University of Nairobi, Department of Surgery, Plastics surgery. Paediatric urology Bladder Exstrophy Epispadias Complex.

1. PRINCIPLES OF
EEC
MANAGEMENT
DR. ESIRU JOHN -PRAS PGY3 UON
31/05/2023
1

2. OUTLINE
• DEFINITION
• EPIDEMIOLOGY
• HISTORICS
• EMBRYOLOGY
• PATHOPHYSIOLOGY
• CLINICAL PRESENTATION
• DIAGNOSIS
• MANAGEMENT
• COMPLICATIONS
2

3. Introduction.
The exstrophy-epispadias complex,(EEC), a spectrum of GU
malformations can be as simple as a glanular epispadias or as
overwhelming as a multisystem defect, cloacal exstrophy.
It is one of the most complex anomalies in Pediatric Urology.
It is a rare spectrum of defects of GU, GI, MSKS(bony pelvis & mm).
3

4. Introduction:
Utmost distress to patient & family due to physical anomaly &
constant urine soiling.
Common presentations of the spectrum:
1.Epispadias -30%
2.Classic bladder Exstrophy (CBE) – 60%
3.Cloacal Exstrophy (CE) – 10%
Diagnosed in T2 of pregnancy may be reason to terminate.
4

5. Spectrum…
Epispadias:, least severe,
+Epispadias & mild diastasis pubis.
Classic bladder exstrophy, CBE:, most common,
+Epispadias, wide pubic diastasis, AWD & open bladder.
Cloacal exstrophy, CE:, most severe,
+Portion of caecum or hindgut separates the hemi bladders.
5

6. EEC spectrum…
CE with GI,MSK & CNS malformations is called OEIS complex
(Omphalocele, Exstrophy, Imperforate anus & Spine anomalies).
The spectrum also includes EEC Variants:
Pseudo-exstrophy,
Duplicate bladder exstrophy,
Superior vesicalfistula/fissure and
Covered exstrophy
6

7. Definitions
 EEC; is an anterior midline defect with variable expression
involving the infraumbilical AW including the pelvis, urinary
tract, & external genitalia (Gearhart and Jeffs, 1998)
Continence is correctly defined as being dry >3 h.
Social continence is daytime continence, with bed-wetting
incidences at night.
7

8. History -Descriptive
The First account of BE is ascribed to Assyro-Babylonia sources
dating from 1st & 2nd millennia 2000BC.
First recorded case of epispadias is attributed to the Byzantine
Emperor Heraclius (AD 610 – 641).
First description of bladder exstrophy to V.G Schenck in 1595.
1597: Von Grafenberg first described the medical condition
1748: Complete description by Mowat
8

9. History - Management
1780: Francois. Chaussier first coined the term “exstrophie” a Greek
“Ekstriphein” for inside out.
Early management of BE: 1. Application of external urinary receptacle,
2. Ureterosigmoidostomy, 3. Transplantation of the bladder trigone into
rectum; 4. Coverage with lateral skin flaps .
1892:Trendelenburg: described bilateral SI osteotomies & pelvic sling to
support bladder & abdominal wall closure.
1942: Hugh Hampton Young : succesful 1° closure of exstrophy patient.
1970’s: Jeffs & M.Cendron (MSRE): Mordern closure, modified in the
last decade & considered today standard Care.
9

10. Epidemiology
African data:
Complete epispadias 1 in 117,000 male vs 1 in 484,000 female
births.
CBE: 1 in 10,000 – 50,000 births, with M:F ratio of 2:1.
1 in 40,000 infants***
CE: 1 in 200,000 births
Isolated epispadias is <CBE & is diagnosed 1 in 200,000 -
400,000LBs.
***Boston Children’s Hospital in the United States of America 10

11. Presentation of Exstrophy epispadias
complex
A) Complete male epispadias B) Complete female epispadias 11

12. C) Classic bladder exstrophy D) Cloacal exstrophy
12

13. Causes
Genetic factors.
Master control gene ISL1 probably a susceptibility gene for
bladder exstrophy.
Also important in regulating urinary tract development.
13

14. Risk Factors,
Family history,
• Firstborn children,
• Children of a parent with bladder exstrophy,
• Siblings of a child with bladder exstrophy.
Race. More common in whites –Caucasians.
Sex. More males than females.
Tobacco exposure -Maternal
Young maternal age <20yrs
Multiparity
IVF & high dose progesterone, clomiphene citrate use. 14

