![Cloacal Exstrophy
• Omphalocele, Exstrophy, Imperforate anus, Spinal abnormality [OEIS].
• Non visualization of the bladder, a low-lying umbilicus, lower
abdominal wall mass, kidney (number, location, and/or appearance)
and lumbosacral spine abnormalities.](https://image.slidesharecdn.com/oshibafetalurology-221028124756-85b5cdc3/85/oshiba-fetal-urology-pptx-25-320.jpg)
oshiba fetal urology.pptx
- 1. Prenatal and Postnatal Urology Ahmed oshiba, Assistant lecturer, Pediatric Surgery and Urology Department
- 3. Antenatal hydronephrosis • It is identified in 1-3 % of all pregnancies. • APD of the renal pelvis more than 5 mm. • Measurement of the APD has been used widely. • The APD can be affected by gestational age, hydration status of the mother, bladder hypertonicity, and degree of bladder distention. • The APD fails to describe pelvic configuration, calyceal dilation, and the laterality of findings.
- 4. Diagnostic Accuracy • The risk of VUR per degree of ANH is similar, implying that ANH is not an appropriate indicator of VUR. • Obstruction may be diagnosed prenatally in 88%.
- 8. Kidney • Number, location, size, duplication, renal parenchyma, pelvic dilation, calyceal dilation, urothelial thickening and cystic disease. • Echogenicity should be less than liver and spleen. • Increased echogenicity is indicated in renal disease. • Renal cystic disease: APPKD, MCDK.
- 10. Ureter, Bladder, Urethra • Ureteral dilation, bladder filling and emptying, bladder wall thickness, intravesical cystic structures, urinoma. • Snowman bladder, megalourethra (Prune Belly syndrome). • Keyhole sign (PUV). • Abnormal genitalia. • Bladder exstrophy, bladder not identified. • Perirenal urinoma, Urinary ascites indicate BOO. • BOO in female fetus, indicate cloacal anomalies.
- 13. Amniotic fluid • By 20 to 22 weeks the vast majority of amniotic fluid is fetal urine • Oligohydramnios identified after 18 to 20 weeks’ gestation may be a result of urinary tract obstruction or poor renal development.
- 14. Special Cases
- 15. UPJO • Only hydronephrosis. • No hydroureter. • Perinephric urinoma, urinary ascites… severe form of PUJO.
- 16. Ureterovesical Obstruction • Primary obstruction of the UVJ. • Ectopic ureter inserting into the bladder neck. • High-grade VUR. • VUR.. Not easily diagnosed prenatally.
- 17. Renal Cystic Disease • Multiple noncommunicating cysts, minimal or absent renal parenchyma, and the absence of a central large cyst are diagnostic of MCDK. • Bilaterally enlarged echogenic kidneys without renal cystic disease, particularly if associated with hepatobiliary dilatation or oligohydramnios, suggest ARPKD. • Moderately enlarged hyperechogenic kidneys with increased corticomedullary differentiation suggest ADPKD. • Congenital multilocular cystic nephroma (Cystic Wims Tumor).
- 19. Duplication anomalies and Ureterocele • Upper pole: ureterocele, ectopic. • Lower pole: VUR, UPJO.
- 22. PUV • Bilateral hydroureteronephrosis. • A thick-walled bladder with dilated posterior urethra (keyhole sign). • In more severe cases, dysplastic renal parenchymal changes with perinephric urinomas and urinary ascites.
- 24. Bladder Exstrophy • Non visualization of the fetal bladder. • A lower abdominal wall mass immediately inferior to a low-lying umbilicus. • Diminutive genitalia. • Absence of bladder filling. • Normal kidneys in orthotopic position, normal vertebrae and spinal cord. • Abnormal symphyseal diastasis. • Anteriorly displaced anus. • Termination of pregnancy ??????????
- 25. Cloacal Exstrophy • Omphalocele, Exstrophy, Imperforate anus, Spinal abnormality [OEIS]. • Non visualization of the bladder, a low-lying umbilicus, lower abdominal wall mass, kidney (number, location, and/or appearance) and lumbosacral spine abnormalities.
