Spontaneous Splenic Rupture due toPlasmodium vivaxin a Traveler: Case Report and Review

During acute and chronic malaria, there are changes in splenic structure and function that can result in either an asymptomatic enlargement or a wide variety of complications. Physicians are usually aware of the complications caused by Plasmodium falciparum malaria, but they are less familiar with the complications of Plasmodium vivax malaria. As an example, distress respiratory syndrome has recently been described in the course of P vivax malaria, most frequently after treatment instauration.1 Splenic rupture, though a rare complication of P vivax malaria, can be a life‐threatening event. Historically, splenectomy has been the treatment of choice in cases of spontaneous rupture of the spleen, but due to recognition of complications of this practice, new conservative strategies are considered in selected cases. We present a case of splenic rupture in a traveler that was successfully treated with a conservative approach. A 34‐year‐old patient had traveled to Indonesia for 1 month (visiting the islands of Sumatra, Java, Kalimantan, and northern region of Sulawesi), without taking malaria prophylaxis. He presented to the emergency department because of a 4‐day history of fever, with onset 24 hours after return. He was diagnosed with noncomplicated malaria due to P vivax (parasitemia 1%). Treatment with cloroquine (25 mg/base/kg) was prescribed, and the patient was discharged. The next day he returned to hospital because of sudden onset of upper left quadrant pain. Though on admission the patient was conscious, he appeared severely affected by abdominal pain and referred to having fainted several times during the previous hours. His blood pressure was 80/60 mm Hg, and his temperature was 36 °C. Abdominal examination was remarkable for tenderness in the left hypochondrium. Laboratory tests showed a hemoglobin level of 10.9 g/dL, a platelet count of 17.000/μL, and a normal white blood cell count (previous values on initial evaluation at the emergency department the day before had showed: hemoglobin 14.7 g/dL and platelets 26.000/μL). Creatinine value was at 1.69 mg/dL. Urea and electrolytes were normal, and liver function tests were as follows: AST 49 U/L, ALT 110 U/L, and total bilirrubin 1.86 mg/dL. Thin and thick blood films demonstrated no remaining parasites. Peritoneal lavage revealed hemoperitoneum. Image studies were performed: the chest X‐ray showed a left pleural effusion, abdominal ultrasonography showed a 15 cm nonhomogeneous spleen with hypoechogenic lines suggesting splenic rupture, and computerized tomography (CT) scan of the abdomen further showed abundant perihepatic and perisplenic fluid, perisplenic hematoma, and a peripheric splenic tear that spared the hilium (Figure 1). Because of stable hemodynamics, no surgical procedure was carried out and he was admitted to the intensive care unit where he underwent scrupulous monitoring and received blood and platelet transfusions and liquid replacement. The patient was discharged 6 weeks later, with a control CT scan showing only residual perisplenic hematoma and having completed oral standard treatment with cloroquine diphosphate (25 mg/base/kg, total dose) followed by primaquine (0.6 mg/base/kg/day during 14 days). Six months after admission, the patient remains asymptomatic, presenting no further splenic complications. Big encapsulated splenic hematoma. Malaria is the most frequent tropical infectious cause of spontaneous splenic rupture.2 It is a rare complication of P falciparum malaria, most frequently associated with P vivax malaria. There are no structured studies to measure the incidence of spontaneous splenic rupture in malaria, and rates in naturally occurring infection have been estimated to range, following the only two published series, from 0% (0/5,870 total cases of infection in Howard and colleagues series in 1973) to 2% (1/51 cases in Kahn and colleagues series in 1970).3,4 We consider that these are only rough approximations of real rates, as an incidence of 0% is difficult to understand and an incidence of 2%, given that the incidence of malaria attacks around the world, would imply a huge total number of splenic ruptures. Nevertheless, it is known that spontaneous splenic rupture is not a frequently communicated and diagnosed complication, and several review articles since 1993 suggest a significant underreporting and underdiagnosis of this entity.2 In fact, there are only 19 malaria cases complicated with spontaneous splenic rupture described in review articles in the English literature since 1960 (Medline database).5–7 Twelve cases were due to P vivax. Among these cases, only eight patients were travelers. The other cases of splenic rupture described were due mainly to P falciparum. Spleen enlargement is one of the characteristic clinical signs of malaria. Within 3 to 4 days of the onset of symptoms, a palpable asymptomatic enlargement of the spleen can be found in up to 50% to 90% of malaria patients. Other splenic complications due to malaria changes in splenic structure include torsion, cyst and hematoma formation, infarction, hypersplenism, ectopic spleen, and the hyperreactive malarial syndrome (previously known as tropical