15. Embryology - Mechanism.
 Cloacal membrane: bilaminar layer at caudal end of germinal
disk that occupies the infraumbilical abdominal wall.
 Mesenchymal ingrowth between ectodermal & endodermal
layers results in formation of LAW muscles & pelvic bones.
 Muecke, 1964: Failure of the cloacal membrane to be reinforced
by mesodermal ingrowth leads to the defect.
 Ambrose & O’Brien, 1974: Premature rupture defines the extent
of infraumbilical defect,
 Stage of development during the rupture, defines the defect.
15

16. Embryology
16

17. Etiopathogenesis.
The timing and location of rupture of cloacal membrane dictates
the presentation along the EEC spectrum.
Epispadias: if the rupture produce a division/nonunion at the distal
end of the urinary tract. Or failed migration of genital tubercle to
midline.
CBE: if rupture occurs after uro-rectal septum divides GIT from GUT.
CE: results if the rupture occurs before the separation,
17

18. Theories.
 Embryonic maldevelopment: (Marshall and Muecke (1968)
Abnormal overdevelopment of the cloacal membrane, prevents medial
migration of mesenchymal tissue for proper LAW devt.
 Patton and Barry, 1952; Ambrose and O’Brien, 1974:
Abnormal development of genital hillocks caudal to normal position, with
fusion in the midline below rather than above the cloacal membrane.
 Mildenberger et al, 1988.
Abnormal caudal insertion of body stalk, results in a failure of interposition
of mesenchymal tissue in the midline
18

19. Molecular & Genetic - Etiopathogenesis
P63, a member of the p53 tumor suppressor family, is highly
expressed in stratified epithelium including the bladder &
overlying skin. Its expression seen ↓d in CBE patients.
(Thus insertion & deletion polymorphisms of ΔNp63 lead to
the reduced p63 expression that may cause EEC)
19

20. ASSOCIATED ANOMALY
1.Gastrointestinal;
2.Urogenital Anomalies
3.Neurospinal abnormalities
4.Musculoskeletal Anomalies.
20

21. 1. Urogenital anomalies.
1.Bladder: Normal in epispadias, anteriorly exposed in both CBE & CE.
Histologically; bladder appears immature, few myelinated nerves,
thus potential for normal development after successful closure.
2. Ureters, abnormal VU-angle, thus VUR following bladder closure,
ureters are reimplanted into bladder at the time of AC or BNR.
3. PUJO, horseshoe kidney & ectopic kidney
21

22. Urogenital anomalies.
4. Spatulous dorsal urethral opening.
5. Shorter & broad-base phallus with dorsal chordee.
6. 50% Corporal bodies shortening. laterally displaced under the
pubic bones.
In CE, bifid phallus between diastatic pubis, each ½ often equal.
22

23. Signs
Low set umbilicus
Anteriorly displaced anus
Genitalia defects (cryptoorchydism)
Inguinal hernia
Urine incontinence.
Symphyseal diastasis
Abnormal bladder:
Epispadias:
VUR after the bladder is closed.
23

24. Male Genital Defect
24

25. Female - Urogenital anomalies
Patulous bladder neck: dorsally open distal urethra remains open.
Flattened Mons: & displaced laterally,
Vagina & introitus are displaced anteriorly.
Bifid clitoris in anterior vaginal wall, surrounded by divergent labia.
Mullerian anomalies: CE patients, short/stenotic vaginal or uterine
duplication or agenesis.
25

26. Female - Urogenital anomalies
26

27. 27

28. 2. Musculoskeletal Anomalies
AAW, intact in Epispadias
CBE & CE, bladder & urethra exposed thru’ a Δ defect In LAW.
Umbilical hernias, common, but often insignificant.
Indirect IH, 2° persistent PV, large inguinal rings, & relatively
straight direction of the inguinal.
Diastatic pubic rami (mean 4.8cm), with divergent rectus muscle,
28

29. 29
Anterior pelvic segment: each ½
 Externally rotated 18° &
 Average 30% shorter
Posterior segment, each ½
 Externally rotated 12°,
 Acetabular retroverted, yielding
an increased intertriradiate
distance.
Pubic diastasis:
Wider SI joint angles, more inferiorly rotated pelvis, & larger sacrum.
Pelvic deformities cause waddling gait.

