SlideShare une entreprise Scribd logo
1  sur  62
Télécharger pour lire hors ligne
PRUNE-BELLY SYNDROME
Gaurav Nahar
DNB Urology Resident
MMHRC, Madurai
INTRODUCTION
• A constellation of anomalies with variable degrees of severity.
• Three characteristic findings:
1. a deficiency of abdominal musculature,
2. bilateral intra-abdominal testes, and
3. an anomalous urinary tract.
• Single most important determinant of long-term survival-
severity of the urinary tract anomaly, in particular, degree of
renal dysplasia.
Also known as:
• Triad syndrome,
• Eagle-Barrett syndrome, and
• Abdominal musculation syndrome
Urinary tract anomalies:
variable degrees of
• Hydronephrosis,
• Renal dysplasia,
• Dilated tortuous ureters,
• Enlarged bladder, and
• Dilated prostatic urethra
Additional associated
anomalies:
• Respiratory tract,
• Gastrointestinal tract,
• Cardiac system, and
• Musculoskeletal system.
...flashback
• Frolich(1839) first described the characteristic
abdominal wall in PBS.
• Parker(1895) described the full triad of
anomalies.
• Osler(1901) coined the term Prune-belly
syndrome d/t characteristic abdominal wall
findings.
EPIDEMIOLOGY
• Incidence: 1 in 29,000 to 1 in 40,000 live
births(similar as bladder exstrophy).
• 95% cases in males.
• PBS females exhibit abdominal wall deficiency and
urinary tract dysmorphism without any gonadal
anomaly.
• Higher incidence noted in twins, blacks, and children
born to younger mothers.
• Incidence declining in developed countries because of
prenatal diagnosis and a decision to terminate the
pregnancy.
GENETICS
• Most cases sporadic with normal karyotype.
• Reported association with
 Turner syndrome
 Monosomy 16
 Trisomy 13
 Trisomy 18
 Beckwith Wiedemann syndrome.
Proposed inheritance patterns- X-linked recessive, 2-
step autosomal dominant, sex-influenced autosomal
recessive & polygenic transmission.
EMBRYOLOGY
• Exact mechanism unclear.
• Four main theories:
(1) Early in utero posterior urethral obstruction resulting
in severe dilation of urinary tract and possible fetal
ascites and oligohydramnios.
(2) Primary defect in lateral plate mesoderm (precursor
of ureters, bladder, prostate, urethra, and
gubernaculum).
(3) Intrinsic defect of urinary tract leading to ureteral
dilation and fetal ascites
(4) A yolk sac defect.
CLINICAL FEATURES
GENITOURINARY ANOMALIES:
Kidneys:
• Spectrum of anomalies range from normal
renal parenchyma to dysplasia.
• Dysplasia in 50%.
• Severe degree of renal collecting system
dilatation- characteristic.
• Calyceal morphology well-preserved.
• Severely dysplastic or dilated kidney.
• Non-obstructive hydronephrosis.
• Primary or secondary UPJO.
Ureters:
• Dilated, tortuous & redundant.
• Distal ureters more severely affected.
• Vesicoureteral reflux(VUR) in 75% pts.
• Histology- a lack of smooth muscle cells and
an increase in fibrous connective tissue.
• Ratio of collagen to smooth muscle cells in
prune-belly ureters is elevated.
• Decreased number of thick and thin myofibrils
(ultrastructural examination) contributes to
poor peristalsis.
• UPJ & UVJ obstruction- uncommon.
• Ineffective ureteral peristalsis because of poor
ureteral wall coaptation.
• Severity of urinary tract abnormalities is not
proportional to flaccidity of abdominal wall.
Prune belly syndrome
• Excretory urogram (A to
C) demonstrating the
variable degree of HUN in
PBS.
• Note the preservation of
calyceal architecture,
despite severe ureteral
dilation in C.
• D, Dilated tortuous
refluxing ureters as seen on
a voiding
cystourethrogram
Bladder:
• Massively enlarged + Urachal
pseudodiverticulum.
• Patent urachus in 25-30%.
• Despite being very thick, bladder wall is
smooth.
• Increased ratio of collagen to muscle fibers in
the absence of obstruction.
• Delayed first sensation to void and a large
capacity.
• A significant postvoid residual may result
from a relative outlet obstruction and inability
of bladder to generate sufficient pressure with
a detrusor contraction.
• Despite these limitations, 50% of PBS patients
void spontaneously with normal voiding
pressures, normal flow rates, and low postvoid
residuals.
• Trigone is splayed with ureteric orifices
displaced laterally and superiorly(? cause of
VUR).
VCUG of a child with PBS demonstrating urethral
atresia, urachal diverticulum, and VUR
Prostate and Accessory Sex Organs:
• Posterior urethral dilatation d/t prostatic hypoplasia,
k/a type 4 valve- angulation of urethra during
voiding.
• Related to abnormal mesenchymal-epithelial
development.
• Reduction of both epithelial and smooth muscle cells
and increase in connective tissue cells.
• Various obstructive lesions of distal posterior
urethra:- urethral atresia, valves, urethral stenosis,
urethral membrane, and urethral diverticulum- occur
in 20% of cases.
• Prostatic hypoplasia may cause ejaculatory failure.
