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Gu anomaly for nursing school
- 1. Genitourinary Problems Prof. Samir Gouda Dr Mokhtar Mahdy Pediatric surgery dept.
- 5. Genitourinary Problems • Obstructive uropathies • Bladder exstrophy • Cloacal exstrophy • Ambiguous genitalia • Hypo/Epispadias
- 6. Differential of Abdominal Mass - RenalRenal • Hydronephrosis • Cystic diseases of kidney – PCKD – MCDK • Renal vein thrombosis • Solid tumors of kidneys – Mesoblastic Nephroma – Wilm’s Tumor • Horseshoe or ectopic kidneys
- 7. Imaging Evaluation • Imaging • Start with plain film • Abdominal/Renal U/S • MRI • CT +/- contrast
- 10. Hydronephrosis • Most common congenital condition on prenatal ultrasound (1:500 to 1:700 deliveries) • Causes are varied – Physiologic (usually mild, up to 15%) – UPJ or UVJ obstruction – Vesicoureteral reflux – Posterior urethral valves
- 13. Hydronephrosis
- 14. Hydronephrosis vs Pyelectasis • 1 in 100 pregnancies w/ some evidence of dilation • Hydronephrosis: Dilation of renal pelvis >1 cm • Pyelectasis: Mild enlargement of renal pelvis 4-10 mm – Often resolves – F/u postnatal ultrasound – May suggest increased T21 risk in older mom
- 15. Cystic Diseases of Kidneys • Multicystic Dysplastic Kidney (MCDK) • Polycystic Kidney Disease – Autosomal Recessive – Autosomal Dominant
- 16. Multicystic Dyplastic Kidney MCDK • Most severe form of renal dysplasia • Multiple large cysts/ureteral atresia • Unilateral • Sporadic (+/- VACTERL) • Involutes over time • Follow with serial U/S & remove if doesn’t involute • Urological testing on healthy kidney
- 17. Polycystic Kidney Disease • Genetic, with variable expression • Most commonly inherited kidney disease • Bilateral involvement • Cysts may also be in liver • Results in hypertension ‘‘Snowstorm’ appearance ofSnowstorm’ appearance of infantileinfantile polycystic diseasepolycystic disease
- 18. Polycystic Kidney Disease Autosomal Recessive ‘Infantile’ PKD Autosomal Dominant Adult PKD Frequency 1/40,000 1/10,000 Chromosome 6p21 16 (Codes for polycystin) Diagnosis Fetal U/S: large echogenic kidneys, oligohydramnios Rarely findings at birth Cysts Kidneys liver, as older Large>2cms Kidney, liver, pancreas, spleen. Variable size Problems Severe HTN, hepatic fibrosis, biliary dysgenesis, Potter’s Sequence HTN, renal insufficiency. Worse as older
- 19. Renal Vein Thrombosis Flank mass, hematuria, and renal failure: • Usually w/in first 3 DOL, dx RUS w/ doppler • Risk factors: Maternal diabetes, dehydration, sepsis, hypovolemia, DIC, polycythemia, hypercoagulable state • Conservative, non-operative mgmt • Thrombocytopenia (consumptive) • Most frequent vascular condition
- 20. Renal Vein Thrombosis (flank mass can be felt from congestion)
- 21. Tumors of Neonate • Renal: – Mesoblastic Nephroma – Less common: • Wilms (aka nephroblastoma) • Clear cell • Rhabdoid Tumor • Adrenal: – Pheochromocytoma (medulla of adrenals) – Neuroblastoma
- 22. Ectopic Kidney • Kidney not located in usual position • 1 in 1,000 births, but only about one in 10 of these are ever diagnosed; up to 10% bilateral Most common: Horseshoe Kidney Unilateral renal agenesis Pelvic kidney (Left kidney more likely to be abnormal)
- 23. Ectopic Kidney • Function is generally normal initially, but… • Abnormal position leads to obstruction in 50% of ectopic kidneys • Increased risk UTI, kidney stones, VUR • Frequently associated with abnormalities of other organ systems (uterine, cardiac, skeletal)
- 25. Ectopic Kidney (simple renal ectopia)
- 26. Horseshoe Kidney • Most common renal fusion anomaly (1:400) • As kidneys rise from pelvic area they fuse at lower pole (90%) • 33% without symptoms, although associated w/ Turner Syndrome, Trisomy 18 • Commonly present with UTI • Diagnosis: Renal Ultrasound, VCUG • Supportive Management, prophylactic antibiotics, surgical intervention (stones)
- 27. Horseshoe Kidney
- 28. Differential of Acute Renal Failure • Prerenal • Intrinsic • Obstructive Annabelle N. Chua and Minnie M. Sarwal. Acute Renal Failure Management in the Neonate.
