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Disclosure Statement of Financial Interest
Dr Maghrebi Houcine
“Nothing To Disclose”
EP01B-088
Budd-Chiari Syndrome Secondary to Hepatic Echinococcosis
H. Maghrebi, A. El Heni, R. Rhaiem, A. Makni, A. Daghfous, F. Fteriche,
M. Jouini, M. Kacem, Z. Bensafta
Department of general surgery , Rabta Hospital, Tunisia
Introduction :
The hydatid cyst is a worldwide zoonosis caused by Echinococcus
granulosus. It remains a major public health problem in ourTunisia. Budd-Chiari
syndrome (BCS) is a very rare complication. We herein report the clinical
features, radiological investigations and therapeutic management.
Patients and methods:
•we reviewed retrospectively all the patients seen between January 1990 and
December 2015 who presented an hydatid cyst of the liver causing Budd-
Chiari syndrome
•An abdominal US was available for all patients and confirmed the diagnosis
of hydatid disease. All patients underwent surgical treatment usually after a
period of medical management
•Clinical, radiological, operative and postoperative Findings were recorded as
well as follow-up data
Results:
•The series included 14 patients: 11 females and 3 males
with a mean age of 33 years.
•The predominant symptom was pain in the right
Hypochondrium.
•Budd-Chiari syndrome was subacute in 75% of cases.
•The physical examination revealed hepatomegaly in all the
patients, collateral venous circulation , ascites (50%), and
cutaneomucosal jaundice (2patients)
•Laboratory tests showed cholestasis in 8 patients and
elevated serum transaminase levels in all patients. Four
patients presented coagulation disorders
•The radiologic diagnosis of BCS in a patient with a hydatid
cyst can be established using ultrasonography (US), color
Doppler, CT, and magnetic resonance imaging (MRI)
Figure . Intraoperative view : dysmorphic liver
and hypertrophy of segment I,
Results:
•Average cyst diameter in our series was13 cm. The cysts were
located in the hepatic dome in all patients
•Preoperative care was necessary : it consist specially on anti-
coagulation (heparin 3-5 mg/kg/d), ascites paracentesis,
correction of anemia and hemostasis (infusion of fresh-frozen
plasma). A provision of at least three units of packed red cells
was available at surgery in all patients.
•Laparotomy was performed in all cases. The surgical
procedure consisted in a conservative treatment in all patient
(resection of the protruding hepatic tissue and puncture
aspiration). The residual cavity was drained in all patients
•Operative mortality was about 7% (septic shock on the fourth
postoperative day.) and morbidity was more than 50% due
specially to biliary fistula.
Fig. venous compreession by the hydatid cyst (CT), MRI.
Discussion:
• Hydatid disease is a parasitic infection caused by several species of the
Echinococcus cestode. The most common in Tunisia is E. granulosus
•The clinical signs and symptoms of hydatid disease vary from incidental
findings to severe, life-threatening complications such as Budd-Chiari
syndrome
•Budd-Chiari syndrome is an uncommon disorder characterized by the
obstruction of hepatic venous outflow leading to progressive liver damage and
portal hypertension. The obstruction has to involve at least two of the main
suprahepatic veins to produce BCS
•The etiology of Budd-Chiari syndrome is classified as primary (intrinsic
intraluminal thrombosis ) or secondary caused by intraluminal invasion (by a
malignant tumor) or extraluminal compression (by a solid tumor, or cyst, such
as a hydatid cyst). Hydatid causes are exceptional
•In our series, none of the patients had a personal or family history of venous
thrombosis and hematology tests were normal (excepting two patients with
inflammatory anemia).
Discussion:
•According to the duration of disease, BCS can be classified as fulminant,
acute, subacute, or chronic.
•Abdominal pain, fever, ascites, leg edema, and hepatomegaly are
present in almost all patients with BCS. Jaundice, gastrointestinal
bleeding, and hepatic encephalopathy are less common
•The radiologic diagnosis of BCS shows : defective hepatic perfusion,
hypertrophy of segment I, and thrombus formation in the inferior vena
cava or the hepatic veins
•Preoperative management is necessary: heparin in the event of venous
thrombosis, treatment of ascites, and treatment of anemia
•The conservative surgical approach (resection of the protruding dome )
for the hydatid cyst should be used to eliminate compression . Radical
surgical options should be avoided due to the high risks of hemorrhage
and liver injury.
