Case Report: A giant ruptured splenic hydatic cyst in a patient with a complete situs inversus: Diagnostic challenge and intra-operative difficulties

Patient information

A 50-year-old female, with no medical history presented to the emergency department with right hypochondrium pain.

Clinical findings

On physical examination, the patient was febrile at 38.4°C; anicteric, with tenderness of the right hypochondrium on abdominal examination. The hemodynamic status was stable.

Diagnostic assessment

Blood analysis showed a biological inflammatory syndrome. The liver test was normal.

In the face of a 50-year-old obese female who consulted for a painful and febrile syndrome of the right hypochondrium, an abdominal ultrasound was performed, which showed a complete situs inversus and a mass of the right hypochondrium with a membrane detachment, measuring 152 mm, evoking a type II splenic hydatic cyst.

Computed tomography (CT) revealed a large cystic formation in the spleen, measuring 15 cm in its largest dimension. The cyst showed a detached internal membrane, which is typical of a type II hydatid cyst according to the Gharbi classification. Additionally, there was evidence of surrounding fat stranding and infiltration, indicative of local inflammatory changes. These findings strongly suggest a complication, specifically a hydatid cyst cracking (Figures 1, 2).

Figure 1. Splenic hydatid cyst cracking in a complete situs inversus 1.

Figure 2. Splenic hydatid cyst cracking in a complete situs inversus 2.

Therapeutic intervention

The patient underwent an emergency midline laparotomy. The exploration revealed a situs inversus, a voluminous splenic cyst occupying over 80% of the splenic volume. Exposition of the splenic pedicle is difficult. The cysto-parietal and cysto-visceral adherences, giant size of the cyst, and obesity prevented good exposure, which led to the decision to empty the cyst content after protecting the operating field with a field soaked in hypertonic serum. The cyst was infected.

Equally, the choice of the type of surgery, whether a total splenectomy or a protruding dome resection in an emergency context with complications such as cracking and surinfection, was not easy.

However, in the face of an emergency, the primary localization in the spleen, we performed a total splenectomy that allowed healing of the infested organ and avoided recurrence and surinfection of the residual cavity.

The overture of the cyst objectified the proligere membrane (Figure 3).

Figure 3. The splenectomy specimen and the proligere membrane of the hydatic cyst.

Follow up and outcomes

The post-operative period was unproblematic, and the patient was discharged with antibiotic and antiparasitic treatment and habitual vaccination.

Treatment with albendazole 400 mg twice daily was initiated from post-operative day 1, with cycles consisting of three 28-day treatments at 2-week intervals. Serum anti-Echinococcus antibody titers (hemagglutination test and ELISA) were positive in the immediate postoperative period (titers of 1/1280 and 1.9, respectively). Subsequently, the titers gradually decreased until achieving a negative serology at 2 years post-operatively. Liver function tests and complete blood counts were performed after each cycle and did not reveal any abnormalities during the treatment period. The abdominal CT scan did not reveal any recurrence of cystic echinococcosis in the thoraco-abdominal regions, particularly in the peritoneal area, with a current follow-up period of 3 years.

Epidemiology

Hydatic cysts are a common pathology in endemic countries. The most frequent locations are the liver (70%) and lungs (20-30%).² Splenic localization is extremely rare, with a worldwide incidence rate of 0.5%-4%.¹

To our knowledge, this is the first reported case of a giant splenic hydatid cyst associated with situs inversus. In our case, the cyst was also ruptured into the abdomen, which posed both a diagnostic and therapeutic challenge.

Clinical and diagnostic aspects

Based on the literature of published cases of splenic primary localization, the most frequent circumstances of discovery include pain, a left hypochondrium mass, and fortuitous findings. These circumstances also occur during complications, such as infection and splenic abscess, rupture with anaphylactic shock, and dissemination to other organs.^1,2

Ultrasound, computed tomography, and magnetic resonance imaging of the abdomen allow for diagnosis by objectifying membrane detachment and calcifications on the daughter vesicle wall.^2,3 In the case of a complicated cyst, cross-sectional imaging, particularly CT scans, can establish the diagnosis in an emergency while also allowing for the assessment of the cyst’s location and its anatomical relationships.^2,3

Therapeutic management

The treatment of splenic hydatic cysts is surgical. Total splenectomy has the advantage of avoiding recurrences. Protruuding dome resection has the advantage of being a conservative intervention of the organ and its functions and is slightly hemorrhagic at the cost of a considerable rate of residual cavity surinfection.^4–6

The surgical approach depends on the localization of the splenic hydatic cyst(s) and its association with other cystic localizations.^4,7 The laparoscopic approach is realizable in almost all cases, with good short-term and long-term results.^6–8

Regarding complicated cysts, the treatment of a ruptured hydatid cyst typically relies on urgent surgical intervention,^4–6 followed by medical therapy to prevent and manage peritoneal echinococcosis.⁹ However, Carola Buscemi et al.⁹ reported a case where prolonged treatment with albendazole was employed over 10 years for peritoneal, hepatic, and splenic hydatidosis, including a ruptured cyst. The albendazole protocol consisted of 400 mg administered twice daily for three cycles of 28 days each, with a 14-day break between cycles. This treatment was well-tolerated, and the hydatidosis showed favorable progression under this regimen.

Such cases highlight the potential of medical therapy as a complementary or alternative approach in select instances, especially when surgery carries significant risks or is incomplete. However, additional randomized prospective studies are necessary to establish standardized protocols and enhance the management of this common yet complex condition.

Our case contribution

This case provides valuable insights into the spectrum of hydatid disease by highlighting a rare localization and an exceptional anatomical context. To our knowledge, this is the first documented instance of a giant splenic hydatid cyst associated with situs inversus, further complicated by intra-abdominal rupture. Such an association underscores the importance of maintaining a high level of suspicion for hydatid disease even in atypical locations, particularly in endemic regions. Furthermore, the presence of a ruptured cyst emphasizes the diagnostic and therapeutic urgency posed by the complications of splenic hydatidosis.

Surgical intervention in patients with situs inversus presents unique challenges due to reversed anatomy. This anomaly complicates both the orientation and execution of standard procedures, especially in minimally invasive approaches. Altered anatomical landmarks, including variations in vascular and lymphatic structures, increase the risk of intraoperative complications and may prolong operative time. To mitigate these risks, thorough preoperative imaging is essential to delineate the reversed anatomy and guide operative planning. Additionally, interdisciplinary coordination and careful intraoperative navigation are crucial. This case thus reinforces the need for meticulous planning and surgical adaptability when managing complex hydatid disease in patients with congenital anatomical variations.