2002 Número: 1
RARE LOCATIONS OF HYDATID DISEASE.
Echenique-Elizondo, M., MD, FACS.
Amondarain Arratíbel, J.A., MD.
Lirón de Robles, C., MD
Basque Country Universty. School of Medicine.
Donostia Hospital. Deparftment of Surgery
Paseo Dr. Begiristain 105. 20014 San Sebastián. Spain.
Phone: Int 34-943462890
Fax: Int 34-943468385
Rare forms of presentation in hydatic disease may posse important diagnostic and therapeutic problems.
Material and Methods.-
Retrospective review of the cases attended at Arantzazu Hospital between the years 1979 – 1999 to determine the incidence of atypical localization of the disease.
405 patients have been included in the study. 274 (65%) had hepatic disease and 62 (15,3%) pulmonary cysts. Rare presentation forms were encountered in 69 patients: bone in 9 (2,2%), soft tissues in 15(3,7%), spleen: 5 (1,23%), pancreas: 1(0,24%), peritoneum: 23(5,67%), brain: 3 (0,74%), thyroid: 3(0,74%), kidney: 6(1,48%), heart: 1(0,24%), thymus: 2(0,48%) and adrenal gland: 1(0,24%).
Of 69 (17,5%) atypical cases (6,9%) were primary forms and (10,1%) associated to one or more different additional locations.
Hydatid disease continues to be a medical and sanitary problem in endemic areas and is been observed more frequently in regions were previously was unknown because of current migratory flows and travel uses Proteiform presentation is the rule when hydatid cyst is located in non-usual sites and locations. Hydatid disease presence must be considered for a correct management of the problem.
Hydatid disease produced by Echinococcus granulosus remains an important sanitary problem in many regions of the world - Mediterranean Sea bordering countries, South America and Oceania – despite wide knowledge on parasite biology leading to adequate sanitary preventive measures and the introduction and widespread use of chemotherapeutic agents (1).A variety of hydatid disease produced by Echinococcus multilocularis is also endemic in some specific areas (Bavaria, Alaska). Liver in first place and lung are the organs most commonly involved by parasitic growth as an unique site (primary form) or associated with other locations (associated form). Correct diagnosis is crucial in distinguishing it from other cystic processes and has important therapeutic and prognostic consequences. Moreover current population flows and travel uses are the reason why new cases of hydatid disease are being observed in areas with no previous prevalence where it can be considered a clinical rarity (2). Atypical localization has been reported in a growing incidence ranging from 3,9% to 12,5% according registered series (3,4).
Material y Methods .-
A retrospective review of the cases of hydatic disease treated between 1979 and 1999 at Azantzazu Hospital. San Sebastian. Gipuzkoa. Spain has been done.
The objective of the review was: 1/ Determine the incidence of cases per year and organs where disease was observed . 2/ Know the real incidence of atypical forms and infrequent sites of location of the disease apart of the pulmonary and hepatic distribution. 3/Evaluate diagnostic and therapeutic problems encountered.
405 patients affected with hydatic disease were studied. 274 (65%) had cysts in the liver and 62 (15,3%) pulmonary hidatidosis. Other sites were : bone in 9 (2,2%), soft tissues: 15(3,7%), spleen: 5(1,23%), pancreas: 1(0,24%), peritoneum: 23(5,67%), brain 3 (0,74%), thyroid: 3(0,74%), kidney: 6(1,48%), heart: 1(0,24%), thymus: 2(0,48%) and adrenal gland: 1(0,24%).
Rare locations were found in 69 (17,5%) cases. In 28 (6,9%) no other cyst was found and were considered pure primary forms and in 41 (10,1%) other cyst existed in two or more organs: one in 23 cases and two or more in 18 patients.
Association was synchronic in 32 (78%) cases and in 9(22%) metachronic in presentation.
Pure primary forms - 28 cases – were situated in: bone: 6,soft tissues: 3 ,spleen: 4, pancreas: 1 ,peritoneum: 2, brain: 1,thyroid: 2 , kidney; 6, heart:1, thymus:1, adrenal gland: 1.
The average number of patients treated each year was of 19 +/-8. With a maximum of 31 and a minimum of 7cases.
Hydatid disease produced by Echinococcus granulosus still represents an important medical problem in many regions and a challenge in common surgical practice worldwide (5). Commonly affects the liver and the lung. When located in non-usual sites posses serious diagnostic problems. Correct diagnosis is essential for an adequate treatment and prevent serious misleading therapeutic attitudes and complications. Affected sites reported in the literature includes adrenal gland (6), muscle and teguments (7,8), mesentery, thymus (4), uterus (9), bone (10), brain (11), kidney (12), heart (13), breast (14), spleen (15), pancreas (16,17), seminal vesicles (18), pericardium and subcutaneous tissue. Before reaching this sites the parasite must pass through the hepatic filter first and the pulmonary filter thereafter before it implants elsewhere. In every case of rare no usual localization pulmonary and hepatic involvement must be discarded. Similarly the diagnosis of pulmonary hydatic disease must be followed by hepatic examination to dismiss any associated hydatic pathology. From a theoretical point of view any anatomic structure may be affected during Echinococcal infestation (19).
