Ascites In A 20-Year-Old Man With Dandy-Walker Syndrome, Hydrocephalus, And Ventriculoperitoneal Shunt

Hyojeong Han, MD*2. Kristen Critelli, MD†3. Amy W. Davis, MD‡4. James E. Squires, MD†5. Michael D. Fox, MD* 1. *Department of Pediatrics,2. †Department of Pediatric Gastroenterology, Hepatology, and Nutrition, and3. ‡Department of Pediatric Pathology, Children’s Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PAA 20-year-old man with Dandy-Walker syndrome, cerebral palsy, epilepsy, and congenital hydrocephalus with ventriculoperitoneal (VP) shunt presents with 4 months of progressive abdominal distention and 1 day of lower-extremity swelling. Treatment for constipation was tried before his presentation, with no improvement. He is uncomfortable when supine, which his mother attributes to the abdominal fullness. Otherwise, he has been asymptomatic.Physical examination is notable for a distended abdomen and pitting edema up to his ankles. There is no dyspnea and no stigmata of chronic liver disease.Laboratory evaluation is notable for a hemoglobin level of 10.5 g/dL (105.0 g/L), an albumin level of 2.0 g/dL (20.0 g/L), a total protein level of 5.2 g/dL (52.0 g/L), and an erythrocyte sedimentation rate of 39 mm/hour. Results of additional studies, including serum electrolytes, liver function, kidney function, thyroid hormones, coagulation profile, urinalysis, pancreatic enzymes, serum uric acid, serum lactate dehydrogenase, serum ceruloplasmin, and serum α1-antitrypsin levels, are all normal. Tuberculin skin test results are negative at 48 hours. Abdominal ultrasonography shows a large amount of fluid throughout the abdomen and pelvis, with coarse echotexture of the liver but patent hepatic vessels with normal directional flow (Fig 1). Computed tomographic (CT) scan of the abdomen is notable for a large volume of ascites but normal intra-abdominal organs, including the liver and spleen (Fig 2). Paracentesis …