1. 報告者： fellow 1 陳筱惠

3.  Name: 游 O 琴  Sex: female  Age: 56-year-old  Occupation: 餐飲業  Chart number: 8970369  Date of admission: 2011/09/25

4.  Right flank pain and black urine for 1 week

5.  Small kidney with kidney stones was told at 亞東 hospital 2~3 years ago. She received URS + SM then.  Right flank/low abdominal pain and black urine for 1 week; associated symptoms: dysuria, frequency, and urgency; no fever or hematuriablack urine  LMD visit twice, but no improvement under analgesic + oral antibiotic  At ER, foley was inserted for urine retention

6.  Small kidney with kidney stones was told at 亞東 hospital 2~3 years ago. She received URS + SM then.  Urinary tract infection or chronic kidney diseases: denied  No hypertension, diabetes mellutis, heart, liver, or other significant systemic diseases  Current medicine: nil

7.  Allergy: no known allergy  Alcohol: denied; betel-nut: denied; cigarette: denied  Over-the-counter medication or chinese herb: nil

8.  No family history of diabetes mellutis, malignancy, bleeding diathesis, heart, liver, kidney, or hereditary diseases

9.  Vital signs: blood pressure: 142/81mmHg; temperature: 36‘C; pulse rate: 90/min; respiratory rate: 17/min  General apperance: acute ill looking  Eye: conjunctiva: not pale, sclera: no icteric  Neck: supple, no lymphadenopathy or jugular vein engorgement  Chest: symmetric expansion breathing sound: bilateral clear heart sound: regular heart beats, no S3 or S4, no murmurs  Abdomen: soft, flat, diffuse tenderness, no muscle guarding or rebounding liver/spleen: impalpable bowel sound: normoactive  Back: right flank knocking pain  Extremities: no lower limb pitting edema  Skin: intact, no rash

10. WBC8.7x1000/ul Hgb13.2 g/dl Hct38.2 % MCV89.3 fl PLT442 x1000/uL Segment43 % Band21 % Urea N17.6 mg/dl Creatinine0.75 mg/dl GPT53 IU/L NA138 mEq/L K3.8 mEq/L Sugar127 mg/dl

11. ColorRed TurbidityTurbid SP. Gravity1.016 PH6.5 Leukocyte1+ Nitrite- Protein1+ Glucose- Ketone1+ Urobilinogen0.1 Bilirulin- Blood3+ bacteria+ RBC78/uL WBC129/uL Epithelial cell0/uL  9/24 urine culture: Viridans streptococcus (> 100,000)  9/25 blood culture: negative

15.  Left kidney Length: 11.2 cm  Mild dilatation of the pelvocalcyeal systems  A peri-pelvic echo-free lesion (2.0cm) in the lower pole  Right kidney Length: 14.4 cm  Irregular in contour, increased cortical echogenicity and decreased thickness  Severe dilatation of the pelvocalcyeal systems and ureter; multiple tiny hyperechoic lesions without acoustic shadow kidney and soft tissue-like density

16.  Bladder: distended  foley within it.  A protruding mass (4.9x2.7cm) with connection of a peri-bladder lumen near right vesicle-ureter junction, a iso-echoic lesion (1.2cm)

20.  Right hydronpehrosis and hydroureter, due to ureterocele; complicated with infection and probably pyonephrosis and pyoyreter  Multiple tiny stones inside  Left minimal hydronephrosis  Urinary bladder mucosal thickening and enhancement, suggesting chronic cystitis

21.  Infected purulent urine in an obstructed collecting system  S/S: typically associated with fever, chills, and flank pain, although may be asymptomatic, too  Etiologies:  Ascending infection of the urinary tract  Hematogenous spread of a bacterial pathogen

22.  Incidence: relatively uncommon  The risk of pyonephrosis is increased in patients with upper urinary tract obstruction secondary to various causes (eg, stones, tumors, ureteropelvic junction [UPJ] obstruction  Pathogen:  Escherichia coli, Enterococcus species, Candida species, Enterobacter species, Acteroides species, Staphylococcus species, Salmonella species, Tuberculosis

