ABSTRACT
Beaver tail liver is an anatomical liver variant presenting as elongated left lobe of liver which extends laterally to the spleen. It can present with symptoms or be detected accidentally. We present a case of a 2-year-old asymptomatic patient who was had an X-ray of the chest describing a shadow of unknown origin located left paracardial and the diagnosis of "beaver tail liver" was confirmed after a multi-slice computed tomography of thorax and abdomen was done. We give an overview of very sparse available literature on this anatomical variant while emphasizing the rarity of diagnosis in pediatric population and clinical importance of this variant.
ABSTRACT
BACKGROUND: McCune-Albright is a rare syndrome, caused by mutation of the GNAS1 gene, and is characterized by an appearance of multiple endocrinopathies, most commonly premature puberty, polyostotic fibrous dysplasia and skin changes called cafe au lait macules. CASE REPORT: We present the case of a patient who is, to the best of our knowledge and after extensive review of literature, the youngest McCune-Albright syndrome patient with growth hormone excess, diagnosed at 8.9 months of age. An extensive diagnostic procedure was done upon the diagnosis. Hormonal assessment was performed and all hormone levels were within reference range, and an additional oral glucose suppression that noted the presence of growth hormone excess. Magnetic resonance imaging of the pituitary gland did not detect a tumor process. The genetic analysis of the GNAS1 gene from skin punch biopsy came back negative. Octreotide was administered as therapy for growth hormone excess at 9.8 months. After the introduction of therapy, we noted a decrease in growth rate from 29.38 to 16.6 cm/year. CONCLUSION: This case report emphasizes the lack of available data on treatment of growth hormone excess and follow-up in pediatric population and the need for further research.