• Vas deferens and seminal vesicles are atretic; may be
dilated or thickened.
• Epididymis may be poorly attached to the testis (as is
seen commonly in abdominal undescended testes).
• Lack of continuity between efferent ductules and rete
testis.
• Retrograde ejaculation because of an incompetent
bladder neck.
Anterior urethra:
• Usually normal.
• Most common anomalies: urethral atresia or
hypoplasia and megalourethra.
• Unless associated with a patent urachus,
urethral atresia is lethal.
• Spontaneous bladder rupture with fistula
formation also may occur.
In PBS, two types of megalourethra seen.
Fusiform type:
• a deficiency of corpus
cavernosum + spongiosum.
• Entire phallus dilates with
voiding.
• results from a mesenchymal
deficiency of urethral folds.
Scaphoid variety:
• a deficiency of spongiosum
only with preservation of
glans and corpora
cavernosa.
• Ventral urethra dilates with
voiding.
• results from a mesenchymal
deficiency of urethral
supportive tissues.
• Megalourethra is more commonly seen in PBS
than any other syndrome.
• Transient in utero obstruction of junction
between glanular & penile urethra- proposed
cause of megalourethra.
Testes:
• Bilateral intra-abdominal testes lying over iliac
vessels and adjacent to dilated ureters- most
typical findings.
• Some authors found no difference in germ cell
counts, Ad spermatogonia, and Leydig cells
between PBS testes and non-PBS intra-
abdominal testes; others found decreased
numbers.
Prune belly syndrome
• Infertility caused by a combination of testicular
histologic abnormalities, structural defects of the
ducts, and prostatic abnormalities.
• No PBS pts.have fathered a child. More recently,
paternity achieved by sperm retrieval techniques and
intracytoplasmic sperm injection(ICSI).
• Normal pregnancy with assisted vaginal delivery-
described in a female PBS patient.
EXTRA GENITOURINARY
ABNORMALITIES:
75% have non–urinary
tract
abnormalities
Abdominal wall defect:
• Most characteristic feature in newborn-
appearance of abdominal wall.
• M.c.- uneven involvement, medial and inferior
musculature most deficient.
• Totally absent abdominal wall musculature, in
some cases.
• Appearance at birth is that of wrinkled,
redundant skin with bulging flanks. Intra-
abdominal organs can be discerned through the
thinned abdominal wall.
• The most severely affected areas may have
skin, subcutaneous fat, and a single fibrous
layer on the peritoneum.
• More vulnerable to respiratory illness because
their cough effectiveness is compromised
• Good wound healing.
• "Pot-belly" appearance in adults.
Wrinkled abdomen in PBS newborns
Cardiac Anomalies:
• Occur in 10% of children with PBS
• Patent ductus arteriosus,
• Atrial septal defect,
• Ventricular septal defect, and
• Tetralogy of Fallot.
Pulmonary:
• Pulmonary hypoplasia (sec. to severe
oligohydramnios related to renal dysplasia or
severe bladder outlet obstruction)
• Pneumothorax and pneumomediastinum can
be seen
• Pneumonia and lobar atelectasis( ineff. cough)
Gastrointestinal Abnormalities
• 30% of cases.
• Result from incomplete rotation of midgut
giving way to a wide mesentery
• Int. malrotation, volvulus, atresia, stenosis
• Splenic torsion
• Omphalocele, gastroschisis, and anorectal
abnormalities
• Chr.constipation and acquired megacolon( sec.
to dec. intra abd. pressure)
Orthopedic:
• 30% to 45%,
• Result from compressive effects of
oligohydramnios
• Dimpling of lateral aspect of the knees is a
common finding.
• Talipes equinovarus (26%), hip dysplasia
(5%), and congenital scoliosis(4%).
PRENATAL DIAGNOSIS & MANAGEMENT
• Prenatal ultrasonography- major role
• Fetal hydronephrosis,
• Distended bladder,
• Irregular abdominal circumference.
classic
findings
30 weeks
Massively dilated bladder filling most of the abdominal cavity.
Note the lack of amniotic fluid.
A dilated bladder with a urachal diverticulum(arrow) and an
elongated and dilated posterior urethra
Neonatal Presentation
Spectrum of disease
• Cardiac or pulmonary often should take precedence
over the urinary tract( in the absence of BOO)
Category 1 Category 2 Category 3
Oligohydramnios Marked
Moderate to
severe
mild/nil
BOO severe( uret. atresia) nil
Pulm Hypoplasia severe mild nil
Prognosis
succumb within few
days / still born
variable good
Urologic
intervention
Not usual- cath. drain Mx controversy
nil or if rec.
UTI/VUR
Early reconstr. - for VUR/ red.
cystoplasty (after 3 mths age)
cons.mx/limited sx
Adult Presentation
• Incomplete forms of PBS- present into
adulthood.
• Symptoms of renal failure and hypertension.
Female Syndrome- 5%
• BOO with anorectal anomalies similar to
males
EVALUATION & MANAGEMENT
• Requires a team consisting of a neonatologist, a
nephrologist, and a urologist.
• Major initial concern is that of management of
cardiac and respiratory issues- do CXR.