•Mortality is high for surgical treatment of hydatid cysts of the liver in
patients with BCS (7% in our series). Morbidity is also high especially
when Preoperative management is insufficiant. It is generally related to
deep abscess formation, biliary fistulae..
References
1-Dumortier J, Conord S, Henry L, Trzeciak MC, Boillot O, Partensky C, et al. Syndrome de Budd-Chiari. Prise en charge diagnostique et
thérapeutique des formes aigues et subaiguës. Presse Med 1999;28: 802-8.
2. Heykal B, Kais N, Sofiene A. Budd-Chiari syndrome secondary to hepatic echinococcosis. Gastroenterol Clin Biol 2007;31:721-724
3 N. Maàmouri · F. Ben Hariz · N. Belkahla. Syndrome de Budd-Chiari : complication rare du kyste hydatique du foie : à propos de trois cas. J. Afr.
Hépatol. Gastroentérol. (2011) 5:193-197
4 Sarawagi R, Keshava SN, Surendrababu NR. Budd-Chiarri syndrome complicating hydatid cyst of the liver managed by venoplasty and stenting.
Cardiovasc intervent radiol 2011; 34 Suppl 2:S202-05.
5- Mahajan D, Mandeep K. Hydatid Cyst of liver: a rare cause of secondary Budd-Chiarri Syndrome. Journal of Postgraduate Medecine, Education
and research, July-September 2013;47 (3): 159-161
Conclusion :
Budd-Chiari syndrome (BCS) is an uncommon complication of hydatid cyst of the
live wich causes a high mortality and morbidity. The improvement of prognosis
needs an early diagnosis and a careful preoperative preparation. Prevention of
echinococcosis in endemic zones is the best approach for improved treatment

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Budd-Chiari Syndrome Secondary to Hepatic Echinococcosis.ppt

  • 1. Disclosure Statement of Financial Interest Dr Maghrebi Houcine “Nothing To Disclose”
  • 2. EP01B-088 Budd-Chiari Syndrome Secondary to Hepatic Echinococcosis H. Maghrebi, A. El Heni, R. Rhaiem, A. Makni, A. Daghfous, F. Fteriche, M. Jouini, M. Kacem, Z. Bensafta Department of general surgery , Rabta Hospital, Tunisia Introduction : The hydatid cyst is a worldwide zoonosis caused by Echinococcus granulosus. It remains a major public health problem in ourTunisia. Budd-Chiari syndrome (BCS) is a very rare complication. We herein report the clinical features, radiological investigations and therapeutic management.
  • 3. Patients and methods: •we reviewed retrospectively all the patients seen between January 1990 and December 2015 who presented an hydatid cyst of the liver causing Budd- Chiari syndrome •An abdominal US was available for all patients and confirmed the diagnosis of hydatid disease. All patients underwent surgical treatment usually after a period of medical management •Clinical, radiological, operative and postoperative Findings were recorded as well as follow-up data
  • 4. Results: •The series included 14 patients: 11 females and 3 males with a mean age of 33 years. •The predominant symptom was pain in the right Hypochondrium. •Budd-Chiari syndrome was subacute in 75% of cases. •The physical examination revealed hepatomegaly in all the patients, collateral venous circulation , ascites (50%), and cutaneomucosal jaundice (2patients) •Laboratory tests showed cholestasis in 8 patients and elevated serum transaminase levels in all patients. Four patients presented coagulation disorders •The radiologic diagnosis of BCS in a patient with a hydatid cyst can be established using ultrasonography (US), color Doppler, CT, and magnetic resonance imaging (MRI) Figure . Intraoperative view : dysmorphic liver and hypertrophy of segment I,
  • 5. Results: •Average cyst diameter in our series was13 cm. The cysts were located in the hepatic dome in all patients •Preoperative care was necessary : it consist specially on anti- coagulation (heparin 3-5 mg/kg/d), ascites paracentesis, correction of anemia and hemostasis (infusion of fresh-frozen plasma). A provision of at least three units of packed red cells was available at surgery in all patients. •Laparotomy was performed in all cases. The surgical procedure consisted in a conservative treatment in all patient (resection of the protruding hepatic tissue and puncture aspiration). The residual cavity was drained in all patients •Operative mortality was about 7% (septic shock on the fourth postoperative day.) and morbidity was more than 50% due specially to biliary fistula. Fig. venous compreession by the hydatid cyst (CT), MRI.
