Pleural complications of hydatid disease (Echinococcusgranulosus) M! von Sinner King Falsiil Specialist Ilospitiil and Hescaicli Ceiitre. tiiyacih. Saudi Arabia
Siimrnary
Pleurale Komplikationen bei Hydatidenbefall (Eehinokokkose) -
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Seventy patients with hydatid disease were referred to our institution during the last ten years. In all cases the clinical and radiological findings were confirmed by pathology. In three of seventy patients pleural complications were foiind consisting in hydropneumothorax and a life-threatening tension pneumothorax following pleural perforation of ruptured pulmonary hydatid cysts in two cases and prolapsed hepatic hydatid cyst in one case. Bronchial rupture with anaphylactic syrnptoms and collapse occurred in one patient. In another, the pathologic anatomic examinatiori gave clues of a n old bilio-bronchial fistiila. In all cases. lobectomies had to be carried out due to involvement and extensive destruction of surrounding lung tissue. CT visualized and localized the lesion and was helpful for a correct diagnosis. Ruptured and infected hydatid cysts are often confused with tumors and/or abscesses or empyema. Early recognition is important to prevent complications and if dissemination has occurred, to combine surgery with mebendazole or albendazole treatment.
lntroduction P
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Primary pleural hydatid disease is very rare (1-4, 13). Secondary involvement of the pleura occurs more frequently. usually as a complication of ruptured or infected pulmonary hydatid cysts (6, 10. 11, 14) and less frequently as a complication of liver cysts arising near the diaphragm, secondarily involving the basal or posterior pleura (1. 7, 8, 15.17, 19). The diagnosis of echinococcosis of the pleura is dificult because of its non-specific appearance. Clinical signs and symptoms and conventional radiography are Fortschr.Höntganstr. 152.6(1990)718-722
O GeorgThiemeVerlagStuttgart . New Ynrk
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70 Patienten mit Hydatidenbefall wurden wahrend dcr letzten zehn Jahre zur Behandlung eingewiesen. In allen Fällen bestätigte der pathologische die klinischen und radiologischen Befunde. Bei drei vor1 70 Patienten wurde Hydropneumothorax als pleurale Komplikation festgestellt: bei 7wei Patienten trat ein leberisbedrohender Spannungspneiimothorax nach pleuraler Perforierung aufgebrochener pulmonaler Hydatidzysten auf und bei einem Patienten als Folge eines Prolapsus einer hepatischen Hydatidzyste. Bei einem Patienten wurde ein Bronchialriß mit anaphylaktischen Schocksymptomen und Kollaps beobachtet. Bei einem weiteren Patienten ergab die pathologisch-anatomische Untersuchung Hinweise auf eine bereits früher bestehende Biliobronchialfistel. In allen Fällen mußten Lobektomien vorgenommen werden, weil das umliegende Lungengewebe befallen und weitgehend zerstört war. Die Compiitertoinographie veranschaulichte und lokalisierte die Läsion und erleichterte die Diagnosestellung. Aufgebrochene und infizierte Hydatidzysten werden oft mit Tumoren und/oder Abszessen oder Empyemen verwechselt. Die Früherkennung ist wichtig, um Komplikationen zu vermeiden. 1st bereits eine Streuung eingetreten, ist deren frühzeitige Erkennung ebenfalls von großer Bedeutung, um den chirurgischen Eingriff ggf. mit einer Mebendazol- bzw. Albendazolbehandlung zu kombinieren.
seldomly helpful. lntracutaneous and serologic tests. if the possibility of echinococcosis is taken into consideration a t all, are often unreliable, c?specially in non-complicated pulmonary hydatid cysts. False positives and false negative tests may confuse the diagriosis. Although specific diagnosis by modern imaging methods, such a s CT and MRI. may not always be conclusive, there is a higher chance to recognize the disease or to suspect its true etiology. This is important for appropriate surgical and/or chemotherapeutic management and post-therapeutic control. I'atients and Methods P-
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During ttie last ten ycars seventy cascs of tiydatid disease, all vcrified by pathologic-analomic findings, have been referred to out institution wliich serves as a tertiary referral centre. The age and Sex distribution showed a predominance oi
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Fig. 1 A26 year old male with intrapleural perforated hydatid cyst causing tension pneumothorax. a) Chest x.ray PA-view:before rupture. b)Chest x-ray PA-view:after rupture. C) CT: expansity containing speck of airwith moderate pleural effusion. d) Microscopic pathology (operated specimen):Collapsed hydatid cyst with cystic membraneswithoutgerminal layerand with large amount of bile pigment and inflammatory round cell infiltration,bacterial growth.
fcmalc paticnts with a Sex relation of 1 : 1.5 rnale:female. Hydatid cysts occurred morc frequently in younger paticnts with a peak in the age group 20-29 years: however. another less promincnt penk w a s found in the age group 50 5 9 ycars. Sixty per cent orthe tiydatid cysts occurrcd in the liver. 20% in lung parcnchyma. and 20% in the rest of the body (brain. spleen. pelvis. pnncrcas. abdominal wall, skeletori. etc.). 'Thirty paticnts had Single cysts arid 20 patients showcd multiple cysts within the snrne Organ o r within different Organs or tissues. In 20% a pleural reactioii was foiind, usually manifcsted by pleural effusion. In three cases, major pleural complications arose which are presented in thrcc rcprcsentative cases.
