António Menezes da Silva CYSTIC ECHINOCOCCOSIS HYDATID CYST - Defintion and nomenclature HYDATID CYST IN THE LIVER - Therapeutic strategy António Menezes da Silva

HYDATID CYST definition Un kyste hidatique se compose de deux parties: - le PARASITE échinococcique, et - l’ADVENTICE qui l’encapsule Félix Dévé (1905) The hydatid cyst is composed by two parts: - the echinococcal parasite (or hydatid), and - the adventitia that involves it

Hydatid cyst (schematic)

Anatomical nomenclature gl – germinal layer; adv - adventitia bc – brood capsules scol - scolices

X The adventitia is NOT pericyst It IS part of the cyst Anatomical nomenclature X The adventitia is NOT pericyst It IS part of the cyst “Pericyst” can be ALL normal tissue around the cyst

Anatomical nomenclature Scolice Brood capsule Daughter vesicles

UNIVESICULAR (simple) cyst (type 1 WHO) Anatomical nomenclature UNIVESICULAR (simple) cyst (type 1 WHO) ONE vesicle only (mother vesicle)

MULTIVESICULAR cyst (type 2 WHO) Anatomical nomenclature MULTIVESICULAR cyst (type 2 WHO) MORE than ONE vesicle (daughter vesicles) X NOT daughter cysts

HYDATID CYSTS IN THE LIVER THERAPEUTIC STRATEGY

What cysts to treat? Therapeutic options? There is no “best” treatment option for CE and no clinical trial has compared all the different treatment modalities, including “Watch and Wait” One important difference is between complicated and uncomplicated cysts (TJ, AMS, JH, PC, EB – AJTMH, 2008)

Cysts characteristics What cysts to treat? ► COMPLICAED CYSTS ! ► Uncomplicated cysts ? - Asymptomatic - Symptomatic Cysts characteristics (US Classification)

Complicated cysts Therapeutic options SURGERY (Always ?) Innovative interventions Double drainage technique (Benazouz) - internal – endoscopic - external – percutaneous 12

Complicated cysts:

Complicated cysts:

Uncomplicated cysts Therapeutic options Non-invasive treatment ● Anti-parasitic drugs Invasive treatment ● Conservative procedures - Non-surgical approaches - Surgical approaches ● Radical procedures ► ONLY surgical approaches 15

Invasive approaches Conservative procedures Radical procedures Cyst sterilization Parasite (hydatid) removal Partial cyst removal Radical procedures Total cyst removal Organ resection 16

Conservative procedures Cyst sterilization - Percutaneous (PAIR/Radiofrequency) - Surgery (Laparotomy/Laparoscopy) Parasite (hydatid) removal - Percutaneous (PEvac) Partial cyst removal 17

Cyst sterilization Puncture and scolicidal solution injection puncture/aspiration scolicidal agent injection total aspiration Parasite death Thermo-ablation (radiofrequency)

Removal of the parasite (hydatid) ONLY (HYDATIDECTOMY) Percutaneous scolicidal agent injection total aspiration including the parasite puncture/aspiration Surgical puncture/aspiration Removal of the parasite (hydatid) Adventitia is not removed

Removal of the ENTIRE cyst (parasite + adventitia) Radical procedures Removal of the ENTIRE cyst (parasite + adventitia) Total cystectomy - Open method - Closed method (Napalkoff operation) Organ resection - Segmentectomy - Lobectomy 20

entire cyst “wall” (adventitia) removed TOTAL CYSTECTOMY Open method entire cyst “wall” (adventitia) removed open the cyst content aspiration begning cyst removal complete cyst removal 21

Closed method (Napalkoff) TOTAL CYSTECTOMY Closed method (Napalkoff)

TOTAL CYSTECTOMY In the past the dissection was made in sane hepatic tissue (Pericystectomy) (more bloody dissection and risk of lesion of the hepatic structures) Nowadays the dissection is made in the virtual space between the adventitia and sane hepatic tissue (less bloody dissection, less risk of lesions) (Peng Xinyu technique)

Closed total cystectomy (Peng Xinyu procedure)

Removal of the ENTIRE cyst Organ resection Segmentectomy II/III

Anti-parasitic drugs (ABZ) Approach proposal Type (WHO) Anti-parasitic drugs (ABZ) Percutaneous treatment Surgery Suggestion 1 X - 1st – ABZ 2nd - PAIR 2 X (?) 1st – Pevac (?) 2nd – Surgery 3 a 3 b 2nd – Pevac (?) 3rd – Surgery 4 & 5 Watch & wait

In conclusion The indications for hydatid cysts in the liver treatment are complex and are based on cyst characteristics, available medical/surgical expertise and equipment, and adherence of patients to long-term monitoring. Because treatment involves a variety of options and requires specific clinical experience, patients should be referred to recognized reference and national/regional CE treatment centres, whenever available.

Lisbon – Portugal Thank you