Surgical management of congenital urological conditions

1. Congenital conditions of the
male GUT
Dr. Munyaga Byanjo
Gen Surg III
2021

2. Outline
• Urethral anomalies
• Hypospadias
• PUV
• AUV
• Urethral strictures
• Urethral duplication
• DSD
• Renal conditions
• Renal agenesis
• MCDK
• Ureter conditions
• Ureteric duplication
• Ureterocele
• Ectopic ureter
• Bladder anomalies
• BEEC
• Urachal abnormalities

3. Hypospadias
• Abnormal ventral placement of the urethral opening anywhere along
the penis, scrotum, or perineum.
• Epidemiology 1/250
• 3 penile abnormalities:
• ventrally sited urethral meatus
• Ventral penile curvature
• Ventral deficient hooded foreskin

4. Embryology
• Y chromosome, SRY region on its short arm
• 9woa, leydig cells in testis appear, produce testosterone
• Masculinises the genital tubercle, urogenital sinus(Increase distance btn anus and
genital structures, elongate genital tubercle and formation of urethral plate)
• Urethral plate canalizes from prox to distal to form wide diamond shaped urethral
groove within the penile shaft
• Penile shaft forms as a result of fusion of medial edges of urethral folds
• 11 to 12 woa, coronal sulcus seperates penile glans from the shaft
• 16 woa, urethral folds have fused in ventral midline of penile shaft
• Normal ventral curvature or chordee resolves by 20th week
• Glanular urethra forms as a result of direct canalization of urethral plate

5. Classification
• Glanular or subcoronal meatus 50%
• Midshaft meatus 30%
• Proximal meatus 20%
This may underestimate severity in
some cases.

6. GMS score

7. • Etiology
• ?genetic susceptibility
• Maternal exposure to endocrine disruptors
• Genetic defect in 5 alpha reductase type 2(converts testosterone to DHT)
7% associated with UDT
• All boys with unilateral or bilateral undescended testes and severe proximal
hypospadias must undergo additional testing to rule out a disorder of sexual
differentiation (DSD)
• Objective of treatment
• Reconstruct straight penis for normal coitus
• Allow a forward directed stream

8. • If both gonads not palpable, consider DSD
• Age of operation 6/12 to 1 year
• Administer parenteral testosterone to increase penis size, once or
twice 3 weekly before operation

9. Steps
• Orthoplasty (straightening)
• Urethroplasty
• Meatoplasty and glanuloplasty
• Scrotoplasty
• Skin coverage

11. Plication techniques

12. Techniques
• TIP
• MAGPI
• Snodgrass
• Onlay island flap repair
• Staged repairs(Bracka..)

13. TIP

14. MAGPI

15. 2 stage

16. Prevention and management of urethroplasty
complications
• Fistulas
• Prevention
• Use of dartos flap over the neourethra was found in some study to reduce
fistula rate from 27% to 3.5%
• Correction
• Assess for distal obstruction.
• Midline ventral incision to excise the fi stula tract, repair the urethra with a
one‐layer subepithelial closure, and cover the defect with a ventral dartos
flap.
• Coronal fistulas can either be primarily repaired or require reoperative
urethroplasty.

17. Glans dehiscence
Complete separation of the glans wings resulting in a subcoronal meatus.
• Prevention
• Adequate mobilization of glans wings, reduced tension on approximation.
• Correction
• Treatment is reoperative urethroplasty and glansplasty(reoperative TIP), and
use of the extended glans wings mobilization in cases with prior dehiscence.

18. Meatal stenosis
Neomeatus <8Fr 6 months after surgery to be stenotic regardless of
symptoms
• Prevention
• Plate incision should extend to near the surface of the corpora, and
tubularization should end within approximately 3mm of the distal edge of the
plate, leaving an oval, not rounded, opening
• Correction
• Meatotomy, incise dorsally to minimize risk for recurrent hypospadias.
• May require reoperative urethroplasty and glansplasty, by reoperative TIP,
inlay grafting, or two‐stage oral graft repair depending on the extent of distal
fibrosis.