Case 1. A 26 ycar oid male was rekrred to our hospitnl for sudden pairiiri the right shnuldcr followed by fever and unprodiictive cough. It was assurned that tlie patient had suffered from a n acute prieunionia which rapidly responded to erythrorny-
cin leaving local pleural thickening and scarring. Tuberculosis was a remote possibility. The patient was. therefore, kept under surveillance. Hali a year Inter (Fig. 1 a. b). the patient felt sudden severc pain along the trachea accompnnied by forceful coughing. Multiple Sputum exarns were negative for malignant cells. CT of the chest (Fig. 1 C) showed the lesion to bc in contact with the pleura and appearcd to be associated with a pleural effusion: it contained at least one small speck of air. 'l'he sonography of the right hernithorax showed aii aneclioic lesion thought to contain fluid. DiiTerential diagnostically. a lung turnor o r a Iiing absccss w a s suspected. Smcars and cultures were negative for AFB. Yhe total blood cell count was normal except for a borderline eosinophilia of 5%. A fine iieedle nspiration biopsy and bronchial washiiigs were ncgative Tor malignant cells. Since the paticnt had not further iniproved after a ycar, h e was finally operated under the clinical diagnosis of a chroriic Iiing abscess. Thc macroscopic exarniriation oi' the opcrated specimen (from right lnwer lobcc-
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Pl>leurnl(,'ornplicaiions q//&jdc/tid Diseuse (Fchinococcus Gru~iuloscts)
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Case 2. A 28 year old foiiiale was rcpeatedly operated for recurrent hydatid disease: Six years ago she had a left hcpatic lobectomy for a hydatid cyst. Onc year later she was reoperated for recurrence ofthe disease. After an intcrirn tiine of four years she was again operated fur a hydatid cyst involving the righl adnexal area. Six rnonths ago a chest X-rayshowed a large opaquc cystic structure in the left lung. Two wceks prior to her adrnission she had an acute attack. coughing out largc arnounts of white sputuni accornpanied by streaks of blood. She collapsed and was in acute distrcss, and was subseqiiently rel'erred to our hospital for further treatrnent. On physical exarnination. shc had a pale chronically ill appearance. Laboratory investigations were uncharacteristic except for IHA which was positive for echinococcosis. A chest X-ray showed a left pneumothorax of approximately 80% or rnore. The left lung had collapsed rnedially into the left lower zone. There were soveral pleural bands and a large arnount of fluid a t thc leit lung base (Fig. 2 a). A shift of thc rnediastinurn to the right was consistent with tension pneurnothorax rollowing a ruptiire of thc patient's known left pulmonary hydatid cyst IFig. 2 b). Cl' of the chest showed a collapsed infected hydatid cyst which had ruptured into the pleural space (Fig. 2 C).An ultrasound and CT scan of thc liver showed a space occupying lesion in the posterior superior aspect of the right lobe of the liver rneasuring 4,s crn in greatest diarncter. A left lowcr lobectorny of the lung with dccortication of the left iipper polc and evacuation of aii infected hydatid cyst of the left lower lobe was carried out. Tlie patient was dischargcd aiid followed-up a t home. Pathology confirmed the presencc of a hydatid cyst which had ruptured into the pleura causiiig extensive pleuritis. Larninated parasitic niernbranes and riurnerous scolices werc found. rnany showing degenerative ctiariges. The sections of thn lobe with Open bronchus rcvealed a non-specific chronic iiiflarnrnation. 'rhe surrounding lung tissuc revealed the presence of bronchopiieurnonia. extensive non-specific chronic inflarnrnation. focal ernphysema, and atclcctasis, iri addition to Iarge arcas of fibrosis. Thc pleural surfacc showed thc presence of fibrosis and exudate. Case 3. A I 3 year old girl had a routinc chest xray which sliowed findings cornpatible with a hydatid cyst. Shc was to be refcrrcd for consultation to our hospital. Urifortunately, she had an niilornobile accident in the interiin. This rcsulted in referral to an outside hospital wherc a cliest tube was inserted. On adiriission to our liospital. shc had continuation of an air-leak and a cornplcto ateleclasis of tho left lung with hydropneumothorax. An IHA was done and showed a titre of 2.048 (normal up 1.0 1-32). A thoracotomy rcvealed a hugc ruptured hydatid cyst with contarniriation of the eiitire chcst cavity by hydatid fluid. 'l'hc pleura was markedly thickcncd. The left lowcr lobe was fixed iii a fibrous encasing. 'l'hc left upper lobc was alinost completely destroyed diie to cornprossioii by thc hydatid cyst. A left upper loboctoiiiy with cxcision ofthe hydatid cyst, decorticat,iori oft the lcft lower lobc and pleurectomy of tlie left parictal pleura was perforrned. The left lower lobe expanded niccly. Post-opcrntively, a persistent air leak was present for apprnxiniately 8 to 10 days until i t was healcd. A high dose of meheridazole trcatrnent was initiated and continiied during two inoiiths.
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'l'ho surgical spcciiiieri consisted or the left upper lobo. which was replaced by a large collapsed cystic struc,turc ineasuring 8 x 10 x 5 crn in size. Pathology corifirrncd the presencc of a large ruptured hydatid cyst with coritarnination orthe left chesi cavity. The microscopic oxaiiiination revealed larninated eosiriophilic rneiiibrancs witliout definite germinal layrr. In other parts of the specimen. liaginents ol'parasitic gcrminal epithcliurr~ and si:olices werc seeii.
