Postpartum rupture of Splenic hydatid cyst: An extremely rare presentation of a rare condition

We present a case of splenic hydatid cyst detected late in pregnancy, which presented with intraperitoneal rupture in the postpartum period. A brief review in the is also presented. On follow up patient is doing well. Follow up USG is unremarkable. Histopathological examination conrmed hydatid cyst of spleen (Echinococcus granulosus) with areas of haemorrhage and inammation in the surrounding parenchyma. Post splenectomy vaccination was carried out after two weeks.


Abstract Background
Hydatid disease is endemic in some regions of the world. Even in endemic regions, splenic hydatid cysts are rare, especially in pregnancy. The most serious presentation is intra peritoneal rupture, which is a surgical emergency. Exploration with splenectomy is the favoured management.

Case presentation
We present a case of splenic hydatid cyst detected late in pregnancy, which presented with intraperitoneal rupture in the postpartum period. A brief review in the context is also presented.

Conclusion
Cystic lesions located anywhere in the body in endemic regions could be hydatid cysts. Prompt treatment should be planned immediately on detection in order to prevent potentially serious complications like rupture.

Background
Hydatid disease is fairly common in endemic areas. The most frequent location of the hydatid cysts is liver. Splenic cysts are uncommon. Majority of these are hydatid cysts in endemic regions. Splenic cysts are extremely rare in pregnancy with only few reports of hydatid cyst spleen in pregnancy and negligible data of its presentation in the postpartum period. Among the varied presentations of primary hydatid cyst spleen, intraperitoneal rupture is the most serious and potentially life threatening. Although the risk of rupture is more during pregnancy but it can occur any time, even in the postpartum period. Management during pregnancy is not standardized but in postpartum period, routine is followed, with the favoured treatment being splenectomy. Antenatal detection should entail a clear plan and if non-operative treatment is decided upon till the termination of pregnancy, surgical treatment should ensue as soon as possible in the postpartum period to avert rupture and its potentially lethal consequences.

Case Presentation
A 23 year old female, para 1, home-maker, reported to our department with complaints of pain in left upper abdomen for one day. The pain was constant, progressively increasing in intensity and distribution and unaccompanied by any other symptom. Patient had a normal vaginal delivery 25 days prior. Just a week before her delivery a USG at some other centre had revealed a cyst measuring 102.5 × 93.1 × 104.6 mm cyst between spleen and left kidney. (Fig. 1) Patient underwent normal vaginal delivery at a rural centre and was lost to follow up till she reported to our department. Patient denied any history of trauma or drug intake.
On examination she was found to be conscious, oriented with respect to time, place and person. Her pulse was 104 bpm, regular with a blood pressure of 126/76 mmHg, respiratory rate of 16/min and a temperature of 99 o F. Abdominal examination revealed a mildly distended abdomen with tenderness, rigidity and guarding predominantly on left side of the abdomen. Examination of other systems was unremarkable. Digital rectal examination was normal. Gynaecological assessment was also normal.
Investigations revealed a total leucocyte count of 9.8 × 10 3 /µL, with 89..5% neutrophils, 3% lymphocytes, haemoglobin of 12.6 G/dL and platelet count of 261 × 10 3 /µL. Blood glucose was 67 mG/dL, urea 33 mG/dL, creatinine 1.34 mG/dL. LFT was normal except for total protein of 5.5G/dL and albumin of 2.99 G/dL. Serum lipase was 24 U/L. Chest radiograph was unremarkable. (Fig. 2) USG revealed a cyst in the spleen measuring 82.1 × 51 × 63.8 mm with detached membrane and pelvic ascitis. CECT abdomen and pelvis showed Intra-splenic and subcapsular, partially collapsed, ruptured hydatid cyst along the medial aspect with mild pelvic and minimal abdominal ascitis. There was no evidence of any cyst elsewhere.
( Fig. 2,3) Patient was operated upon, Exploratory laparotomy was done. Intra operative ndings were an enlarged spleen with a ruptured cyst, approximately 10 × 8 cm in the inferomedial aspect with membranes seen exuding through the site of rupture. (Fig. 4 Dense adhesions were found between cyst, omentum and stomach. About 600 ml off white, turbid uid was found in abdomen and pelvis. Liver and other intra-abdominal viscera were grossly normal. Splenectomy followed by peritoneal lavage and scolicidal irrigation was carried out. Drains were placed in splenic fossa and pelvis. Post-operative period was uneventful. On follow up patient is doing well. Follow up USG is unremarkable. Histopathological examination con rmed hydatid cyst of spleen (Echinococcus granulosus) with areas of haemorrhage and in ammation in the surrounding parenchyma. Post splenectomy vaccination was carried out after two weeks.

