Malaria-associated splenic haemorrhage requiring emergency splenectomy: a rare complication managed in a resource-constrained setting

Abstract

Falciparum malaria and Vivax malaria are notorious for the development of multiple complications, including acute kidney injury, acute liver injury, severe hemolysis, bone marrow suppression, severe acidosis, hypoglycemia and CNS complications. We hereby present two cases of rare complications associated with Malaria, which were managed in a resource-constrained setting in Eastern Africa. Two individuals working in the malaria endemic region of South Sudan were detected with Mixed Plasmodium falciparum–Plasmodium vivax when they reported to the hospital with acute febrile illness for two days. They were noted to have palpable hepatosplenomegaly. Both patients suffered spontaneous rupture of the spleen, leading to gross hemoperitoneum and hemodynamic instability. They were taken up for an emergency explorative laparotomy as a life-saving measure. Of the two patients, one survived following timely surgical intervention and supportive care, whereas the other succumbed despite emergency management.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12936-026-05854-8.

Introduction

Malaria is endemic to many countries in Africa, Southeast Asia and the Americas. The prevalence of this disease is strongly affected by climatic conditions. Falciparum malaria has been one of the most common causes of death in Africa for many decades. African countries face significant challenges in malaria management and control owing to widespread poverty, poor health coverage and emerging resistance to antimalarial drugs and insecticides [1]. Falciparum and vivax malaria are associated with multiple disease-related complications, although these occur less frequently in isolated vivax malaria. Multiple pathogenetic factors, including parasite-derived toxins and surface antigens on infected erythrocytes, mediate sequestration of these parasites in the capillaries of vital organs, resulting in complications [2]. The spectrum of malaria-related complications includes acute kidney injury, acute hepatitis, cerebral malaria, severe hemolysis, hypoglycemia, metabolic acidosis, splenic infarcts, bone marrow suppression and multiple treatment-related complications.

Spontaneous splenic rupture in malaria is a potentially life-threatening, rare complication. In a systematic review published by Osman et al. in 2012, they reported 252 cases of this rare complication [3]. Given the paucity of published data, there is a lack of standardised management protocols. A high index of suspicion, prompt detection, close monitoring and emergent surgery/radiological intervention on hemodynamic deterioration, along with appropriate resuscitation and supportive care, is imperative for a positive outcome. We hereby present two cases with this complication and describe their management in a resource-constrained setting.

Patient 1

A 29-year-old male of Indian ethnicity has been residing in the malaria-endemic region of South Sudan for the last six months before the onset of his symptoms. He presented to a field hospital in the northern part of South Sudan with a history of fever of two days duration. He complained of intermittent fever recurring every 6–8 h associated with chills and rigour. Each episode was associated with frontal headache and myalgia. He tested positive for both Plasmodium vivax and Plasmodium falciparum via a rapid diagnostic kit. He was started on oral artemisinin-based therapy (Artemether 80 mg and Lumefantrine 480 mg 1 tablet twice a day) along with primaquine (15 mg BD for 14 days) at the primary health care centre after testing for G6PD activity. He showed a poor response to the ACT given for three days and continued to mount febrile episodes; therefore, he was referred to our hospital. His clinical examination revealed an enlarged liver and spleen (4 cm and 2 cm below the right and left costal margins, respectively). He was also noted to have tenderness on palpation of the spleen. His initial investigations revealed anaemia (Hb 10.2 g/dL) and thrombocytopenia (40,000/cm³). He also had unconjugated hyperbilirubinemia (Total bilirubin 2.2 mg/dl and unconjugated bilirubin 1.7 mg/dl) and transaminitis (ALT/AST – 68/87 IU/L). His peripheral smear revealed the presence of asexual forms of Falciparum. The results of his investigations are tabulated in Table 1. In accordance with the institutional protocol, the patient underwent ultrasonography of the abdomen, which revealed an enlarged liver and spleen and a suspicious perisplenic collection. For confirmation, the patient underwent a CT scan of the abdomen, which revealed a splenic hematoma and a spleen of 15 cm in size (Fig. 1a).

Table 1.