- 26. Cloacal Malformations • Considered in any female fetus with hydronephrosis and a large pelvic cystic mass. • Calcified meconium is an important sign. • Fetal ascites, multicystic pelvic mass, bilateral hydronephrosis, and oligohydramnios associated with meconium peritonitis. • If polyhydramnios, suspect OA and TOF.
- 28. Congenital Adrenal Hyperplasia • Genital ambiguity in females can be reduced or eliminated with prenatal dexamethasone treatment. • As early as gestational week 12 by profiling maternal urine for steroid metabolite excretion.
- 29. Megalourethra and Prune-Belly Syndrome Association
- 30. Myelomeningocele • α-fetoprotein (AFP). • Sonographic findings of myelomeningocele.
- 31. Renal Mass • Congenital Mesoblastic Nephroma (CMN): is the most common neonatal mass, described as a hypoechoic homogeneous or heterogeneous solid renal mass with an echogenic rim that is not often well defined, a Vascular ring sign, usually in the 3rd trimester. • Rhabdoid tumor of the kidney: large mass in the left renal area with concomitant massive polyhydramnios. • Beckwith-Wiedemann syndrome (BWS): macrosomia, polyhydramnios, omphalocele, macroglossia, hepatomegaly, and renal enlargement
- 32. Renal vein thrombosis • Renal enlargement, loss of corticomedullary differentiation, echogenic streaks, lack of definition of renal sinus echoes, and loss of venous flow in the affected kidney by Doppler imaging.
- 34. Adrenal mass • DD: neuroblastoma, adrenal hemorrhage, adrenal and cortical renal cysts, adrenal adenoma and carcinoma, subdiaphragmatic pulmonary sequestration, BWS, duplication of the renal system, Wilms tumors, CMN, and mesenteric and enteric duplication cysts. • Neuroblastoma is most often cystic, right sided, and identified in the third trimester.
- 36. Antenatal Management of Fetal Uropathies
- 37. • Normal fetal growth parameters. • Amniotic fluid volume. • Renal appearance (echogenicity, degree of hydronephrosis, cystic changes). • Extrarenal fluid collections.
- 38. Options 1. Medical treatment. 2. Termination of pregnancy (TOPFA). 3. Early induction of delivery. 4. Surgical intervention.
- 39. Why Fetal Interventions? • Fetal urine comprises more than 90% of amniotic fluid volume by the 16th week of gestation. • Oligohydramnios during the second trimester is often associated with pulmonary hypoplasia. • Prevent or delay ESRD.
- 45. Unilateral hydronephrosis • It is important to keep in mind that a postnatal ultrasound evaluation performed within the first 48 hours of life may not yet demonstrate hydronephrosis or may underestimate the degree of hydronephrosis secondary to physiologic oliguria in the newborn. • Infants with severe ANH should be placed on a prophylactic antibiotic (amoxicillin, 10 to 25 mg/kg/day) and undergo VCUG. • Diuretic renography.
- 46. Bilateral hydronephrosis • PUV, Bilateral VUR, Neurogenic bladder, Bilateral UPJO. • BOO , in male suspect PUV, in female suspect ectopic ureter in the bladder neck. • In PUV, bladder decompression and chemoprophylaxis (amoxicillin 10 to 25 mg/kg/day) started immediately. • Ultrasound and VCUG should be done immediately.
- 47. Solitary kidney • Infants born with solitary kidneys (renal agenesis), renal ectopia, or unilateral multicystic dysplasia should be evaluated postnatally by ultrasonography. • 30% have VUR, 11% UPJO, and 7% UVJO. • VCUG and DMSA.
- 50. Perineal mass in a female • Periurethral cyst : whitish, covered with epithelium, drainage. • Imperforate hymen. • Prolapsed ureterocele: eccentric, posterior, edematous, congested, necrotic, distended bladder. • Urethral prolapse: skin moisturizers, hot compresses. • Botryoid sarcomata of the vagina: pelvic abdominal mass.
- 51. Scrotal mass • Hydrocele. • Inguinal hernia. • Meconium in scrotum. • Seeding scrotal mass secondary to neuroblastoma, Wilms tumor or adrenal hemorrhage. • Perinatal torsion (extravaginal): unilateral, contralateral fixation (early or late ????) • Para testicular abscess: staph, salmonella, klebsiella. • Testis tumor: yolk sac, teratoma, alfa fetoprotein, radical orchiectomy.
