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CONGENITAL UROLOGICAL ANOMALIES DETECTED IN ADULTHOOD

CONGENITAL UROLOGICAL ANOMALIES DETECTED IN ADULTHOOD. Sarel Halachmi MD Pediatric Urology Service, Rambam Medical Center Faculty of Medicine Technion Israeli Institute of Technology, Haifa, Israel. Detection of congenital anomalies.

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CONGENITAL UROLOGICAL ANOMALIES DETECTED IN ADULTHOOD

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  1. CONGENITAL UROLOGICAL ANOMALIES DETECTED IN ADULTHOOD Sarel Halachmi MD Pediatric Urology Service, Rambam Medical Center Faculty of Medicine Technion Israeli Institute of Technology, Haifa, Israel.

  2. Detection of congenital anomalies • The urology system is easily demonstrated sonographicaly even in utero. • The prenatal ultrasound era changed the approach and improved the outcome of various congenital urological anomalies. • Aigrain Y Fetal Diagn Ther. 11, 181-90, 1996.

  3. Congenital anomalies diagnosed in adulthood • Not all pregnant women undergo prenatal US. • Some of the anomalies are missed – human error. • Most prenatal systematic scans performs around 17-22nd gestational week, however 20% of the hydronephrotic anomalies appears after the 24th week

  4. UPJO • Ureterocele • Cryptorchidism • Hypospadias • Reflux

  5. Adult UPJ Obstruction

  6. Adult UPJ Obstructionincidence • The incidence of pediatric UPJO is well defined, affecting around 60-70% of all antenatally dilated systems. • The incidence of adult UPJ is not known. • Adult UPJ is not a rare finding.

  7. Adult UPJ Obstructionpresentation • Flank pain, back pain. • UTI/ Pyelonephritis • Hypertension (rare) • Asymptomatic Incidentally detected during evaluation of other symptoms

  8. Adult UPJ Obstructiondiagnosis • Same diagnostic modalities used for the pediatric age group are applicable for adults: • Nuclear renogram • IVP • CT

  9. Pediatric UPJmanagement decisions • Function • Need for preservation of growth & function potential.

  10. Adult UPJ Obstructionmanagement decisions • Symptoms • Function • Age • Co-morbidities.

  11. Adult UPJ Obstructionmanagement options • Nephrectomy. • Reconstructive surgery: • Open surgery / Laparoscopic / Robotic • Endoscopic • Direct incision • antegrade/retrograde; • cold knife/electrocautery/laser • Acucise balloon dilatation and cutting.

  12. Adult open pyeloplasty • gold standard treatment with success rate of 90-95%. • Gogus C. et all, Urol Int. 2004 • Long term results of open pyeloplasty in adults. • 180 patients • Mean age 33 (16-65) • Mean follow up 9.4 years (1-17) • Success rate 91% • Success for poor function grade 4/4 hydronephrosis 62.5% • Success rate good function grade 1-3/4 hydronephrosis 100%

  13. Laparoscopic pyeloplasty. • Bauer JJ et al, J. Urol 1999; compared laparoscopic to open pyelolasty. • 42 lap, 35 open • Comparable success rate of 98% vs. 94% • Complication rate 12% vs. 11% • Lap pyeloplasty is comparable to open surgery in adults.

  14. Robotic pyeloplasty • Palese,M.A. J.Endourol 2005 • Robot-assisted laparoscopic dismembered pyeloplasty • 35 patients • Mean operative time was 3.6 hrs. • Minimal blood loss of 73.9 mL. • Short hospitalization of 2.8 days. • No intra-operative complications • No conversions. • Mean follow-up was 7.9 months. • success rate of 94%.

  15. Adult pyeloplasty“Formal” dismembered pyeloplasty • Open=Lap=Robotic

  16. Endopyelotomies • This group of minimally invasive procedures could be applied only in the adult patient with UPJO. • Technical issues • Success rate

  17. Antegrade endopyelotomy • First described by Wickham and Kellet Eur Urol, 1983. • percutanous pyelolysis. • Antegrade procedure. • Rigid nephroscop. • Cold knife. • 3 patients were operated and 2 achieved improved renal drainage. • Motola and Smith j urol 1993. Result of 212 consecutive endopyelotomies: 8 year follow-up. • 212 cases, 110 primary UPJ • Follow up 3-6 years • Success rate 85%

  18. Retrograde ureteroscopic endopyelotomy Originally described by Bagley J Urol 133, 1985. • Results in terms of UPJ drainage were promising, however distal ureteral stricture significantly complicated this procedure. • Clayman RV, J Urol 1990.

