Isolatedextra Hepatic and Extra Pulmonary Hydatid Cyst: Report of 33 Rare Cases
- 1. Department of Thoracic & General Surgery, Guilan University of Medical Sciences, Iran
- 2. Resident of General Surgery, Guilan University of Medical Sciences, Iran
Abstract
Background: Hydatid disease is a parasitic infestation caused by Echinococcus granulosus and caused by the larval stage of the Echinococcus tapeworm and it primarily affects the liver and lung but involvement of other organs is also possible secondary to peritoneal seeding or hematogeneous dissemination. About extra hepatic and pulmonary hydatid cyst (EHPHC) there are not basic studies. we want to discuss this entity and review the literatures.
Materials and methods: A systematic retrospective review was performed for all extra hepatic and pulmonary hydatidcyst (EHPHC) patients treated in our province between 1990and 2016. Inclusion criteria in this study, was all patients with (EHPHC) organ involvement without simultaneously involvement liver and lung by hydatid cyst. Final diagnosis confirmed with pathologist. The patient’s records were studied for gender, age, site of involvement, diagnosis and results of treatment. All data was analyzed by using SPSS version 21.
Results: In this study record of 33 patients who were evaluated within this period, were reviewed. 21 patients were male and 12 patients were female. Age of patients was 25 to 56 years old. Hydatid cyst of spleen was the most common all of (EHPHC). Others were Kidney, pancreas, soft tissue and mediastinum. The most common tools for diagnosis was U/S and CT-scan. All patients with hydatid cyst of spleen underwent splenectomy. Others (EHPHC) underwent radical resection. Albendazole used in all patients just post operation. Outcomes of surgery were good and no recurrences were occurred.
Conclusion: Although liver and lung are the most commonly involved organs, in 10% of cases it occurs in other locations. Hydatid cysts should be included in the differential diagnosis of any cystic mass of all organs in body, especially in endemic regions. Total remove of the cyst component without any spillage is the best treatment option.
Citation
Aghajanzadeh M, Mosafayi O, Karimi B, Torabi H, Ziabari M, et al. (2018) Isolatedextra Hepatic and Extra Pulmonary Hydatid Cyst: Report of 33 Rare Cases. Ann Clin Pathol 6(1): 1129.
INTRODUCTION
Hydatid cyst (HC) is a zoonotic infection caused by Echinococcus granulosus, or less frequently, E. multilocularis which occurs worldwide [1,2]. However, (HC) remains a considerable public health problem in several mediterranean countries [3,4]. The dog or other carnivore is the definitive host while sheep is the intermediate host [3,4]. The adult worm lives in the intestine of the definitive host and discharges eggs in their feces [1,4]. The intermediate host ingests the ovum while vegetable on contaminated ground. The developed embryo, passes through the intestinal wall to enter the portal venous system and embedded in the liver which is the first organ most frequently involved [4,5]. The life cycle is completed when an infected intermediate host dies and their viscera which contain the larval are consumed by a definitive host [3,4]. Man becomes infected by contact directly with a definitive host or by eating contaminated water or vegetables [1,4,5]. (HC) disease has been involve almost every organ of the human body, but approximately 70% of the cysts are involve in the liver, followed by the lung (15%–47%) and 10% rest of body [2-4]. The disease is less frequently found in the spleen, pancreas, heart, brain, kidney, bones, adrenal and muscles, soft tissue and rib [1,2,4,5,6,9]. The liver or lung involvement may by rollout of (HC), if a cyst is present where else in the body [1-3,5]. The fluids in the cyst are crystal and clear [2,4,5]. It is transudate of serum contains protein and may be high antigenicity [5,6]. As far for diagnosis of (HC) according serology tests have little role, due to its high false positive or false negative rate up to 15% - 20% [6]. Ultrasonography and CT scan provide characteristic appearances for diagnosis especially in hepatic disease while extra hepatic disease may pose a diagnostic challenge [6]. The suspected diagnosis was confirmed by histopathological examination [5-7]. The choice of treatment is surgery for eradication and diagnosis of (HC). The aim of this study is to discuss presentation, diagnosis and treatment of extrahepatic and pulmonary hydatid cyst, because this cyst can affect any part of the body and any age groups, except the hair and nails with various symptoms and sign and is challenging [3,8-10].
