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Bladder Exstrophy Epispadias Complex .pptx

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Pradeep Pande
Pradeep Pande

Lecture notes for MBBS UG

1 of 50
Tips on using my ppt. 1. You can freely download, edit, modify and put your name etc. 2. Don’t be concerned about number of slides. Half the slides are blanks except for the title. 3. First show the blank slides (eg. Etiology ) > Ask students what they already know about etiology of today's topic. > Then show next slide which enumerates etiologies. 4. At the end rerun the show – show blank> ask questions > show next slide. 5. This will be an ACTIVE LEARNING SESSION x three revisions. 6. Good for self study also. Display blank slide> Think what you already know about this > Read next slide. 7. See notes for bibliography.
Learning Objectives At the end of this session the learner will be able to describe- • Etiology • Clinical Features • Management Of Bladder exstrophy aka. Bladder- exstrophy-epispadias-complex (BEEC). / Ectopia vasicae.
Learning Objectives 1. Introduction & History 2. Relevant Anatomy, Physiology 3. Etiology 4. Pathophysiology 5. Pathology 6. Classification 7. Clinical Features 8. Investigations 9. Management 10. Controversies 11. Prevention 12. Guidelines 13. Take home messages
Etiology • Idiopathic • Traumatic • Infections /Infestation • Neoplastic (Benign/Malignant) • Congenital/ Genetic • Nutritional Deficiency/excess • Autoimmune • Degenerative / lifestyle • Iatrogenic • Psychosomatic • Poisoning/ Toxins/ Drug induced
Etiology • Idiopathic • Traumatic • Infections /Infestation • Neoplastic (Benign/Malignant) • Congenital/ Genetic • Nutritional Deficiency/excess • Autoimmune • Degenerative / lifestyle • Iatrogenic • Psychosomatic • Poisoning/ Toxins/ Drug induced
Etiology • Maternal smoking • Maternal irradiation. • Incidence less if Folate is taken during in periconceptional period.
Pathophysiology
Pathophysiology Multiple Theories- • An abnormally large cloacal membrane causes a wedge effect and prevents the medial migration of the mesenchymal tissue. • An abnormally large cloacal membrane causes a wedge effect and prevents the medial migration of the mesenchymal tissue. • Premature rupture of the cloacal membrane • Caudal displacement of the genital tubercle, • Abnormal fluid retention leading to rupture of the embryonic bladder • Pubic diastasis
Pathology •
Pathology • The lower portion of anterior abdominal wall and anterior wall of urinary bladder is missing. • Bladder-exstrophy-epispadias-complex (BEEC) depicts a spectrum of birth defects, ranging from epispadias to cloacal exstrophy at the milder and the most severe ends of the spectrum. • The classical bladder exstrophy is characterized by abnormalities involving the lower urinary tract, abdominal wall, bony pelvis, genitalia, pelvic floor, spine, and the anus. • The 'open book configuration' of the pelvis
Clinical Features •
Clinical Features • Demography • Symptoms • Signs • Prognosis • Complications
Demography
Demography • Incidence & Prevalence • Geographical distribution. • Race • Age • Sex • Socioeconomic status • Temporal behaviour
Demography • Incidence & Prevalence-
Demography Incidence & Prevalence- • 3.3 per 1,00,000 live births,
Demography • Race.
Demography Race- • More common in whites.
Demography • Sex
Demography Sex- • predominance in males.
Signs
Signs
Signs Local Examination- • A fleshy, red mass prolapsing out of the suprapubic region represents the open bladder plate. • There is continuous urine leakage from the mass. • In male newborns, the phallus is short, stubby, and has a dorsal chordee (pointing upwards). • The glans is flattened with widely separated (divergent) corpora due to the wide pubic diastasis.
