Bladder and urethral congenital disorders Notes

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This Osmosis High-Yield Note provides an overview of Bladder and urethral congenital disorders essentials. All Osmosis Notes are clearly laid-out and contain striking images, tables, and diagrams to help visual learners understand complex topics quickly and efficiently. Find more information about Bladder and urethral congenital disorders by visiting the associated Learn Page.
NOTES NOTES BLADDER & URETHRAL CONGENITAL DISORDERS GENERALLY, WHAT ARE THEY? PATHOLOGY & CAUSES ▪ Congenital abnormalities in bladder, urethra ▪ Benign/kidney failure/systemic involvement CAUSES ▪ Interferences in fetal development SIGNS & SYMPTOMS DIAGNOSIS DIAGNOSTIC IMAGING Prenatal ultrasound ▪ Diagnosis difficult OTHER DIAGNOSTICS ▪ Bladder exstrophy, hypospadias, epispadias: visible at birth TREATMENT ▪ May be asymptomatic until complications develop SURGERY ▪ See individual disorders BLADDER EXSTROPHY osms.it/bladder-exstrophy the mesenchymal tissue towards midline → rupture of cloacal membrane → herniation of lower abdominal components through the lower abdominal wall surface PATHOLOGY & CAUSES ▪ Congenital disorder, inside-out bladder protruding out of abdomen ▪ Part of the exstrophy-epispadias complex (EEC) that includes epispadias and cloacal exstrophy ▪ Bladder fails to fully form anteriorly, pushed through front anterior abdomen wall CAUSES ▪ Occurs during embryological development: overdevelopment of cloacal membrane disrupts development of the lower abdominal wall → prevents migration of RISK FACTORS ▪ ▪ ▪ ▪ Genetic predisposition Biological males > biological females Firstborn > subsequent births Infants born to white parents COMPLICATIONS ▪ Urinary and/or fecal incontinence, UTIs, abnormal gait, hip dysplasia, rectal prolapse; inguinal hernia, uterine prolapse OSMOSIS.ORG 769
SIGNS & SYMPTOMS ▪ Observable changes in pelvis, pelvic floor, genitalia ▫ Exposed bladder and urethra ▫ Low-set umbilicus ▫ Abnormalities of the pelvic bone, vertebral column, and spinal cord ▫ Flattened puborectal sling, anus anteriorly displaced ▫ Biological males: epispadias, absent dorsal foreskin, open prostate, shortened penis ▫ Biological females: vagina wider, shorter, more vertically oriented; displaced, narrowed vaginal orifice; bifid clitoris; divergent labia ▫ Epispadias DIAGNOSIS DIAGNOSTIC IMAGING Figure 107.1 A newborn baby with a severe case of bladder exstrophy. The genitals are also grossly irregular. TREATMENT SURGERY ▪ Performed within first weeks of life ▪ Staged surgeries required over months/ years CT scan ▪ CT scan detects skeletal abnormalities Ultrasound and MRI ▪ Often made by prenatal ultrasound, can be confirmed by MRI OTHER DIAGNOSTICS ▪ Clinically recognizable at time of delivery Figure 107.2 Illustration of the bladder pushing through the symphysis pubis and abdominal wall during bladder exstrophy. 770 OSMOSIS.ORG
Chapter 107 Bladder & Urethral Congenital Disorders Figure 107.3 A plain pelvic radiograph demonstrating a wide symphysis pubis in a case of bladder exstrophy. HYDRONEPHROSIS osms.it/hydronephrosis PATHOLOGY & CAUSES ▪ Dilation of renal pelvis, calyces associated with kidney atrophy ▪ Severe, long-standing hydronephrosis → kidney failure ▪ Urinary tract obstruction/compression → build up of urinary pressure → progressive dilation ▪ Dilation starts at blockage, continues up towards kidneys ▫ Hydroureter: dilation of ureter ▫ Hydronephrosis/hydroureteronephrosis: dilation of ureter, renal pelvis, calyces ▪ Grading ▫ 0: no dilation ▫ I: dilation of renal pelvis ▫ II: dilation of renal pelvis, calyces ▫ III: moderate dilation of renal pelvis, calyces; mild cortical thinning, flattening of papillae ▫ IV: severe renal dilation; cortical thinning CAUSES ▪ Fetus: antenatal hydronephrosis ▫ Often unknown, may disappear on own ▫ Congenital malformation: ureteropelvic junction obstruction, vesicoureteral reflux ▪ Children: ▫ Congenital malformation: ureterocele, posterior urethral valves ▪ Adults: ▫ Acquired disease: kidney stones (most common cause), benign prostatic hyperplasia, blood clot, contiguous malignant diseases (prostate/bladder/cervix cancer, retroperitoneal lymphoma), contiguous inflammation (prostatitis, ureteritis, urethritis, retroperitoneal fibrosis), tissue scarring from injury/surgery, uterus enlargement during pregnancy OSMOSIS.ORG 771