  • 6. Discussion: • Hydatid disease is a parasitic infection caused by several species of the Echinococcus cestode. The most common in Tunisia is E. granulosus •The clinical signs and symptoms of hydatid disease vary from incidental findings to severe, life-threatening complications such as Budd-Chiari syndrome •Budd-Chiari syndrome is an uncommon disorder characterized by the obstruction of hepatic venous outflow leading to progressive liver damage and portal hypertension. The obstruction has to involve at least two of the main suprahepatic veins to produce BCS •The etiology of Budd-Chiari syndrome is classified as primary (intrinsic intraluminal thrombosis ) or secondary caused by intraluminal invasion (by a malignant tumor) or extraluminal compression (by a solid tumor, or cyst, such as a hydatid cyst). Hydatid causes are exceptional •In our series, none of the patients had a personal or family history of venous thrombosis and hematology tests were normal (excepting two patients with inflammatory anemia).
  • 7. Discussion: •According to the duration of disease, BCS can be classified as fulminant, acute, subacute, or chronic. •Abdominal pain, fever, ascites, leg edema, and hepatomegaly are present in almost all patients with BCS. Jaundice, gastrointestinal bleeding, and hepatic encephalopathy are less common •The radiologic diagnosis of BCS shows : defective hepatic perfusion, hypertrophy of segment I, and thrombus formation in the inferior vena cava or the hepatic veins •Preoperative management is necessary: heparin in the event of venous thrombosis, treatment of ascites, and treatment of anemia •The conservative surgical approach (resection of the protruding dome ) for the hydatid cyst should be used to eliminate compression . Radical surgical options should be avoided due to the high risks of hemorrhage and liver injury. •Mortality is high for surgical treatment of hydatid cysts of the liver in patients with BCS (7% in our series). Morbidity is also high especially when Preoperative management is insufficiant. It is generally related to deep abscess formation, biliary fistulae..
  • 8. References 1-Dumortier J, Conord S, Henry L, Trzeciak MC, Boillot O, Partensky C, et al. Syndrome de Budd-Chiari. Prise en charge diagnostique et thérapeutique des formes aigues et subaiguës. Presse Med 1999;28: 802-8. 2. Heykal B, Kais N, Sofiene A. Budd-Chiari syndrome secondary to hepatic echinococcosis. Gastroenterol Clin Biol 2007;31:721-724 3 N. Maàmouri · F. Ben Hariz · N. Belkahla. Syndrome de Budd-Chiari : complication rare du kyste hydatique du foie : à propos de trois cas. J. Afr. Hépatol. Gastroentérol. (2011) 5:193-197 4 Sarawagi R, Keshava SN, Surendrababu NR. Budd-Chiarri syndrome complicating hydatid cyst of the liver managed by venoplasty and stenting. Cardiovasc intervent radiol 2011; 34 Suppl 2:S202-05. 5- Mahajan D, Mandeep K. Hydatid Cyst of liver: a rare cause of secondary Budd-Chiarri Syndrome. Journal of Postgraduate Medecine, Education and research, July-September 2013;47 (3): 159-161 Conclusion : Budd-Chiari syndrome (BCS) is an uncommon complication of hydatid cyst of the live wich causes a high mortality and morbidity. The improvement of prognosis needs an early diagnosis and a careful preoperative preparation. Prevention of echinococcosis in endemic zones is the best approach for improved treatment