Signs Local Examination- • The testis may not be descended at birth, and occasionally there may be the presence of bilateral inguinal hernias. • In females, the clitoris is bifid with a slightly anterior vaginal opening. • Uterine and vaginal duplications . • Anterior ectopic anus wlth patulous anal orifice with mucosal prolapse
Signs Local Examination- • It is not uncommon for some children to present later in life. • Severe excoriation in the para-exstrophy skin, hypertrophied-polypoidal bladder plate with squamous metaplasia, poor control over bowel movements, and waddling gait are common. • Social problems due to the continuous wetting of clothes
Prognosis
Prognosis • Morbidity • Mortality rate • 5 year survival in Malignancy
Complications
Complications • Incontinence: • Upper tract damage • Upper tract damage • Sexual dysfunction • Infertility.
Investigations • Laboratory Studies – Routine – Special • Imaging Studies • Tissue diagnosis – Cytology • FNAC – Histology – Germ line Testing and Molecular Analysis • Diagnostic Laparotomy.
Investigations • Laboratory Studies – Routine – Special • Imaging Studies • Tissue diagnosis – Cytology • FNAC – Histology – Germ line Testing and Molecular Analysis • Diagnostic Laparotomy.
Investigations • Laboratory Studies –
Investigations • Laboratory Studies –
Diagnostic Studies Imaging Studies • X-Ray • USG • CT • Angiography • MRI • Endoscopy • Nuclear scan
Diagnostic Studies Imaging Studies • X-Ray • USG • CT • Angiography • MRI • Endoscopy • Nuclear scan
Diagnostic Studies Imaging Studies •
Diagnostic Studies •
Differential Diagnosis
Differential Diagnosis • Epispadias • Cloacal exstrophy -omphalocele, exstrophy of bladder, imperforate anus, and spinal abnormalities • Pseudoexstrophy • Superior Vesical Fissure • Duplicate exstrophy • Covered exstrophy
Operative Therapy
Operative Therapy • The aims of the surgical treatment- 1. To provide a competent receptacle (bladder) for storage, 2. To prevent upper tract damage 3. To provide cosmetically acceptable genitalia providing good functional outcomes in terms of continence and sexual function.
Operative Therapy • Modern staged repair of exstrophy (MSRE) • Complete primary repair of exstrophy (CPRE),
Operative Therapy • Two main categories: – Reconstructive procedures – Diversion procedures.
Operative Therapy • Bladder closure, • Epispadias repair • Bladder neck reconstruction • Bladder augmentation • Ureteric reimplantation. • Correction of abnormalities in the bony pelvis and the pelvic floor. • Bladder augmentation.
Take home messages
Take home messages • Bladder exstrophy is a rare congenital anomaly characterized by a spectrum of anomalies involving the ventral body wall, urinary tract, genitalia, bony pelvis, spine, anus, etc • Its diagnosis is clinical and does not require any additional investigations • Precise surgery to provide acceptable continence rates remains the greatest challenge for pediatric surgeons.
Get this ppt in mobile 1. Download Microsoft PowerPoint from play store. 2. Open Google assistant 3. Open Google lens. 4. Scan qr code from next slide.
Bladder Exstrophy Epispadias Complex .pptx
Get this ppt in mobile
Get my ppt collection • https://1drv.ms/u/s!AvOWIE3I3JkugQ7qQv9vsY8 pGHLf?e=CSNFK2 • https://t.me/surgerypresentation • https ://www.slideshare.net/drpradeeppande/edit_my_u ploads • https://www.dropbox.com/sh/x600md3cvj85woy/ AACVMHuQtvHvl_K8ehc3ltkEa?dl=0 • https://www.facebook.com/doctorpradeeppande/?r ef=pages_you_manage • https://t.me/+eqNYT21gmWZjMjI9