23.  Complications:  Sepsis and septic shock  Irreversible damage to the kidneys  Treatment: surgical emergency for decompression  Disadvantages of retrograde decompression: ▪ General anesthesia, contraindicated in unstable patients ▪ Smaller-caliber urinary drainage catheter than with percutaneous access

24. ▪ Increased irritative urinary symptoms ▪ Lack of antegrade access for radiologic studies or inability to administer medications such as antibiotics via nephrostomy tube ▪ Bypassing the obstruction may not be possible in some patients. ▪ Pyelovenous, pyelolymphatic, and pyelosinus backflow of infected urine into the systemic circulatory system

25.  Ultrasonographic features of pyonephrosis:  Dilated collecting system  Echogenic debris in the in dependent areas of collecting system ▪ Strong echoes with acoustic shadowing ▪ Change position when patient moves  Air can be seen in these infections. Ultrasonographic Evaluation of Renal Infections Radiol Clin N Am 44 (2006) 763–775

27.  CT: depicts both hydronephrosis and often the underlying cause  Contrast-enhanced imaging is more desirable as in infection parenchymal and functional changes can be assessed. ▪ Pelvic and ureteral wall thickness ▪ Renal enlargement ▪ Perinephric fat stranding ▪ Fluid–fluid levels and gas within the collecting system Imaging of urinary tract infection in the adult Eur Radiol (2004) 14:E168–E183

29.  Name: 徐 O 華  Sex: female  Age: 63-year-old  Occupation: nil  Chart number: 6425429  Date of admission: 2011/09/05

30.  Low abdominal pain for 4 days

31.  Underlying diseases: rheumatoid arthritis, diabetes mellitus, and history of infectious spondylitis with left anterior epidural abscess post operation in 2011/03 (stool/urine incontinence under foley use and bedridden status since then)  Turbid urine, suprapubic and right flank pain for 4 days; associated symptoms: poor appetite, nausea/vomiting; no fever

32.  Underlying diseases:  Rheumatoid arthritis  Hypertension  Diabetes mellitus  Osteoporosis  Iatrogenic adrenal insufficiency  History of infectious spondylitis with left anterior epidural abscess post operation operation at 802 hospital in 2011/03  No heart, liver, or other significant systemic diseases  Current medicine: from our Rheuma OPD

33.  Allergy: no known allergy  Alcohol: denied; betel-nut: denied; cigarette: denied  Over-the-counter medication or chinese herb: nil

34.  No family history of diabetes mellutis, malignancy, bleeding diathesis, heart, liver, kidney, or hereditary diseases

35.  Vital signs: blood pressure: 124/92mmHg; temperature: 36.6‘C; pulse rate: 110/min; respiratory rate: 18/min  General apperance: acute ill looking  Eye: conjunctiva: mild pale, sclera: no icteric  Neck: supple, no lymphadenopathy or jugular vein engorgement  Chest: symmetric expansion breathing sound: bilateral clear heart sound: regular heart beats, no S3 or S4, no murmurs  Abdomen: soft, flat, low abdominal tenderness, no muscle guarding or rebounding liver/spleen: impalpable bowel sound: normoactive  Back: right flank knocking pain  Extremities: no lower limb pitting edema  Skin: intact, no rash

36. WBC7.2x1000/ul Hgb9.6 g/dl Hct29.2 % MCV92.7 fl PLT258 x1000/uL Segment79.8 % Creatinine0.58 mg/dl GPT8 IU/L NA136 mEq/L K3.9 mEq/L Sugar104 mg/dl Lactate9.1 mg/dl

37. ColorYellow TurbidityCloudy SP. Gravity1.010 PH8.5 Leukocyte3+ Nitrite+ ProteinTrace Glucose- Ketone- Urobilinogen0.1 Bilirulin- Blood2+ bacteria+ RBC10/uL WBC65/uL Epithelial cell5/uL  9/3 urine culture: Proteus mirabilis (>100,000)  9/3 blood culture: negative

40.  Left Kidney Length: 10.2 cm  One isoechoic band extending from the cortex to central sinus  Right Kidney Length: 10.4 cm  One mass-like lesion (7.0x3.5cm) over middle portion  The both kidneys are normal in size and contour. The cortical echogenicity and thickness are normal.  No evidence of renal stone or cyst exists.