Summary of case presentation with examination findings, investigation findings and Treatment administered for both patients

Parameter	Case 1	Case 2
Age/Sex	29/Male	45/Male
Ethnicity/Region	Indian ethnicity/South Sudan	Indian ethnicity/South Sudan
Comorbidities	None	None
Initial symptoms	Intermittent fever with chills, myalgia, frontal headache	Intermittent fever, chills, myalgia
Duration of symptoms	2 days before presentation	2–3 days before presentation
Initial diagnosis	Malaria (Rapid positive for P. falciparum and P. vivax)	Malaria (Rapid positive for P. falciparum and P. vivax)
Initial treatment	Oral ACT + Primaquine	Oral ACT + Primaquine
Reason for referral	Poor response to conventional oral treatment	Poor response to conventional treatment
Key clinical findings	Liver 4 cm & Spleen 2 cm below costal margins), Left upper quadrant tenderness	Liver 4 cm and spleen 3 cm below the respective costal margins, Left upper quadrant tenderness
Initial labs	Hb 10.2 gm%, Platelets 40,000/mm3, increased bilirubin(2.2 mg/dl), ALT/AST – 68/87 IU/L Normal INR	Hb – 13.6 gm%, Platelets – 1.4 Lakh/mm3, Increased Bilirubin (3.0 mg/dl), ALT/AST- 56/64 IU/L Normal INR
Imaging	USG: Spleen of 14 cm and peri splenic collection, CT Abdomen: Splenic hematoma and spleen of 15 cm in size	USG Abdomen: Enlarged spleen 16 cm in size CT Abdomen: Enlarged liver of 17 cm and Spleen of 19 cm in size
Complication	Spontaneous splenic rupture (confirmed via FAST and USG guided aspiration)	Spontaneous splenic rupture with massive hemoperitoneum (confirmed via FAST and USG guided aspiration)
Surgical intervention	Emergency laparotomy and splenectomy	Emergency laparotomy and splenectomy
Intra-op findings	Hemoperitoneum, Enlarged, friable spleen (15 cm), active bleeding from ruptured spleen	Hemoperitoneum, Friable enlarged spleen of 22 cm in size and 1.2 kg gross weight. Active bleeding from splenic bed
Post-op course	Persistent fever, parasitemia with P. falciparum → Switched to IV quinine (responded well)	Cardiac arrest, revived after 15 min Cardio-Pulmonary Resuscitation; re-exploration for persistent bleeding, likely coagulopathy
Post -OP lab values	Transient Cytopenias were noted with unconjugated hyperbilirubinemia and transaminitis	Rapid fall in Hb ranging between 4–7 gm% despite multiple transfusion Thrombocytopenia (< 50,000/cumm) and Increased INR values – 1.8
Further complications	Transient leukocytosis & thrombocytosis post-op which recovered spontaneously	Multi organ dysfunction, coagulopathy, hypotension, persistent bleed and death
Definitive anti-malarial therapy	IV artesunate → IV quinine	IV artesunate → Oral Atovaquone-Proguanil
Supportive treatment	6 PRBC + 2 whole blood transfusions; inotropes	10 PRBC + 8 whole blood transfusions; dual inotropes, mechanical ventilation
Vaccinations post-splenectomy	PCV-13, Meningococcal (ACWY), Hib, Influenza, COVID-19	Not applicable
Outcome	Survived and discharged in stable condition	Died due to hemorrhagic shock and coagulopathy

Fig. 1.

a An abdominal transverse section CT scan showing a Splenic hematoma in case 1. b An abdominal coronal section showing enlarged liver and spleen in case 2

Given the presence of malaria parasites in the blood and associated complications, he was started on intravenous artesunate (2.4 mg/kg) along with doxycycline (100 mg BD). He was continued on Primaquine, the dose was changed to 0.5 mg/kg OD. Since he was hemodynamically stable, conservative management of the splenic hematoma with close hemodynamic and sonological monitoring was planned. He continued to mount fever despite treatment with IV artesunate. He was managed with supportive anti-pyretics and tepid sponging. He also continued to experience persistent nausea and vomiting despite treatment with proton pump inhibitors and antiemetics. Sonological monitoring done on a daily basis, before clinical deterioration, did not reveal any increase in the size of the hematoma and was consistent with the initial findings. The complete blood count was monitored twice daily.