• Pts.with BOO- SPC initially.
• BUN,creat,electrolytes - assess renal insuff, met.
acidosis.
• S.Creat <0.7 % predictive of adeq.renal function.
• Circumcision is advisable- reduce risk of
infant urinary tract infections.
• Prophylactic antibiotic therapy is
recommended- before VCUG.
• VCUG- assess Bladder emptying, outlet( esp.
in renal insuff)
• Avoid early VCUG - in normal renal function,
patent urachus.
• DTPA/MAG3 to assess outflow obstruction in
massive HN & stasis(4-6 weeks of age).
SURGICAL MANAGEMENT
Three components:
• Urinary tract reconstruction,
• Abdominal wall reconstruction, and
• Orchidopexy
Supravesical Urinary Diversion
• Indications: Repeated upper tract infections or
deterioration of renal function.
• Cut. Pyeloplasty/ureterostomy( UPJO/UVJO)
Cutaneous Vesicostomy
• Indications: Acute renal failure, urinary sepsis, or
bladder outlet obstruction from urethral atresia.
• OR Excise large urachal diverticulum
Internal Urethrotomy
• True anatomic obstruction- rare in PBS
• Used in "Unbalanced Urethrovesical function"- with
large PVR.
• Does not result in incontinence.
Reduction Cystoplasty:
• Poor bladder contractibility leads to
incomplete and infrequent emptying.
• Remodeling into a more spherical shape to
better direct the contractible forces.
• Simple excision of urachal diverticulum or
excision of redundant mucosa with overlaping
between flaps.
Anterior Urethral Reconstruction:
• Urethral atesia/hypoplasia- progresive UD
not successful.
• Urethroplasty with skin flaps / grafts
• Megalourethra- redundant urethra excised,
and reconstructed over catheter.
Prune belly syndrome
Ureteral Reconstruction:
• Indications: Repeated nonsuppressible UTI or
with progressive upper tract deterioration.
• Ureteral reimplantation may be technically
challenging- abnormal bladder.
Orchidopexy:
• Timing of orchidopexy- early: to preserve
normal hormonal function.
• Transabdominal Orchidopexy- at 6 months
current approach of choice.
• If adequate mobilisation not possible -
• Fowler-Stephens orchidopexy(Single or
multi-staged)
• Microvascular autotransplantation.
Operative photograph showing increased vacularity along vas
deferens 4 months after first-stage Fowler-Stephens orchidopexy
Reconstruction of Abdominal Wall:
• Mild degree- may show improvement with
age.
• Benefits- improved bladder emptying, more
effective cough and improvement in
defecation.
• Timing - can be combined with other urinary
tract reconstructions, even at 6 months.
Prune-belly syndrome patient demonstrating preoperative
appearance of abdominal wall (A), estimated extent of abdominal
wall resection (B), and immediate postoperative appearance (C).
A and B, Anterior and lateral views of the abdomen of a 14-year-old boy who underwent
major surgical remodeling of the urinary tract during early infancy with good results.
Note typical abdominal configuration. C and D, Anterior and lateral views of the same
boy 1 month after undergoing abdominoplasty with the technique described by Monfort
Techniques:
Randolph Technique-
• Transverse incision from 12th rib to pubic
symphysis to opposite 12th rib with full-
thickness removal of skin, lower abdominal
musculature, and peritoneum.
• Healthy fascia is then approximated to anterior
iliac spines, pubic tubercle, and inferior fascia.
• Disadv.- Lateral abdominal bulge persists.
Ehrlich Technique-
• Vertical midline incision, preservation of
umbilicus on a vascular pedicle from inferior
epigastric artery.
• Skin and subcutaneous tissues are elevated off
the muscle and fascial layers, and
• an overlapping, vest-over-pants advancement
of each side to contralateral flank is
performed, preserving the less affected lateral
muscles and fascia.
Monfort Technique-
• An elliptically oriented incision isolates the redundant skin,
extending from the tip of xiphoid to pubis. A second incision
is made around umbilicus to preserve it in situ.
• Skin and subcutaneous tissue are dissected off the attenuated
fascia and muscle with dissection extending laterally to
anterior axillary line.
• Vertical fascial incisions are made lateral to superior epigastric
arteries, leaving a central fascial bridge.
• If intraabdominal surgery is necessary, excellent exposure to
urinary tract or abdominal testes is afforded through these
lateral fascial incisions.
• The lateral fascia is then advanced over central fascial bridge
from both sides, alleviating redundancy and increasing
thickness of abdominal wall.
Prune belly syndrome
Prune belly syndrome
Modification of Ehrlich & Monfort technique
Prune belly syndrome
LONG-TERM OUTLOOK
• If the nadir value is less than 0.7 mg/dL, renal
function tends to be stable
• 30% of patients- with impaired renal function -
develop ESRD at adolscence - need renal
transplant.
Thank you !!!
Prune belly syndrome

