Littoral cell angioma with splenic rupture in pregnancy

Case

We present the case of a 38-year-old woman gravida 2, para 0 + 1. She had a background history of infertility and this pregnancy was after a successful 4th in vitro fertilisation attempt. Her medical history was significant for hypothyroidism for which she was taking eltroxin 75 μg. In addition to this, concomitant medications included aspirin, folic acid, low-molecular-weight heparin and progesterone. She had previously had a laparoscopic salpingectomy due to a hydrosalpinx.

She had a normal body mass index (BMI) of 24 kg/m² when she presented to the hospital at 9 weeks’ gestation, and was a non-smoker. The antenatal course was uneventful until 34 + 3 weeks’ gestation when she presented to the emergency department at our institution following a fall on the stairs at home. She had slipped on the stairs and landed onto her left side, leading to some discomfort along her left side from the rib to the hip. Her rhesus status was positive and she was reviewed in the emergency department. She was haemodynamically stable; a physical examination, foetal ultrasound scan and cardiotocograph (CTG) were reassuring, and she was discharged home. Nine days later, she attended the emergency department again; she had been attending physiotherapy for ongoing left-sided rib pain post her fall and the pain had worsened that day. On arrival to the emergency department she collapsed. She regained consciousness immediately. A foetal bradycardia for 3 minutes was observed on CTG which recovered. She reported left shoulder pain and mild abdominal pain with no per vaginal bleeding. On examination, she was haemodynamically stable with a blood pressure of 118/73 mm Hg, pulse rate of 85 bpm and oxygen saturations of 100%. Abdominal examination revealed a soft abdomen with mild tenderness elicited in the left upper quadrant, there was no rebound tenderness or guarding present. A bedside ultrasound scan was performed which was reassuring for the foetal well-being; however it revealed a large amount of free fluid in the maternal abdominal cavity of unknown origin. On CTG a foetal tachycardia was now evident with a baseline rate of 180 bpm. The decision was made to proceed to an emergency category 1 caesarean section under general anaesthetic. Two units of cross-matched blood were ordered from the laboratory. The pre-operative maternal blood parameters were:

In the operating theatre, a massive haemoperitoneum of approximately 3 L was observed on opening the abdomen. A lower segment caesarean section was performed and a female infant weighing 2.74 kg in good condition was delivered. There was clear liquor at delivery. Neonatal Apgar scores were 9 at 1 minute and 10 at 5 minutes. The uterus was examined and was intact with no rupture. Active bleeding continued originating from the left upper quadrant, and following suctioning, a ruptured spleen was visualised. An upper midline laparotomy was performed and a general surgeon attended and carried out a splenectomy. On removal of the spleen, a large haematoma was evident. The total blood loss was 3 L and the patient received 2 units of O rhesus-negative blood and 2 g of fibrinogen. She made an uneventful post-operative recovery and was discharged home on day 11 post-operative. She was reviewed at six weeks post-operative in the outpatient department. Histology was available at that time.

Histology of the spleen described a spleen weighing 374 g with dimensions of 13 × 11 × 6.5 cm. The outer surface appeared haemorrhagic and ruptured. The specimen was serially sectioned revealing an area of haemorrhage, 11 × 8 × 2 cm. The specimen consisted of spleen showing marked congestion of the red pulp. In addition, there appeared to be anastomosing vascular channels resembling splenic sinuses that were lined by endothelial cells admixed with muscularised larger vessels containing thrombi in varying stages of evolution. An area with dilated vascular spaces with enlarged endothelial cells was present at the periphery of the splenic red pulp adjacent to the area of haemorrhage. Immunohistochemistry revealed positive staining of the cells lining these spaces for CD31 and CD68. These cells were negative for CD34. The findings were those of a subcapsular littoral cell angioma with secondary haemorrhage. There was no evidence of malignancy.