30. Musculoskeletal
Anomalies
 Levator ani:
has large mean area,
More posterior to the rectum,
Externally rotated and flattened
resulting in a “boxlike, open
book” pelvis with an anteriorly
positioned bladder.
 Obturator int and ext:
outwardly rotated
These lead to incontinence &
uterine prolapse. 30

31. 3. Gastrointestinal Abnormalities
In CBE & CE Anteriorly displaced anus & sphincter, predisposed
to fecal incontinence.
CBEs, occational omphalocele, imperforate anus, rectal stenosis
or prolapse.
CE; always:
1)Omphalocele
2)Imperforate anus
3)Rudimentary hind gut,
4)Malrotated bowel
5)Short gut syndrome.
31

32. 4. Neurospinal abnormalities
7% of CBE have spinal abnormalities; Spina bifida occulta, scoliosis,
& hemivertebrae, most uncomplicated, but spinal dysraphism may
cause neurologic dysfunction.
CE: nearly all demonstrate significant neurospinal deficits including
NTDs, Vertebral anomalies, Spinal myeloplasia, Spinal dysraphism, &
tethered cord.
Necessitating neurologic eval’ with spinal US & MRI
Exercerbate urinary & bowel incontinence, lower extremity
immobility & erectile dysfuntion.
32

33. Diagnosis
Family medical history and Physical Exam.
Prenatal U/S and Ultra-fast Fetal MRI scan
• *Targeted Hight-resolution Fetal transabdominal US @≥15 WoG:,
1)Absent bladder filling,
2)Low set umbilicus +/- omphalocele
3)Pubic diastasis, & increased pelvic diameter
4)Small genitalia,
5)LAAW mass increases through pregnancy,
6)Prolapsed ileum in CE = elephant trunk appearance
7)Associated abnormalities, Myelomeningocele, limb abnormalities 33

34. Diagnosis - Prenatal
a). Sagittal US image: b) Sagittal T2-weighted fetal MR 34

35. Tests for associated anomaly
 Blood tests
 IVP
,
 Spinal x-ray of KUB, Pelvic X-Ray
 MRI and CT scans, depending
on condition
A pre-operative ultrasound evaluation of the upper tracts is
mandatory for renal and genital assessment.
35

36.  Delivery room: Umbilical cord tied
with 2-0 silk close to abdominal wall;
 Cover bladder with a nonadherent
film of plastic wrap;
 Bladder plate irrigation with warm
saline with each diaper change.
 A humidified air incubator or mist
tents to moisten the bladder plate.
 Prophylactic antibiotics
Evaluation and Management:
36

37. Management
• Multidisciplinary care surgeons, anaesthesiologists, psychologists
& nursing guarantee the most favorable outcome.
Primary principles in surgical management are:
 A secure, initial abdominal closure,
 Reconstruct a functional & cosmetically satisfactory ext-genitalia,
 Achieve urine continence while preserving renal function.
37

38. Goals of reconstruction
PRINCIPAL OBJECTIVES:
• Volitional Voiding
• Low-pressure urine storage
• Preservation of kidney function
• Functionally & cosmetically
acceptable external genitalia
SECONDARY OBJECTIVES
• Avoid UTIs
• ↓ risk of calculi.
• Minimize risk of malignancy
associated with UT
• Integrity of abdominal wall fascia &
Pelvic floor.
38

39. Perioperative
 Create a latex free environment in the operation room, many
children with BE are prone to latex allergies.
 Peri-operative broad-spectrum antibiotics are administered &
continued throughout the first post-operative week.
39

40. 3 Types of epispadias:
A. Glanular: Affects distal part of urethra.
B. Penile: Entire penile urethra,
C. Penopubic/Complete: Total deficiency of dorsal wall of urethra
& anterior wall of bladder.
40

41. A. EPISPADIAS Repair.
i. Modified Cantwell-Ransley Approach
a) Chordee release by mobilizing urethral plate from underlying glans down to prostatic
urethra, tubularized & placed in a dorsa groove incision in the glans. Doted lines an
incisin site of cvernostomies;
b)Corporal carvanosa anastomosis at dorsomedial aspects over tabularized urethra;
c) Glans closure over urethra, then skin closure. 41