19. Urethral stricture
• Prevention
• careful tissue handling, especially in pedicle flaps
• Correction
• Direct vision internal urethrotomy (DVIU) for hypospadias strictures <1cm
• If it fails, open urethroplasty

20. Diverticulum
• Ballooning of the neourethra during voiding, typically with postvoid
dribbling.
• Seen more when Byar’s flaps are used.
• Prevention
• Use of alternative repair techniques such as TIP or two‐stage prepucial grafts
if not experienced
• Correction
• Diverticulum repair involves assessment for distal obstruction, and then
excision of excessive neourethra with retubularization and coverage with a
dartos or tunica vaginalis flap.

21. Posterior Urethral Valves
• Abnormal mucosal folds at the prostatic urethra level that function as
a valve to obstruct urine flow.
• Formed due to the failure of the Wolffian ducts to properly integrate
into the urethra resulting in a membrane which obstructs the lower
urinary tract.
• 1/25000 live male births
• Prenatal ultrasonography may show bilateral hydronephrosis, a thick-
walled bladder, dilated posterior urethra, and/or oligohydramnios.

22. Classification
• Type I: 95% - Plicae colliculi, arise from the caudal verumontanum
along the lateral margins of the urethra fuse anteriorly causing an
obstruction.
• Type II: Membranes cranially attached to the bladder neck originating
from the verumontanum. These are now thought to be due to
hypertrophy of the plicae colliculi and not obstructive valves.
• Type III: 5% - Round membrane at the caudal verumontanum with a
hole in the middle that is either above (type IIIa) the verumontanum
or below it (type IIIb). Neither subtype's hole communicates directly
with the verumontanum.

24. Clinically
• Antenatally
• Bilateral hydroureteronephrosis,
• Dilated prostatic urethra (keyhole sign)
• Distended bladder with thickened wall greater than 3 mm, with poor
emptying over 30 minutes.
• Focal renal parenchymal cysts demonstrate renal dysplasia. Other findings
include oligohydramnios, fetal ascites, or urinoma.
• Postnatal Presentation
• Delayed voiding, weak stream, urosepsis, or a palpable bladder.
• Resp distress due to pulmonary hypoplasia.

26. Management
• Evaluation
• Antenatally, maternal ultrasound is the mainstay of evaluation
• A neurogenic bladder has a keyhole appearance on ultrasound due to
the hypotonic neck.
• Serum Cr only after 48 h
• CUG

28. Management
• Antenatally:
• Vesicoamniotic shunting (preserve on pulmonary functioning)
• Drain bladder with 5 or 7 FR feeding tube. Avoid catheters with
ballooning, may cause spasms which close off ureteric orifice
• Ventilatory support in case of pulmonary hypoplasia
• Vesicostomy (for LBW)
• Cystoscopy with PUV ablation
• Upper tract diversion

29. • Preferred is ablation with vesicostomy if ablation not possible.
• In the unique case in which upper tract dilation persists after
vesicostomy and is accompanied by worsening renal parameters,
upper urinary tract diversion with a cutaneous ureterostomy or renal
pyelostomy

30. Anterior urethral valves
• 25 to 30 times less common than PUVs.
• Described as a semilunar fold draping down from the wall of the
anterior urethra interrupting urinary flow
• The embryology of anterior urethral valves is not clear, but
hypothesized to involve faulty union between urethral mucosa and
the epithelium of the fossa navicularis.
• The valves may be located at the bulbar urethra, penoscrotal junction,
or penile urethra
• Patients present with anterior urethral valves at different ages based
on the severity of the obstructive process.

31. • CP
• Postvoid dribbling andmild incontinence,
• Significant bulging of the distal penis
• Palpable bladder with obstruction
• Renal insufficiency and urinary tract infections
• CUG, shows dilated ant urethra with prox signs of chronic obstruction

32. Management:
• In a premature or small infant, a vesicostomy.
• Cystoscopy with valve ablation
• Surgical reconstruction in case of concomitant urethral diverticulum

33. Urethral Stricture
• Congenital urethral strictures are a rare urethral anomaly.
• After birth, patients present with obstructive and irritative symptoms,
such as urinary retention, dysuria, or weak stream.
• Diagnosis is made with VCUG, and treatment is with urethral
dilatation, endoscopic repair, or vesicostomy if severe.