Contenu connexe

Tendances

Management and surgical procedures of Hirschsprung disease
Management and surgical procedures of Hirschsprung disease Management and surgical procedures of Hirschsprung disease
Management and surgical procedures of Hirschsprung disease Thorlikonda Sasidhar
 
Posterior urethral valves- Pediatric Surgery
Posterior urethral valves- Pediatric SurgeryPosterior urethral valves- Pediatric Surgery
Posterior urethral valves- Pediatric SurgerySelvaraj Balasubramani
 
Omphalocele vs gastroschisis
Omphalocele vs gastroschisisOmphalocele vs gastroschisis
Omphalocele vs gastroschisisRusila Divere
 
Ectopic ureter & ureterocoele
Ectopic ureter & ureterocoeleEctopic ureter & ureterocoele
Ectopic ureter & ureterocoeleGAURAV NAHAR
 
Pediatric urology:Prune Belly Syndrome(PBS)
Pediatric urology:Prune Belly Syndrome(PBS)Pediatric urology:Prune Belly Syndrome(PBS)
Pediatric urology:Prune Belly Syndrome(PBS)GovtRoyapettahHospit
 
Congenital diaphragmatic hernia by Dr. Varsha Atul Shah
Congenital diaphragmatic hernia by Dr. Varsha Atul ShahCongenital diaphragmatic hernia by Dr. Varsha Atul Shah
Congenital diaphragmatic hernia by Dr. Varsha Atul ShahVarsha Shah
 
vesicourethral reflux
vesicourethral refluxvesicourethral reflux
vesicourethral refluxRia Saira
 
Undescended testis
Undescended testisUndescended testis
Undescended testisGAURAV NAHAR
 
Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis & management
Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis &  managementPediatric urology :Posterior Urethral Valve (PUV)- diagnosis &  management
Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis & managementGovtRoyapettahHospit
 

Tendances (20)

Management and surgical procedures of Hirschsprung disease
Management and surgical procedures of Hirschsprung disease Management and surgical procedures of Hirschsprung disease
Management and surgical procedures of Hirschsprung disease
 
Posterior urethral valves- Pediatric Surgery
Posterior urethral valves- Pediatric SurgeryPosterior urethral valves- Pediatric Surgery
Posterior urethral valves- Pediatric Surgery
 