On day 4 of admission, he complained of giddiness and was hypotensive and pale, with cool and clammy extremities. An urgent bedside USG revealed a gross heteroechoic collection in the abdominal cavity. USG-guided needle aspiration confirmed the presence of blood in the peritoneal cavity. Given the patient’s hemodynamic instability and unresponsiveness to fluid resuscitation, he was taken up for an emergency exploratory laparotomy. Intraoperatively, the patient was noted to have hemoperitoneum (approximately 2 L) with active profuse bleeding. The source of the bleeding was the ruptured spleen. The splenic vessels and short gastric vessels were clamped and doubly ligated, and splenectomy was performed (Fig. 2a). The spleen was noted to be significantly enlarged (15 cm) and friable. Once hemostasis was achieved, mass closure of the abdomen over an abdominal drain was performed. Intraoperatively, the patient had fluctuating blood pressure, which was managed with the transfusion of six units of packed RBCs and two units of whole blood (plasma and other blood components were unavailable). He also required inotropic support to maintain an optimal mean arterial pressure. Postoperatively, the patient was closely monitored for any recurrence of bleeding manifestations. During the postoperative period, the patient continued to be febrile, and his peripheral smear revealed the persistence of Falciparum trophozoites (Fig. 3). Given the suboptimal response to artesunate, he was started on intravenous quinine therapy. A baseline ECG and blood sugar measurement were performed, and the patient was started on quinine via an infusion pump. The rate of infusion was limited to 5 mg/kg/hr, and a loading dose of 20 mg/kg/hr was administered over 4 h. His cardiac rhythm was monitored along with hourly blood sugar throughout the infusion. The loading dose was followed by a maintenance dose of IV Quinine at 10 mg/kg every 8 h. Oral Doxycycline 100 mg BD was continued along with IV Quinine. He responded well to the treatment. He showed a clinical response by day 2 and parasitic clearance after day 3 of treatment. Once he was asymptomatic, his vaccination was initiated. He received vaccines for Pneumococcus (PCV-13), Meningococcus (ACWY), Haemophilus influenzae type b, influenza and COVID-19. Post-splenectomy, the patient was noted to have transient leucocytosis and thrombocytosis along with anaemia, which was expected and gradually resolved. He was given a post-splenectomy wallet card for emergencies, and prophylactic antibiotics were continued. He has been advised to receive periodic vaccinations. A viral panel (PCR test) was performed postoperatively to rule out infection with endemic hemorrhagic fever, which was negative.

Fig. 2.

a Intraoperative image of an enlarged spleen with ruptured capsule in Case 1. b Post-operative enlarged and friable spleen in case 2

Fig. 3.

Peripheral smear showing multiple Falciparum Trophozoites despite treatment with Artemisinin-based therapy

Patient 2

This 45-year-old male of Indian ethnicity has been residing in South Sudan for last six months before the onset of his symptoms. He was also referred to our hospital with complaints of intermittent fever with chills and myalgias for a 2–3-day duration. His clinical examination revealed a palpable liver 4 cm below the right costal margin, and a palpable spleen 3 cm below the left costal margin. He also tested positive for Mixed Plasmodium falciparum–Plasmodium vivax disease. His initial ultrasound of the abdomen revealed an enlarged spleen measuring 16 cm. He was started on oral ACT along with primaquine (after testing for G6PD activity). He was later switched to injectable artesunate (2.4 mg/kg) in view of clinical worsening in the form of persistent fever, recurrent episodes of vomiting, poor oral intake and deranged liver function (S. Bil 3.0), and declining platelets (50,000–60000/cumm) while on oral ACT and supportive medications like antipyretics and antiemetics. His follow-up investigations revealed the presence of trophozoites of P. falciparum in the peripheral blood even after 5 days of therapy with intravenous artesunate. He also complained of left upper quadrant pain, for which a contrast-enhanced computed tomography of the chest and abdomen was performed, which revealed a significantly enlarged liver and spleen (Liver −17 cm and Spleen −19 cm in size) (Fig. 1b). His investigations on day six of admission revealed parasitic clearance on peripheral smear, mild transaminitis and hyperbilirubinemia. Given symptomatic improvement and parasitic clearance, he was shifted to oral anti-malarials (Tab Atovaquone and Proguanil). A quinine-based combination was selected as he had shown a poor response to initial oral artemisinin-based therapy. On day 7 after admission, the patient collapsed in the ward. A Code Blue was initiated, and the critical care team attended to the patient. He was unresponsive and had an absent carotid pulse. He was revived after 15 min of high-quality cardiopulmonary resuscitation. A bedside FAST (focused assessment with sonography in trauma) assessment followed by USG-guided needle aspiration revealed hemoperitoneum. He was taken up for an emergency explorative laparotomy. The patient was noted to have significant hemoperitoneum with approximately 4 L of blood in the peritoneal cavity. He was found to have a massive spleen(22 cm and 1.2 kg), which was friable, and there was spurting of blood from the splenic bed (Fig. 2b). He underwent splenectomy, and hemostasis was attempted. He received a massive transfusion of 10 units of packed RBCs and two units of whole blood to maintain hemodynamics. Once hemodynamically stabilised, the patient was transferred to the intensive care unit and was kept on mechanical ventilation and inotropes. His haemoglobin levels kept fluctuating between 4–7 gm% despite repeated transfusions. His platelet count fell to around 50,000/cumm and INR was deranged at 1.8. He remained critical with high inotropic requirements, poor urinary output and multiorgan dysfunction. The unavailability of blood components made the treatment of coagulopathy extremely difficult. He underwent a re-exploration laparotomy after 6 h of initial surgery, as he continued to bleed out into the abdominal drain (100 ml/hr of fresh bleed).

