L14- pediatric urinary disorders Year 4 Paediatrics Notes

L14- pediatric urinary disorders Year 4 · Paediatrics Collection: 4TH YEAR ALL NOTES / PAEDS / OTHER NOTES -- Pediatric Urinary Disorders \n\nObjectives: ü Recall normal anatomy. ü Identify the common congenital anomalies ü How to detect this anomaly on radiological\ninvestigations ü Important steps in management\n\n 2 \n\nAnatomy of urinary and genital tract:\n\n Urinary tract Genital tract\n\n Upper Lower Male Female\n\n Kidney Bladder\nInternal External Internal External\nUreter Urethra\n\n Ovary Labia majora\nProstate scrotum\nFallopian tube Labia minora\nSeminal vesicle Testis\nUterus Clitoris\nVas deference penis\nvagina vestibule \n3 \n\nIntroduction: -Most common anomalies of all organ systems 10% of the population have some type of urogenital anomaly. 14:1000 births have an antenatal diagnosis of urogenital anomaly. The antenatal diagnosis is done by ultrasound after 28 weeks of gestation because at 24-28 weeks the urinary system starts to be clear on ultrasound. Anomalies of the Upper Urinary Tract: 1- kidney number -involve anomalies of: ascent\nForm and fusion \nrotation \nRenal vasculature \nThe collecting system 4 \n\n1-Anomalies of number: First:Unilateral Renal Agenesis. -1 in 1100 births. -Male: Female of 1.8: 1 -The left side is absent more frequently. -The ipsilateral ureter is completely absent in 50%. -mostly found incidentally -Anomalies of other organ systems are found frequently in affected individuals which include: cardiovascular, gastrointestinal, musculoskeletal. Müllerian duct abnormalities occur in 25% to 50% of cases of females with URA (urinary renal agenesis) compared with wolffian duct anomalies, which are seen in 10% to 15% of males with URA. -Approximately one fourth to one third of women with müllerian duct anomalies are found to have URA.\n\n Müllerian duct Wolffian duct\nforms uterine tubes,uterus, pair of tubes that carry urine\ncervix, and upper one-third of from primitive kidneys to a\nvagina. Disappears in male. primitive bladder. \n5 \n\nSecond:bilateral renal agenesis: -40% are stillborn. -Do not survive beyond 48 hours due to respiratory distress associated with pulmonary hypoplasia. Characterized by: 1-Potter facies. 2- Oligohydramnios(low amniotic fluid). -Ureters are almost always absent. -Bladder is either absent or hypoplastic. -Adrenal glands are usually positioned normally. -Müllerian duct anomalies are commonly observed\n\n Potter Facies:\n- Abnormal ear lobation\n- Micrognathia (undersized jaw)\n- Flattened nasal tip\n- Prominent infra orbital folds \n6 \n\nThird: Supernumerary Kidney: Definitive accessory organ with its own collecting system, blood supply, and distinct encapsulated parenchyma. Due to the presence of multiple ureteric buds on the same side. Either completely separate or loosely attached to the kidney\non the ipsilateral side. The ureteral inter-relationships on the side of the supernumerary\nkidney can be variable. 2-Anomalies of ascent and position: First: simple renal ectopia. A kidney that is outside the renal fossa. Can be in pelvic region (commenest), lumbar or sacral. The left side is more occurring than the right. Pelvic ectopia has been estimated to occur in 1 of 2100 to 3000 autopsies. 50% present with hydronephrosis:\nüObstruction: UPJO and UVJO\nüReflux: grade III or greater\nüMalrotation Vesicoureteral reflux is found in 30%. The incidence of genital anomalies in the patient with ectopia is about 15%. Most ectopic kidneys are clinically asymptomatic. Second: Cephalad Renal Ectopia. Third: Thoracic Kidney Kidney in chest.\n7 \n\n 3-Anomalies of form and fusion:\nFirst:Crossed Renal Ectopia with and without Fusion.\n-Crossed ectopia: kidney is located on the side opposite from that in which its ureter inserts into the\nbladder.\n-90% are fused with their mate\n-the superior pole of the ectopic kidney usually joins with the inferior aspect of the normal kidney.\n-The ureter from each kidney is usually orthotopic.\n\nA) Crossed renal B) Crossed\nC) Solitary D) Bilaterally\nectopia renal ectopia Right to left\ncrossed crossed\nwith fusion without fusion ectopia\nrenal ectopia renal ectopia\n\n 8 \n\nSecond: horseshoe kidney: -Occurs 1 in 400 persons. 2 kidneys fused and connected together. 90% by the lower lobes. -10% upper lobes connected. -The isthmus is bulky and consists of parenchymatous tissue. -The blood supply is abnormal. -The calyces: \nPelvis remains\nNormal in Atypical in in the vertical\nnumber orientation or obliquely\nlateral plane -The blood supply can be quite variable. -Horseshoe kidney is frequently found in association with other congenital anomalies. -Ureteropelvic junction obstruction is seen in one third of the cases. -60 % asymptomatic.\n\n 9 \n\n4-Anomalies of rotation: -The kidney and renal pelvis normally rotate 90 degrees ventromedially during ascent\nüthe calyces point laterally.\nüthe pelvis faces medially. -When this alignment is not exact, the condition is known as malrotation. Frequently associate