Wandering Spleen with Niemann-Pick Disease

Wandering spleen is a rare entity that develops as a result of splenic hypermobility caused by congenital absence or acquired laxity of splenic suspensory ligaments that hold the spleen in its normal position. It is usually described in women of childbearing age and rare in children. Symptoms include acute or chronic intermittent abdominal pain due to the torsion of the pedicle. It may also be asymptomatic and discovered incidentally on clinical or radiological examination done for other reasons. Niemann-Pick disease is a lipid storage disorder. It is characterized by sphingomyelin and cholesterol accumulation in many organs and predominantly the liver and spleen. Hepatosplenomegaly may develop due to the disease. A 16-year-old girl with Niemann-Pick disease has admitted to our hospital with complaint of amenorrhea. A 10 cm pelvic mass possibly related to the left ovary was detected in her examinations. Laparotomy was performed and a 12 cm wandering spleen was discovered at the left lower quadrant of abdomen. And 5 cm faint, solid mass was found to be fused with the spleen at hilus. Splenectomy was performed. Histopathological examination revealed ceroid histiocytosis throughout spleen. The hilar mass was found to be a necrotic and sclerotic tissue. Although Niemann-Pick disease is reported as an etiological factor for splenomegaly as a cause of wandering spleen in the literature, their association is not reported yet. Asymptomatic wandering spleen can be regarded as abdominal or ovarian mass as in our case. We discussed this case to point out the differential diagnosis of wandering spleen and its rare association with Niemann-Pick Disease.