- 31. Genitourinary Problems • Obstructive uropathies (PUV,UPJ,UVJ) • Vesicourethral reflux • Bladder exstrophy • Cloacal exstrophy • Ambiguous genitalia • Hypo/Epispadias • Urachal Anomalies
- 32. Obstructive Uropathies • Posterior Urethral Valves (PUV) • Ureteropelvic Junction Obstruction (UPJ) • Ureterovesical Junction Obstruction (UVJ) –Ureterocele
- 34. Posterior Urethral Valves • Most common cause of obstructive uropathy in males – Does not affect females • Incidence: 1/5000-1/8000 • Cause: congenital membrane which (partially) obstructs urethra
- 35. Posterior Urethral Valves • Clinical presentation: – bilateral flank masses (hydronephrosis) – distended bladder – poor urinary stream (+/- dribbling) • Diagnostic test: VCUG • Therapeutic Goal: Preserve renal function, avoid renal failure – 30% at risk for progressive renal insufficiency
- 36. PUV
- 38. Posterior Urethral Valves Hydronephrosis Dilated ureters Thickened trabeculated bladder Dilated proximal urethra
- 39. UPJ Obstruction
- 40. UPJ Obstruction (Ureteropelvic) • Most common cause of congenital hydronephrosis • Cause: Abnormal muscle development at UPJ • Males > Females • Can be associated with other congenital abnormalities (e.g. VATER)
- 41. UPJ Obstruction – Surgical Repair • Pyeloplasty - involves removing the blockage, and reconnecting the ureter to the renal pelvis. – Stent may be left across the pyeloplasty or a nephrostomy may be left above the repair to decompress the kidney
- 42. Genitourinary Malformations • Bladder exstrophy • Cloacal exstrophy • Ambiguous genitalia • Hypo/Epispadias • Urachal Anomalies
- 44. Bladder Exstrophy • Spectrum of severity – Small defect can result in epispadias – large defect can result in exposure of posterior bladder wall • Occurs in 1 in 30,000 births • Exposed bladder mucosa is edematous and friable
- 46. Bladder Exstrophy • Moist, fine-mesh gauze or vaseline gauze to cover exposed bladder • Antibiotics • Renal ultrasound • Transfer infant to surgical center – Want surgical correction by 48-72 hours of life – Sacroiliac joints are still pliable and the pelvis can be “molded” to allow better approximation of the pubic rami • IVP of limited utility b/c of poor concentrating ability of neonatal kidney
- 47. Bladder Exstrophy - Surgery • Turn-in of the bladder to preserve bladder function • Symphysis pubis is approximated • Iliac osteotomies aren’t necessary if repair is within 48 HOL (bones are still pliable from circulating maternal estrogens) • Epispadiac urethra is reconstructed later
- 49. Cloacal Exstrophy • Very rare: 1 in 200,000 births • Sporadic occurrence • Complex of GI and GU anomalies: – Imperforate anus – Exstrophy of the bladder – Omphalocele – Vesicointestinal fistula – Frequently w/ prolapse of bowel thru the fistula on bladder mucosa
- 50. Cloacal Exstrophy – Preop • Cover exposed mucosa (vaseline/saline gauze) and/or plastic wrap to minimize heat loss • Gender assignment / diagnosis – Karyotype / FISH – Controversial regarding assignment • NG suction to relieve partial intestinal obstruction. – Stool frequently excreted through a vesico- intestinal fistula that is often partially obstructed
- 51. Cloacal Exstrophy - Operative • Survival 80% - mortality due to sepsis and bowel obstruction • Prompt surgery to separate fecal and urinary streams • Bladder can be closed during initial procedure if baby stable • Subsequent procedures to reduce the number of stomas and create genitalia
- 52. Cloacal Exstrophy • Multiple Long term problems: • Psychosocial / Gender Identification • Recurrent UTIs • Bowel/Bladder Incontinence • Sexual function in later life
- 54. CAH – Multiple Types
- 55. Hypospadias • Incidence: up to 1:250 births • Associations: undescended testicles (DSD), inguinal hernias • Management: recognition, avoidance of circumcision • Surgery: usually by 2 years of life
- 56. Epispadias • Incidence: 1:10,000 to 1:20,000 • Associations: Bladder exstrophy, VUR (40%) • Management: recognition, avoidance of circumcision • Surgery: multistaged reconstruction
- 59. Hypo/Epispadias
- 60. Hypospadias
- 62. Epispadias
- 63. Urachal abnormalities • Patent urachus: – Connection remains between allantois and fetal bladder – Drainage/urine leakage through umbilicus – Surgically remove @ 2 mo • Urachal sinus: – Drains to umbilicus – Intermittent drainage from umbilicus (may be serous or serosanguinous • Urachal cyst: – Fluid-filled structure occurring in between the two obliterated ends of the urachus (distal end)
- 65. The End
Editor's Notes
- If AD at birth- really bad- resp failure- sepsis. HTN may respond to ACE/ Loop diuretics. Decreased renin and angiotensin levels.
