Congenital Anomalies Lecture Notes PDF- University of Baghdad College of Medicine 2023-2024

University of Baghdad College of Medicine 2023-2024 Title: Abnormalities of the kidney position and number, duplication anomalies, ureterocoeles and ectopic ureters Grade: Fourth Grade Module: Surgery Module Speaker: Dr. Firas Sabah Al-Ali M.B.Ch.B, F.I.C.M.S (URO.), C.A.B.M.S (URO.) Date: 19-2-2024...

University of Baghdad College of Medicine 2023-2024 Title: Abnormalities of the kidney position and number, duplication anomalies, ureterocoeles and ectopic ureters Grade: Fourth Grade Module: Surgery Module Speaker: Dr. Firas Sabah Al-Ali M.B.Ch.B, F.I.C.M.S (URO.), C.A.B.M.S (URO.) Date: 19-2-2024 University of Baghdad/ College of Medicine 2022-2023 University of Baghdad/ College of Medicine 2022-2023 Learning Objectives To list the abnormalities of the kidney position and number To describe the embryology and classification of duplication anomalies. To clarify the associated pathology with duplication anomalies. To explain the most important radiological signs. To know some points on the management of these conditions. Abnormalities Of The Kidney Position And Number University of Baghdad/ College of Medicine 2022-2023 1. Simple ectopia 2. Thoracic ectopia 3. Crossed ectopia and fusion 4. Horseshoe kidney 5. Bilateral renal agenesis 6. Unilateral renal agenesis 7. Supernumerary kidney University of Baghdad/ College of Medicine 2022-2023 Duplication Anomalies 7 University of Baghdad/ College of Medicine 2022-2023 Introduction  Found in 0.8% of postmortem examinations.  Higher proportion (2–3%) of patients undergoing intravenous urography (IVU) for various indications.  Approximately 40% of cases the condition is bilateral.  Transmitted as an autosomal dominant trait with incomplete penetrance. University of Baghdad/ College of Medicine 2022-2023 Introduction cont.  Great majority of duplications are of the incomplete variety.  Complete duplication anomalies are often of clinical significance in terms of symptoms, renal function or both. University of Baghdad/ College of Medicine 2022-2023 Embryology  A single ureteric bud which arises normally from the mesonephric duct but which then undergoes bifurcation leads to a variable degree of incomplete duplication of the upper renal tract.  Complete duplication occurs when two ureteric buds arise separately from the mesonephric duct. University of Baghdad/ College of Medicine 2022-2023 Embryology cont.  As a rule, the more extreme the degree of ectopia of the ureteric bud the greater the likelihood that it will come to penetrate an abnormal zone of metanephric tissue with resultant dysplasia. University of Baghdad/ College of Medicine 2022-2023 Meyer–Weigart law  An accessory ureteric bud which arises in a caudal position on the mesonephric duct and connects with the lower renal pole is absorbed into the urogenital sinus, so that the ureteric orifice opens in a superior and lateral position in relation to the trigone, an anatomical configuration commonly predisposing to vesicoureteric reflux to the lower pole of the kidney. University of Baghdad/ College of Medicine 2022-2023 Meyer–Weigart law cont.  An accessory bud that arises from the mesonephric duct in a more cephalad position than the normal site and which drains the upper renal pole will come to enter the urinary tract in a distally ectopic location. It may drain into either the bladder, the urethra or the urogenital sinus. University of Baghdad/ College of Medicine 2022-2023 Meyer–Weigart law cont. University of Baghdad/ College of Medicine 2022-2023 Associated Pathology 1. Coexisting vesicoureteric reflux to the ipsilateral lower pole. 2. Obstruction of the ipsilateral lower pole ureter. 3. Dilatation of the ipsilateral upper pole ureter. 4. Bladder outflow obstruction. 5. Contralateral vesicoureteric reflux. 6. Prolapse of the ureterocoele into the urethra. IVP with duplicated system University of Baghdad/ College of Medicine 2022-2023 University of Baghdad/ College of Medicine 2022-2023 University of Baghdad/ College of Medicine 2022-2023 Ureterocoele University of Baghdad/ College of Medicine 2022-2023 Duplex-system ureterocoele  Cystic dilatation of the terminal portion of the ureter draining the upper pole.  80% are female.  A slight majority are left sided.  10% of cases are bilateral. University of Baghdad/ College of Medicine 2022-2023 University of Baghdad/ College of Medicine 2022-2023 Duplex-system ureterocoele cont.  60% of children with these anomalies are currently identified by prenatal ultrasonography.  Clinical presentation is most commonly with urinary infection.  Acute or chronic urinary retention.  In females, urethral prolapse of the ureterocoele. University of Baghdad/ College of Medicine 2022-2023 Prolapsed ureterocoele University of Baghdad/ College of Medicine 2022-2023 Single-system ureterocoeles  Occur more commonly in boys.  Almost always lie entirely within the bladder. Cobra head appearance University of Baghdad/ College of Medicine 2022-2023 University of Baghdad/ College of Medicine 2022-2023 Ectopic Ureter University of Baghdad/ College of Medicine 2022-2023 Ectopic Ureter  In females, ectopic ureters may be sited above the sphincter mechanism (suprasphincteric), close to or at the level of the striated sphincter (sphincteric), or distal to the sphincter (infrasphincteric), either at the introitus or in the distal vagina.  In males, the termination is always suprasphincteric. University of Baghdad/ College of Medicine 2022-2023 Management University of Baghdad/ College of Medicine 2022-2023 Management  Indications for surgical intervention comprise: symptoms. upper renal tract obstruction. University of Baghdad/ College of Medicine 2022-2023 Management cont.  Endoscopic ureterocoele incision  Upper pole heminephrectomy  Pyelopyelostomy  Ureterocoele excision and reimplantation  Nephrectomy University of Baghdad/ College of Medicine 2022-2023 Intraoperative appearance University of Baghdad/ College of Medicine 2022-2023 Intraoperative appearance University of Baghdad/ College of Medicine 2022-2023 Summary  Incomplete duplication is relatively common and is often of little or no clinical significance. By contrast, complete duplication is rarer and is more frequently associated with symptoms, impaired function or both.  Complete duplication anomalies can be divided, on the basis of their embryology, into those affecting principally the upper renal pole (ureterocoele, suprasphincteric or infrasphincteric ectopic ureter) or those affecting the lower renal pole (vesicoureteric reflux, pelviureteric junction obstruction). University of Baghdad/ College of Medicine 2022-2023 Summary cont.  Renal dysplasia of the upper or lower pole is common in all forms of complete duplication. University of Baghdad/ College of Medicine 2022-2023 Thank You