34. Urethral duplication
• Caused by abnormal Mullerian duct termination and growth arrest of the
urogenital sinus or misalignment of the termination of the cloacal
membrane with the genital tubercle
• Classification
• Type I abnormality includes a blind incomplete urethral duplication or accessory
urethra.
• Type II is a complete patent urethral duplication
• Type III refers to urethral duplication as a component of partial or complete caudal
duplication
• Diagnosis
• Clinical
• VCUG

36. Management
• Surgical management is complex and may require a variety of single-
or multiple-stage repairs.
• A small, blind-ending accessory urethra may be conservatively
monitored or treated expeditiously with simple coagulation of the
mucosal tract with a bugbee electrode.
• Septum between the two urethral channels is incised and the dorsal
defect is repaired with reverse glans wings to cover a dorsal
urethroplasty
• In cases inwhich the urethral openings are separated by the “Y”-type
arrangement, a staged reconstruction using preputial or buccal
mucosa flaps may be required

37. Disorders of Sexual differentiation
• Embryology: inheritance of Y chromosome, has SRY gene. This drives
the bipotential gonad to differentiate to testes
• SOX 9 gene: responsible for differentiation of Sertoli cells
• DHH gene: Desert HedgeHog plays role in testicular differentiation
• Differentiated gonad(Leydig cells and Sertoli cells) give off
testosterone and anti-mullerian factor----causes development of male
internal organs
• Male external genitalia requires presence of DHt for normal
phenotypic appearance

38. Pathophysiology
• Mutations in SRY – 46XY gonadal dysgenesis
• Translocation in SRY in females – results in male or ambiguous
genitalia
• SOX 9—mutation here causes autosomaldominant campomelic
dysplasia, 46 XY genotype, ambiguous or female genitalia

39. Clinical assessment
• Examine genitalia
• Note average penile length in a terma male infant (2.8cm to 4cm)
• Palpable testis
• Measure 17-hydroxyprogesterone(exclude CAH), blood electrolytes,
serum anti-mullerian hormone and gonadotropin leves
• Karyotype (46 XXDSD, 46 XYDSD, mixed type)

40. 46xx
• Do FISH(Fluorescent In situ hybridization)
• If SRY tve ---have a gene translocation
• If SRY –ve---- measure concentration of 17 hydroxyprogesterone, if
high then CAH usualyy as a case of deficiency of 21 alpha
rhydroxylase
• If Normal 17…, pelvic USS to ascertain anatomy, if anatomy normal
then was probably exposed to androgens during gestation

41. Prader classification

42. • If USS abnormal, test for AMH levels, testosterone and HCG. Elevated
levels---ovotesticular DSD
• Mgt: Upto Prader 1-4 virilisations, recommend female gender
• Diagnosis delayed or severe virilisation, male gender
• Surgery: Restores vaginal and urethral position and corrects fistula
between vagina and urogenital sinus

43. 46xy
• If SRY negative, there was a deletion
• If SRY positive----CAH, !& alpha hydroxylase deficiency, 3-beta
hydroxyosteroid dehydrogenase
• Do ACTH stimulation to measure steroid precursors
• Or look for testicular tissue on USS or measure AMH
• If low AMH then gonadal dysgenesis, testicular regression or
vanishing testicular syndrome
• If normal AMH then there’s normal testicular reserve
• MGT: hormonal stimulation for phallic growth, hypospadias
correction and orchiopexy

44. 45X/46xY mosaic
• Virilised
• Male upbringing
• Corresct hypospadias

45. Kidney Embryology
• Urogenital ridge forms in 4th week, later forms nephrogenic cord and gonadal
ridge.
• Pro-, meso- and metanephros form sequentially innephrogenic cord.
• Metanephros persists, functional in 5th week
• Mesonephros in males forms the epididymis, vas deferens, seminal vesicles, and
the ejaculatory duct.
• The mesonephric duct extends to fuse with the cloaca and later develops an
outgrowth, the ureteric bud,
• Ureteric bud branches by week 6 to form collecting system
• Kidneys Start in pelvis and migrate to lumbar region by 8th week and by the tenth
week, they start producing urine.
• At the time of birth, each healthy kidney measures approximately 4.0 to 4.5 cm in
the longest dimension.