On re-exploration, diffuse ooze was noted from multiple tissue beds and surgical incisions owing to coagulopathy. No major bleeding vessel was identifiable. Bleeding could not be controlled despite efforts to suture friable tissue, thus worsening the hypotension. An additional 6 units of fresh whole blood were transfused. Peritoneal packing was performed with a midline incision sutured with a temporary sterile plastic bag (Bogota bag). He was shifted to the ICU for damage control resuscitation. Postoperatively, the patient required dual inotropic support in view of intractable hypotension. After 6 h of the second surgery, he succumbed to his illness despite all efforts and could not be revived.

Discussion

Spontaneous splenic rupture is associated most commonly with infections like malaria, infectious mononucleosis and haematological malignancies [4]. Malarial splenic rupture arises from acute pathological changes in the spleen, which increase intrasplenic tension and predispose to capsular disruption even in the absence of external trauma. Malarial splenic ruptures are mostly underreported, with a few series claiming rupture rates upto 2% [5]. In our experience of treating 2124 cases of Falciparum and Vivax malaria over the last 3 years, we have encountered only 2 cases (0.09%) of spontaneous splenic rupture. The previously quoted data seems to be an overestimation. Modern trauma care protocols, including splenic preservation, have substantially improved reported mortality to 7–18% in traumatic splenic ruptures [6]. In comparison, mortality in malarial splenic rupture can be as high as 38% in non-natives [7].

The spleen plays a critical role in immune response to the malarial parasite and pathophysiology of the disease. Splenic enlargement (splenomegaly) is a common manifestation noted in both acute and chronic malaria. In endemic regions, splenic enlargement is caused by vascular congestion, cellular hyperplasia and phagocytic activity. In most cases, splenic enlargement resolves gradually after parasite clearance. However, the splenic enlargement makes it more fragile and susceptible to complications. Splenic rupture is a rare complication, and it is potentially life-threatening. It can lead to severe intra-abdominal haemorrhage and hypovolemic shock. An extensive literature search revealed a few sporadic case reports and small case series, highlighting the rarity of this condition. Splenic rupture has been reported with Mixed Plasmodium falciparum–Plasmodium vivax disease as well as isolated infections with P. falciparum or P. vivax [8].

Several mechanisms for this complication have been proposed, and most often act together. These include:

1. Massive cellular hyperplasia and venous congestion leading to increased size and turgor, thus compounding the stress on the splenic capsule.

2. Microvascular occlusions leading to infarction within the spleen weaken the tissue integrity.

Minor external pressure during palpation or an increase in intra-abdominal pressure (coughing, vomiting, retching, attempted Valsalva, or physical activity) in such cases can precipitate rupture of the fragile organ. The patient may present with severe left-upper-quadrant (LUQ) or generalised abdominal pain. The pain is often referred to the left shoulder (Kehr’s sign). The pain may be associated with abdominal distension, dizziness, syncope or collapse. On examination, there may be hypotension, tachycardia and signs of reduced peripheral perfusion. Abdominal examination generally reveals tenderness/guarding in the left upper quadrant, along with signs of generalised peritonitis (if a large hemoperitoneum develops) [9].

