Giant intra-abdominal hydatid cysts with multivisceral locations

Discussion:

Hydatid disease is a parasitic disease caused by the larval
stage of Echinococcus granulosus. Hydatid disease remains
a continuous public health problem in endemic countries
including India. The most frequently involved organs are
the liver (55-70%) followed by the lung (18-35%); these two
organs affected simultaneously in about 5-13% of cases, the
spleen (5%) and other organs (5%).(1-2 ) Apart from com-
mon sites such as liver and lungs in humans, hydatid cysts
can present in unusual sites which include peritoneum, kid-
ney, muscle, adrenal gland, ovary, pancreas, thyroid gland,
pleura, diaphragm, uterus and brain.(1) The peritoneal hy-
datid disease represents an uncommon occurrence with an
overall frequency of peritoneal disease in cases of abdomi-
nal echinococcus at approximately 13%. Peritoneal hydati-
dosis is almost always secondary to hepatic disease, al-
though some unusual cases of primary peritoneal hydati-
dosis have been described. Peritoneal involvement is usual-
ly undetected unless cysts are large enough to cause symp-
toms. Most of the cases of peritoneal hydatid disease are
secondary to previous surgery for liver hydatidosis; howev-
er, spontaneous microrupture of a hepatic cyst into peri-
toneum has also been reported. Intraperitoneal rupture of
hepatic or splenic cysts results in release of brood’s cap-
sule, scolices and daughter cysts which implant and devel-
op independently leading to multiple disseminated in-
traperitoneal hydatid disease. This phenomenon is called
secondary echinococcosis.(3)

Peritoneal hydatid disease may grow and occupy the entire
peritoneal cavity, simulating a multilobulated mass. This
pathological condition is known as encysted peritoneal hy-
datidosis. Peritoneal hydatid disease represents an uncom-
mon occurrence and its diagnosis is more accurate today
due to the new imaging techniques.(4) A double contrast
CT scan is 90-100% accurate for diagnosing secondary
echinococcosis and is superior to USG in identifying addi-
tional extrahepatic intra abdominal cysts.(3) CT is the
modality of choice for these patients because it permits
imaging of the entire abdomen and pelvis.(5)

The goal of treatment is to prevent secondary complica-
tions of the disease. In cases of disseminated systemic dis-
ease, inoperable cases, cyclic therapy with a benzimidazole
compound is preferred and has been found to cure disease
in 10-30% of cases, to slow progression in 50%-70% and to
yield no change in 20%- 30%.(6) Albendazole seems to be
more effective owing to better penetration and absorption.
These agents have actually been used in several studies as
a conservative treatment, leading to some decrease or sta-
bilization of the cyst size, especially in cases with small
cysts. Albendazole is combined with surgery to prevent
postoperative recurrence.(2) After medical therapy, follow
up is advisable with USG or CT scan. After medical treat-
ment, hydatid cysts shows gradual reduction in cyst size
and number. The cyst becomes poorly defined and denser.
Daughter cysts may disappear or may rupture and the lam-
inated membrane may separate from the pericyst and col-
lapse. Thickening and calcification of the cyst wall can be
taken as therapeutic response if it was absent before treat-
ment. Cyst disappearance should be regarded as the crite-
ria with most promising prognostic value.

Surgical removal of the cyst is customized to each patient
depending on the patient's general condition, the number
and localization of cysts, and the surgeon's expertise.(1-2)
Recurrence of hydatid disease after after radical proce-
dures is very low, but not zero and the possibility of recur-
rence always remains.(2) PAIR( puncture, aspiration, instil-
lation of scolicidal agents and reaspiration) technique is
also possible in cysts relapsing after surgery or failed
chemotherapy. However PAIR is contraindicated in cysts
that communicate with biliary tree, superficial cysts and
cysts with thick internal septal divisions.(7)

The purpose of reporting this case is to highlight the un-
common presentation of hydatid disease due to multivis-
ceral and peritoneal locations.

References:

1. Syed Abdullah Iqbal, Masood Jawaid, Fareya Us-
mani: Disseminated Intra-Abdominal Hydatidosis:
A Very Rare Presentation. The Internet Journal of
Surgery. 2007;11:1.

2. Safioleas MC, Misiakos EP, Kouvaraki M, et al.
Hydatid Disease of the Liver. Arch

Surg. 2006;141:1101-8

3. Sahu SK, Singh P, Sachan PK,et al. Secondary
echinococcosis. Indian J Surg 2006;68:334-5.

4. Tarcoveanu E, Dimofte G, Bradea C,et al. Multi-
ple Peritoneal Hydatid Disease after Rupture of a
Multivesicular Hepatic Hydatid Cyst. Case report.
J Gastrointestin Liver Dis 2006;15:301-5

5. Yuksel M, Demirpolat G, Sever A, et al. Hydatid
disease involving some rarelocations in the body:
a pictorial essay. Korean J Radiol 2007;8:531-40

6. Karmali S, Thompson SK, McKinnon G, et al. A
37-year-old woman with fever and abdominal-
pain. CMAJ 2005;172:1683.

7. White CA, Weller PF. Cestodes. In: Kasper DL,
Braunwald E, Fauci SA, Hauser SL, Longo DL,
Jameson JL, eds. Harrison’s Principles of Internal
Medicine. 16th ed. New York. McGraw-Hill.2005.
pp 1275-76

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