46. Kidney - embryology

47. Renal Agenesis
• Renal agenesis or aplasia is a missing kidney.
• 1/1000, more on the left. 1/4000 for bilateral
• Pathology:
• Early vascular insult on the developing ureteric bud around 6-7 weeks
• Failure of the proper development of the metanephros
• Contralateral kidney undergoes hypertrophy as compensatory mechanism
• Bilateral agenesis not compatible with life

48. • CP: Asymptomatic, incidental finding. May develop secondary
hypertension.
• Contralateral kidney may have anomalies such as pelviureteric
junction obstruction (PUJO) and vesicoureteral reflux (VUR).

49. Management
• Investigations
• RFTs
• USS
• Voiding CUG
• MAG 3 renography
• DMSA scintigraphy

51. Multicystic Dysplastic Kidney
• Characterized by isolated cysts of differing sizes with dysplastic
stroma associated with an atretic ureter on gross examination, have
minimal functional nephrons
• The incidence ranges from 1 in 1000 to 4300 live births
• More in males and on the left
• May involute without intervention(60% by age 10 years)
• The contralateral kidney may be affected by low-grade VUR in 15% to
28% of patients or by UPJ obstruction in 4% of patients.
• Conservative management with regular screening for hypertension
and proteinuria is needed in patients with MCDK.

52. Duplicated Collecting System
• Embryology
• Early division of the ureteric bud leads to a duplication. The more
complete the division, the more complete the duplication.
• The lower pole ureter is shorter and inserts into the bladder in a
proximal location, whereas the upper pole ureter is longer and inserts
distally—the so-called Meyer–Weigart law.
• VUR is usually present in the lower moiety of a duplicated collecting
system, whereas the upper moiety is associated with an obstructive
ectopic ureter
• Ectopic ureters in boys always lie above the sphincter, and so boys
never present with incontinence.

53. Ureteric duplication
• Complete (double ureter) or partial (bifid ureter).
• Duplication of the ureter is common. It is seen in 1% of the
population.
• Antenatal diagnosis.
• Management
• A common sheath Cohen type re-implantation is undertaken, as the ureteric
adventitia and blood supply is common between both ureters.
• If the renal function of the lower pole is very poor (dysplastic moiety)a lower
pole hemi-nephrectomy is performed, taking care not to damage the upper
pole ureter.

54. Ureterocele
• Cystic dilatation of the terminal intravesical segment of the ureter.
• It occurs in 0.2% of the population and is four to seven times more
common in females.
• They are bilateral in 10%, and 80% are associated with a duplex
system, usually being associated with the upper pole.
• Sixty per cent are located in an ectopic location.
• Ureteroceles are classified as intravesical or ectopic.
• They usually present antenatally (on maternal ultrasound scan) with
fetal hydronephrosis or clinically are associated with a UTI or, rarely,
urethral obstruction.

56. • Investigations:
• Renal USS
• CUG for VUR.
• A DMSA renal scan will determine differential parenchymal function of each
kidney and the respective poles.
• Cystoscopy.

57. Management
• Treatment is required only where obstruction or significant reflux is
present.
• Initial treatment: endoscopic incision with cold knife or cautery,
relieves obstruction
• The aim is to relieve obstruction and avoid reflux. However, most do
reflux post-operatively and antibiotic prophylaxis is usually required.
• Do a DMSA renal scan, if function is adequate (>10%) reconstruct the
distal ureters with a double barrel re-implant and occasionally
bladder wall repair.

