prenatal and postnatal assessment of posterior urethral...

40
Prenatal and Postnatal Assessment of Posterior Urethral Valve Katie Rose Clapham, HMS Year III Gillian Lieberman, MD July 19, 2010

Upload: lamnguyet

Post on 13-Mar-2018

223 views

Category:

Documents


6 download

TRANSCRIPT

Page 1: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Prenatal and Postnatal Assessment of Posterior

Urethral ValveKatie Rose Clapham, HMS Year III

Gillian Lieberman, MDJuly 19, 2010

Page 2: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Outline

• Normal kidney and urinary tract development• Normal fetal kidney and urinary tract on

ultrasound • Types of kidney and urinary tract malformations• Case of posterior urethral valve

- prenatal evaluation- postnatal evaluation

• Interventions and long-term outcome

Page 3: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Development of the Kidney

Image from UpToDate. Overview of CAKUT. Waters, A. and Rosenblum, N. http://www.uptodate.com/online/content/topic.do ?topicKey=pedineph/18797&selectedTitle=1%7 E150&source=search_result. 7/15/10.

Part of kidney

Appears Functional Gives rise to

Pronephros 4th week Never functional

Pronephric ducts used by mesonephros

Mesonephros 4th week Functions for 4 weeks

Contributes to male genital structures and bladder

Metanephros 5th week Starts functioning at 9th week

UG bud gives rise to ureters and collecting ductsMetanephric blastema gives rise to the rest of the kidney

Urogenital (UG) Sinus

Bladder and urethra

Page 4: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Development of Urethra• Mesonephric (Wolffian) duct fuses with UG sinus and

contributes to urethra• Caudal UG sinus (genital tubercle) forms phallic urethra• Urethra formation is complete by 14 weeks

Image from Lawrentschuk N., and Frydenberg M. Benign Prostate Disorders. Endotext. http://www.endotext.org/male/male9/male9.html. Accessed [7/18/10]

Page 5: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Outline

• Normal kidney and urinary tract development• Normal fetal kidney and urinary tract on

ultrasound • Types of kidney and urinary tract malformations• Case of posterior urethral valve

- prenatal evaluation- postnatal evaluation

• Interventions and long-term outcome

Page 6: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Normal Fetal Kidney on Ultrasound

•Cannot reliably see fetal kidney on ultrasound during 1st trimester.

•Visualized by 16-22 weeks.

•Can visualize cortex and pyramids by 23-26 weeks, and fine anatomy by 30 weeks.

•Fetal kidney length in mm is roughly equal to the fetal menstrual age in weeks.

Page 7: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Normal Fetal Bladder on Ultrasound (18 wks)

Bladder

PACS, BIDMC

*

Transverse View

Page 8: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Normal Fetal Kidney on Ultrasound (18 wks)

SpineKidneys

PACS, BIDMC

*

*

Transverse View

Page 9: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Ultrasound Evaluation of Kidney Function

• Fetal kidney function is assessed by-visualization of urine in the bladder by 13- 16 weeks (the ureters are not normally seen)- measurement of amniotic fluid (subjective measures appear to be as good as summing the depth of amniotic fluid pockets in four quadrants)

Page 10: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Outline• Normal kidney and urinary tract development• Normal fetal kidney and urinary tract on

ultrasound • Types of kidney and urinary tract

malformations• Case of posterior urethral valve

- prenatal evaluation- postnatal evaluation

• Interventions and long-term outcome

Page 11: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Types of Kidney Malformations: Parenchymal

•Hypoplasia

•Dysplasia

•Renal agenesisGross Hypoplastic Kidney

Massey, H.D. Lab I.j Congenital Malformations. Renal Pathology for Medical II Students. http://www.pathology.vcu.edu/education/renal/lab1.j.ht ml. Accessed [7/15/10].

Multicystic Kidneyon Ultrasound

PACS, BIDMC

•Multicystic kidney

•Genetic cystic disease (ARPKD, ADPKD, NPH)

Page 12: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Types of Kidney Malformations: Migration

• Ectopia• Fusion

Crossed Fused Ectopic Kidneyon CT with contrast

PACS, BIDMC

Horseshoe Kidney on IVUIrshad, A, Ackerman, S., Ravenel, J.G. Horseshoe Kidney. http://emedicine.medscape.com/article/378396-overview. Accessed [7/15/10].

