

The Blackmore obturator probe:
in pediatric practice, it is practically not
used due to its poor tolerance by
children, although in isolated cases, to
temporarily stabilize the child's
homeostasis before surgical treatment,
the use of such probes can be useful.

The position of the Blackmore probe in the
esophagus a - correct; b - wrong.

Surgical treatment
All surgical interventions can be divided into
portodecompressive interventions and portoasical
separation operations.
Dissociative interventions include gastric
transsection and devascularization operations.
Portodecompressive interventions combine
various types of portosystemic shunting.

Surgical treatment
Depending on the direction of the operations of portoasical
uncoupling, there are distinguished operations of transection and
reanastomosis of the esophagus (transection) and operations of
devascularization of the esophagus and stomach.
Depending on the access, there are transabdominal (Tanner,
Hassab, Paquet and others), transthoracic (Boerema-Crile and
others) and combined (Suguira-Fatagawa) interventions.
The method of choice from a large number of disconnections is
circular suturing of the fundus of the stomach according to Tanner,
with ligation of the splenic artery and left phrenic vein and ligated
transection of the fundus of the stomach with fundogastric
anastomosis according to Nazirova F.G.

Postoperative treatment
is a continuation of the activities carried
out before the operation.Parenteral
nutrition - within 2-3 days.Gradual
expansion of the diet (kefir, 5% semolina,
broth, etc.).Blood and plasma transfusions
- until anemia is eliminated.

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