58. • The procedure is essentially the same as a Cohen re-implant
• Excise ureterocele and cannulate both ureters with feeding tubes
• Mobilize ureters to common sheath
• The ureters are mobilized into the bladder and the wide ureter
tapered by loose plication over an 8Fr catheter. If tapering is
undertaken, a JJ stent is usually left.
• If the renal function is poor, hemi-nephrectomy is performed.

59. Ectopic ureter
• An ectopic ureter is usually found in a position caudal to the normal
ureteric orifice, along the pathway of normal development.
• More in girls
• In boys this may mean that the ureter opens at the bladder neck, prostate,
or epididymis.
• In girls it may open at the bladder neck, urethra, or vagina, and possibly
lead to incontinence.
• The further the ureter opens from the normal site, the worse the renal
function.
• In boys epididymo-orchitis may be present, and in girls continuous
incontinence with a normal voiding pattern

60. Management
• Investigations
• Renal ultrasound may show a dilated ureter behind the bladder with a duplex
kidney and a variable amount of cortex.
• CT and MRI.
• Surgical management
• If the kidney is small and non-functioning a nephro-ureterectomy is
performed
• If the kidney is functioning, the ureter is re-implanted.

61. Embryology of the bladder
• In week four, the urogenital septum divides the cloaca into two parts, the
rectum and UG sinus
• The urogenital sinus will continue to grow to form the bladder, with the
inferior end forming the urethra.
• As the mesonephric duct fuses with the cloaca, part of the duct gets
incorporated into the posterior wall of the bladder.
• As the kidneys ascend, the ureters elongate and open into the bladder
superiorly, while the roots of the mesonephric ducts are carried inferiorly,
before fusing to form the trigone region.
• Endodermal cells from the urogenital sinus soon replace the mesodermal
cells epithelium of the trigone region, thus completing development.

63. Bladder Exstrophy Epispadias Complex
• Bladder extrophy is the most common in a spectrum of conditions
ranging from the less severe epispadias through to the most severe
cloacal extrophy.
• Incidence of 1 in 100,000 live births, with a male preponderance.

64. Embryology
• The cause of this complex is thought to be the failure of the cloacal membrane to be
reinforced by ingrowth of mesoderm
• Mesenchymal ingrowth between the ectodermal and endodermal layers of the cloacal
membrane results in formation of the lower abdominal muscles and the pelvic bones.
• The cloacal membrane is subject to premature rupture, and, depending on the extent of
the infraumbilical defect and the stage of development during which the rupture occurs,
bladder exstrophy, cloacal exstrophy, or epispadias results
• Persistence of the cloacal membrane prevents medial migration of mesoderm.
• When the cloacal membrane disappears, there is no anterior bladder wall and the
posterior wall of the bladder is exposed.
• Failure of the urethra to fold on its dorsal surface leads to epispadias.
• In cloacal extrophy, the most severe form of the condition, the cloacal membrane
perforates before division of the cloaca by the urorectal septum into an anterior bladder
and a posterior rectum.

65. Associated abnormalities
• Vesico-ureteric reflux (once the bladder has been closed).
• Pubic diastasis up to 4cm. (normal 1cm)
• In boys, a short dorsally curved penis (chordee), with separated corporal
bodies which diverge to attach to the inferior pubic rami.
• In girls, the clitoris is divided on either side of the urethral plate.
• The vagina is tilted anteriorly and may be stenosed.
• Uterine duplication may occur.
• Inguinal hernias are common.
• Upper tract abnormalities occur in 60–70% of those with cloacal extrophy.
• Rectal prolapse in 10–20%.

66. Management
• Covering of the bladder plate with a non-adherent wrap or film
• Surgically:
• Modern staged repair of exstrophy (MSRE)
• Complete primary repair of exstrophy (CPRE).

67. Modern staged repair of exstrophy (MSRE)
• 3 stages
• Primary turn in to protect the lower abdominal skin from ammoniacal
contents, and prevent the bladder from trauma.
• Epispadias repair or genitalia reconstruction, is performed at 6 to 9
months of age..
• Bladder neck reconstruction is the third procedure and is performed
at a time when the child has sufficient bladder capacity (at least 100
ml) and has attained a certain age.