42. ii. Complete “penile disassembly”.
Mitchell and Bagli modification of Cantwell-Ransley repair.
 The urethral plate & each corporeal body each with its
hemiglans dissected completely off each other.
 Urethra is the tubularized & ventralized.
“Complete penile disassembly” performed at primary bladder
closure, the combination is called “CPRE”
42

43. Complete penile disassembly
43

44. Female epispadias repair:
Due to shorter urethra, repair of isolated female epispadias is
generally done along with BNR, Monsplasty & clitoroplasty.
44

45. EVOLUTION OF SURGICAL APPROACHES
Widely practiced Surgical Approaches:
1.Mordern Stage repair of Exstrophy(MSRE)- Gearhart and Jeff, 1970.
2.Complete Primary repair of Exstrophy (CPRE)-Grady & Mitchell, 1988.
Other approaches.
1.Radical Soft Tissue Mobilization (RSTM), Kelly -1995(staged)
(detachment of Voluntary and involuntary sphincter muscles + bony attachment & wrapped
around Posterior urethra).
2. Warsaw approach:
(Two staged: 1st Bladder plate, 2nd BNR+Epispadias repair)
3. Erlangen Repair: (complete repair @ 8 weeks)
45

46. Correction CBE.
Epispadias Exstrophy
Complex (EEC)
Complete primary Repair
for Extrophy (CPRE)
Modern Staged Repair
for Exstrophy (MSRE)
Bladder Neck
Reconstruction (BNR)
Bladder Turn-in
CR Epispadias
Repair
Augmentation Cystoplasty
46

47. B). Classic Bladder Exstrophy-Repair:
This begins with closure of bladder & abdominal wall by either:
i. Modern staged repair of exstrophy (MSRE)
ii. Complete primary repair for Exstrophy (CPRE)
Timing:
 Early: (72 HoL) closure argue allows for earlier bladder cycling,
improved expansion, reduced risk of precancerous changes.
 Delayed closure: argue, no metaplasia, allows for concomitant
epispadias repair, increases likelihood of post-clossure growth
47

48. Classic Bladder Exstrophy-Repair:
Successful primary closure is of utmost importance since it is
associated with decreased overall costs, decreased
inflammation & bladder fibrosis, improved bladder growth, &
decreased need for urinary diversion.
Pelvic osteotomies: in order to deepen the flattened pelvis,
close diastasis pubis, and release tension on the abdominal wall.
48

49. i). Modern Staged Repair of Exstrophy (MSRE)
Gearhart
Involves 3 Stages:
A.Stage I 72Hrs: Bladder, posterior urethra and abdominal wall closure+ Pelvic
Osteotomy if >72hrs, or >4cm Diastasis for tension free closure
 Females: Genitoplasty & urethroplasty.
 Stage maybe delayed if; bladder template small, 3cm, polypoid or inelastic.
B. Satege II (6-12Mo):Male Epispadias closure(Phalloplasty & urethroplasty)
(after local testosterone stimulation)
C. Satge III: (Continence procedure BNR) e.g. Young-Dees-Leadbetter, delayed till
 bladder with adequate capacity (80mls) & Continence desire(5 – 9yrs age)
Its combined with ureteral reimplantation to repair VUR.
49

50. Stage I MRSE: - Bladder closure.
50

51. Suture approximation of the Pubic diastasis.
51

52. iii). Continence/antireflux procedure.
At age 5 – 9 years, when continence is required,
Bladder capacity ≥85mls
Young-Dees-Leadbetter BNR
Bilateral Cohen Ureteral reimplantation
Bowel &/or stomach segment used for AC or Continent
diversion with abdominal/perineal stoma.
Vagina Recons or augmented using colon, ileum, or FTSG
52

53. Stage III of MSRE: - BNR
The Principles of Young–Dees-Leadbetter Procedure:
Ureters reimplanted, prevents
VUR, Moves them off BNR-site.
Base of bladder reconstructed
to lengthen the urethra and
reinforce Bladder neck.
53

54. Combined Exstrophy & Epispadias
repair
In Selected newborn cases; however, requires:
 Good phallic length,
 Deep urethral & plate groove,
 Adequate penile skin.
 Reasonable bladder template.
 For patients undergoing delayed primary or re-operativ closure.
 The pre-op use of IM testosterone in re-operative exstrophy patients
improves vascularity & penile skin for recon.
54

