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Spontaneous external fistula of a hydatid liver cyst in a diabetic patient

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Spontaneous external fistula of a hydatid liver cyst in a diabetic patient

M. Florea, H. Silaghi, S.T. Barbu, M. Crisan, A. Butnaru / M. Lupsor
Clinical case, no. 6, 2008
* Diagnosis and Treatment Center, University of Medicine and Pharmacy, Cluj- Napoca, Romania
* Diagnosis and Treatment Center
* IVth Surgical Clinic
* Hystology Department
* Radiology Departament / Ultrasonography Departament


Introduction
Hydatid disease is a parasitic tapeworm infection, caused by the metacestode (the larval stage) of Echinococcus species, primarily affecting the population of developing countries. The disease occurs mostly in sheep grazing areas, but the distribution is worldwide, since the dog (a definitive host) is a common animal all over the world (1). Romania has still a quite high incidence of hydatid disease: between 5 and 6 cases /100000 inhabitants (2, 3). The most frequent form (cystic echinococcosis) is due to Echinococcus granulosum, with the most common sites being the liver (60 - 70%) and lungs (30%) (1, 4-7).
Complications of liver hydatid disease include cyst rupture and infection. Rupture is the most common complication, and may represent the clinical presentation of the disease (1, 6, 8). Sites of rupture include physiologic channels (bile ducts, gastrointestinal tract, bronchi), free body cavities (peritoneal and pleural cavity), or adjacent organs (1, 2, 6, 9, 10). Cyst rupture into the subcutaneous tissue followed by spontaneous external fistula is an extremely rare complication.

Case report
 A 71- year old woman was admitted presenting a mass in the right hypochondrium, and a fistula draining clear liquid. During the last 6 weeks, the mass had a progressive growth (from 1 to 6 cm) and was covered by a pruritic rush. No history of fever or jaundice was present. Twelve days before presentation (after a minor trauma), a spontaneous fistula opening appeared, draining clear liquid containing cystic elements and (two days before) a quite large white membrane that was pulled out by the patient. Clinical examination revealed the non-tender, fluid-containing mass, and the fistula opening (fig. 1).
The patient's general condition was very good, wihtout signs of sepsis such as fever or tachycardia. Her medical history was remarkable for type 2 diabetes mellitus, hypertension and ischemic heart failure. Routine blood analysis revealed mild leukocytosis, eosinophilia (11%), and a glycemia of 183 mg% (no glucosuria).
Abdominal ultrasound (US) showed a circumscribed (6/5,5 cm), hypoechoic, inhomogeneous cystic lesion containing daughter vesicles and "hydatid sand", in the right lobe of the liver. The lesion was protruding into the anterior abdominal wall, invading the subcutaneous tissue. The biliary tree and the surrounding liver tissue were normal (fig. 2).
Soft tissues high frequency US showed a 6 cm hypoechoic mass in the subcutaneous tissue communicating with the hydatid cyst of the liver, and the parietal fistula track. Both lesions were containing echogenic hydatid structures (fig. 3).
Figure 1
Figure 2
Figure 3

Computed tomography (CT) scan showed the hydatid cyst of the liver communicating with another cystic mass in the abdominal wall (fig. 4, 5).
Exam of the cystic fluid drained on the fistula opening revealed protoscolices and serological ELISA test was positive for hydatid disease. Based on the clinical presentation, US, CT, and laboratory findings, the diagnosis of a cyst - cutaneous fistula due to a hydatid cyst of the liver was established.
At surgery, the lesion was approached through a right subcostal incision starting from the fistula site. About 250 ml clear liquid containing hydatid material and pieces of the germinative membrane were drained from the subcutaneous tissue. There were many adhesions preventing any communication between the peritoneal cavity and the cystic lesions in the liver and subcutaneous tissue. The fistula track and the parietal collection were totally resected. The liver cyst was treated by partial cystectomy. The residual cystic cavity was cleared of its contents, irrigated with 20% saline solution, and drained by a closed suction system. The postoperative course was uneventful.
Six months of antiparasitic postoperative treatment with Albendazol 10 mg/kg and adjuvant synergic treatment with Praziquantel 5 mg/kg were applied. The patient is doing well, 3 years after surgery.
Figure 4
Figure 5