METHOD AND MATERIALS
In this retrospective study, we reviewed the data of 33 patients with extra hepatic and extra pulmonary hydatid cyst (EHPHC) In Guilan province, Razi, Arya and Golsar Hospitals, Inflammatory, Lung Disease Research Center, Guilan University of Medical Sciences, Rasht, Iran between 1990 to 2016. Inclusion criteria in this study, was all patients with (EHPHC) organ involvement without simultaneously involvement liver and lung by hydatid cyst. The data including symptoms, signs and imaging’s feature and surgical approaches (Table 3), and outcome of surgery. Ultrasonography and CT scan was performed in all patients because this imaging, provide characteristic appearances for diagnosis especially in hepatic disease while extra hepatic disease may pose a diagnostic challenge [6] and MRI was used in soft tissue cysts. Serology tests were not performed routinely. Because has little role to help for diagnosis, due to its high false positive or false negative rate up to 15% - 20% [1,6]. In three of soft tissue (EHPHC) needle aspiration and biopsy was performed and was diagnostic and in others the diagnosis was obtained intra-operatively [7-9]. All patients treated with albendazole 10 mg/kg twice a day in three cycle of 28 day with 14 day interval in early postoperatively. Data were collected forced of patients and analyzed by using SPSS version 21.
RESULTS
21of patients were male and 12 of them were female. The average age at the time of the treatment was 34 years and ranged from 12 to 58 years. Eight of patients present with abdominal masses. The most common organ involvement was spleen, pancreas and kidney. The distribution of organ involvement was shown in Table 1. Four of patients present with left upper quadrant pain due to spleen hydatid cyst. Two of patients present with a long history mass with pain and difficulty in shoulder movements due to axillary region and trapezius muscle cyst, other symptoms and signs were shown in Table 2. Ultrasonography and CT scan was the most common tools for diagnostic. MRI used in two patient with brachioradialis muscle and cervical cyst (Figures 5,11). Three of patients with soft tissue cystic mass to underwent needle aspiration biopsy and was diagnostic. All patients underwent surgical resection. In four spleen (HC) splenectomy was the surgical procedure and in two cases evacuation and per cystectomy was performed. Surgical approach in two cases with pancreatic (HC) was puncture, evacuation and cystojejunostomy and in one case distal pancreatectomy was performed. In two cases with (HC) of kidney (Figure 4), the surgical method was puncture and pericystectomy and capitonnage and in one case nephrectomy was performed. In mediastinal cyst we performed cyst aspiration and evacuation and pericystectomy. Inrib (HC), aspiration, evacuation, pericystectomy and rib resection was performed. In rest organ, we performed puncture, evacuation and pericystectomy. We have not any morbidity and mortality. In average five year follow-up there was not recurrence.