55. ii). Complete Primary Repair Of
Exstrophy (CPRE) Howell,Zderic
Primary bladder, abd wall & Epispadias repair & partial tightening BN.
Bilateral ureteral reimplantation can be done safely(safeguards
Hydronephrosis & UTI).
D↓ cost, morbidity a/w multiple operations, stimulates early bladder
growth.
Epispadias repair is done by complete “penile disassembly”.
55

56. Initial dissection lines for male CPRE – Ventral & dorsal Views.
CPRE
56

57. Disassembly of the urethral wedge (plate+
spongiosa) from corporeal bodies.
Dissection plane maintained on the corporeal
bodies, allows the corpus spongiosa to remain
with the urethra.
Distal separation of corporeal bodies & urethral
wedge. (occasionally).
“Disassembly”, allows maximal exposure to
pelvis, optimizes dissection & posterior
positioning of bladder, neck & urethra in pelvis.
CPRE
Step 1 – Penile disassembly
57

58. Division of the intersymphyseal band (Condensation of anterior
pelvic fascia) allows tensionless posterior placement of bladder.
Deep pelvic dissection.
Suprapubic tube brought out through umbilicus.
CPRE…
Step 2 – Pelvic dissection
Ureteral catheters brought out through the urethral closure
Bladder & uretral closed in 2-layers using absorbable suture.
58

59. If the bladder capacity does not increase sufficiently following
closure (insufficient bladder capacity), augmentation
cystoplasty,(AC) maybe needed.
When the bladder is excessively fibrotic or is too small,
orthotopic neobladder or a continent catheterizable pouch
maybe needed.
59

60. Augmentation Cystoplasty & Diversion
After failed CBE closure, chance of achieving adequate bladder
capacity for a BNR & continent urethral voiding, falls to 60%.
Insufficient bladder capacity or noncompliant may need AC.
Commonly used: Colon segment, stomach or redundant ureter
for AC.
Continent diversion: required during AC for Clean intermittent
Catheterization (ileal conduit, Mitrofanoff)
60

61. Mitrofanoff Procedure
61

62. Modern Functional Recon of CE
ONE-STAGE REPAIR (FEW ANOMALIES)
Omphalocele excision
Cloacal plate separation from bladder halves
Joining bladder halves and urethroplasty
Osteotomies(BAInnominate and Vartical iliac)
Gonadectomy in males with unreconstructable
phallus
Terminal ileostomy/colostomy
Genital revision if needed.
TWO-STAGE REPAIR
• Stage I: (new born period)
Omphalocele excision
Cloacal plate separation from bladder halves
Joining bladder halves and urethroplasty
Gonadectomy in males with unreconstructable
phallus
Terminal ileostomy/colostomy.
Stage II:
Closure of joined bladder halves and urethroplasty
Osteotomies(BAInnominate and Vartical iliac)
Genital revision if needed.
62

63. C). Pelvic Osteotomies & Immobilization
 Once pelvis is no longer malleable(>72hrs of age)., ↑Surgery time, & Post-Op
complications increase, Improves success of primary closure,
 A combination of bilateral anterior transverse innominate & vertical
posterior iliac osteotomies.  Fixation pins & ExoFix devices can be left 4 – 6 weeks as pt is immobilized.
63

64. Modified Bryant’s Traction, where the hips are
placed into 90° of flexion, used if there is no
osteotomy.
Modified Buck’s traction exerts pull
longitudinally on the lower extremities, used
after osteotomy.
Spica cast also immobilize the pelvis without
need for ext-Fix or traction 64

65. Kelly Procedure, RSTM
Includes full mobilization of:
• Bladder plate,
• Urogenital diaphragm, and
• Corpora cavernosa from the medial pelvic walls,
Followed by:
• Anatomical recon with antireflux procedure,
• Bladder closure,
• Urethrocervicoplasty,
• Muscle sphincter approximation, and
• Penile/clitoral reconstruction.
65

66. Challenges in management of BEEC
This paper, applied a holistic approach to highlight the challenges faced in our institution while managing
a series of 34 cases with classic BEEC treated 2010 and 2017 and reviewing literature on these challenges. 66

67. Jumbi T1 et al
2020
67

68. Outcome Factors - Challenges
Patient factor.
• Timing of diagnosis, (either prenatal or postnatal)
• Age at presentation,
• Associated anomalies.
• Quality of bladder plate (poor quality =fibrotic, polypoid or
too small for primary repair) = unfavorable
68

