Tuesday urology conference

2/12/2014
By Dr. Anas Hindawi
MGH PGY 3 Urology Resident

45 y.o married female

Admitted for :

Bilat. flank pain

Palpable abdominal masses
Fever and chills recurrent despite IV antibiotics /one
month ago/

PMH :

Asthma
HTN
autotsomal dominan polycystic kidney disease / Dx since 3
months /
on haemodilysis /3* per week/ with baseline Cr 3

PSH :

Lt AV fisulta
Adenoidectomy

 Vitals

T: 38 ,P 90 ,BP 150/90

P.E :

Chest : bilat. basal crackles

Abdomen : soft ,enlarged
Palpable bilat. Mildly tender masses
Bilateral CVA tenderness /more on the left/
DRE normal
Lower extremeties +2 pitting oedema

Labs :

Bun 41 ,Cr 3 ,electrolytes 140 ,3.8 ,104 ,21

Hg/Hct 11.5/35.6 ,MCV 82
U/A turbid amber ,+1 LE ,6-8 rbc ,numerous wbc
U/Cx Ecoli /ESBL/ > 100.000 CFU ,Sensitive on Amikacin & Imipinem

Next ?

Next ?

Next ?
IV Antibiotics
percutaneous drainage /unroofing/
partial nephrectomy
simple unilateral nephrectomy

radical unilateral nephrectomy

bilateral nephrectomy

ADULT POLYCYSTIC KIDNEY

DISEASE

Adult polycystic kidney disease is an autosomal dominant hereditary

condition /bilateral in 95% /

5% to 10% of ADPKD cases are due to spontaneous mutations without a

positive family history

ADPKD 3rd leading cause for ESRD

Accounting for 5% to 10% of all dialysis cases

Affecting chromosomes 16 and 4

Do not appear until after age 40 ,no preference of sex or race

May be associated with cysts in other organs

Risk of renal cell carcinoma is the same of general population

Smith and Tanagho's General Urology, 18th Edition

Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross sectional Imaging Findings and Management
AUA update series 2013 ,volume 32 ,lesson 2

Pathogenesis
It is now established that hereditary renal cystic
diseases, so-called ciliopathies, are secondary to
genetic abnormalities that influence the formation and
function of primary cilia of the renal tubular epithelial
cells resulting in inappropriate epithelial proliferation and
subsequent development of renal cysts.

Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross sectional Imaging Findings and
Management
AUA update series 2013 ,volume 32 ,lesson 2

Clinical findings
Symptoms :
Pain over one or both kidneys
Gross or microscopic total hematuria
Colic may occur if blood clots or stones are
passed
Abdominal mass
Infection
Vesical irretability
Smith and Tanagho's General Urology, 18th Edition

Clinical findings
Signs :

Palpable kidney/s with or without nodularity

Tenderness justified by infection
HTN in 60-70 %
Cardiomegaly
Fever with pyelonephritis or infected cyst
Uremia ,anemia and weight loss

Laboratory Findings
hematopoietic depression accompanying uremia ,proteinuria and
microscopic (if not gross) hematuria with developping renal
impairment

Smith and Tanagho's General Urology, 18th Edition

Clinical findings
X-Ray Findings

Enlarged on a plain film of the abdomen, even as much as five times normal
The calyces are broadened and flattened, enlarged, and often curved, as they
tend to hug the periphery of adjacent cysts.

Ultrasonography

1s method of investigation non expensive ,safe and effective

CT scanning

ADPKD is characterized by bilateral enlarged kidneys with innumerable cysts of

various sizes ,in addition complications related to ADPKD, such as cyst infection,
hemorrhage or rarely cyst rupture
Extrarenal manifestations of ADPKD including hepatic, pancreatic, seminal
vesicle and splenic cysts; intracranial arterial aneurysms; aortic aneurysms;
abdominal wall hernias; colonic diverticulosis; and aortic/mitral valve abnormalities
can be diagnosed

Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross

sectional Imaging Findings and Management
AUA update series 2013 ,volume 32 ,lesson 2
Smith and Tanagho's General Urology, 18th Edition

Complications of ADPKD

Refractory HTN
Chronic pain
Stones
Tumour
Cysts rupture
Pyelonephritis /most common complication/
Intracranial Berry aneurysms rupture

Campbell walsh urology 10th edition

General mesaures
the new drugs target fluid secretion, whereas
others target cellular growth and proliferation of
the cysts
ACEI's & ARB's
NSAID's has to be avoided
Narcotics has to be limited
Campbell walsh urology 10th edition

Surgical managment of ADPKD

There is no evidence that excision or decompression of cysts
improves renal function
In patients infected with gas forming organisms
Recurrent, recent or refractory infections who are about to undergo
renal transplantation ,In this setting, nephrectomy will minimize the
risk of post-transplant infection when immunosuppressive agents
are administered to prevent rejection .
Patients with a staghorn calculus causing recurrent UTIs in a
relatively nonfunctioning kidney.
Hemodialysis and ultimately renal transplantation is the treatment of
choice for patients with ADPKD and renal failure

Uptodate :Urinary tract infection in autosomal dominant polycystic

kidney disease ,literature review by Mar 2013

Pyelonephritis & ADPKD

30 % to 50 % of ADPKD
Distinguishing between infections of the bladder, renal parenchyma, and cysts
is important
Often leads to serious complications, including perinephric abscess,
septicemia, and death.
Predisposing factors include age, female sex, and recent instrumentation of
the urinary tract.
Most commonly caused by gram-negative enteric organisms.
Diagnosis of these infections may be difficult since some patients do not have
bacteriuria.

Eradication of cyst infections with conventional antibiotic therapy can be difficult

despite in vitro sensitivity of responsible organisms to the agents administered.

Where fever persists beyond 1-2 weeks of appropriate antibiotic therapy,

percutaneous or surgical drainage of infected cysts should be considered.
Where there is end-stage disease, nephrectomy may be indicated

American Journal of Kidney Diseases ,Volume 10, Issue 2, August 1987, Pages 8188
Polycystic Kidney Disease Treatment & Managemen ,Author: Roser Torra, MD, PhD; Chief
Editor: Vecihi Batuman, MD, FACP, FASN

References

Campbell walsh urology 10th edition

Smith and Tanagho's General Urology, 18th Edition

Urology Board review 4th edition

Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross sectional Imaging Findings
and Management ,AUA update series 2013 ,volume 32 ,lesson 2

Uptodate :Urinary tract infection in autosomal dominant polycystic kidney disease ,literature
review by Mar 2013

American Journal of Kidney Diseases ,Volume 10, Issue 2, August 1987, Pages 81
88

Pubmed : Polycystic Kidney Disease Treatment & Managemen ,Author: Roser Torra,
MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN

Thank you