Abstract

Background To date, there is limited knowledge of patients with idiopathic splenomegaly in pregnancy, and the literature lacks cases of a combined cesarean section with splenectomy. The documented cases of splenomegaly in pregnancy often have infectious etiology such as that of malarial infection or an autoimmune component such as idiopathic thrombocytopenic purpura. This condition is life-threatening and complicates medical management, but its rarity has led to a paucity of management guidelines for patients with idiopathic splenomegaly in pregnancy. Objective The aim of this case is to provide novel insight into the management and outcomes of patients with idiopathic massive splenomegaly in pregnancy. Study Design This study is a case report with a review of the current literature on the reported cases of splenomegaly in pregnancy, splenectomy in pregnancy, and cesarean section with splenectomy. This case describes a history of pancytopenia and massive splenomegaly in a pregnant patient who underwent extensive workup with no clear etiology. This case additionally describes the course of care for this patient, including a combined cesarean section with splenectomy. We searched PubMed for all English language articles from 2000 to 2023, with search terms including “splenomegaly in pregnancy,” “splenectomy in pregnancy,” and “cesarean section and splenectomy.” Results In this patient’s case, despite an exhaustive infectious disease workup and evaluation for hematologic, inflammatory, and neoplastic causes, no clear etiology was identified. Delivery was deemed necessary at 34 weeks and 2 days gestation due to the patient’s severe, persistent pain, her worsening pancytopenia, and the risk of spontaneous splenic rupture during labor and delivery; our multidisciplinary team concluded that it was safer for both mother and baby to proceed with cesarean delivery and splenectomy in the early pre-term period. This patient’s pancytopenia improved within hours of spleen removal and has remained stable to date in outpatient follow-up. Baby Boy completed a NICU course of 22 days after requiring surfactant and respiratory support due to prematurity as well as monitoring due to maternal splenomegaly and thrombocytopenia. Conclusion This case offers valuable insights into managing patients with idiopathic splenomegaly during pregnancy and underscores the need for further investigation. Establishing clear delivery indications for these patients remains crucial, and prospective studies should evaluate the long-term impacts on both mother and child following combined splenectomy and cesarean delivery in the context of pancytopenia.

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