Symptomatic splenic hamartomas are rare in the pediatric age group, with only four previous reports in the literature. Splenic hamartoma has been reported as a solid homogeneous mass without calcification on CT and ultrasound (US), and only one previous report of the findings on MRI has been published. We report a case of a large symptomatic splenic hamartoma in a 14-year-old girl who presented with splenomegaly, pancytopenia and growth retardation. A solid mass with multiple punctate foci resembling calcifications was seen on US. The mass was heterogeneous and better demarcated on enhanced CT. Radiocolloid scintigraphy demonstrated uptake within the lesion, but less than that of normal spleen. The mass was isointense relative to normal splenic tissue on T1-weighted MRI (0.5 T) and of increased intensity with T2 weighting. At splenectomy, a red pulp hamartoma was identified, which contained nodules of hyalinization and necrosis thought to account for the punctate foci seen on US.
In these children as in other studies, the commonest presentation of segmental MCDK is in the upper pole of a duplex kidney associated with a ureterocele at the end of the atretic ureter. Atypical segmental MCDK may present a diagnostic dilemma and should be included in the differential diagnosis of multiloculated cystic masses and cystic kidneys.
PUV represents a spectrum of disease severity. A normal prenatal ultrasound does not preclude PUV. The majority of patients with a normal prenatal examination have good outcomes. The OR suggests that there may be increased risk for poor outcome in those with an abnormal prenatal examination. A multicenter study is necessary to obtain a larger sample size and more precise ORs.
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