Discussion
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Primary pleural hydatid disease is rare even in countries where the disease is endemic. In the past, the possibility of a priiriary pleural involvement was a matter of controversy; sonie aiithors rejected this possibility until its existence was firmly established (2, 4, 5, 9, 13). Apezteguia (2) reported on 11 cases of primary pleural echinococcosis. 1t is assumed that these cysts stem from embolized hexacanth embryos carried there by the arterial blood stream through the intercostal arteries after bypassing the liver and lung (5). The cysts develop frorn the embryos under the endothoracic fascia in close contact with the parietal pleura. The sitc of the interpleural parietal space varies from subpleural tissues, the intercostal muscles to the periosteum (9). Although Rakower and Milwidsky (13) reported 1 9 cases of pleural echinococcosis, only one case was thought by the authors to be of primary pleural origin. In our series of 70 patients containing all kinds ofhydatid cysts, 20% had pleural reactions with pleural effusions, but only in three patients (5%),rnajor secondary pleural cornplications arose. While in one of our cases (case 1) clinically and radiographically, a primary pleural ecliinococcosis could have been suspected, the pathologicanatomic findings of the surgical specimen revealed a secondary involvement of the pleura. The pleural effusion usually is secondary to infection of the cyst below the diaphragrn and may be sterile (13). Ruptiired and/or infected pulmonary hydatid cysts with parenchymal reaction (inflammatory or allergicj abutting the pleura (Fig. 1 C, d) may cause pleuritis with pleural effusion (6,lO. 11, 14). Perforation of a ruptured or unruptured hydatid cyst into the pleural space (Fig. 1, 2), may occasionally occur. In such a relatively rare event. severe hydropneurnothorax or pyopneumothorax - as also demonstrated in our cases 2 and 3 - is relatively frequent (4,9).At times, the diagnosis of echinococcal disease can be rnade after insertion of a chest tube from which hydatid vesicles and membranes are voided. In some cases. the condition eventually Progresses to pleural echinococcosis supporting the theory that this condition is usually secondary to perforation of a neighboring cyst. Even in our cascs, the pathologic-anatomic findings confirmed this etiology (Fig. 1 d, 2 d). Some ofthe cases which in the past were claimed to be primary pleural hydatid cysts may actually have beeil caused by prolapse of cysts arising in the vicinity of the pleura. In case 1 , pathologic-anatomic findings of bile pigment gave evidence that once a bilio-bronchial or hepatobronchial fistula had been present (Fig. 1 dj; this suggestrd that thc original hydatid cyst rnay have primarily arisen in the liver and secondarily prolapsed into the basal pleural. We have observed this event to develop in another case monitored by ultrasound, computed tomography and rnagnetic resonance imaging (17). A growing hydatid cyst near the dorne of the liver causes pressure atrophy of the
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toniy) showcd two large cavities ( 3 x 2 and 2 x 2 cm in size) containing parasitic inernbranes wich grcen-yellowish color. No fluid was found in the cysts. Microscopically. cystic rneiiibrancs pattiognornonic for hydatid discase were found. The collapscd nature of the cysts and extensive bacterial growth and loss of germinal epithclium (Fig. 1 d ) were consistent with a dcad cyst and inrlicative of previous loss ofcystic contents via a broiichus which opened ~iitothe superior wall of the ryst. Subsnquent infcction tiad occurred. The yellowish pignientation due to bile. indicated that there inust have becn at oiie time a coiiiiection with ttie biliary tract. 'l'hc pi~st-operativeCourse was iineventful aiid the patient was dischargcd in an improved condition. Oiie year later a chcst X-ray showed a residual pleural thicknnirigat thc riglit lung base wilh thc appearanco o f a rounded atelectasis or a pleurorna but iio evidcncr of recurrence.
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hernidiaphragrn which successively rnay lead to prolapsc int,o the lung parenchyma or the pleural space depcnding on the presence or abscnce o i pleural adhesions (1 5, 17, 19). l'his mechanism may, if bile ducts and brorichi have been eroded by thc cyst, lead to a bilio-bronchial or hepatopleural ristula (1, 7 . 8). Infection of thc cyst usually ensues (Fig. 1 C). Pleural eifusion. hydropnournolhorax. pyopncumothorax, erlcapsulated fluid colleclions. abscess forrnatioii and/or ernpyema (Fig. 1, 2), arid frequently cncapsulated lluid dua to adhcsioris, with pleural thickening and scarring, atelcctatic pseudotumors or plouroma (16, 17). also callcd rouiided alelectasis may be a consequence. 'l'hcsc: hatures usually develop slowly and have a chronic Course ii no appropriate thcrapy is iriitiated. With sudden
release of parasitic fluid and membranes into the pleura. immediate allergic manifestations (urticaria, fever. pain) and liie-threatening anaphylactic: shock inay occur. Secondary pleiiral seeding oiviable parasitic material may follow. If the parasitic coritents are voided into riiptured blood vessels, widespread metastatic dissemination of the disease may ensue. 111two of our cases (case 1 and 21, the history of tlie patients supported the occurrence of a bronchial rupture and evacuation of'parasitic contents and fliiid through the airways. I t is true, tliat in some cases endobronchial ruptiire may lead to a spontaneous ciirc; however, thc patient may also be subjected to a dangerous anaphylactic reaction. Acute siiffocation by large amounts of fluid or asphyxia with blockagc of major airways due to
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Fig. 2 A 28 yearoldfernale with rnultilocated recurrent hydatid diseaseand bronchial and pleural perforation causingtension pneurnothorax. a) Chest x-ray PA-view: collapsed cyst with pleural adhesions. b) Chest x-ray PA-view: tension pneumothorax with a large amount of pleural fluid and rnarked displacernent of mediastinurn and heari to right. C)CT showingcollapsed hydatid cyst oft lefi lung perforated into pleural space. d) Microscopic section showingnurnerous scolices, some with degenerative changes.