43.  Multifocal ill-defined low denity of bilateral renal parenchyma, C/W acute pylonepheritis  Dilatation of bilateral renal pelvis and ureters to right middle ureter and left upper ureter level  No definite dilatation of bilateral renal calyces  No definite ureteral stones or tumor could be identified.  DDx: extrarenal pelvis, retroperitoneal fibrosis/ adhesion, or ureteral stricture

45.  Ureteral catheter passing up to the left upper ureter at L4 level and right middle ureter at S3 level  Mild bilateral hydronephrosis.  No obvious filling defect in the collecting system. The right upper ureter and right renal collecting system are not well opacified.  No definite radiopaque stone in the urinary tract

46.  The presence of extrarenal calyces is a very rare anomaly of the upper urinary tract.  First described in 1925  The total number of cases reported so far is only 20.  Kidney with extrarenal calyces is usually associated with other anomalies like bifid kidney, renal ectopia, horseshoe kidney and renal dysplasia. Extrarenal calyces: A rare anomaly of the renal collecting system Indian J Pathol Microbiol. 2009 Jul-Sep;52(3):368-9.

47.  The calyces were long and extrarenal in position. They drained into a cystic structure which represented either a grossly dilated pelvis (pelviureteric junction) or a ureteral cyst.  The exact cause of extrarenal calyces is not very clear.  Hypothesis: a disparity resulting from slow development of the metanephric tissue or to a relatively rapid development of the ureteric bud

48.  Many cases of collecting system anomalies including extrarenal calyces are detected incidentally or may be diagnosed because of its complications.  Excretory urography often provides good anatomic information.  A false impression of hydronephrosis or chronic pyelonephritis

49.  Rare disease, incidence of idiopathic form about 0.1~1.3 per 100,000 person-years  Etiology:  Idiopathic form: 70%, 40 ~ 60 years of age, 2 to 3:1 male-to-female predominance  Secondary form ▪ Drugs: ergot-derivatives, methysergide, bromocriptine, beta blockers, methyldopa, hydralazine, analgesics ▪ Malignancy: carcinoid, Hodgkin's and non-Hodgkin lymphoma, sarcomas

50.  Infections: tuberculosis, histoplasmosis, actinomycosis  Radiation therapy for testicular seminoma, colon, pancreatic cancer  Surgery: lymphadenectomy, colectomy, aortic aneurysmectomy

51.  Pathology:  Macroscopically: a hard, white plaque of varying thickness ▪ Typically, around the abdominal aorta and iliac vessels, as well as the inferior vena cava and the ureters  Microscopically: sclerosis and infiltration of mononuclear cells in varying proportions, depending on the stage of disease

52.  Pathogenesis: chronic inflammation, fibroblast proliferation, and excessive extracellular matrix deposition  Clinical manifestations:  Early stage: pain in the lower back, flank or abdomen; characteristically dull, noncolicky, in a girdle distribution (> 90%) ▪ Other nonspecific symptoms: weight loss, malaise, anorexia, testicular pain, claudication, edema, and gross hematuria  Late stage: vessel compromise and characterized ureteral obstruction

53.  Laboratory abnormalities:  Elevated ESR and CRP  Positive ANA (60%)  Anemia, possibly related to renal insufficiency or chronic inflammation  No hematologic or biochemical abnormalities diagnostic of this condition.  The urinary sediment is most often normal.

54.  Ultrasonography: a poorly marginated, periaortic mass that is typically echo-free or hypoechoic and may be associated with hydronephrosis  CT scan:  The mass is confluent, encasing the anterior and lateral sides of the aorta and often encircling the inferior vena cava.  Similar attenuation numbers to that of muscle

55.  Magnetic resonance imaging: comparable to those with CT scanning  Intravenous urography: proximal hydroureteronephrosis, medial deviation of the ureters, and extrinsic compression of the ureters  Retrograde or percutaneous antegrade pyelography: a smooth tapering of the ureters that is most pronounced at the level of the pelvic brim

56.  Biopsy: no guidelines  The location of the mass is atypical  Clinical, laboratory or radiologic findings suggest the presence of an underlying malignancy or infection  Local experience is limited  The patient does not respond to initial therapy. UpToDate