69. Factors affecting outcome - Challenges
Surgical factor. (include complications)
Repair breakdown,
Incontinence after repair.
Loss to treatment follow up.
Complication after urine diversion.
“ We try to close the neck of the bladder in exstrophy
incontinence but it recanalizes and doesn’t close, and when we
try to open it in strictured urethra it closes and doesn’t open.
That makes the urethra the craziest thing to deal with”
69

70. Reconstructive Surgery
Bladder wall,
External genitalia.
Abdominal wall:
Flap surgery for wall closure.
Component dissection.
70

71. Abdominal wall closure
 Hypogastric skin, rectus
anterior fascia, & Ext obliquus
myofascial flaps.
 Are rotated medially, flipped
over, & sutured with Prolene,
 Permits tensionless abdominal
closure.
Groin Flaps
One-stage reconstruction: A) Closure of the bladder and tubularized urethra; B) Penile closure; C) Groin
71

72. 72
Anterior rectus sheath flap + paired inguinal flap- Retrograde turndown

73. A: 3 cm extent of advance upon traction;
B: Final aspect after midline closure with a 2 cm gain on each side;
C: Another case in which an even larger extent of approximation was achieved.
Anterior component separation technique
73

74. 74

75. RAM Flap Dehiscence, management post BE repair
75

76. PENILE lengthening
Z-PLASTY and Length.
76

77. Complete reconstruction: -Neophallus
Indication: Severe penile inadequacy (length & function) for sexual
intercourse.
After puberty in sexually active patients
1. Free radial forearm flap(FRFF): Gold standard
2. Pedicled ALT flap: no microsurgery, forearm scar, altered
recipient vasculature from previous surgeries.(also as free flap).
3. Radial forearm flap (RFAF) urethra & ALT wrap-around
4. Fibular flap
5. Muscle flaps:
77

78. FRFA Flap
 The design modifications include
skin paddle for reconstruction of the
navicular fossa.
Neophallus with modifications of standard
design;
 Interdigitating inset skin flaps along the
ventral suture line minimizes contraction, and
 Oblique fashioning of the coronal sulcus.
78

79. FRFF - Neophallus.
Neurovascular anastomoses
79

80. FRFF – Phalloplasty.
Tube-in-a-tube technique while flap still attached to the forearm
by vascular pedicle.
Small skin flap & a skin graft are used to create a corona &
simulate the glans.
Donor: FTSG/STSG
SPC for 10 days,
Bed rest x 1 week
Glans tattooing = before penile sensation return, 3/12 months.
Erection prosthesis = once sensitivity has fully returned
80

81. 81
Outline of the radial forearm free flap phalloplasty
on the arm. The flap is designed to include the
lateral and medial antebrachial cutaneous nerves
as well as the radial artery and veins and the
cephalic vein (A); representation of the flap
following inset (B)
The flap is divided into three sections:
The outer skin envelope of the neophallus; the de-
epithelialized portion, (separates skin & urethra);
and 3. The ulnar-sided skin paddle, which serves
as the neourethra.

82. Free ALT Phalloplasty
Candidates:
Have a pinch test of <2 cm in lateral thigh,
Insufficient ulnar artery on Allen’s test.
Adequate septocutaneous, perforator on angio-CT (or MRI).
Distal most perforator is chosen, & flap is drawn with the perforator
lying close to its proximal margin in order to gain length.
Suprafascial dissection preferred.
Flap tunneled underneath the rectus femoris +/- sartorius & a
subcutaneous tunnel to the groin.
82

83. Free ALT Phalloplasty
Urethral reconstruction:
Rarely needed, the tube-within-a-tube technique cannot be
used for urethra reconstruction unless patient is very thin.
Recon options:-prefabricated STSG; poor quality, pedicled flaps-
peritoneal, groin , superficial circumflex iliac (artery) perforator
FRFF-thin.
Coronoplasty: Performed 10/7 post-Op due to different
vascularization of the ALT compared to the RFF.
83

84. Free ALT Phalloplasty
Glandular, penile, &
cavernosal tissues are
kept at the base of the
neophallus to facilitate
sexual stimulation &
pleasure.
84