P l r ~ ~ rLOnlpliculions fll ofH!/dal~dOisease (Erh~nococcus Crflnc~losc~sl - -- . - --
References
crumpled parasitic m e m b r a n e s m a y occur. lnfection a n d toxemia may ensue. If evacuation of cystic contents is incomplete, residual cavitary lesions a n d bronchiectases may be left behind as a potential focus for further infections.
Acknowledeements I wish to thank Dr. Ashraf Ali, Department of Pathology for pathological analysis of hydatid cyst specimens, and Miss Catherine Devane and Miss Catherine Healy Tor skilfull secretarial assistance.
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Aleslig. K., L: Holrn, G. Nystroni. T. Schersten: Biliobronchial
fistiilasecondary to echinococcal abscess of the liver. Acta. Chir. Scand. 138 (1972) 90-94 Apezteguia, J. L. M.: Hidatidosis interpleuroparietal. Thorax 19 (1970) 211 -21 7 Aynso, L. A., C. T. de Peralta, H. B. Lazaro. A. J. Stein. J. A. Sanchez. D.F. Aymerich: Surgical treatrnentofpulinoiiary hydatidosis. J. Thorac. Cardiovasc. Siirg. 82 (1981) 569-575 Bakir, I.:. M. (41-Omeri: Echinococcal tension pneurnothorax. Thorax 24 (1969) 547-556 .' Beggs. 1.:The radiology of hydatid disease. .2nier. J. Hoentgenol. 145 (1985) 639-648 Borrie, J.: Fifty thoracic hydatid cysts. Br. J. Surg. 50 (1962) 268-283 Borrie, J., J. If. F. Shaw: Hepatobronchinl fistula caused by hydatid disease. Thorax 36 (1981) 25-28 Boyd, D. P.: Bronchobiliary and bronchopleural fistulas. Ann. Thorac. Surg. 24 (1977) 481 -487 Jesitor. M., H. RomanofJ Pneumothorax following riiptiire of a prirnary pleural hydatid cyst. J . Thorac. Cardiovasc. Surg. 63 (1972) 594-598 IU Kegel, R. F. C.. A. Futerni: The ruptured pulrnonary hydatid cyst. Radiology 76 (1961) 60-64 Leruall, D. B.. S. J . McCorkell: Rupture of echinococcal cysts; diagnosis, classification and clinical implications. Amer. J. Roentgenol. 146 (1986) 391 -394 Morris, D. L., P. W. Dykes, B. Dickson. S. E. Marriner. J. A. Bogan, F. G. 0. Burrows: Albendazole in hydatid disease. Br. Med. J. 286 (1983) 103-104 Rnkower. J.. H. Milwidsky: Hydatid pleural disease. Am. Rev. Resp. Dis. 90 (1964) 623-630 Ramos, L.. M. Hernandez-Mora, M. Illanas. M. T. Lloriente. J. Marcos: Radiological characteristics of perforated pulmonary hydatid cysts. Radiology 116 (1975) 539-542 '"euenlo's, J., F. M. Nogueras. X. Rius, T. Lorenzo: Hydatid
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disease ofthe liver with thoracic involvement. Surg. tiyn. Obstet. (1976) 570-574 Sinner, W. N.: Pleuroma - a cancer mimicking atelectatic pseudotumor of the lung. Röfo 133 (1980) 578-582 l 7 Sinner, W. N.: Computed tomography of pleuroma - a cancer mimicking pseudotumor of the lung. Eur. .J. Radiol. 1 (1981) 266-269 ' W o o d t l i , W., J. Bircher, F. Wtassek. J. Eckerl, B. Weithrich, R. W. Ammann: Effect ofplasma mebendazoleconcentrations in
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the treatment of human echinococcosis. Am. J. Trop. Med. Hyg. 34 (1 985) 754-760 Yacoubian, H. D.: Thoracic problems associated with hydatid cyst of the dome of the liver. Surgery 79 (1976) 544-548
Walther N. von Sinner
Department of Radiology King Faisal Specialist Hospital and Research Centre P. 0 . Box 3354 RIYADH 11211
Saudi Arabia
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Although non-complicated pulmonary o r hepatic cysts a r e best treated by total excision o r a t least by evacuation of the parasitic cyst (endocyst and ectocyst) a n d partial excision, with suture o f t h e pericyst (provided by the host) lobectomy in complicated (ruptured and/or infected) cysts c a n often not be avoided. In all our cases, lobectoniy had to b e performed because of extensive darnage to t h e surrounding tissue. Mebendazole treatrnent w a s combined in case 2 where previously several hydatid cysts had been surgically removed a n d cyst recurrence had occurred. It w a s also used in case 1 a n d 3 because of contamination of the thoracic cavities with hydatid fluid d u e to perforation of viable hydatid cysts. Modern imaging modalities (ultrasound, CT, MRI) a r e valuable diagnostic methods which may now allow recognition of t h e disease before t h e abovernentioned cornplications have occurred. This allows appropriate surgical treatment a n d eradication of the disease. They a r e also helpful to rule out further cysts elsewhere in the body a n d may be used in monitoring the future Course of t h e disease in post-operative a n d post-chemotherapeutic treatment by mebendazole o r albendazole (12, 18) which in o u r experience is more successful d u e to its scolicidal effect. Recurrences may be discovered by CT a n d MRI before invalidizing damage h a s been done and/or other complications have occurred.