Thyroid hydatic disease usually appears a ”cold” nodule on radionuclide scan and of liquid content on US examination . Aspiration-biopsy-cytology –ABC – techniques pose a risk of anaphylaxis and hydaid seeding in cervical soft tissues. This problem must be considered in thyroid evaluation in endemic areas.
Hydatic disease in the pancreas may be a disturbing diagnostic problem and must be separated from cystic neoplasms of pancreatic origin. (20,21). Pancreas may participate also in inflammatory processes – acute pancreatitis - secondary to liver hydatid disease when intracystic debris is eliminated through the biliary tree and results in biliary reflux into the pancreatic duct when impacted on the Oddi’ s sphincter.
Pelvic forms may be misdiagnosed as neoplasm of the ovary or anexae.
Cyst in soft tissues may be misinterpreted as common surgical diseases (lipoma, fibroma) as it happens in the ossifluent could abscesses derived from Echinococcosis of osseous structures mimicking slow growing soft masses containing hydatid cysts.
Spleen involvement of the disease may produce diagnostic errors with benign splenic cysts. Same problem may be encountered in the hydatid disease of the kydney. (22,23, 24,25).
Progress has been made on the biological diagnosis and immunological changes in hydatidosis due to Echinococcus granulosus (26,27,28,29) but its value remains complementary to imaging diagnostic procedures because of the number of false negative results due to no existing hypersensitivity to the antigen even in the presence of demonstrable disease.
Diagnosis of hydatid disease of atypical location must be suspected when associated to liver or pulmonary cysts. However in unusual pure forms without any other organ involved diagnosis and correct management may represent a challenge.
Surgery remains the main treatment in hydatidosis with well founded criteriae and approach in the most frequent locations of the disease: liver (30) and lung (31). Surgical planning and techniques are dependent on the number of cysts, the anatomical relations and anatomical changes produced by the parasite growth. Surgery is also the principal form of treatment in atypical forms of hydatid disease. Surgical approach and technique depends on correct diagnosis and if it has been made before or during operation. Every case is unique and different and must be considered in this manner. The use of antiparasitic medication or solutions during operation varies according to hospitals and medical tradition – hypertonic saline, ether, formic aldehyde, oxigen peroxide, cetrimide, rivanol, alcohol - (32) . We employ hypertonic saline solution –33% NaCl- as a routine when indicated by contamination or unexpected opening of the cyst content.
Chemotherapeutic agents - Mebendazol and Albendazol - without surgery have demonstrated reduction in the cysts size and occasional elimination of the parasite but not demonstrable benefit has been described so far in large homogeneous series and not scientific conclusions can be drawn (33,34,35,36). Their mechanism of action is known to be through a blockade of the glucose intake and glucogen deprivation of the parasite with growth retardation and even sterilization of the content. As the purpouse of the treatment includes elimination of the Echinococcus and handling of the resulting cavity and associated damage it may happen that this cannot be accomplished in every case with chemotherapy alone. Antiparasitic agents can be used prior to surgery as a safeguard measure and after surgery to prevent further implants and secondary hydatid seeding but not as a sole therapeutic purpose. In a neoadjuvant manner before surgery and coadjuvant thereafter are the correct uses of antipararasitic drugs.
Several recent publications propose minimally invasive approach for diagnosis – fine needle guided aspiration - and treatement (37,38, 39, 40) contrary to the classical surgical attitude that prohibits any blind procedure because of the important and serious risk of anaphylaxis (41) and parasite seeding . In addition secondary organic damage produced by de parasite growth cannot be treated adequately this manner. Demonstration of its existence is extremely difficult to be determined or suspected prior to surgery in many cases. Moreover a complicated or mutilocular cyst dismiss this form of treatment We think that this techniques must be carefully employed in selected patients and cannot be generalized as the first step in the treatment in hydatidosis.
Hyadatid disease continues to be a medical and sanitary problem. Its knowledge remains crucial for diagnostic algorithms. The slow growing pattern of the parasite combined with current migratory flows and travel uses are the reason why is observed more widely even in the less expected sites where was previously unknown . Proteiform pressentation is the rule when hydatid cyst is located in non-usual sites and locations. Presence of hydatid disease must be considered for a correct management of the problem when considering cystic pathology anywhere.
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