DISCUSSION
Hydatid cysts (HC), primarily affects the liver and lung.The extra hepatic disease is considered to be the consequence of a secondary infection via metastatic spread from the hepatic focus [6,10,11], (HC) has been involve almost every organ of the human body, but approximately 70% of the cysts are involve in the liver, followed by the lung (15%–47%) and 10% involve rest of the body [2-4]. Spleen, pancreas, heart, brain, kidney, bones, adrenal glandmuscles, soft tissues and rib may frequently found in (HC) [1,2,4,5,9]. Isolated extra hepatic hydatid without hepatic and pulmonary involvement is a rare phenomenon, the explanation given are hepatic omission and passage of oncospheres through hepatic sinuses without carrying disease, and passage via lymphatic vessels or via portocaval anastomosis and the vascular passage in a retrograde fashion [6,11]. Splenic involvement in (HC) is uncommon, representing less than 2% of all human involved by Echinococcus [12]. In this study we revived six case of primary splenic cysts. Splenic (HC) may be primary or secondary to ruptured (HC) of liver [13]. Possible routes of primary (HC) of spleen include arterial route after passing through liver and lung [9]. The differential diagnosis includes cystic lesions of adjacent organs as Pancreas, liver and omentum, intrasplenic aneurysm [14]. Ultrasonography and CT-scan, are the most valuable imaging tools for the diagnosis and evaluation of splenic diseases as our cases (Figures 1,2) [14]. Total splenectomy, partial splenectomy, cyst evacuation and pericystectomy with omentoplasty are the surgical techniques to treat splenic (HC) [14]. In our cases, total splenectomy, aspiration, evacuation of cyst and pericystectomy was the preferred methods for splenic (HC). Chemotherapy and Puncture, Aspiration, Injection, and Re-aspiration (PAIR) is another newer technique for splenic (HC) [12,14]. We did not used this method. Hydatid cyst of the pancreas (PHC) is rare, since it accounts for less than 1%, even in countries where (HC) is endemic [15]. In this series we treated three (PHC) (Figure 12). The clinical presentation of (PHC) are, obstructive jaundice, fistulization and spillage of scolices into the biliary tree and acute pancreatitis [16]. Cysts located in the body and tail of pancreas may present with abdominal pain, vomiting, fullness and early satiety, due to compression of the stomach by cyst [15,17]. Two cases of our patients presented with epigastric pain and in one case with (PHC) of tail and body of pancreas which presented with back pain (Figure 3). The diagnosis of (PHC) can be performed by ultrasonography, CT scan, MRI (Figures 5-10) and endoscopic ultrasound. The radiological imaging features are useful for distinguishing hydatid cyst from other cystic lesions of pancreas [15-17]. We used ultrasonography and CT scan in our patient. Surgery remains the treatment of choice in (PHC). Partial or total cystectomy, marsupialization and external drainage have also been reported in the management of pancreatic hydatidcysts, [15-18]. Many surgical procedures are available to remove the cyst. We used cystojejunostomy in two cases without any complication and distal pancreatectomy in one case. We do not use any scolex agent in the cavity of cyst because risk of fatal pancreatitis is high. In external drainage long time discharge may occur and we don’t recommended [19,37].
Table 1: Organ distributions of (HC) in 33patients.
| Organ No |
| Spleen 6 |
| pancreas 3 |
| kidney3 |
| omentum 2 |
| rib 2 |
| mediastinum 2 |
| muscles(Tr,Qu,Br)* 3 |
| breast 1 |
| axilla 1 |
| inguinal 1 |
| chest wall 1 |
| retroperitoneal1 |
| incision site(CW)** 1 |
| incision site(AW)*** 1 |
| meso of intestine 1 |
| cervical spine 1 |
| occiput region(SC)**** 1 |
| stomach 1 |
| pericardium 1 |
| *Trapezius,Quadriceps,Brachioradialis |
| **chest wall |
| ***abdominal wall |
| ****subcutaneous |
Table 2: Show Symptoms and sign in 33 case with (HC).
| Organ symptoms sign |
| Spleen Pain incidental(no=2) |
| Soft tissue pain mass |
| Kidney pain. hematuria mass |
| Pancreas pain mass |
| Rib pain mass |
| Mediastinum pain --- |
| Abdomen wall pain mass |
| Chest wall pain mass |
| cervical pain Horner SY* |
| occipitalpain mass |
Table 3: Surgical approach in 33 case with(HD).
| Spleen splenectomy(n=4) AEO*(N=2) |
| Pancreas AECJ** distal pancreatectomy (n=1) |
| KidneyPPC***(N=2) nephrectomy(n=1) |
| Muscles radical resection ---------- |
| Rib evacuation ,rib resection ----------- |
| mediastinum radical resection ----------- |
| chest wall radical resection ----------- |
| abdominal wall radical resection ---------- |
| others radical resection ---------- |
| *AEO=aspiration,evacuation,omentoplasty |
| **aspiration,evacuation, cystojeujonostomy |
| ***puncture,pericystectomy,capitonnage |
ACKNOWLEDGMENT
We thank of Dr. Mehdi Karimyan, Dr. Kiyani, Dr. Mohamadreza Kohsari and Dr. Shahrokhyosefzadeh for referring of some case in this study to our hospital