- Patient evaluation in ureteropelvic junction obstruction Evaluation. Most children are first imaged by renal ultrasonography. Although ultrasonography cannot directly assess function and obstruction, several variables, when considered in association, can be predictive of a ureteropelvic junction obstruction (UPJO). These variables include increased echogenicity of the renal parenchyma, contralateral hypertrophy, a decreased rim of parenchyma of less than 5 mm, and a resistive index ratio of greater than 1:1 [ref]. Nuclear renal scintigraphy remains the most reliable imaging study in the assessment of a UPJO, but it is not definitive. When determining the significance of hydronephrosis, several parameters are calculated, including the differential uptake of activity between the two kidneys, the time to peak activity, excretion, and the half-time for drainage of the renal pelvis. No one particular feature of the renal scan indicates obstruction. There are multiple pitfalls when using nuclear scintigraphy, and for that reason, standardized protocols have been established to eliminate as many variables as possible [ref]. Upper urinary tract urodynamic evaluation combining fluoroscopic assessment of the ureteropelvic junction and pressure manometry has been helpful in selected cases to further delineate the UPJO. The Whitaker study is performed by infusing the testing medium into the renal pelvis at a constant rate. The change in pressure is recorded as the renal pelvis fills and drains [ref],[ref],[ref]. The pressure perfusion study is an alternative to constant infusion [ref]. In this study, a preselected filling pressure is used to fill the renal pelvis, and the change in renal pelvic pressure is determined as the pelvis fills and drains. This technique eliminates the infusion rate as a variable. Vesicoureteral reflux (VUR) occurs in approximately 20% of children [ref][ref] with a UPJO. It is important to determine whether a true UPJO exists or whether hydronephrosis is due to significant tortuosity and dilatation of the ureter and renal pelvis. The difference in the density of contrast between the renal pelvis and the ureter can help in defining a true obstruction. When an obstruction exists, the renal pelvis is usually much less dense than the ureter because refluxed contrast is mixed with retained urine. When a dilated renal pelvis is secondary to VUR, the contrast density in the pelvis and in the ureter is similar. Delayed images show poor drainage with true obstruction. Both ultrasonography and renal scintigraphy are helpful in postoperative assessment. If imaging studies are obtained within 4 weeks of the repair, it is not unusual to see either no apparent change or a small increase in hydronephrosis. This finding can be due to operative edema at the site of the anastomosis. It may take several months for improvement to be visible on renal ultrasonography. 99mTc DTPA—99mTc diethylene triamine penta-acetic acid; 99mTc MAG3—99mTc mercaptoacetyltriglycine.
- Other findings: single umbilical artery, vertebral anomalies (hydromelia), club foot and ambiguous genitalia (in boys, the penis is divided and duplicated) – very difficult to repair male genitalia
- A canal connecting the bladder of the fetus with the allantois, a structure that contributes to the formation of the umbilical cord. The lumen (inside) of the urachus is normally obliterated during embryonic development, transforming the urachus into a solid cord, a functionless remnant. Children may present with periumbilical tenderness, a wet umbilicus, or granulation tissue at the level of the umbilicus. In many instances, these children have undergone multiple sliver nitrate cauterizations under the mistaken notion that this is simply granulation tissue after severance of the remnant umbilical cord