A 45-year-old female presented to a local emergency department (ED) with complaints of acute onset upper abdominal pain and distension associated with nausea and vomiting.
Her medical history included well-controlled hypertension, hyperlipidemia and hypothyroidism for approximately two years. Her medications included daily enalapril 10 mg, rosuvastatin 10 mg, levothyroxine 50 mg and vitamin D supplement. She worked full-time as a physician and was mother to five children (ages 5 to 14 years) with the last two children being twins. Her pregnancies were uneventful except for the last pregnancy (with the twins) about six years ago; she had transient thrombocytopenia and mild liver enzyme elevation prior to the delivery which resolved promptly after the delivery. Reports of ultrasound examinations during her pregnancies did not indicate presence of hepatic or renal cysts. She had been on hormonal contraceptives intermittently with a cumulative duration of about 20 years. She denied a known family history of ADPKD.
On the day of her presentation to the local ED, while at work, she developed a sudden-onset sharp upper abdominal pain, associated with shortness of breath and a feeling of abdominal fullness. She felt nauseated and vomited several times. She was rushed to the ED, where her vital signs were found to be normal. Her abdomen was mildly distended and tender on palpation in the upper quadrants with some guarding. Laboratory studies showed hemoglobin 12.9 mg/dL, leukocytes 7.3 × 109 cells/mL, albumin 4.2 mg/dL, aspartate aminotransferase 26 U/L, alanine aminotransferase 45 U/L, alkaline phosphatase 66 U/L, bilirubin 0.6 mg/dL, blood urea nitrogen 19 mg/dL, creatinine 1.1 mg/dL, and urinalysis was unremarkable. Her serum amylase and lipase were normal. Ultrasonography of the abdomen revealed perihepatic ascites, multiple cysts throughout the liver with the largest cyst being 1.7 cm in diameter, and numerous cysts in the kidneys. Contrast-enhanced abdominal computed tomography (CT) confirmed perihepatic ascites and the benign-appearing cysts in the liver and kidneys (Figure , left). She was hospitalized for monitoring and treated with intravenous fluids and antiemetics (ondansetron). The following day, an esophago-gastro-duodenoscopy with random biopsy was performed which was without abnormality. Her pain, nausea and vomiting subsided, and she was discharged. A week later, she underwent an elective laparoscopic cholecystectomy. Intra-operative inspection showed no abnormality except for the visible fluid-filled hepatic cysts. The pathology of the gallbladder was normal.
Figure 1 Contrast-enhanced abdominal computed tomography. Left panel: Serial images from an axial computed tomography (CT) scan at the time of the patient’s presentation to the local emergency department, showing perihepatic ascites and multiple liver (more ...)
Five months later, she presented to our institution for a second opinion. She was asymptomatic and physical examination was unremarkable. Her medications were unchanged. An abdominal CT scan showed both liver cysts and bilateral kidney cysts (Figure , right), consistent with ADPKD. In retrospect, her acute abdomen and ascites were consistent with hepatic cyst rupture.