EXSTROPHY-EPISPADIAS COMPLEX

Deepak K Thakur
MS, MCh(Urology)
• Introduction
• Presentation
• Evaluation
• Management
 Introduction

• Spectrum :
• Epispadias
• Bladder extrophy
• Cloacal extrophy
 Embryology

• Theory of Muecke, 1964:

– failure of the cloacal membrane to be reinforced by
ingrowth of mesoderm
• Theory of Marshall and Muecke,1968:
– the basic defect is an abnormal overdevelopment of the
cloacal membrane during the 4th week of gestation,
which prevents medial migration of the mesenchymal
tissue and proper lower abdominal wall development
• The timing of the rupture of this defective cloacal
membrane determines the variant of the
exstrophy-epispadias complex
Embryology
 Risk Factors

• Genetic: breakpoint disruption in CASPR3gene on

chromosome 9
• ART
• Progesterone in the early part of the first
trimester
• Periconception maternal exposure to smoking
 Manifestations

• Skeletal Defects:
– rotational and dimensional abnormalities of pelvis
– spina bifida occulta,
– lumbarization or sacralization of vertebrae
• Pelvic Floor Defects:
– Posterioriorly displaced pelvic floor muscles
– Anterior paucity of pelvic floor muscles
• Abdominal Wall Defects:
– triangular defect occupied by extrophy bladder & posterior
urethra
– indirect inguinal hernias
 Manifestations
• Anorectal Defects:
– The perineum is short and broad and the anus is displaced anteriorly
– imperforate anus/rectal stenosis/congenital rectal prolapse
• Male Genital Defects:
– corporeal bodies separated and triangular in shape
– a long convex ventral surface and a short wedge-shaped dorsal
surface
• Female Genital Defects:
– The vagina is shorter than normal, hardly greater than 6 cm in
depth, but of normal caliber
– The vaginal orifice is frequently stenotic and displaced anteriorly,
– the clitoris is bifid, and the labia, mons pubis, and clitoris are
– divergent
– The cervix enters the vagina superiorly
 Manifestations

• Urinary Defects:
• The upper tract is usually normal but malformations
may occur as
– duplicated collecting system
– hypoplastic or absent kidney,
– pelvic kidney,
– ureteropelvic junction obstruction
 Presentation

• Incidence: to be 2.2 cases/100,000 live births

• M:F= 5 : 1 to 6 : 1
• Classical picture
• Urine coming from lower abdominal wall
 Evaluation

• Prenatal Diagnosis: USG

• Postnatal Diagnosis:
 C/F
 USG
 CT/MRI
 Management

• Modern staged repair of exstrophy (MSRE):

 At birth: functional closure to convert the bladder
exstrophy into a complete epispadias

 At 6 months: epispadias repair

 At 4 to 5 years: Bladder neck repair

 Management-Epispadias Repair

• Components:
 correction of dorsal chordee
 urethral reconstruction
 glanular reconstruction, and
 penile skin closure
• Techniques:
Cantwell-Ransley repair
 modified Cantwell-Ransley repair (1995),
 penile disassembly technique(Mitchell and Bägli )