CC: flank pain
HPI: 56 year old Korean man with no past medical history reports 2 months of worsening bilateral flank pain. He states it was of sudden onset with gradual worsening of the pain over 2 months. It radiates to upper back, but there is no radiation to groin. Intensity is 10/10 at its worst, intermittent, sharp in quality, and worse when lying down. In addition, he complains of subjective fevers, abdominal swelling, shortness of breath and decreased exercise tolerance. He is only able to walk up 1 flight of stairs when he previously could walk multiple flights. He denies chest pain, palpitations, lower extremity swelling, and orthopnea. He also denies any changes to urination, dysuria, frequency, gross hematuria or difficulty urinating. The patient has been seen at several outside emergency departments, however, he has not had further work up due to lack of funding. He reports he has always been healthy and has never been hospitalized, never had surgeries, and never been treated for any diseases. Exam is pertinent for decrease breath sounds at the bilateral lung bases with dullness to percussion. The patient also has moderate tenderness to palpation to bilateral flanks.
Labs/Studies:
BUN/Cr: 19/1.61
K: 5.3
Pro-BNP: 277
UA: moderate blood, 4-5 RBCs
Urine Cr 45
Urine urea 219
Feurea 41%
IgG 1046 mg/dL (690-1600)
Abdominal US:
Moderate bilateral hydronephrosis. No obstructing lesion is identified within the renal collecting systems.
Bilateral pleural effusions.
CXR:
Pleural Fluid:
Yellow color
Cell count = 1073 (PMN 5%, lymph 23%, 9% mono, 63% other)
Protein 4.3, ratio: 4.3/6.0 = 0.72
LDH 221, ratio: 221/154 = 1.44
Glucose 72
Gram stain negative, no organisms
Fungal culture negative
Pleural Fluid Cytology:
CT Abdomen/Pelvis with Contrast:
The patient had evidence of bilateral hydronephrosis and pleural effusions. CT A/P demonstrated retroperitoneal fibrosis encasing the bilateral renal arteries and abdominal aorta, with hepatosplenomegaly, and mild T12 vertebral body compression fracture. Urology performed cystocospy with ureteral stent placement however renal function worsened requring IR to place nephrostomy tubes. Pleural fluid was obtained with cytology positive for adenocarcinoma. The patient’s IgG4 levels were not elevated. Overall, it was though that the patient had retroperitoneal fibrosis secondary to malignancy which was discovered to be signet ring cell adenocarcinoma of the gastrointestinal tract.
Morning Report Pearls:
Retroperitoneal fibrosis is a rare entity that involves fibrotic tissue encasing abdominal organs including the ureters. Hydronephrosis without any obvious causative lesion on abdominal ultrasound is a classic finding that was seen in this patient.
It can be primary or secondary in nature. Primary disease includes IgG4 disease. Secondary causes include: Drugs, Biologics, Malignancy, Infections, Autoimmune disease, Radiation, Retroperitoneal hemorrhage, and Surgery