68. Complete primary repair of exstrophy
(CPRE): (Mitchell’s repair).
• The procedure aims at performing the complete repair, including
bladder turn-in, bladder neck repair, and epispadias repair in a single
sitting.
• The ideology is the initiation of early bladder cycling to ensure the
optimal development of the bladder.
• The procedure utilizes complete penile disassembly and division of
the intersymphyseal bands.

69. Complications
• Fascial dehiscence
• Requires immediate correction unless compounded with infection
• Catheter malfunction
• Urethrocutaneous fistula formation
• May occur in 21% of patients
• May spontaneously resolve within 7 months
• Persistent fistulae should be evaluated with urethroscopy to determine the
extent of fistula and rule out the presence of distal urethral stricture.
• Surgical repair should be delayed at least 6 months following initial closure

70. • Bladder dehiscence
• Rare
• Isolated bladder dehiscence requires a recovery period of at least 6
months prior to reclosure.
• Tension‐free reclosure with osteotomy, preoperative testosterone (for
boys), and combined epispadias‐bladder repair are important factors
in subsequent closures.
• The risk of subsequent failure increases with each reclosure.

71. • Orthopedic complications
• Within the first 48–72 hours, the pubis remains elastic and can often
be reapproximated without signifi cant tension during an exstrophy
repair. If there is any doubt regarding the pliability of the pubic bones,
osteotomy is indicated.
• Evidence suggests that osteotomy decreases the risk of developing
prolapse or wound dehiscence
• [Osteotomy can result in femoral nerve palsy, delayed union of
osteotomy, persistent sacroiliac pain, leg length inequality, and/or
osteomyelitis in up to 6% of patients.

72. • The glans, urethra, and corporal bodies are at risk for
devascularization if the terminal arteries to the penis or bulbar artery
are damaged during dissection of the corporal bodies from the pubic
symphysis

73. • Bladder outlet obstruction
• Patients are at risk for obstruction secondary to neourethral stricture or a
narrow bladder neck after the initial repair.
• Vesicoureteral refl ux
• Almost all patients experience vesicoureteral reflux following bladder closure.
Spontaneous resolution can be seen in up to 16%
• Renal deterioration
• Incontinence

74. Urachal abnormalities
Introduction
• The urachus is a normal embryonic remnant of the primitive bladder
• There are four clinical entities relating to the incomplete involution of
the urachus during embryogenesis.
• Patent urachus,
• Urachal cyst,
• Urachal sinus
• Vesicourachal diverticulum
• More common in males

75. Patent urachus
• Usually presents itself at or soon after birth when the umbilical cord
is ligated and urine drains from the umbilicus.
• Diagnosis
• Clinical
• Fluid analysis
• Management:
• Observation for up to 6 months
• Investigate and manage BOO
• If persistent, complete excision of urachus.

76. Urachal cyst
• The majority of urachal cysts develop in the lower third of the
urachus.
• Most urachal cysts go undetected unless they become infected or
enlarge to a size that causes mechanical symptoms.
• US is the most common diagnostic modality
• Treatment of urachal cysts involves complete excision.

77. Urachal sinus
• A urachal sinus most likely represents a urachal cyst that becomes
infected and dissects to the umbilicus
• These patients typically present in childhood with periumbilical pain
and tenderness and may have umbilical erythema, excoriation or
reactive granulation tissue.
• A fistulogram is usually diagnostic and will help delineate the extent
of the sinus tract.
• After treatment of the acute infection, surgical excision of the sinus
tract is recommended.

78. Vesicourachal diverticulum
• A vesicourachal diverticulum is thought to occur when there is
incomplete obliteration and closure of the lower portion of the
urachus and the bladder apex.
• A vesicourachal diverticulum is frequently seen in a child with prune
belly syndrome.
• Asymptomatic
• No treatment is usually necessary since this anomaly is primarily
morphological and bears no functional consequences.