Page 13: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Types of Kidney Malformations: Collecting System

• Pelvis: UPJ obstruction• Ureter: megaureter, ectopic ureter, VUR• Bladder: exstrophy• Urethra: posterior urethral valve

(PUV)

Ectopic ureter on CTPACS, BIDMC

Page 14: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Outline

• Normal kidney and urinary tract development• Normal fetal kidney and urinary tract on

ultrasound • Types of kidney and urinary tract malformations• Posterior urethral valve

- prenatal evaluation- postnatal evaluation

• Interventions and long-term outcome

Page 15: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 1: Posterior Urethral Valve

• Congenital defect• Affects males• The ‘valve’ is a membranous fold between

urethral wall and verumontanum (crest where seminal vesicles enter urethra) that obstructs the urethra

Page 16: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 1: Posterior Urethral Valve

Image from Belman, A., King, L.R., and Kramer, S.A. Clinical pediatric urology. 4th

edition. London, England: Martin Dunitz Ltd; 2002.

Dilated ureter

Hydronephrotic kidney

Dilated bladder

The valve may originate from incomplete canalization or an aberrant insertion of the

Wolffian duct into the cloaca

Site of membranous ‘valve’

Image from Lawrentschuk N., and Frydenberg M. Benign Prostate Disorders. Endotext. http://www.endotext.org/male/male9/male9.html. Accessed [7/18/10]

Prostate

Gross Appearance

Page 17: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Posterior Urethral Valve

Key Findings on Ultrasound

HydronephrosisDilated bladder with possible

wall thickening

Dilated posterior urethra

Page 18: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 2: Male Fetus on Ultrasound (18 wks)

PACS, BIDMC

GenitaliaLegs

Page 19: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 2: “Keyhole Sign”: Dilated Bladder and Prostatic Urethra on Ultrasound

PACS, BIDMC

Dilated bladder

Dilated prostatic urethra

**

Sagittal View

Page 20: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 2: Bladder Dilatation on Ultrasound (20 wks)

Bladder (20 wks) measures 4.14 by 2.04 cm on sagittal view

PACS, BIDMC

Normal bladder (18 wks) measures 0.44 cm on transverse view

Anechoic space demonstrates no flow on Doppler imaging, suggesting a dilated bladder rather than a vascular structure

Sagittal View Sagittal View

Transverse View

*

Page 21: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 2: Enlarged Hydronephrotic Kidneys on Ultrasound (20 wks)

Left kidney measures 3.42 cm

Right kidney measures 2.80 cm

PACS, BIDMC

SpineAmniotic

Fluid

Bladder

Normal kidney size: 1 mm/week x 20 weeks = 20

mm or 2 cm

Sagittal view

Sagittal view

*

*

Page 22: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 2: Dilated Renal Pelvis on Ultrasound (20 wks)

Dilated Pelvis of Right Kidney (0.66 cm)

Dilated Pelvis of Left Kidney (0.53 cm)

PACS, BIDMC

At 20 wks: Pelvis <5 mm is normal but can be 6-9 mm and normal.

Here, pelvis>5 mm plus other findings suggest obstruction.

Transverse view

Transverse view

*

*

Page 23: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 2: Amniotic Fluid Measurement on Ultrasound

Amniotic Fluid estimate: 14.23 mL at 20 weeks

5-25 mL is the normal range for most of pregnancyPACS, BIDMC

Page 24: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 3: Visible Ureters and Dilated Renal Pelvices on Ultrasound

Renal Pelvis

BladderUreter

Image courtesy of Dr. Tejas Mehta, BIDMC

*

*

*

Page 25: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Differential Diagnosis for Ultrasound Findings

• Mechanical obstruction – PUV– Urethral atresia– Caudal regression syndrome– Megacystis-microcolon-intestinal hypoperistalsis syndrome– Prune Belly Syndrome (hypotonic abdominal wall, megacystis,

ureterectasis, cryptorchidism). • Functional obstruction

– Abnormality in the sphincter, innervation, or musculature of the bladder

PUV is suggested by the keyhole sign, the absence of other associated findings, and the severity of obstruction

Diagnosis is confirmed postnatally

Page 26: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Accuracy of Ultrasound Evaluation

• In one study:- 6 fetuses had catheters placed for dilated

urinary tracts thought to be caused by PUV (5) or obstructed megaureter (1)

- 2 were found to have PUV postnatally

Page 27: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Prognostic Indicators

Patients with PUV often have chronic kidney disease, VUR, and bladder dysfunction.