Discussion

Splenic rupture in pregnancy is rare and is usually due to trauma or an underlying splenic pathology. There are normal physiological changes that occur in pregnancy which can increase the risk of splenic rupture. First, there is a significant overall linear growth pattern of the splenic area in pregnancy with increasing gestational age. In fact, the splenic area enlarges by up to 50% compared to its size in the first trimester, by the end of the pregnancy [1]. An increase in the maternal blood volume by up to 45% above the nonpregnant levels and reduced space in the peritoneal cavity during pregnancy could also be implicated in the pathogenesis of splenic rupture during pregnancy [2]. Circulating hormones such as oestrogen and progesterone cause structural changes to the spleen that may increase the risk of splenic rupture during pregnancy even after minor trauma [3].

First described by Falk et al. [4] in 1991, a littoral cell angioma is a vascular tumour which arises from the littoral cells in the splenic red pulp sinuses. Although vascular neoplasms are the commonest primary tumour of the spleen, littoral cell angiomas are rare. These rare vascular tumours can occur at any age and have no gender predilection. Although the majority of these tumours are benign, rare malignant variants have been described [5], [6]. Patients typically present with haematological abnormalities such as anaemia or thrombocytopenia. Splenomegaly is seen in almost all patients with this condition. In many cases these tumours are found incidentally during abdominal surgery for a nonrelated reason.

Radiological features include splenomegaly with multiple splenic lesions of similar size and appearance. On ultrasound the echotexture can be mottled with the splenic lesions appearing as hypoechoic, hyperechoic or isoechoic in nature. The differential diagnosis on sonographic assessment of a hyperechoic splenic nodular lesion includes haemangiomatosis, hamartoma and Kaposi sarcoma in patients with acquired immunodeficiency syndrome (AIDS) [7]. Computed tomography (CT) scanning can help establish a diagnosis. On delayed contrast-enhanced CT imaging, littoral cell angiomas homogeneously enhance and become isoattenuating relative to the remaining splenic parenchyma [8]. The delayed filling allows the nodules to become isodense surrounded by the enhancing splenic tissue. Magnetic resonance imaging (MRI) is the best radiological imaging method to differentiate between littoral cell angioma and other angiomatous vascular lesions. The nodular lesions of littoral cell angioma typically appear markedly hypointense with both T1- and T2-weighted pulse sequences. This finding reflects the presence of haemosiderin in the lesions. Haemosiderin accumulates in the cytoplasm of these neoplastic cells because of their haemophagocytic capacity [9].

Definitive diagnosis of a littoral cell angioma can only be made at histopathological examination. There are characteristic morphologic and immunophenotypic features that distinguish it from other vascular splenic tumours [10]. On gross examination at histopathology, a littoral cell angioma will typically reveal splenomegaly. On the cut surface of the spleen, multiple focal nodules which differ in colour from red to black depending on the chronicity of the blood in the lesion are present. The nodules are of similar size and are well delineated from the surrounding splenic tissue but do not have a surrounding capsule. It is characterised histologically by anastomosing vascular channels lined by tall or flat endothelial cells, which may anastomose with normal splenic sinuses at the periphery [11]. Like haemangiomas, littoral cell angiomas can express immunoreactivity for vascular endothelial markers CD31 and factor VIII. However, almost all cases also express histiocytic marker CD68.

Conclusion

To the best of our knowledge, this is the first case of a splenic rupture in association with a littoral cell angioma in pregnancy. We believe that the fall that the patient suffered at home led to splenic trauma and in association with the littoral cell angioma resulted in a haematoma formation and subsequent massive haemoperitoneum and maternal collapse. Splenic rupture is an important clinical entity to discuss as the clinical presentation can mimic that of a ruptured ectopic in early pregnancy and uterine rupture or placental abruption in later pregnancy. Splenic rupture in pregnancy is associated with a high morbidity and mortality rate for both mother and foetus. Timely recognition and prompt surgical intervention is crucial to allow for optimal maternal and perinatal outcome.