splenomegaly syndrome).7 Rupture is more frequent in nonimmune persons than in those living in endemic areas in which multiple attacks result in gradual splenic enlargement, making rupture less likely. Spontaneous splenic rupture (nontraumatic rupture) is most commonly associated with acute malaria, and formation and breakage of a contained hematoma are believed to be the underlying cause.2 During the primary attack, hematoma formation and rupture occur practically at the same time. Vomiting or chills, both frequent during the acute phase, or other minor abdominal pressures, like vigorous abdominal palpation, have also been suggested to play a role in the pathogenesis of splenic rupture during acute malaria.8 Other factors like thrombocytopenia, treatment with aspirin, rapid hyperplasia of lymphoid tissue with small infarctions and hemorrhages, and loss of capsule elasticity have been considered to contribute to hematoma formation and breakage. In chronic splenomegalies secondary to malaria (hyperreactive malarial splenomegaly) or other parasites (Leishmania sp) or virus (Epstein‐Barr), rupture of the spleen usually follows an obvious trauma. In these cases, hematoma formation precedes rupture in several days or weeks. Severe thrombocytopenia has been described in recent series of cases of severe P vivax malaria and could have acted as a contributing factor to hematoma formation in this patient. A high index of suspicion is needed for an early diagnosis, as sometimes rupture‐presenting symptoms do not differ from acute‐phase symptoms or from other nonsplenic surgical emergencies. Presence of hypotension, abdominal pain, or signs of intraabdominal bleeding should alert one to the possibility of splenic rupture, even if thick and thin blood films are already negative. Abdominal ultrasonography, CT scan, and arteriography can provide confirmation of the diagnosis in a hemodynamically stable patient.9 In the case presented, fainting and hypotension concomitant to upper left quadrant pain and anemia prompted an urgent peritoneal lavage. The patient had started treatment the previous day, and blood smears showed no malaria parasites. Nowadays, optimal treatment for P vivax malaria from Indonesia should take into account the possibility of a cloroquine‐resistant and/or a primaquine‐tolerant strain. This implies the use of mefloquine or quinine + doxicicline and high doses of primaquine (0.6 mg/base/kg/day). This patient had not visited the island of Irian Jaya, where cloroquine‐resistant strains are widespread, and his response to cloroquine, prescribed at the emergency department, was excellent. A left‐sided pleural effusion, as in this case, may be present on a chest X‐ray. This patient presented a normal white cell count, which is frequent in uncomplicated malaria but complications such as suprainfection and splenic rupture are often accompanied by an increased white blood cell count. Treatment options include splenectomy, which has long been the treatment of choice of splenic ruptures of different origins. This procedure can lead to severe complications such as infections due to widespread bacteria like Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae and other encapsulated bacteria, specially as patients living in malaria‐endemic regions frequently have no access to meningococcal and pneumococal vaccines. It can also increase the risk of complicated malaria, and patients should be advised to take antimalarial prophylaxis. On the other hand, the surgical procedure itself implies significant morbidity and mortality. Conservative management includes nonoperative management or splenic repair and vascular tamponade. This is the prevailing approach in splenic trauma with stable hemodynamics. Nonoperative management consists of bed rest in the hospital under strict monitoring, during 1 to 2 weeks with fluid and blood replacement as necessary, which was the choice in the case presented. Delayed rupture is rare. However, when there is uncontrolled bleeding and hypotension, surgery is required but most cases can be managed with splenic repair and tamponade.10,11 Successful medical management has been reported in various recent publications, but data regarding treatment of malarial splenic rupture are still scarce.5–7,12,13 Plasmodium vivax malaria is the second most common cause of imported malaria and is generally regarded as a disease that follows a benign course. However, physicians should become familiar with its rare life‐threatening complications such as spontaneous spleen rupture given the growing number of international travelers, migrants, and migrants who travel (visiting friends and relatives), which will probably lead to an increase in the incidence of complications in malaria patients in nonendemic areas. Prognosis depends on early diagnosis demonstrable by both ultrasound and CT scan, though the latter is the preferred confirmation method. Knowledge of splenectomy complications, specially increased susceptibility to bacterial and plasmodium infections, has favored the development of conservative procedures under strict monitoring, which should be considered in selected patients, such as frequent travelers to endemic areas. The authors state that they have no conflicts of interest.