Risk factors for poor renal function:

•Diagnosis of PUV before 24 weeks

•Oligohydramnios – when present in 2nd

trimester, mortality is 90-95%

•Serum creatinine >1.0 mg/dL

•Bladder dysfunction

Page 28: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Renal Outcome

It is unclear whether PUV and renal dysplasia are associated because there is:

- A common developmental injury- Damage caused by high back pressure- Recurrent infection causing scarring

Page 29: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Outline

• Normal kidney and urinary tract development• Normal fetal kidney and urinary tract on

ultrasound • Types of kidney and urinary tract malformations• Case of posterior urethral valve

- prenatal evaluation- postnatal evaluation

• Interventions and long-term outcome

Page 30: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Clinical Presentation

Neonates: respiratory distress, dilatated bladder, urinary ascites, difficulty urinating

Infants: Failure to thrive, urosepsis, difficulty urinating

Boys: UTIs, incontinence, difficulty urinating

Page 31: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Postnatal Evaluation: Menu of Tests

Voiding Cystourethrogram (VCUG) is the study of choice

• Contrast is injected via a catheter into the bladder • During micturition, the flow of urine is observed on

fluoroscopyUltrasound can be used to assess hydrouteronephrosis

and renal parenchymaIf VCUG is not diagnostic, cystourethroscopy can be usedMRI is a developing technology

-fast sequences or patient sedation required in pediatric population

Page 32: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 4: VCUG Demonstrates Obstruction and Unilateral Reflux Postnatally

1/3-1/2 of patients with PUV have reflux, which may be unilateral or bilateral

*

*

***

*

Dilated renal pelvisDilated and torturous ureterDilated bladderDilated prostatic urethraTrabeculationsDiverticulum

Children’s Hospital Boston

Page 33: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Unilateral or Bilateral Reflux

• In one retrospective study of 200 patients with PUV, 27% had unilateral vesicoureteral reflux on VCUG and 37% had bilateral reflux

• Unilateral reflux may be due to protective mechanisms increasing reflux in one kidney in order to preserve contralateral kidney function (vesicoureteral reflux and dysplasia syndrome)

Page 34: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Patient 4: Lateral VCUG shows Dilated Bladder and Prostatic Urethra

Dilated bladder with trabeculations and diverticula

Dilated prostatic urethraChildren’s Hospital Boston

**

Page 35: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Outline

• Normal kidney and urinary tract development• Normal fetal kidney and urinary tract on

ultrasound • Types of kidney and urinary tract malformations• Case of posterior urethral valve

- prenatal evaluation- postnatal evaluation

• Interventions and long-term outcome

Page 36: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

InterventionsPrenatalIn general, prenatal intervention is avoided because the benefits have not been shown to outweigh prenatal and maternal morbidity

Vesicoamniotic shuntOpen fetal surgery Cystoscopic ablation

Newborn Bladder drainage via feeding tube or catheterCystoscopic ablationVesicotomy if needed

Older boys Cystoscopic ablation

Page 37: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Cystoscopy

The cystoscope allows visualization of the

urinary tract

Surgical instruments may be passed

through the cystoscope

Cystoscopy. Mayo Clinic. http://www.mayoclinic.com/health/medical/IM00166/undefine d. Accessed [7/18/10]

Page 38: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Long-Term Outcome

• Perinatal mortality, caused by pulmonary hypoplasia and sepsis, has decreased to <10% due to better management

• Long term outcome depends on renal parenchymal function

• Renal transplant has been shown to be effective in a setting where the bladder permits transplant survival