Discussion
In humans, as the Echinococcus oncosphere is carried from the duodenum by the portal bloodstream into the first capillary filter (the liver parenchyma), over 70% of hydatid cysts develop in the liver, and most of them in the right lobe (1). Complications of liver hydatid disease depend on the site, size, stage of development, whether the cyst is alive or dead and whether the cyst is infected or not (1,6-10).
Rupture of hepatic hydatid cysts occurs in 35-58% of cases, usually into the biliary ducts, but sites of rupture may include other physiologic channels, free body cavities, or adjacent organs (1, 2, 5, 6, 9, 10). While in the western countries echinococcal disease is quite rare and is diagnosed at an early stage, in developing countries patients are usually admitted with complicated hydatid cysts (6, 11, 12). Rare types of rupture are fistulisation into the duodenum, perforation of the diaphragm, infiltration of ribs and rupture into the vascular and urinary systems (8, 10, 11). Although cyst rupture may be clinically silent, complications like acute anaphylaxis, contamination of the cystic fluid, and obstructive symptoms, such as jaundice (biliary tree), or hydronephrosis (urinary tract) may occur (1, 10).
Subcutaneous rupture followed by spontaneous cyst-cutaneous fistula is an extremely rare presentation of liver hydatid disease. To the best of our knowledge, there are only six such case-reports in the literature (12, 15-19). Two of them are cutaneous-cysto-bronchial fistula of hydatid disease (15, 17). Three additional cases of liver hydatid cyst ruptured into the subcutaneous tissue without external opening to the skin were reported (13, 14, 20).
In our patient, such a fistula was produced by direct abdominal wall invasion by a cyst located in the anterior segments of the liver. A viable hydatid cyst is a space-occupying mass with a tendency to grow in the direction of the least resistance. Continued expansion of the cyst towards the anterior abdominal wall with increasing intracystic pressure, and permanent frictions to the rough plan of a rib could have produced pressure erosions and adhesions to adjacent structures. Erosion and inflammation leaded to necrosis and caused fistulisation (1). Adhesions blocked the fistula track and prohibited the spillage of the cyst's content. The entire process developed without any noticeable symptoms in an old, diabetic patient. The growing mass in the right hypochondrium was produced by cyst rupture in the subcutaneous tissue. Before external opening occurs, the cyst usually passes through a small orifice adopting an "hourglass configuration" (13, 14).
Two imagists, Lewall and McCorkell (21) had classified the liver hydatid cyst rupture into 3 types: contained, communicating and direct. Contained rupture occurs when only the endocyst ruptures and the cyst contents remain confined to the pericyst. Communicating rupture consists of a rupture of the endocyst with spillage of cyst contents into small bile ducts incorporated into the pericyst. Direct rupture is when both the endocyst and pericyst tear, causing the content's leakage into the pleural or peritoneal cavities or other adjacent tissues (21). In communicating and direct ruptures, the cyst empties, becomes smaller and loses the spherical configuration (1). In our patient, a direct type of rupture was present.
Ultrasound and CT scan are the modalities of choice in diagnosing liver hydatid cysts. Both clearly show (with high sensitivity and specificity) the existence, number, site and location of the cysts and explain the relationship between the cysts and neighboring organs. Even if imagistic diagnosis is difficult without concomitant clinical signs of direct rupture, US and CT could clearly establish the diagnosis in our patient, showing important details like the subcutaneous collection and the fistula track.
In 2005, a new, comprehensive classification of hydatid liver cyst was proposed (22), based on 4 criteria: location (T), natural history (N), recurrence (R) and complications (C) of the liver cyst. The TN(R)C classification provides a standardized description formula of every liver hydatid cyst, permitting multiple comparisons and analyses, aiming to allow prediction of the treatment outcome, or making clinical decisions regarding medical treatment, open surgery, PAIR (punction, aspiration, injection, reaspiration), or laparoscopy.

Conclusions
 Spontaneous cyst-cutaneous fistula is an extremely rare complication of hepatic hydatid cyst, usually occurring silently, in elder people. Diagnosis can be done easily by usual imaging modalities like US and CT. Open surgery is required to achieve complete evacuation of the cyst contents, resolution of the residual cavity and complete resection of the fistula track. To the best of our knowledge, this is the seventh case published, and the first one in the Romanian literature.

References
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