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Congenital anomalies ppt Congenital anomalies ppt Presentation Transcript

  • Urology Department Undergraduate coursesCongenital Genitourinary anomalies
  • For our Lectures and Scientific resourcesvisit our web sites, Uroainshams.blogspot.com Uronotes2012.blogspot.com ©
  • Common congenital anomaliesCommon Renal anomalies Common ureteral & renal• Abnormal number: agenesis pelvis anomalies• Abnormal form or position: • UPJ obstruction. horseshoe kid. • Vesico-uretral reflux. • Duplication.Common Bladder anomalies • Uretrocele.• Bladder Extrophy. • Ectopic ureter. Common genitalCommon Urethral & penile anomaliesanomalies • Undescended testis.• Hypospadias. • Intersex (disorders of• Epispadias. sexual differentiation). View slide
  • Renal agenesisBilateral renal agenesis• both mesonephric ducts fail to develop.• Incompatible with life.Unilateral renal agenesis• the mesonephric duct fails to develop.• Usually there is absent ureter, trigone, kidney and (in boys) vas deferens. View slide
  • Horseshoe kidney• both metanephros are fused together.• both kidneys rotated & their lower poles are joined in the shape of a horseshoe.• As the fetus grows, the joined kidneys are held up by the inferior or superior mesenteric arteries at L3.
  • Pelviureteric junction obstructionObstruction of the junctionbetween the renal pelvis & ureter.Aetiology• aperistaltic segment of ureter due to absent muscles.or• crossing vessels over UPJ.
  • Pelviureteric junction obstruction- Presentation• may present at any time (before birth, in childhood, or in adulthood) by:• abdominal mass.• abdominal pain.• Haematuria after fairly minor abdominal trauma.
  • Pelviureteric junction obstruction- Evaluation• IVU shows delay in appearance of contrast and dilated renal pelvis and calices.• Renal scan shows differential renal function and confirms obstruction.
  • Pelviureteric junction obstruction- Treatment• Surgery is indicated for: 1. obstructive symptoms, 2. stone formation, 3. recurrent urinary infection, 4. progressive renal impairment.• Pyeloplasty is the treatment of choice• Nephrectomy is performed if the affected kidney is <10% of total renal function.
  • Pelviureteric junction obstruction• Minimally invasive alternative techniques: 1. antegrade endopyelotomy. 2. Laparoscopic pyeloplasty is becoming more used now.
  • Vesicoureteric junction reflux• Reflux can be defined as the retrograde flow of urine into upper urinary tract.• incidence of reflux is equal in both sexes.• Reflux can be classified into 5 grades according to severity.
  • Vesicoureteric junction reflux- evaluation• Micturating cystourethrography is the gold standard for diagnosis and evaluation of VUR grade.• Diuretic Renal scan (DMSA) is used to visualize scarring and quantify differential renal function.
  • Vesicoureteric junction reflux- Management• antibiotic prophylaxis is recommended for children with reflux of grades I-II.• Surgery (uretro-vesical reimplantation or endoscopic injection) is recommended in reflux of grades III-V and persistent reflux despite a trial of antibiotics.
  • Duplication of urinary system• Ureteral duplication is the most frequent anomaly of urinary tract• Female: male = 2 : 1• ureteral orifice of the upper renal segment drains inferiorly and medially to the orifice of the lower segment (Weigert –Myer law).
  • Duplication of urinary system• The orifice draining the upper segment is often obstructed.• The orifice of the lower segment generally refluxes.• Duplication is usually discovered on an IVU.• Management is according to segment affected and its function.
  • Ectopic ureter• An ectopic ureter is one that opens in some location other than the bladder.80% associated with duplicated system.20% associated with single system. Single system• Most common sites (in female): urethra, vestibule, and vagina• In female present as urinary incontinence.• Most common sites (in male): posterior urethra and seminal vesicles. duplex system
  • Uretrocele• A congenital cystic ballooning of the terminal submucosal ureter.• It is classified as simple or ectopic.• Simple (Orthotopic) Ureterocele: in trigone.• Ectopic Ureterocele: can obstruct bladder neck or even prolapse from female urethra.
  • Undescended testis• The incidence ranges from 3.4% to 5.8% in full term boys• It decreases to 0.8% in one year old boys.• Cause is unclear, but androgens may have an important role.
  • Undescended testis vs Ectopic• Undescended testis can be classified by its location: 1. upper scrotum, 2. superficial inguinal pouch, 3. inguinal canal, 4. abdomen.• In 80% of cases, the undescended testis will be palpable in the inguinal canal.
  • Undescended testis vs Ectopic• Ectopic testis (testis outside path of normal descent) may be: 1. penile, 2. femoral, 3. perineal, 4. retrovesical. 5. Superficial inguinal pouch.
  • Undescended testis- Complications• Patients with undescended testes have two major concerns: 1. increased incidence of testicular cancer: up to 10 times higher than in a normal testis 2. subfertility.• Spontaneous descent of undescended testis is rare after the age of one year.
  • Undescended testis-• Examination (esp, under anesthesia) is more accurate than imaging.• Laparoscopy is the investigation of choice for non-palpable testes to differentiate intra-abdominal from vanished from inguinal testis.• Imaging: Ultrasound, CT, MRI (inaccurate).• chromosomal and endocrine evaluation if bilateral non-palpable testes.
  • Undescended testis Patent internal ring Spermatic vessels VasLaparoscopy showing intra-abdominal testis
  • Undescended testis- Management• Orchidopexy if testis is felt in inguinal canal or below, it should be performed as soon as possible.• Orchidectomy If an atrophic intra- abdominal testis is detected especially after puberty, as the testis is incapable of spermatogenesis and the risk of malignancy.
  • Retractile testis• Commonly confused with undescended testis.• the testis can be delivered into bottom of scrotum.• low undescended testis will immediately pop back to its undescended position after being released.
  • Hypospadias• congenital condition results in underdevelopment of urethra.• affects 3 per 1000 male infants.• Consists of 3 anomalies:(1) Abnormal ventral opening of the urethralmeatus.(2) Ventral curvature of the penis (chordee).(3) Deficient prepuce ventrally.
  • Hypospadias- Evaluation- Site Of the meatus.- Circumcised or not.- Associated anomalies: meatal stenosis, or undecended testis.- Penile curvature.
  • Hypospadias- Examples
  • Hypospadias- Treatment• The child should be referred for urological assessment and surgical treatment.• The ideal age for surgery is 6–12 months.
  • Epispadias• Congenital condition in which the urethra opens on dorsal surface of penis..• Usually associated with bladder extrophy (ectopia vesicae).
  • Bladder Extrophy (Ectopia vesicae)• Failure of development of the lower abdominal wall.• Anomaly include defect in anterior abdominal wall, defect in anterior bladder wall and epispadias (dorsal penile opening).
  • Posterior Urethral Valves• Posterior urethral valves are congenital membranel like structures located in the distal prostatic urethra.• most common cause of BOO in male children.• Diagnosis is made on voiding cystourethrography (VCUG) & cystourethroscopy.
  • Posterior Urethral ValvesTreatment• Fulgration of posterior valves by endoscopy.• Urinary diversion.• Management of renal failure.
  • Thank You