I
P
Siimrnary
Pleurale Komplikationen bei Hydatidenbefall (Eehinokokkose) -
P -
Seventy patients with hydatid disease were referred to our institution during the last ten years. In all cases the clinical and radiological findings were confirmed by pathology. In three of seventy patients pleural complications were foiind consisting in hydropneumothorax and a life-threatening tension pneumothorax following pleural perforation of ruptured pulmonary hydatid cysts in two cases and prolapsed hepatic hydatid cyst in one case. Bronchial rupture with anaphylactic syrnptoms and collapse occurred in one patient. In another, the pathologic anatomic examinatiori gave clues of a n old bilio-bronchial fistiila. In all cases. lobectomies had to be carried out due to involvement and extensive destruction of surrounding lung tissue. CT visualized and localized the lesion and was helpful for a correct diagnosis. Ruptured and infected hydatid cysts are often confused with tumors and/or abscesses or empyema. Early recognition is important to prevent complications and if dissemination has occurred, to combine surgery with mebendazole or albendazole treatment.
lntroduction P
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Primary pleural hydatid disease is very rare (1-4, 13). Secondary involvement of the pleura occurs more frequently. usually as a complication of ruptured or infected pulmonary hydatid cysts (6, 10. 11, 14) and less frequently as a complication of liver cysts arising near the diaphragm, secondarily involving the basal or posterior pleura (1. 7, 8, 15.17, 19). The diagnosis of echinococcosis of the pleura is dificult because of its non-specific appearance. Clinical signs and symptoms and conventional radiography are Fortschr.Höntganstr. 152.6(1990)718-722
O GeorgThiemeVerlagStuttgart . New Ynrk
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70 Patienten mit Hydatidenbefall wurden wahrend dcr letzten zehn Jahre zur Behandlung eingewiesen. In allen Fällen bestätigte der pathologische die klinischen und radiologischen Befunde. Bei drei vor1 70 Patienten wurde Hydropneumothorax als pleurale Komplikation festgestellt: bei 7wei Patienten trat ein leberisbedrohender Spannungspneiimothorax nach pleuraler Perforierung aufgebrochener pulmonaler Hydatidzysten auf und bei einem Patienten als Folge eines Prolapsus einer hepatischen Hydatidzyste. Bei einem Patienten wurde ein Bronchialriß mit anaphylaktischen Schocksymptomen und Kollaps beobachtet. Bei einem weiteren Patienten ergab die pathologisch-anatomische Untersuchung Hinweise auf eine bereits früher bestehende Biliobronchialfistel. In allen Fällen mußten Lobektomien vorgenommen werden, weil das umliegende Lungengewebe befallen und weitgehend zerstört war. Die Compiitertoinographie veranschaulichte und lokalisierte die Läsion und erleichterte die Diagnosestellung. Aufgebrochene und infizierte Hydatidzysten werden oft mit Tumoren und/oder Abszessen oder Empyemen verwechselt. Die Früherkennung ist wichtig, um Komplikationen zu vermeiden. 1st bereits eine Streuung eingetreten, ist deren frühzeitige Erkennung ebenfalls von großer Bedeutung, um den chirurgischen Eingriff ggf. mit einer Mebendazol- bzw. Albendazolbehandlung zu kombinieren.
seldomly helpful. lntracutaneous and serologic tests. if the possibility of echinococcosis is taken into consideration a t all, are often unreliable, c?specially in non-complicated pulmonary hydatid cysts. False positives and false negative tests may confuse the diagriosis. Although specific diagnosis by modern imaging methods, such a s CT and MRI. may not always be conclusive, there is a higher chance to recognize the disease or to suspect its true etiology. This is important for appropriate surgical and/or chemotherapeutic management and post-therapeutic control. I'atients and Methods P-
-
During ttie last ten ycars seventy cascs of tiydatid disease, all vcrified by pathologic-analomic findings, have been referred to out institution wliich serves as a tertiary referral centre. The age and Sex distribution showed a predominance oi
Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
Departmeni of Hadiology.
...
.. .
..
Fortschr. Iföntgenslr. 752.h
Fig. 1 A26 year old male with intrapleural perforated hydatid cyst causing tension pneumothorax. a) Chest x.ray PA-view:before rupture. b)Chest x-ray PA-view:after rupture. C) CT: expansity containing speck of airwith moderate pleural effusion. d) Microscopic pathology (operated specimen):Collapsed hydatid cyst with cystic membraneswithoutgerminal layerand with large amount of bile pigment and inflammatory round cell infiltration,bacterial growth.
fcmalc paticnts with a Sex relation of 1 : 1.5 rnale:female. Hydatid cysts occurred morc frequently in younger paticnts with a peak in the age group 20-29 years: however. another less promincnt penk w a s found in the age group 50 5 9 ycars. Sixty per cent orthe tiydatid cysts occurrcd in the liver. 20% in lung parcnchyma. and 20% in the rest of the body (brain. spleen. pelvis. pnncrcas. abdominal wall, skeletori. etc.). 'Thirty paticnts had Single cysts arid 20 patients showcd multiple cysts within the snrne Organ o r within different Organs or tissues. In 20% a pleural reactioii was foiind, usually manifcsted by pleural effusion. In three cases, major pleural complications arose which are presented in thrcc rcprcsentative cases.