Discussion
Echinococcosis or Hydatid disease is a zoonotic parasitic disease, endemic in certain regions of the world like Mediterranean, Mid East, South America, Australia, South Asia including India. Canines are the de nitive hosts and sheep, goat, cattle are the intermediate hosts. Man is an accidental host with no role in the transmission. Route of transmission is feco-oral, most often from soil or water contaminated by dog feces. Cyst is the larval stage of the parasite that develops in man. Liver is the most common site of these cysts and the infestation is usually caused by Echinococcus granulosus. Spleen is rarely involved, more so when it's an isolated site of involvement, accounting for nearly 4% of abdominal hydatid cysts. 1,2,3,4,5 Primary splenic hydatid cyst is thought to result either from arterial seeding bypassing liver and lungs or via possible venous re ux through the portal vein. Secondary splenic hydatid cysts result from systemic dissemination or following intraperitoneal rupture of liver hydatid cysts. 6,7 Splenic hydatid cysts may be asymptomatic, detected incidentally 8 or may present with vague abdominal pain, early satiety, dyspepsia, dyspnoea due to pushing up of the diaphragm 9 , constipation, local compressive symptoms or as a mass 10,11,12 . Splenic hydatid cyst may present acutely with rupture, which is most commonly due to trauma and less frequently spontaneous. 13 Rupture can be into neighbouring viscera like colon 14 or into the pleural or peritoneal cavity. Free intraperitoneal rupture can lead to anaphylaxis that can be potentially fatal. 15,16 Intraperitoneal rupture can present with clinical features ranging from vague abdominal pain to frank peritonitis with typical signs. Intraperitoneal rupture of splenic hydatid cyst is exceedingly rare. 17,18,19 Although hydatid disease is found in pregnancy with an incidence of 1 in 20000 to 1 in 30000 20,21 but liver is the usual site of involvement in pregnancy as well. Splenic cysts in pregnancy are extremely rare. 22 Most splenic cysts in endemic areas are hydatid cysts. 23 There are very few reports of hydatid cyst of spleen in pregnancy. 24,25 Pregnancy increases the risk of rupture owing to raised intra-abdominal pressure. There is a scant account of spontaneous rupture of hepatic hydatid cysts during pregnancy. However, there is hardly any literature documenting spontaneous rupture in the postpartum period. Ante natal detection should be accompanied by meticulous planning and follow up so as to prevent potentially fatal complication like rupture or haemorrhage. Differential diagnoses include epidermoid, pseudocyst, abscess, haemorrhage, neoplasm. USG and CT are the main stay of diagnosis especially in acute settings to rule out other causes of acute abdomen, with CT being superior. 26,27,28 The characteristic appearance varies as per duration, location, presence of secondary infection or rupture.
Serological tests like ELISA, Immunoelectrophoresis, or Indirect Hemagglutination test have limited feasibility in ruptured hydatid cyst, but nevertheless can be used when the diagnosis is in doubt. MRI has also been used in cases where USG/CT are inconclusive. Management of the ruptured cyst warrants an urgent laparotomy. 19 Splenectomy is the treatment of choice particularly in ruptured cysts. 29,30 Owing to post splenectomy complications including sepsis related deaths in 1.9% of adults and 4% of children, a number of spleen preserving procedures have come up. 31 There appears to be no signi cant difference between the rates of recurrence following splenectomy and spleen preserving procedures. 32,33 Despite this the most feasible option in a ruptured hydatid cyst of spleen continues to be splenectomy. Even though the optimal management of splenic cysts in pregnancy is yet to be established, but the management in postpartum period especially in case of rupture is straightforward. 22 A thorough lavage followed by scolicidal irrigation is necessitated to minimize seeding and recurrence. Various scolicidal agents like cetrimide, povidone iodine, hypertonic saline, chlorhexidine can be used. Laparoscopic procedures have been done in splenic hydatid cyst but in elective, uncomplicated cases. 34,35 Laparoscopic approach on emergent basis in case of a ruptured cyst remains largely unattempted.
Preoperative medical treatment with albendazole is not possible in patients who present with ruptured cysts, but should be started postoperatively as soon as possible. Patients should be followed up with USG and serological tests at shorter intervals.

Conclusion
Hydatid cyst should be included in the differential diagnosis of any cystic lesion in a patient from endemic region. In pregnant patients splenic cysts should be meticulously evaluated. A management plan with emphasis on assessment of the risk of rupture should be formulated at the outset of diagnosis even in ante partum detection. Surgical treatment should be done as early as feasible. In our part of the world, ethical committee approval is not mandated for case reports, provided the identity of the patient is not revealed. We have kept the identity hidden even in radiological images.

Consent for publication
Due consent has been obtained from the patient Availability of data and material The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Competing interests
The authors declare that they have no competing interests     Mobilised spleen delivered through wound before dividing pedicle