  19. Endoscopic proceduresRetrograde ureteroscopic endopyelotomy • With the introduction of smaller endoscopes and laser energy, stricture problem was reduced significantly. • Overall success rate 73-90% • Minimal hospital stay. • Short recovery time. • Considered as first line treatment in many centers. • Danuser H Studer UE, J Urol 1998. • Preminger GM, AUA update 2000. • Urena R, Altas Urol Clin 2003.

  20. Acucise balloon dilatation and cutting • Minimally invasive procedure. • Reported success rate 56%-64% • Downside indirect vision of the UPJ • Inability to adjust the incision to the UPJ angulations. • Intra-operative bleeding • Suboptimal success rate • Chandhoke PS, Clayman RV, J Endourol 1993 • Schwartz BF, J Urol 1999. • Baldwin DD, J Endourol 2003 • Biyani CS, Eur Urol 2002

  21. Endoscopic proceduressummary • Many options are available most are comparable to open pyeloplasy. • For poor functioning symptomatic kidney nephrectomy is a valid option (open / laparoscopic) • Nadu A Isr Med Assoc J. 2005

  22. Adult cryptorchidism

  23. Cryptorchismfactors affecting management • Endocrine function. • Fertility. • Risk of malignancy. • Risk of other complications.

  24. Endocrine function of the adult UDT • Ren L, J Reprod Dev. 2006 • Effects of experimental cryptorchidism on testicular endocrinology. • Bilateral UDT was created in adult male rats • In cryptorchid rats, testosterone, and inhibin B levels were significantly lower. • Testosterone release in response to hCG was decreased. • Heat stress to the testes resulted in a significant changes in testicular endocrine function.

  25. UDT endocrine function • Hadziselimovic F,J Urol. 2005 • Examined the response to HCG stimulation in boys with UDT who had early orchidopexy. • 35% had inadequate response to HCG • 10% did not respond. • Non or inadequate responders had also defective spermatogenesis. • CONCLUSIONS: • Despite early orchidopexy many boys will have insufficient testosterone secretion.

  26. Adult Cryptorchismfertility

  27. Adult Cryptorchismfertility • Rogers E, J Urol 1998. • Analyzed the histology of resected adult UDT • 52 patients with postpubertal cryptorchidism. • mean age of 26 years (15 – 66). • All had orchiectomy • Histology of the UDT • 1 normal spermatogenesis • 15 maturation arrest, • 6 testicular agenesis • 30 Sertoli-cell-only. • the majority of cryptorchid testes cannot contribute to fertility.

  28. Adult Cryptorchism fertility • Postpubertal cryptorchidism: review and evaluation of the fertility. • Grasso M,Eur Urol.1991;20(2):126-8 • Biopsied 22 patients during post pubertal unilateral orchidopexy. • 83.5% of patients were azoospermic or oligospermic, with or without asthenospermia. • orchidopexy is not the best treatment for postpubertal cryptorchid patients.

  29. Adult Cryptorchism location vs. function • Higher location = lower function. • Cryptorchidism in adults. About 81 cases. Ben Jeddou F. Tunis Med. 2005 Dec;83(12):742-5. • Histological changes in the testis following adult orchidopexy for unilateral cryptorchidism. Duvie SO, Arch Androl 12, 231, 1984.

  30. Adult Cryptorchismrisk of malignancy • Effect of age at orchidopexy on risk of testicular cancer. • Pike MS Lancet. 1986 • 724 cases of testicular cancer in 10 years seen in a single institute. • 69/724 (9.5%) had hx of UDT. • 11/69 (16%) had uncorrected UDT on diagnosis. • 58/69 (84%) had orchydopexy • Uncorrected UDT has a high risk of cancer. • Corrected UDT has a high risk of cancer. • The age at treatment of UDT have no effect on the risk of cancer.

  31. Adult Cryptorchismrisk of malignancy • Rogers E, J Urol Mar;159(3):851-4, 1998. • Assessed the histology of adult UDT who underwent orchectomy • 52 patients with postpubertal cryptorchidism. • mean age of 26 years (15 – 66). • 2/52 (4%) had carcinoma in situ of the testicle. • Orchiectomy is the treatment of choice for the majority of postpubertal male presenting with unilateral cryptorchidism.