Case 1. A 26 ycar oid male was rekrred to our hospitnl for sudden pairiiri the right shnuldcr followed by fever and unprodiictive cough. It was assurned that tlie patient had suffered from a n acute prieunionia which rapidly responded to erythrorny-
cin leaving local pleural thickening and scarring. Tuberculosis was a remote possibility. The patient was. therefore, kept under surveillance. Hali a year Inter (Fig. 1 a. b). the patient felt sudden severc pain along the trachea accompnnied by forceful coughing. Multiple Sputum exarns were negative for malignant cells. CT of the chest (Fig. 1 C) showed the lesion to bc in contact with the pleura and appearcd to be associated with a pleural effusion: it contained at least one small speck of air. 'l'he sonography of the right hernithorax showed aii aneclioic lesion thought to contain fluid. DiiTerential diagnostically. a lung turnor o r a Iiing absccss w a s suspected. Smcars and cultures were negative for AFB. Yhe total blood cell count was normal except for a borderline eosinophilia of 5%. A fine iieedle nspiration biopsy and bronchial washiiigs were ncgative Tor malignant cells. Since the paticnt had not further iniproved after a ycar, h e was finally operated under the clinical diagnosis of a chroriic Iiing abscess. Thc macroscopic exarniriation oi' the opcrated specimen (from right lnwer lobcc-
71 9
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Pl>leurnl(,'ornplicaiions q//&jdc/tid Diseuse (Fchinococcus Gru~iuloscts)
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Case 2. A 28 year old foiiiale was rcpeatedly operated for recurrent hydatid disease: Six years ago she had a left hcpatic lobectomy for a hydatid cyst. Onc year later she was reoperated for recurrence ofthe disease. After an intcrirn tiine of four years she was again operated fur a hydatid cyst involving the righl adnexal area. Six rnonths ago a chest X-rayshowed a large opaquc cystic structure in the left lung. Two wceks prior to her adrnission she had an acute attack. coughing out largc arnounts of white sputuni accornpanied by streaks of blood. She collapsed and was in acute distrcss, and was subseqiiently rel'erred to our hospital for further treatrnent. On physical exarnination. shc had a pale chronically ill appearance. Laboratory investigations were uncharacteristic except for IHA which was positive for echinococcosis. A chest X-ray showed a left pneumothorax of approximately 80% or rnore. The left lung had collapsed rnedially into the left lower zone. There were soveral pleural bands and a large arnount of fluid a t thc leit lung base (Fig. 2 a). A shift of thc rnediastinurn to the right was consistent with tension pneurnothorax rollowing a ruptiire of thc patient's known left pulmonary hydatid cyst IFig. 2 b). Cl' of the chest showed a collapsed infected hydatid cyst which had ruptured into the pleural space (Fig. 2 C).An ultrasound and CT scan of thc liver showed a space occupying lesion in the posterior superior aspect of the right lobe of the liver rneasuring 4,s crn in greatest diarncter. A left lowcr lobectorny of the lung with dccortication of the left iipper polc and evacuation of aii infected hydatid cyst of the left lower lobe was carried out. Tlie patient was dischargcd aiid followed-up a t home. Pathology confirmed the presencc of a hydatid cyst which had ruptured into the pleura causiiig extensive pleuritis. Larninated parasitic niernbranes and riurnerous scolices werc found. rnany showing degenerative ctiariges. The sections of thn lobe with Open bronchus rcvealed a non-specific chronic iiiflarnrnation. 'rhe surrounding lung tissuc revealed the presence of bronchopiieurnonia. extensive non-specific chronic inflarnrnation. focal ernphysema, and atclcctasis, iri addition to Iarge arcas of fibrosis. Thc pleural surfacc showed thc presence of fibrosis and exudate. Case 3. A I 3 year old girl had a routinc chest xray which sliowed findings cornpatible with a hydatid cyst. Shc was to be refcrrcd for consultation to our hospital. Urifortunately, she had an niilornobile accident in the interiin. This rcsulted in referral to an outside hospital wherc a cliest tube was inserted. On adiriission to our liospital. shc had continuation of an air-leak and a cornplcto ateleclasis of tho left lung with hydropneumothorax. An IHA was done and showed a titre of 2.048 (normal up 1.0 1-32). A thoracotomy rcvealed a hugc ruptured hydatid cyst with contarniriation of the eiitire chcst cavity by hydatid fluid. 'l'hc pleura was markedly thickcncd. The left lowcr lobe was fixed iii a fibrous encasing. 'l'hc left upper lobc was alinost completely destroyed diie to cornprossioii by thc hydatid cyst. A left upper loboctoiiiy with cxcision ofthe hydatid cyst, decorticat,iori oft the lcft lower lobc and pleurectomy of tlie left parictal pleura was perforrned. The left lower lobe expanded niccly. Post-opcrntively, a persistent air leak was present for apprnxiniately 8 to 10 days until i t was healcd. A high dose of meheridazole trcatrnent was initiated and continiied during two inoiiths.
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'l'ho surgical spcciiiieri consisted or the left upper lobo. which was replaced by a large collapsed cystic struc,turc ineasuring 8 x 10 x 5 crn in size. Pathology corifirrncd the presencc of a large ruptured hydatid cyst with coritarnination orthe left chesi cavity. The microscopic oxaiiiination revealed larninated eosiriophilic rneiiibrancs witliout definite germinal layrr. In other parts of the specimen. liaginents ol'parasitic gcrminal epithcliurr~ and si:olices werc seeii.