Page 39: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

ReferencesBelman A., King L.R., and Kramer S.A. Clinical pediatric urology. 4th edition. London, England: Martin Dunitz Ltd; 2002. Cystoscopy. Mayo Clinic. http://www.mayoclinic.com/health/medical/IM00166/undefined. Accessed [7/18/10]DeFoor W., Clark C., Jackson E., Reddy P., Minevich E., and Sheldon C. Risk factors for end stage renal disease in children with posterior urethral

valves. J Urol. 2008;180(4 Suppl):1705-8; discussion 1708. Goldstein R.B., and Filly R.A. Sonographic estimation of amniotic fluid volume: subjective assessment versus pocket measurements. J Ultrasound

Med 1988; 7:363.Heikkilä J., Rintala, R., and Taskinen S.. Vesicoureteral reflux in conjunction with posterior urethral valves.

J Urol. 2009;182(4):1555-60. Holmes, N. Clinical presentation and diagnosis of posterior urethral valves. UpToDate.

http://www.uptodate.com/online/content/topic.do?topicKey=pediatri/2371&selectedTitle=1%7E150&source=search_result. Accessed [7/14/10].Holmes, N. Management of posterior urethral valves. UpToDate.

http://www.uptodate.com/online/content/topic.do?topicKey=pediatri/2087&selectedTitle=1%7E22&source=search_result. Accessed [7/14/2010].

Irshad A, Ackerman S., and Ravenel J.G. Horseshoe Kidney. eMedicine. http://emedicine.medscape.com/article/378396-overview. Accessed [7/13/10].

Lawrentschuk N., and Frydenberg M. Benign Prostate Disorders. Endotext. http://www.endotext.org/male/male9/male9.html. Accessed [7/18/10]Massey H.D. Lab I.j Congenital Malformations. Renal Pathology for Medical II Students. http://www.pathology.vcu.edu/education/renal/lab1.j.html

Accessed [7/15/10]. Moore K.L., and Persaud T.V.N. The Developing Human: Clinically Oriented Embryology. 8th edition. Saunders Elsevier Health Sciences, 2007. Romero R., Pilu G., Jeanty P., et al. Prenatal diagnosis of Congenital Anomalies. Norwalk: Appleton and Lange, 1998. Rumack C., Wilson S.R., and Charboneau J.W. Diagnostic Ultrasound. 1st edition. St. Louis, Missouri: Mosby-Year Book, Inc.; 1991. Rutherford S.E., Phelan J.P., Smith C.V., et al. The four-quadrant assessment of amniotic fluid volume: an adjunct to antepartum fetal heart rate

testing. Obstet Gynecol 1987; 70:353. Sarhan O., Zaccaria I., Macher M.A., Muller F, Vuillard E., Delezoide A.L., Sebag G., Oury J.F., Aigrain Y., and El-Ghoneimi A. Long-term outcome

of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation. J Urol. 2008;179(1):307- 12; discussion 312-3.

Sholder, A.J., Maizels, M., Depp, R., Firlit, C.F., Sabbagha, R., Deddish, R., Reedy, N. Caution in antenatal intervention. J Urol. 1988; 139(5):1026-9Waters, A. and Rosenblum, N. Evaluation of congenital anomalies of the kidney and urinary tract (CAKUT). UpToDate.

http://www.uptodate.com/online/content/topic.do?topicKey=pedineph/17792&selectedTitle=1%7E40&source=search_result. Accessed [7/11/10].

Waters, A. and Rosenblum, N. Overview of congenital anomalies of the kidney and urinary tract (CAKUT). UpToDate. http://www.uptodate.com/online/content/topic.do?topicKey=pedineph/18797&selectedTitle=1%7E150&source=search_result. Accessed [7/11/10].

Wiener, J.S., Gaca, A. M., Sekula, J. Posterior Urethral Valve. eMedicine. http://emedicine.medscape.com/article/412226-overview. Accessed [7.15.10].

Page 40: Prenatal and Postnatal Assessment of Posterior Urethral Valveeradiology.bidmc.harvard.edu/LearningLab/genito/clapha… ·  · 2011-07-25Prenatal and Postnatal Assessment of Posterior

Acknowledgments

• Dr. Gillian Lieberman• Dr. Mai-Lan Ho• Dr. Iva Petovska• Dr. Tejas Mehta• Maria Levantakis