MNEMONIC: SIP BaN Causes of acquired hydronephrosis Stones Inflammation Prostate hypertrophy Baby (pregnancy) / Blood clot Neoplasm SIGNS & SYMPTOMS TREATMENT SURGERY ▪ Restore urine flow: upper blockage ▫ Acute: nephrostomy tube ▫ Chronic: ureteric stent/pyeloplasty OTHER INTERVENTIONS ▪ Restore urine flow: lower blockage ▫ Urinary or suprapubic catheter ▪ Acute with sudden onset: intense pain in flank, called Dietl’s crisis ▪ Nausea, vomiting DIAGNOSIS DIAGNOSTIC IMAGING Abdominal ultrasound ▪ Dilation of renal calyces ▪ Increased anteroposterior diameter ▪ Dilated ureter, if obstruction is distal Prenatal ultrasound ▪ Oligohydramnios if bilateral obstruction Intravenous (IV) urography/pyelography ▪ Demonstrates distal obstruction 772 OSMOSIS.ORG Figure 107.4 An abdominal CT scan in the coronal plane demonstrating severe hydronephrosis of the left kidney.
Chapter 107 Bladder & Urethral Congenital Disorders POSTERIOR URETHRAL VALVE osms.it/posterior-urethral-valve PATHOLOGY & CAUSES ▪ Congenital disorder, posterior urethra obstructed by membranous folds/tissue flap ▪ Most common cause of bladder outlet obstruction in infants who are biologically male ▪ Obstruction increases bladder pressure → bladder wall hypertrophy → decreases bladder compliance → repeats ▪ Obstruction increases bladder pressure → ureterovesical junction dysfunction → vesicoureteral reflux ▪ Urine retention by obstruction → urine backs up → bilateral hydronephrosis ▪ Severe obstructions in utero → oligohydramnios → Potter syndrome ▫ Limb irregularities, facial anomalies, kidney failure, pulmonary hypoplasia Antenatal ultrasound ▪ Hydronephrosis (10% may be normal) ▪ Trabeculated and thick-walled bladder with elongation and dilation of posterior urethra ▪ Valve may be seen as echogenic line Voiding cystourethrogram (VCUG) ▪ Dilation and elongation of posterior urethra ▪ Vesicoureteral reflux (in half of instances) ▪ Bladder trabeculation or diverticula ▪ Radiolucent linear band (representing valve) TREATMENT SURGERY ▪ Surgical ablation of membrane ▪ Prenatal surgery CAUSES ▪ Unknown; theory: abnormal integration of Wolffian duct → large plicae colliculi fuse anteriorly SIGNS & SYMPTOMS ▪ Posterior urethra obstructed by membranous folds/tissue flap DIAGNOSIS DIAGNOSTIC IMAGING Prenatal ultrasound ▪ Generally seen > 26 weeks gestation ▪ Noticeable distension and hypertrophy of bladder ▪ Possitlbe hydronephrosis and hydroureter ▪ Keyhole sign: distended proximal urethra and thick-walled bladder, resembles keyhole Figure 107.5 A lateral view of a micturating cystourethrogram demonstrating a proximally dilated urethra in case of posterior urethral valve. OSMOSIS.ORG 773
VESICOURETERAL REFLUX osms.it/vesicoureteral-reflux PATHOLOGY & CAUSES ▪ Retrograde flow of urine from the bladder into the ureters and kidneys ▪ Grading ▫ Grade I: urine goes into ureters ▫ Grade II: urine fills entire ureter, renal pelvis ▫ Grade III: urine fills, stretches ureter, renal pelvis ▫ Grade IV: ureter swollen, curvy; renal pelvis, calyces swollen, distorted ▫ Grade V: urine fills ureter, pelvis, calyces; swell completely ▪ Primary vesicoureteral reflux (most common type): due to congenital defect at ureterovesical junction (congenital absence/ shortening of intravesical portion of ureter) ▫ Inadequate closure of the ureterovesical junction → urine builds up in bladder → ureter fails to act as valve → urine returns to ureters ▪ Secondary vesicoureteral reflux: due to failure of the ureterovesical junction to close during bladder contraction; often due to a blockage in urinary tract ▫ Pressure increases in urinary tract → urine follows path of least resistance, back into ureters RISK FACTORS ▪ ▪ ▪ ▪ Genetic predisposition Neonates: prenatal hydronephrosis Children: febrile UTIs Individuals of white, Northern European descent COMPLICATIONS ▪ Recurrent UTIs, pyelonephritis, renal scarring/fibrosis, hypertension, kidney failure ▪ Infants: asymptomatic, fever, lethargy, poor appetite 774 OSMOSIS.ORG ▪ Children: discomfort with urination; bowel and bladder dysfunction SIGNS & SYMPTOMS ▪ Infants: asymptomatic, fever, lethargy, poor apetite ▪ Children: discomfort with urination DIAGNOSIS DIAGNOSTIC IMAGING Abdominal ultrasound ▪ Assesses renal parenchyma for scarring or anatomical abnormalities ▪ Presence of hydronephrosis VCUG ▪ Should be performed on first UTI in child < six years old ▪ Used for grading ▪ Presence of other anatomical abnormalities TREATMENT SURGERY ▪ Primary vesicoureteral reflux ▫ Surgery to repair valve at ureterovesical junction ▫ Infants, children: no intervention; child grows → ureters lengthens → valve function improves ▪ Secondary vesicoureteral reflux ▫ Surgery to remove blockage
Chapter 107 Bladder & Urethral Congenital Disorders Figure 107.6 A voiding cystourethrogram demonstrating bilateral vesicoureteric reflux. OSMOSIS.ORG 775

Osmosis High-Yield Notes

This Osmosis High-Yield Note provides an overview of Bladder and urethral congenital disorders essentials. All Osmosis Notes are clearly laid-out and contain striking images, tables, and diagrams to help visual learners understand complex topics quickly and efficiently. Find more information about Bladder and urethral congenital disorders by visiting the associated Learn Page.