Badd's syndrome — Kiari

Description:

Badda-Kiari's syndrome  - obstruction (thrombosis) of hepatic veins which is observed at the level of their falling into the lower vena cava and leads to disturbance of outflow of blood from a liver.

For the first time the disease was described by the English doctor of G. Budd in 1845. Later, in 1899 the Austrian pathologist N. of Chiari provided information on 13 cases of this syndrome.

Symptoms of the Syndrome of Badda-Kiari:

Current of a syndrome of Badda-Kiari can be acute and chronic.
Clinical manifestations at an acute current of a syndrome of Badda-Kiari.

Acute current of a syndrome of Badda-Kiari meets in 5-20% of cases.

In most cases the clinical symptomatology at Badda-Kiari's syndrome depends on the disease which caused a syndrome and also on duration of existence of venous obstruction.

At acute obstruction of hepatic veins vomiting, megalgias in epigastric area and in right hypochondrium suddenly develop (owing to swelling of a liver and stretching of the glissonovy capsule),  jaundice is noted  . At an acute form symptoms of a disease arise suddenly and have the expressed character with bystry progressing.

The disease quickly progresses, within several days  ascites develops  . In an end-stage the hematemesis appears.

At the joined thrombosis of the lower vena cava hypostases of the lower extremities, a phlebectasia on a front abdominal wall are observed. In hard cases of total obstruction of hepatic veins thrombosis of mezenterialny vessels with pains and diarrhea develops.

At an acute current of a syndrome of Badda-Kiari, as a rule, within several days there comes the death of patients.
Clinical manifestations at a chronic current of a syndrome of Badda-Kiari.

At a chronic form (meets in 80-95% of cases) a disease the long time can proceed asymptomatically.

At a course of a disease, pains in right hypochondrium, vomiting, small  jaundice develop further  .

At a long current collateral circulation which is shown by expansion of superficial veins of skin gradually develops. In the presence of varicose veins of a gullet bleedings can be observed.

Accumulation of liquid in an abdominal cavity (ascites) and expansion of superficial veins on a front abdominal wall with primary (inborn) option of a syndrome of Badda-Kiari.

Reasons of the Syndrome of Badda-Kiari:

Mechanical reasons:
Inborn hymenoid fusion of the lower vena cava.
Type I: the thin membrane is in the lower vena cava.
Type II: lack of a segment of the lower vena cava.
Type III: lack of a blood-groove on the lower vena cava and development of collaterals.
Stenosis of a hepatic vein.
Hypoplasia of suprahepatic veins.
Postoperative obstruction of hepatic veins and lower vena cava.
Posttraumatic obstruction of hepatic veins and lower vena cava.
Parenteral food.

Cases of development of a syndrome of Badda-Kiari as a complication of catheterization of the lower vena cava at newborns are known.
Tumors.
Pochechnokletochny carcinoma.
Hepatocellular carcinoma.
Tumors of adrenal glands.
Leiomyosarcoma of the lower vena cava.
Myxoma of the right auricle.
Vilms's tumor.
Coagulation disturbances:
Hematologic frustration.
Polycythemia.
Thrombocytosis.
Paroxysmal night haemoglobinuria.
Myeloproliferative diseases.
Coagulopathies:
Deficit of a protein of Page.
Deficit of a protein of S.
Deficit of antithrombin II.
Anti-phospholipidic syndrome.
Other coagulopathies.
System vasculites.
Sickemia.
Chronic inflammatory diseases of intestines.
Reception of oral contraceptives.
Infectious diseases:
Tuberculosis  .
Aspergillosis.
Filariosis.
Echinococcosis.
Amebiasis.
Syphilis.

Treatment of the Syndrome of Badda-Kiari:

Drug treatment of a syndrome of Badda-Kiari

Medicamentous therapy of a syndrome of Badda-Kiari gives not expressed and short-term effect. When holding only medicamentous actions 2-year survival of patients with Badda-Kiari's syndrome makes 80-85%.

In therapy diuretics, anticoagulants, thrombolytic drugs are applied.

Can be applied:  эноксапарин sodium      (Kleksan); дальтепарин sodium    (Fragmin).
Thrombolytic therapy.  It is carried out by the following drugs:
Streptokinasa  (Streptodecasum, Streptasum) can be entered through a catheter for 7500 ME/hour or in/in for 100000 ME/hour after bolyusny introduction in a dose 250000 ME.
The urokinase  is entered into 4400 ME/kg within 10 min. then the dose raises to 6000. A maintenance dose - 4400-6000 ME/hour, in / century.
Alteplaza  (Aktiliza) is entered in/in, the adult, on 0,25-0,5 mg/kg, within 60 min.
Diuretic therapy. Diuretic therapy consists in prolonged use  of Spironolactonum  (  Veroshpiron  ,  Veroshpilakton  ) and  furosemide  (  Lasixum  ,  tab. Furosemide  ) in combination with restrictions in a diet (restriction of reception of water, sodium) and administration of salts of potassium.
Spironolactonum  (  Veroshpiron  ,  Veroshpilakton  ) is appointed inside: adult - 25-200 mg/days 2-4 times a day (but no more than 200 mg/days); to children - 1-3,3 mg/kg/days 2-4 times a day.
Furosemide  (  Lasixum  ,  tab. Furosemide  ) is appointed inside: the adult - inside on 20-80 mg/days each 6-12 hours (but no more than 600 mg/days); to children - inside on 0,5-2,0 mg/kg/days each 6-12 hours (no more than 6 mg/kg).

Surgical treatment of a syndrome of Badda-Kiari:

The type of surgical intervention at Badda-Kiari's syndrome is defined by the reason which caused it.

At hymenoid fusion of a gleam of the lower vena cava transdermal installation of a stent after balloon dilatation can be carried out.