Discussion
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Primary pleural hydatid disease is rare even in countries where the disease is endemic. In the past, the possibility of a priiriary pleural involvement was a matter of controversy; sonie aiithors rejected this possibility until its existence was firmly established (2, 4, 5, 9, 13). Apezteguia (2) reported on 11 cases of primary pleural echinococcosis. 1t is assumed that these cysts stem from embolized hexacanth embryos carried there by the arterial blood stream through the intercostal arteries after bypassing the liver and lung (5). The cysts develop frorn the embryos under the endothoracic fascia in close contact with the parietal pleura. The sitc of the interpleural parietal space varies from subpleural tissues, the intercostal muscles to the periosteum (9). Although Rakower and Milwidsky (13) reported 1 9 cases of pleural echinococcosis, only one case was thought by the authors to be of primary pleural origin. In our series of 70 patients containing all kinds ofhydatid cysts, 20% had pleural reactions with pleural effusions, but only in three patients (5%),rnajor secondary pleural cornplications arose. While in one of our cases (case 1) clinically and radiographically, a primary pleural ecliinococcosis could have been suspected, the pathologicanatomic findings of the surgical specimen revealed a secondary involvement of the pleura. The pleural effusion usually is secondary to infection of the cyst below the diaphragrn and may be sterile (13). Ruptiired and/or infected pulmonary hydatid cysts with parenchymal reaction (inflammatory or allergicj abutting the pleura (Fig. 1 C, d) may cause pleuritis with pleural effusion (6,lO. 11, 14). Perforation of a ruptured or unruptured hydatid cyst into the pleural space (Fig. 1, 2), may occasionally occur. In such a relatively rare event. severe hydropneurnothorax or pyopneumothorax - as also demonstrated in our cases 2 and 3 - is relatively frequent (4,9).At times, the diagnosis of echinococcal disease can be rnade after insertion of a chest tube from which hydatid vesicles and membranes are voided. In some cases. the condition eventually Progresses to pleural echinococcosis supporting the theory that this condition is usually secondary to perforation of a neighboring cyst. Even in our cascs, the pathologic-anatomic findings confirmed this etiology (Fig. 1 d, 2 d). Some ofthe cases which in the past were claimed to be primary pleural hydatid cysts may actually have beeil caused by prolapse of cysts arising in the vicinity of the pleura. In case 1 , pathologic-anatomic findings of bile pigment gave evidence that once a bilio-bronchial or hepatobronchial fistula had been present (Fig. 1 dj; this suggestrd that thc original hydatid cyst rnay have primarily arisen in the liver and secondarily prolapsed into the basal pleural. We have observed this event to develop in another case monitored by ultrasound, computed tomography and rnagnetic resonance imaging (17). A growing hydatid cyst near the dorne of the liver causes pressure atrophy of the
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toniy) showcd two large cavities ( 3 x 2 and 2 x 2 cm in size) containing parasitic inernbranes wich grcen-yellowish color. No fluid was found in the cysts. Microscopically. cystic rneiiibrancs pattiognornonic for hydatid discase were found. The collapscd nature of the cysts and extensive bacterial growth and loss of germinal epithclium (Fig. 1 d ) were consistent with a dcad cyst and inrlicative of previous loss ofcystic contents via a broiichus which opened ~iitothe superior wall of the ryst. Subsnquent infcction tiad occurred. The yellowish pignientation due to bile. indicated that there inust have becn at oiie time a coiiiiection with ttie biliary tract. 'l'hc pi~st-operativeCourse was iineventful aiid the patient was dischargcd in an improved condition. Oiie year later a chcst X-ray showed a residual pleural thicknnirigat thc riglit lung base wilh thc appearanco o f a rounded atelectasis or a pleurorna but iio evidcncr of recurrence.
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hernidiaphragrn which successively rnay lead to prolapsc int,o the lung parenchyma or the pleural space depcnding on the presence or abscnce o i pleural adhesions (1 5, 17, 19). l'his mechanism may, if bile ducts and brorichi have been eroded by thc cyst, lead to a bilio-bronchial or hepatopleural ristula (1, 7 . 8). Infection of thc cyst usually ensues (Fig. 1 C). Pleural eifusion. hydropnournolhorax. pyopncumothorax, erlcapsulated fluid colleclions. abscess forrnatioii and/or ernpyema (Fig. 1, 2), arid frequently cncapsulated lluid dua to adhcsioris, with pleural thickening and scarring, atelcctatic pseudotumors or plouroma (16, 17). also callcd rouiided alelectasis may be a consequence. 'l'hcsc: hatures usually develop slowly and have a chronic Course ii no appropriate thcrapy is iriitiated. With sudden
release of parasitic fluid and membranes into the pleura. immediate allergic manifestations (urticaria, fever. pain) and liie-threatening anaphylactic: shock inay occur. Secondary pleiiral seeding oiviable parasitic material may follow. If the parasitic coritents are voided into riiptured blood vessels, widespread metastatic dissemination of the disease may ensue. 111two of our cases (case 1 and 21, the history of tlie patients supported the occurrence of a bronchial rupture and evacuation of'parasitic contents and fliiid through the airways. I t is true, tliat in some cases endobronchial ruptiire may lead to a spontaneous ciirc; however, thc patient may also be subjected to a dangerous anaphylactic reaction. Acute siiffocation by large amounts of fluid or asphyxia with blockagc of major airways due to
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Fig. 2 A 28 yearoldfernale with rnultilocated recurrent hydatid diseaseand bronchial and pleural perforation causingtension pneurnothorax. a) Chest x-ray PA-view: collapsed cyst with pleural adhesions. b) Chest x-ray PA-view: tension pneumothorax with a large amount of pleural fluid and rnarked displacernent of mediastinurn and heari to right. C)CT showingcollapsed hydatid cyst oft lefi lung perforated into pleural space. d) Microscopic section showingnurnerous scolices, some with degenerative changes.