- 52. Abdominal mass • Hydronephrosis. • Cystic renal disease. • Adrenal hemorrhage. • A dilated bladder. • Gastrointestinal duplications. • Tumors. • Ultrasonography ????
- 53. 1. Nonobstructive hydronephrosis 2. UPJO. 3. Unilateral cysts (MCDK). 4. Bilateral cysts (ARPKD/ ADPKD). 5. Midline cystic abdominal masses: a. Hydrometrocolpos. b. Ovarian cyst. c. Distended bladder. d. Urinary ascites.
- 54. Solid abdominal masses: a. Neuroblastoma: the most common malignant abdominal neonatal tumor. b. SCT. c. CMN: the most common solid renal mass < 6 months. d. Wilms’ tumor (WAGR, BWS), rhabdoid tumor, clear cell sarcoma, angiomyolipoma, and ossifying tumor of the kidney. e. Renal vein thrombosis (RVT). f. Renal artery thrombosis. g. Adrenal hemorrhage. h. Rhabdomyosarcoma. i. Germ cell tumor.
- 55. Anorectal Malformations • 75% …. Associated genitourinary and spinal anomalies. • Renal and bladder ultrasound. • Spinal ultrasound. • VCUG.
- 56. Exstrophy epispadias complex • Protect bladder mucosa. • Suturing the cord, no plastic clamp. • Good hydration. • Latex allergy is increasing in those patients. • Complete or staged repair.
- 57. Prune belly syndrome • Triad syndrome. • Eagle – Barret syndrome. • Abdominal wall muscle deficiency, cryptorchidism, urinary tract dilation. • Pulmonary problems. • Sonography, VCUG, nadir creatinine • Management. • Early orchiopexy.
- 58. Potter Syndrome • Oligohydramnios. • Limb contractures (particularly clubfeet). • Compressed facies with low-set ears. • Bilateral renal agenesis. • Respiratory failure.
- 59. Absence voiding • After 24hrs. • Distended bladder. • Examine meatus (pinpoint opening). • Ultrasound renal and bladder. • VCUG.
- 60. Renal vein thrombosis • Enlarged kidneys, hematuria, anemia, and thrombocytopenia. • A history of a prolonged delivery, dehydration, sepsis, birth asphyxia, maternal diabetes, protein C deficiency, umbilical catheter and prematurity. • Male > female, left > right. • Doppler ultrasonography. • Good hydration, electrolytes correction, anticoagulants.
- 61. Adrenal Hemorrhage • 1-2 % of healthy babies. • Right > left side. • Prolonged labor, birth trauma, and large birth weight, associated with RVT, BWS. • Anemia, shock, and an abdominal mass, scrotal hemorrhage. • Ultrasonography, MRI. • Peripheral eggshell calcifications. • DD : neuroblastoma ( urinary catecholamines). • Supportive treatment.
- 62. Renal artery thrombosis • Not common as a neonatal mass. • Hypertension and hematuria in a neonate. • Umbilical artery catheterization is the most common cause. • Renal insufficiency, proteinuria and congestive heart failure. • Doppler ultrasonography, CT Angiography. • Thrombolytic therapy, Nephrectomy in persistent hypertension.
- 63. Inguinal swelling Organs drawn into the hernia sac: Appendix, Bladder, Gonad, Meckel's diverticulum, Omentum, Bowel, Pelvic kidney, Ureter, Uterus. Aberrant rests: Adrenal, Ectopic renal tissue, Accessory spleen.
- 64. Inflammatory lesions: Abscess, Funniculitis, Granulomas, Sarcoidosis, Meconium granuloma, Parasitic disease, Peritonitis, Polyarteritis nodosa, Vasitis nodosa, Xanthrogranuloma. Tumors: Adrenal tumor, Dermoid cyst, Epidermoid cyst, Epididymal cysts and nodules, Calculi, Hemangioma, Lymphangioma, Mesenchymoma, Neuroblastoma, Paratesticular cyst or rhabdomyosarcoma, Spermatic cord tumors, Spermatocele, Tunica albuginea cyst, Tunica vaginalis cyst, Venous thrombosis, Wilms’ tumor.
- 65. Lesions that appear because of patent processus vaginalis: Adrenal hemorrhage, Endometriosis, Meconium.