85. 85
 Markings of ALT flap including glansplasty (black).
 The descending branch of LCFA & its venae
commitantes (red & blue),
 Location of 3 perforators “dopplered”.
 Course of lateral femoral cutaneous nerve (yellow)

86. Muscle flaps.
 Free latissimus dorsi
 Bilateral pedicled gracillis flap.
Possible penile contraction( endogenous in gracilis, regen in LD),some
can therefore have penetrative sex
No skin sensation is restored, penile prosthesis contraindicated
Urethral reconstruction, if attempted, needs multiple stage techniques
86

87. Umbilicoplasty: - AVELAR Technique.
(A, B, C) show plication of the aponeurosis and (D) shortening the pedicle of the
87

88. Prognosis
The prognosis for BEECE- Complex is typically good;
Surgical correction & reconstruction can help maintain normal
kidney function.
Nevertheless, children may have kidney stones, infections,
incontinence after reconstruction
88

89. Complications of BEECE
Long-term urinary abnormalities:
• Kidney and bladder, stones,
• Kidney infections, and
• Urinary incontinence.
Continence problems
Issues with self-image
Wound dehiscence, flap necrosis and fistulation
Sexual dysfunctions and infertility (boys esp)
Prolapse cervix and or uterus
Re-operation.
Adenocarcinoma
89

90. NEW TRENDS IN MANAGEMENT
SUBMUCOSAL COLLAGEN
Periurethral submucosal collagen injection
Indication:
Stress incontinence from cervico-urethral resistance after partial
failure of BNR.
Before BNR after Urethroplasty
Role:
Increases outlet resistance
Allows bladder to stretch(gain volume)
Alternative to a re-do operation.
Can safely be repeated upto 2-3 times, need be.
90

91. FUTURE THERAPIES:
Muscle derived stem cells (MDSC) may offer benefit in
generating differentiated urothelium.
Urine isolated Stem cells.
This urothelium seems comparable with native
urothelium, provides a valuable recon tool for urinary tract
91

92. References..
Bladder Exstrophy and Epispadias Chapter 56: Dominic Frimberger, John
P
.Gearhart
Gearhart J. Exstrophy, Epispadias, and Other Bladder Anomalies, 8th,
WB Saunders, Philadelphia 2002.
Challenges in the management of bladder exstrophy. An African case
series and review of literature. Jumbi T1*, Mwika P2, Shahbal S2, Osawa
F2 and Kambuni F1
http://www.dovemed.com/healthy-living/kidney-health/
Exstrophy-Epispadias Complex | Abdominal Key
Bladder/Cloacal Exstrophy, and Prune Belly Syndrome | Obgyn Key
Hypospadias, epispadias and bladder exstrophy (slideshare.net)
Exstrophy Epispadias complex (slideshare.net)
92

93. References…
Essentials of paediatric urology,2nd edition,edited by David FM Thomas
Patrick G Duffy Anthony MK Rickwood chapter 15 Bladder exstrophy
and epispadias.
Badder Exstrophy and Epispadias William Appeadu-Mensah Piet
Hoebeke.
Essentials in Pediatric Urology, 2012: 39-56 ISBN: 978-81-308-0511-5
Editor: George Sakellaris.
Bladder exstrophy and epispadias complex ,Sofia Barbagadakis and
George Sakellaris ,Consultant Pediatric Surgeon, Department of
Pediatric Surgery, University Hospital of Heraklion Greece
Peter C Neligan Plastic surgery 4th edition CHAPTER 13 •
Reconstruction of male genital defects Stan Monstrey, Salvatore D’Arpa,
Karel Claes, Nicolas Lumen, and Piet Hoebeke.
93

94. References…
Our initial experience with the technique of complete primary repair for
bladder exstrophy Yusuf Kibar*, Christopher C. Roth, Dominic Frimberger,
Bradley P
. Kropp.
Anterior component separation technique for abdominal closure in
bladder exstrophy repair: Primary results. Francisco Nicanor Araruna
Macedo,Eduardo Corrêa Costa Jovelino Quintino de Souza Leão. Rossan
Kepler Alvim Fiorelli Leandro Totti Cavazzola..José Carlos Fraga
Umbilicoplasty | Plastic Surgery Key
Applications of the modified Cantwell–Ransley epispadias repair in the
exstrophy–epispadias complex - Journal of Pediatric Urology
(jpurol.com).
Plastic surgery class repository.
94

95. THE END
95