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Bladder Exstrophy Epispadias Complex .pptx

  • 1. Tips on using my ppt. 1. You can freely download, edit, modify and put your name etc. 2. Don’t be concerned about number of slides. Half the slides are blanks except for the title. 3. First show the blank slides (eg. Etiology ) > Ask students what they already know about etiology of today's topic. > Then show next slide which enumerates etiologies. 4. At the end rerun the show – show blank> ask questions > show next slide. 5. This will be an ACTIVE LEARNING SESSION x three revisions. 6. Good for self study also. Display blank slide> Think what you already know about this > Read next slide. 7. See notes for bibliography.
  • 2. Learning Objectives At the end of this session the learner will be able to describe- • Etiology • Clinical Features • Management Of Bladder exstrophy aka. Bladder- exstrophy-epispadias-complex (BEEC). / Ectopia vasicae.
  • 3. Learning Objectives 1. Introduction & History 2. Relevant Anatomy, Physiology 3. Etiology 4. Pathophysiology 5. Pathology 6. Classification 7. Clinical Features 8. Investigations 9. Management 10. Controversies 11. Prevention 12. Guidelines 13. Take home messages
  • 4. Etiology • Idiopathic • Traumatic • Infections /Infestation • Neoplastic (Benign/Malignant) • Congenital/ Genetic • Nutritional Deficiency/excess • Autoimmune • Degenerative / lifestyle • Iatrogenic • Psychosomatic • Poisoning/ Toxins/ Drug induced
  • 5. Etiology • Idiopathic • Traumatic • Infections /Infestation • Neoplastic (Benign/Malignant) • Congenital/ Genetic • Nutritional Deficiency/excess • Autoimmune • Degenerative / lifestyle • Iatrogenic • Psychosomatic • Poisoning/ Toxins/ Drug induced
  • 6. Etiology • Maternal smoking • Maternal irradiation. • Incidence less if Folate is taken during in periconceptional period.
  • 8. Pathophysiology Multiple Theories- • An abnormally large cloacal membrane causes a wedge effect and prevents the medial migration of the mesenchymal tissue. • An abnormally large cloacal membrane causes a wedge effect and prevents the medial migration of the mesenchymal tissue. • Premature rupture of the cloacal membrane • Caudal displacement of the genital tubercle, • Abnormal fluid retention leading to rupture of the embryonic bladder • Pubic diastasis
  • 10. Pathology • The lower portion of anterior abdominal wall and anterior wall of urinary bladder is missing. • Bladder-exstrophy-epispadias-complex (BEEC) depicts a spectrum of birth defects, ranging from epispadias to cloacal exstrophy at the milder and the most severe ends of the spectrum. • The classical bladder exstrophy is characterized by abnormalities involving the lower urinary tract, abdominal wall, bony pelvis, genitalia, pelvic floor, spine, and the anus. • The 'open book configuration' of the pelvis
  • 12. Clinical Features • Demography • Symptoms • Signs • Prognosis • Complications
  • 14. Demography • Incidence & Prevalence • Geographical distribution. • Race • Age • Sex • Socioeconomic status • Temporal behaviour
  • 16. Demography Incidence & Prevalence- • 3.3 per 1,00,000 live births,
  • 21. Signs
  • 22. Signs
  • 23. Signs Local Examination- • A fleshy, red mass prolapsing out of the suprapubic region represents the open bladder plate. • There is continuous urine leakage from the mass. • In male newborns, the phallus is short, stubby, and has a dorsal chordee (pointing upwards). • The glans is flattened with widely separated (divergent) corpora due to the wide pubic diastasis.
  • 24. Signs Local Examination- • The testis may not be descended at birth, and occasionally there may be the presence of bilateral inguinal hernias. • In females, the clitoris is bifid with a slightly anterior vaginal opening. • Uterine and vaginal duplications . • Anterior ectopic anus wlth patulous anal orifice with mucosal prolapse
  • 25. Signs Local Examination- • It is not uncommon for some children to present later in life. • Severe excoriation in the para-exstrophy skin, hypertrophied-polypoidal bladder plate with squamous metaplasia, poor control over bowel movements, and waddling gait are common. • Social problems due to the continuous wetting of clothes
  • 27. Prognosis • Morbidity • Mortality rate • 5 year survival in Malignancy
  • 29. Complications • Incontinence: • Upper tract damage • Upper tract damage • Sexual dysfunction • Infertility.
  • 30. Investigations • Laboratory Studies – Routine – Special • Imaging Studies • Tissue diagnosis – Cytology • FNAC – Histology – Germ line Testing and Molecular Analysis • Diagnostic Laparotomy.
  • 31. Investigations • Laboratory Studies – Routine – Special • Imaging Studies • Tissue diagnosis – Cytology • FNAC – Histology – Germ line Testing and Molecular Analysis • Diagnostic Laparotomy.
  • 34. Diagnostic Studies Imaging Studies • X-Ray • USG • CT • Angiography • MRI • Endoscopy • Nuclear scan
  • 35. Diagnostic Studies Imaging Studies • X-Ray • USG • CT • Angiography • MRI • Endoscopy • Nuclear scan
  • 39. Differential Diagnosis • Epispadias • Cloacal exstrophy -omphalocele, exstrophy of bladder, imperforate anus, and spinal abnormalities • Pseudoexstrophy • Superior Vesical Fissure • Duplicate exstrophy • Covered exstrophy
  • 41. Operative Therapy • The aims of the surgical treatment- 1. To provide a competent receptacle (bladder) for storage, 2. To prevent upper tract damage 3. To provide cosmetically acceptable genitalia providing good functional outcomes in terms of continence and sexual function.
  • 42. Operative Therapy • Modern staged repair of exstrophy (MSRE) • Complete primary repair of exstrophy (CPRE),
  • 43. Operative Therapy • Two main categories: – Reconstructive procedures – Diversion procedures.
  • 44. Operative Therapy • Bladder closure, • Epispadias repair • Bladder neck reconstruction • Bladder augmentation • Ureteric reimplantation. • Correction of abnormalities in the bony pelvis and the pelvic floor. • Bladder augmentation.
  • 46. Take home messages • Bladder exstrophy is a rare congenital anomaly characterized by a spectrum of anomalies involving the ventral body wall, urinary tract, genitalia, bony pelvis, spine, anus, etc • Its diagnosis is clinical and does not require any additional investigations • Precise surgery to provide acceptable continence rates remains the greatest challenge for pediatric surgeons.
  • 47. Get this ppt in mobile 1. Download Microsoft PowerPoint from play store. 2. Open Google assistant 3. Open Google lens. 4. Scan qr code from next slide.
  • 49. Get this ppt in mobile
  • 50. Get my ppt collection • https://1drv.ms/u/s!AvOWIE3I3JkugQ7qQv9vsY8 pGHLf?e=CSNFK2 • https://t.me/surgerypresentation • https ://www.slideshare.net/drpradeeppande/edit_my_u ploads • https://www.dropbox.com/sh/x600md3cvj85woy/ AACVMHuQtvHvl_K8ehc3ltkEa?dl=0 • https://www.facebook.com/doctorpradeeppande/?r ef=pages_you_manage • https://t.me/+eqNYT21gmWZjMjI9

Editor's Notes

  1. drpradeeppande@gmail.com 7697305442
  2. drpradeeppande@gmail.com 7697305442