Omphalocele vs gastroschisis
Omphalocele vs gastroschisisOmphalocele vs gastroschisis
Omphalocele vs gastroschisis
 
Ectopic ureter & ureterocoele
Ectopic ureter & ureterocoeleEctopic ureter & ureterocoele
Ectopic ureter & ureterocoele
 
Posterior Urethral Valve
Posterior Urethral ValvePosterior Urethral Valve
Posterior Urethral Valve
 
Pediatric urology:Prune Belly Syndrome(PBS)
Pediatric urology:Prune Belly Syndrome(PBS)Pediatric urology:Prune Belly Syndrome(PBS)
Pediatric urology:Prune Belly Syndrome(PBS)
 
Biliary Atresia
Biliary AtresiaBiliary Atresia
Biliary Atresia
 
Congenital diaphragmatic hernia by Dr. Varsha Atul Shah
Congenital diaphragmatic hernia by Dr. Varsha Atul ShahCongenital diaphragmatic hernia by Dr. Varsha Atul Shah
Congenital diaphragmatic hernia by Dr. Varsha Atul Shah
 
vesicourethral reflux
vesicourethral refluxvesicourethral reflux
vesicourethral reflux
 
Undescended testis
Undescended testisUndescended testis
Undescended testis
 
Posterior urethral valve
Posterior urethral valvePosterior urethral valve
Posterior urethral valve
 
Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis & management
Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis &  managementPediatric urology :Posterior Urethral Valve (PUV)- diagnosis &  management
Pediatric urology :Posterior Urethral Valve (PUV)- diagnosis & management
 
Neonatal hydronephrosis
Neonatal hydronephrosisNeonatal hydronephrosis
Neonatal hydronephrosis
 
Choledochal cyst
Choledochal cystCholedochal cyst
Choledochal cyst
 
Malrotation
MalrotationMalrotation
Malrotation
 
Malrotation
MalrotationMalrotation
Malrotation
 
Biliary atresia
Biliary atresiaBiliary atresia
Biliary atresia
 
Puj obstruction
Puj obstructionPuj obstruction
Puj obstruction
 
Exstrophy Epispadias complex
Exstrophy Epispadias complexExstrophy Epispadias complex
Exstrophy Epispadias complex
 
Antenatal hydronephrosis
Antenatal hydronephrosisAntenatal hydronephrosis
Antenatal hydronephrosis
 

En vedette

Urological trauma during O/G procedures
Urological trauma during O/G proceduresUrological trauma during O/G procedures
Urological trauma during O/G proceduresGAURAV NAHAR
 
MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...
MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...
MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...GAURAV NAHAR
 
Flexible Uretero-renoscopy or RIRS
Flexible Uretero-renoscopy or RIRSFlexible Uretero-renoscopy or RIRS
Flexible Uretero-renoscopy or RIRSGAURAV NAHAR
 
Cystic liver lesions - An ultrasound perspective
Cystic liver lesions - An ultrasound perspectiveCystic liver lesions - An ultrasound perspective
Cystic liver lesions - An ultrasound perspectiveSamir Haffar
 
UNDERACTIVE DETRUSOR
UNDERACTIVE DETRUSORUNDERACTIVE DETRUSOR
UNDERACTIVE DETRUSORGAURAV NAHAR
 
Transplant in abnormal bladder
Transplant in abnormal bladderTransplant in abnormal bladder
Transplant in abnormal bladderGAURAV NAHAR
 
Locally advanced Prostate Cancer
Locally advanced Prostate CancerLocally advanced Prostate Cancer
Locally advanced Prostate CancerGAURAV NAHAR
 
Post obstructive diuresis
Post obstructive diuresisPost obstructive diuresis
Post obstructive diuresisGAURAV NAHAR
 
RETROPERITONEAL FIBROSIS
RETROPERITONEAL FIBROSISRETROPERITONEAL FIBROSIS
RETROPERITONEAL FIBROSISGAURAV NAHAR
 
Metabolic Evaluation in Urolithiasis
Metabolic Evaluation in UrolithiasisMetabolic Evaluation in Urolithiasis
Metabolic Evaluation in UrolithiasisGAURAV NAHAR
 
Detrusor Sphincter Dyssynergia
Detrusor Sphincter DyssynergiaDetrusor Sphincter Dyssynergia
Detrusor Sphincter DyssynergiaGAURAV NAHAR
 
