A Novel Presentation of Littoral Cell Angioma and Lymphatic Malformations in a Neonate

Abstract

This case report describes a novel presentation of littoral cell angioma (LCA) and lymphatic malformations involving the omentum and mesentery. To our knowledge, these 2 entities have not been reported in the same patient. A 1-month term infant male presented with chylous ascites. During his workup, imaging detected splenic nodules. Biopsies revealed that the nodules were LCA and the chylous ascites was secondary to microscopic mesenteric and omental lymphatic malformations. Evaluation for a secondary malignancy, an underlying immunologic defect, and genetic causes were unrevealing. The presence of LCA and lymphatic malformations in the same patient suggests a genetic link between these 2 rare vascular disorders and may help elucidate the etiopathogenesis of these 2 poorly understood anomalies.

Clinical Landscape of Littoral Cell Angioma in the Spleen Based on a Comprehensive Analysis

“…The statistical analysis showed a male-to-female ratio of 0.90 in 405 patients with LCA, which indicated the absence of gender predilection. The average age of 267 patients was 48.4 ± 16 years, with the oldest and youngest being 86 years old and 26 days old, respectively ( 11 , 19 ). As shown in Figure 2 , LCA was more prevalent in the 40–60-year age group; however, it was detected in 15 minors ( 1 , 19 – 30 ).…”

“…The average age of 267 patients was 48.4 ± 16 years, with the oldest and youngest being 86 years old and 26 days old, respectively ( 11 , 19 ). As shown in Figure 2 , LCA was more prevalent in the 40–60-year age group; however, it was detected in 15 minors ( 1 , 19 – 30 ).…”

“…A total of 435 participants were diagnosed with LCA with a male-to-female ratio of 0.90 (192/213), indicating no gender predilection. The age of LCA onset showed a normal distribution with the oldest and youngest being 86 y ( 11 ) and 26 d ( 19 ), respectively. Although most of the patients were aged 40–60 years, the incidence of LCA in children was not uncommon (5.7%) ( 1 , 19 – 30 ).…”

“…A total of 18 patients described the natural history of LCA. Detailed description of the period of LCA formation was provided for ten patients (19,26,38,42,45,65,70,76,100,101). The median time for LCA formation was 15 m, with the shortest and longest being 26 d and 4 y, respectively.…”

“…The median time for LCA formation was 15 m, with the shortest and longest being 26 d and 4 y, respectively. Eleven patients with untreated LCA survived during follow-up, which had no obvious symptoms of discomfort (16,19,43,65,71,(102)(103)(104)(105)(106)(107). The median of follow-up time of patients with untreated LCA was 24 m, with the shortest and longest being 0.5 m and 9 y.…”