P l r ~ ~ rLOnlpliculions fll ofH!/dal~dOisease (Erh~nococcus Crflnc~losc~sl - -- . - --
References
crumpled parasitic m e m b r a n e s m a y occur. lnfection a n d toxemia may ensue. If evacuation of cystic contents is incomplete, residual cavitary lesions a n d bronchiectases may be left behind as a potential focus for further infections.
Acknowledeements I wish to thank Dr. Ashraf Ali, Department of Pathology for pathological analysis of hydatid cyst specimens, and Miss Catherine Devane and Miss Catherine Healy Tor skilfull secretarial assistance.
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Aleslig. K., L: Holrn, G. Nystroni. T. Schersten: Biliobronchial
fistiilasecondary to echinococcal abscess of the liver. Acta. Chir. Scand. 138 (1972) 90-94 Apezteguia, J. L. M.: Hidatidosis interpleuroparietal. Thorax 19 (1970) 211 -21 7 Aynso, L. A., C. T. de Peralta, H. B. Lazaro. A. J. Stein. J. A. Sanchez. D.F. Aymerich: Surgical treatrnentofpulinoiiary hydatidosis. J. Thorac. Cardiovasc. Siirg. 82 (1981) 569-575 Bakir, I.:. M. (41-Omeri: Echinococcal tension pneurnothorax. Thorax 24 (1969) 547-556 .' Beggs. 1.:The radiology of hydatid disease. .2nier. J. Hoentgenol. 145 (1985) 639-648 Borrie, J.: Fifty thoracic hydatid cysts. Br. J. Surg. 50 (1962) 268-283 Borrie, J., J. If. F. Shaw: Hepatobronchinl fistula caused by hydatid disease. Thorax 36 (1981) 25-28 Boyd, D. P.: Bronchobiliary and bronchopleural fistulas. Ann. Thorac. Surg. 24 (1977) 481 -487 Jesitor. M., H. RomanofJ Pneumothorax following riiptiire of a prirnary pleural hydatid cyst. J . Thorac. Cardiovasc. Surg. 63 (1972) 594-598 IU Kegel, R. F. C.. A. Futerni: The ruptured pulrnonary hydatid cyst. Radiology 76 (1961) 60-64 Leruall, D. B.. S. J . McCorkell: Rupture of echinococcal cysts; diagnosis, classification and clinical implications. Amer. J. Roentgenol. 146 (1986) 391 -394 Morris, D. L., P. W. Dykes, B. Dickson. S. E. Marriner. J. A. Bogan, F. G. 0. Burrows: Albendazole in hydatid disease. Br. Med. J. 286 (1983) 103-104 Rnkower. J.. H. Milwidsky: Hydatid pleural disease. Am. Rev. Resp. Dis. 90 (1964) 623-630 Ramos, L.. M. Hernandez-Mora, M. Illanas. M. T. Lloriente. J. Marcos: Radiological characteristics of perforated pulmonary hydatid cysts. Radiology 116 (1975) 539-542 '"euenlo's, J., F. M. Nogueras. X. Rius, T. Lorenzo: Hydatid
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disease ofthe liver with thoracic involvement. Surg. tiyn. Obstet. (1976) 570-574 Sinner, W. N.: Pleuroma - a cancer mimicking atelectatic pseudotumor of the lung. Röfo 133 (1980) 578-582 l 7 Sinner, W. N.: Computed tomography of pleuroma - a cancer mimicking pseudotumor of the lung. Eur. .J. Radiol. 1 (1981) 266-269 ' W o o d t l i , W., J. Bircher, F. Wtassek. J. Eckerl, B. Weithrich, R. W. Ammann: Effect ofplasma mebendazoleconcentrations in
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the treatment of human echinococcosis. Am. J. Trop. Med. Hyg. 34 (1 985) 754-760 Yacoubian, H. D.: Thoracic problems associated with hydatid cyst of the dome of the liver. Surgery 79 (1976) 544-548
Walther N. von Sinner
Department of Radiology King Faisal Specialist Hospital and Research Centre P. 0 . Box 3354 RIYADH 11211
Saudi Arabia
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Although non-complicated pulmonary o r hepatic cysts a r e best treated by total excision o r a t least by evacuation of the parasitic cyst (endocyst and ectocyst) a n d partial excision, with suture o f t h e pericyst (provided by the host) lobectomy in complicated (ruptured and/or infected) cysts c a n often not be avoided. In all our cases, lobectoniy had to b e performed because of extensive darnage to t h e surrounding tissue. Mebendazole treatrnent w a s combined in case 2 where previously several hydatid cysts had been surgically removed a n d cyst recurrence had occurred. It w a s also used in case 1 a n d 3 because of contamination of the thoracic cavities with hydatid fluid d u e to perforation of viable hydatid cysts. Modern imaging modalities (ultrasound, CT, MRI) a r e valuable diagnostic methods which may now allow recognition of t h e disease before t h e abovernentioned cornplications have occurred. This allows appropriate surgical treatment a n d eradication of the disease. They a r e also helpful to rule out further cysts elsewhere in the body a n d may be used in monitoring the future Course of t h e disease in post-operative a n d post-chemotherapeutic treatment by mebendazole o r albendazole (12, 18) which in o u r experience is more successful d u e to its scolicidal effect. Recurrences may be discovered by CT a n d MRI before invalidizing damage h a s been done and/or other complications have occurred.
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