Splenic rupture complicating sequestration crisis in a two-year-old with homozygous sickle-cell anaemia

Abstract

Introduction: Splenic sequestration crisis (SSC) is a major cause of early mortality in sickle-cell disease, although rupture of the engorged spleen is rarely reported. Sickled erythrocytes obstruct splenic sinusoids, blood accumulates and intravascular volume collapses, with free intraperitoneal haemorrhage if the capsule fails.

Case report: A two-year-old boy with homozygous sickle-cell anaemia arrived after two weeks of fever, breathlessness, abdominal distension and pallor despite two transfusions. He was tachypnoeic at 66 breaths/min, tachycardic at 136 beats/min, and febrile at 38.2°C; the spleen projected 12cm below the costal margin and packed-cell volume was 14%. Abdominal paracentesis retrieved 250mL of fresh blood, indicating haemoperitoneum. Immediate transfusion, artemisinin therapy and supportive care led to a transient improvement. Within twenty-four hours oxygen saturation and blood pressure deteriorated; ultrasonography showed a ruptured spleen with free fluid. Emergency laparotomy revealed a macerated, actively bleeding spleen. Total splenectomy with two intra-operative transfusions controlled haemorrhage. Post-operative antibiotics, a pneumococcal conjugate vaccine and penicillin prophylaxis were commenced. The child left hospital on day seven with haemoglobin 10g/dL and remained well at two-week review.

Discussion: Rupture can complicate SSC before auto-infarction fibroses the spleen. Clinicians should suspect rupture when rapid splenic enlargement, precipitous anaemia and circulatory compromise coincide. Diagnostic paracentesis and bedside imaging can aid decision-making in resource limited settings, but survival depends on prompt transfusion and surgical control of bleeding. Multidisciplinary management, coupled with stringent post-splenectomy immunisation and parental education, should be standard when this rare complication is encountered.