  32. Adult Cryptorchismrisk of torsion • Torsion of the cryptorchid testis--can it be salvaged? • Zilberman D, J Urol. 2006 • UDT is at higher risk for torsion compared to the normally descended testis. • 11 children with torsion of a UDT. • 5/11 (45%) necrosis -> orchiectomy • 6/11 orchidopexy -> 4 (36%) vanished testis • UDT torsion has a low salvage rate. 2/11 (18%)

  33. Adult Cryptorchismsummary • Low endocrine and fertility capacity. • Increased rate of torsion, low salvage rate. • Increased rate of malignancy.

  34. Adult Cryptorchismrecommendations • Orchiectomy should be offered. • Preservation management demands patient and physician awareness to the possible complications

  35. Adult cryptorchidismsurgical correction

  36. Adult Cryptorchismorchidopexy • Orchidopexy in adult is a safe and feasible procedure • Laparoscopic management of the adult nonpalpable testicle. Corvin S, Urol Int. 2005;75(4):337-9. • Laparoscopic assessment and orchidectomy for the adult undescended testis. Sousa ASurg Laparosc Endosc Percutan Tech. 2000 Dec;10(6):420-2

  37. Adult ureterocele

  38. Pediatric ureterocele • Ureterocele in the pediatric age group is related to a complex of anomalies such as: • Duplex kidneys • Urinary tract obstruction • Incontinence • Reflux

  39. Adult ureterocele • In contrast most of the adult ureterocele are: • Single system. • Intravesical. • The degree of obstruction is less severe.

  40. Adult ureterocelepresentation • Asymptomatic hydronephrosis. • Flank/back pain. • UTI. • Stone formation. • Renal failure (rare single case report).

  41. Adult ureterocelediagnosis • IVP • US

  42. Adult ureteroceletreatment • Factors affecting management • Symptoms • Renal function.

  43. Adult ureteroceletreatment • Chourou M, Prog. In Urol, 2002 • Assessed the treatment in adult ureterocele complicated with calculi. • 12 females, 8 males, mean age 48 (24-75) • Presentation: low back pain • Diagnostic modality: IVP • Single system 16 (80%), duplex 4 (20%) • Treatment: endoscopic ureterocele incision, stone fragmentation. • Complications 1/20 (5%): sepsis • F/U: 6 months • Elimination of pain 100% • 1/20 developed transient reflux.

  44. Adult ureterocelerare complication • Vasu TS, Can J Urol. 2006 • Bilateral ureteroceles progressing to reversible renal failure in an adult. • Renal failure reversed following incision. • Single case report - but should be bared in mind.

  45. Adult hypospadias

  46. Primary repair in adulthood • Adayener C, Urol Int. 2006;76(3):247-51. • Distal hypospadias repair in adults. • Assessed 80 adults with primary hypospadias. • Meatal position • Glanular in 6 • Coronal in 35 • Subcoronal in 56 patients • Operative techniques: • Meatal advancement 6 • Mathieu 41 • Tubularized incised plate 14 • Overall success rate: 73/80 91.3% • Position related success rate: • Coronal 91% • Subcoronal 85% • CONCLUSION: The success rate for primary adult cases is quite acceptable, but it is decreased in patients having longer neourethra

  47. Secondary repair • Failed hypospadias repair presenting in adults. • Barbagali G, Eur Urol. 2006 May;49(5):887-94; • 60 adults with complications following pediatric hypospadias surgery. • 36% of the patients had one complication and 64% had two or more complications • Stricture 34. • residual hypospadias 26. • Fistula 18. • meatal stenosis 11. • penile curvature 9. • Hair 4 • Diverticula 2 • Stone 1 • Operative technique: • 29 one-stage repair with buccal or skin grafts or direct repair. • 31 underwent multistage repairs with buccal or skin grafts • Results • 45 (75%) had a final successful outcome • 15 (25%) failed. • One-stage repair provided 24 (82.7%) successes and 5 (17.3%) failures. • Multistage repair provided 21 (67.7%) successes and 10 (32.3%) failures. • CONCLUSIONS: Adults with complications following childhood hypospadias repair are still a difficult population to treat with a high failure rate for reoperative surgery.

  48. Adult reflux

  49. Adult reflux • VUR may resolve spontaneously, however in 10-50% depending on grade reflux will persist beyond childhood.

  50. Persistent asymptomatic refluxShould we treat with antibiotics?Should we to observe / operate?Observe until when, how? VCUG - ROOM

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