The diagnosis of this condition requires a high index of suspicion in a patient with acute abdominal pain, falling haematocrit or hypotension. A Bedside ultrasound (focused assessment with sonography in trauma—FAST) can quickly identify free fluid and subcapsular collections. A contrast CT of the abdomen (when available and done if the patient is stable) can better define subcapsular hematoma, active bleeding (contrast extravasation) and splenic laceration [10].

There is a lack of standard guidelines on the management of splenic haemorrhage in cases of complicated malaria. We have extrapolated the guidelines given by the American Association for the Surgery of Trauma for the management of splenic injury. The management primarily focuses on early resuscitation with fluids and blood transfusion as needed. The correction of coagulopathy is of paramount importance. The appropriate initiation or continuation of effective antimalarial treatment is also essential. Artemisinin derivatives remain among the most effective treatments for falciparum malaria in most malaria-endemic areas. Resistance to Artemisinin has emerged significantly in many regions of Southeast Asia. Reports have revealed that ACTs are largely efficacious in India and Sub-Saharan Africa. However, epidemiological studies have reported a few validated mutations (i.e., 476I, 539 T and 561H) in these two regions [11]. The authors, after treating more than 300 cases of Falciparum malaria in the past six months, have noted suboptimal clinical response, probably due to resistance to conventional ACT regimens in South Sudan in more than 30% of the cases. Intravenous quinine has been used as an alternative drug in complicated malaria in patients who have not responded to ACT. The drug is associated with serious complications such as anaphylaxis and cardiac arrhythmias. Patients should be carefully screened for a preexisting cardiovascular disease and a prolonged QT interval.

The definitive management option for splenic haemorrhage depends on the patient's hemodynamic stability and the availability of medical resources. An emergency splenectomy is indicated for hemodynamically unstable patients with ongoing intra-abdominal bleeding or peritonitis. The non-operative management is appropriate for hemodynamically stable patients with subcapsular hematomas or minimal bleeding. This entails careful hemodynamic monitoring, transfusions (if required), serial USG imaging and bed rest. Splenic artery embolisation is an alternative option at centres with the facility of interventional radiology for controlling bleeding while preserving splenic function. The outcome depends on the promptness of diagnosis, hemodynamic status at presentation, availability of blood products and availability of surgical/interventional facilities [12].

Post-splenectomy, clinicians must counsel patients about lifelong implications (increased risk of encapsulated bacterial infections) and ensure appropriate vaccinations. Prophylactic antibiotics are indicated as per the guidelines. Strict preventive measures must be followed by the patients against subsequent malaria infections in endemic regions.

As per our experience with these cases, we have developed an institutional protocol for monitoring patients at high risk for splenic rupture. Any patient with a spleen size on USG of 15 cm or above at presentation, and any of the associated features listed below, is advised admission for closer clinical and daily sonographic monitoring of the splenic size or any evidence of bleeding:

• Extremes of age

• Rare blood group

• Mixed malarial disease

• Suspected resistance to ACT with ongoing fever and parasitemia on peripheral smear

• Findings of parenchymal/subcapsular contained bleed/Splenic infarcts

• Comorbidities like anaemia and G6PD deficiency

Their hemogram and biochemical parameters are monitored on a daily basis. They are closely monitored for evidence of coagulopathy. These patients are advised to minimise exertion. Every attempt is made to achieve parasitic clearance in these patients using alternate regimens.

Conclusion

Splenic rupture and haemorrhage are rare and catastrophic manifestations of complicated malaria. A high degree of suspicion and early intervention can be lifesaving. Post-splenectomy vaccinations and patient education ensure correct practices and prevent life-threatening overwhelming infections. Effective prevention strategies directed at eliminating mosquito breeding sites, the use of insecticidal-treated mosquito nets, personal protection with covered clothing and mosquito repellents should be promoted.

Author contributions

AK, DU and VSK - Wrote the manuscript BAA, AAF and AP - Prepared Figures and Tables All Authors - Reviewed the manuscript

Data availability

No datasets were generated or analysed during the current study.

Declarations

Competing interests

The authors declare no competing interests.