Trus biopsy prostate
Trus biopsy prostateTrus biopsy prostate
Trus biopsy prostateGAURAV NAHAR
 
Urodynamic studies
Urodynamic studiesUrodynamic studies
Urodynamic studiesGAURAV NAHAR
 
Premature Ejaculation
Premature EjaculationPremature Ejaculation
Premature EjaculationGAURAV NAHAR
 
Ambiguous genitalia
Ambiguous genitaliaAmbiguous genitalia
Ambiguous genitaliaGAURAV NAHAR
 

En vedette (17)

Urological trauma during O/G procedures
Urological trauma during O/G proceduresUrological trauma during O/G procedures
Urological trauma during O/G procedures
 
MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...
MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...
MANAGEMENT OF BIOCHEMICAL RECURRENCE AFTER RADICAL PROSTATECTOMY & RADIATION ...
 
Nocturia
NocturiaNocturia
Nocturia
 
Flexible Uretero-renoscopy or RIRS
Flexible Uretero-renoscopy or RIRSFlexible Uretero-renoscopy or RIRS
Flexible Uretero-renoscopy or RIRS
 
Wilms tumor
Wilms tumorWilms tumor
Wilms tumor
 
Cystic liver lesions - An ultrasound perspective
Cystic liver lesions - An ultrasound perspectiveCystic liver lesions - An ultrasound perspective
Cystic liver lesions - An ultrasound perspective
 
UNDERACTIVE DETRUSOR
UNDERACTIVE DETRUSORUNDERACTIVE DETRUSOR
UNDERACTIVE DETRUSOR
 
Transplant in abnormal bladder
Transplant in abnormal bladderTransplant in abnormal bladder
Transplant in abnormal bladder
 
Locally advanced Prostate Cancer
Locally advanced Prostate CancerLocally advanced Prostate Cancer
Locally advanced Prostate Cancer
 
Post obstructive diuresis
Post obstructive diuresisPost obstructive diuresis
Post obstructive diuresis
 
RETROPERITONEAL FIBROSIS
RETROPERITONEAL FIBROSISRETROPERITONEAL FIBROSIS
RETROPERITONEAL FIBROSIS
 
Metabolic Evaluation in Urolithiasis
Metabolic Evaluation in UrolithiasisMetabolic Evaluation in Urolithiasis
Metabolic Evaluation in Urolithiasis
 
Detrusor Sphincter Dyssynergia
Detrusor Sphincter DyssynergiaDetrusor Sphincter Dyssynergia
Detrusor Sphincter Dyssynergia
 
Trus biopsy prostate
Trus biopsy prostateTrus biopsy prostate
Trus biopsy prostate
 
Urodynamic studies
Urodynamic studiesUrodynamic studies
Urodynamic studies
 
Premature Ejaculation
Premature EjaculationPremature Ejaculation
Premature Ejaculation
 
Ambiguous genitalia
Ambiguous genitaliaAmbiguous genitalia
Ambiguous genitalia
 

Similaire à Prune belly syndrome

Congenital anomalies of pancreas and hepatobiliary system radiology
Congenital anomalies of pancreas and hepatobiliary system radiologyCongenital anomalies of pancreas and hepatobiliary system radiology
Congenital anomalies of pancreas and hepatobiliary system radiologyVidya TK
 
Evaluation of urogenital sinus and cloacal anomalies
Evaluation of urogenital sinus and cloacal anomaliesEvaluation of urogenital sinus and cloacal anomalies
Evaluation of urogenital sinus and cloacal anomaliesDr. Manoj Deepak
 
Pelvi ureteric junction obstruction in children
Pelvi ureteric junction obstruction in childrenPelvi ureteric junction obstruction in children
Pelvi ureteric junction obstruction in childrenAseesh Varma
 
USG Fetal Gut .pptx
USG Fetal Gut .pptxUSG Fetal Gut .pptx
USG Fetal Gut .pptxdypradio
 
Imaging in Paediatric Intestinal Obstruction
Imaging in Paediatric Intestinal ObstructionImaging in Paediatric Intestinal Obstruction
Imaging in Paediatric Intestinal ObstructionDr. Soe Moe Htoo
 
3.Management of HSD and ARM.pptx
3.Management of HSD and ARM.pptx3.Management of HSD and ARM.pptx
3.Management of HSD and ARM.pptxBedrumohammed2
 
CONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptx
CONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptxCONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptx
CONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptxXavier875943
 
Obstructive jaundice in neonate.ppt
Obstructive jaundice in neonate.pptObstructive jaundice in neonate.ppt
Obstructive jaundice in neonate.pptshashi singh
 
Kelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptx
Kelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptxKelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptx
Kelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptxikaseptyarini2
 
Biliary atresia lulu
Biliary atresia  luluBiliary atresia  lulu
Biliary atresia luluLulu Naushad
 
Common pediatric surgical problems
Common pediatric surgical problemsCommon pediatric surgical problems
Common pediatric surgical problemspune2013
 
Intestinal obstruction by Dr.Usman Haqqani
Intestinal obstruction by Dr.Usman HaqqaniIntestinal obstruction by Dr.Usman Haqqani
Intestinal obstruction by Dr.Usman HaqqaniUsman Haqqani
 
oshiba prune belly syndrome.pptx
oshiba prune belly syndrome.pptxoshiba prune belly syndrome.pptx
oshiba prune belly syndrome.pptxahmed eshiba
 
Disorders of Gu system by Abhi
Disorders of Gu system by AbhiDisorders of Gu system by Abhi
Disorders of Gu system by AbhiAbhishek Joshi
 
Constipation&amp;incontinence
Constipation&amp;incontinenceConstipation&amp;incontinence
Constipation&amp;incontinencemostafa hegazy
 

Similaire à Prune belly syndrome (20)

Congenital anomalies of pancreas and hepatobiliary system radiology
Congenital anomalies of pancreas and hepatobiliary system radiologyCongenital anomalies of pancreas and hepatobiliary system radiology
Congenital anomalies of pancreas and hepatobiliary system radiology
 
Evaluation of urogenital sinus and cloacal anomalies
Evaluation of urogenital sinus and cloacal anomaliesEvaluation of urogenital sinus and cloacal anomalies
Evaluation of urogenital sinus and cloacal anomalies
 
Pelvi ureteric junction obstruction in children
Pelvi ureteric junction obstruction in childrenPelvi ureteric junction obstruction in children
Pelvi ureteric junction obstruction in children
 
USG Fetal Gut .pptx
USG Fetal Gut .pptxUSG Fetal Gut .pptx
USG Fetal Gut .pptx
 
Imaging in Paediatric Intestinal Obstruction
Imaging in Paediatric Intestinal ObstructionImaging in Paediatric Intestinal Obstruction
Imaging in Paediatric Intestinal Obstruction
 
3.Management of HSD and ARM.pptx
3.Management of HSD and ARM.pptx3.Management of HSD and ARM.pptx
3.Management of HSD and ARM.pptx
 
colacal and ARM.pptx
colacal and ARM.pptxcolacal and ARM.pptx
colacal and ARM.pptx
 
CONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptx
CONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptxCONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptx
CONGENITAL RENAL ABNORMALITIES By Dr. Enobong Runcie(0).pptx
 
Obstructive jaundice in neonate.ppt
Obstructive jaundice in neonate.pptObstructive jaundice in neonate.ppt
Obstructive jaundice in neonate.ppt
 
Kelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptx
Kelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptxKelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptx
Kelainan Kongenital pada Sistem Gastrointestinal, Hepatobilier,.pptx
 
biliary atresia
biliary atresiabiliary atresia
biliary atresia
 
Congenital anomalies ppt
Congenital anomalies pptCongenital anomalies ppt
Congenital anomalies ppt
 
Biliary atresia lulu
Biliary atresia  luluBiliary atresia  lulu
Biliary atresia lulu
 
Common pediatric surgical problems
Common pediatric surgical problemsCommon pediatric surgical problems
Common pediatric surgical problems
 
Intestinal obstruction by Dr.Usman Haqqani
Intestinal obstruction by Dr.Usman HaqqaniIntestinal obstruction by Dr.Usman Haqqani
Intestinal obstruction by Dr.Usman Haqqani
 
oshiba prune belly syndrome.pptx
oshiba prune belly syndrome.pptxoshiba prune belly syndrome.pptx
oshiba prune belly syndrome.pptx
 
Disorders of Gu system by Abhi
Disorders of Gu system by AbhiDisorders of Gu system by Abhi
Disorders of Gu system by Abhi
 
Constipation&amp;incontinence
Constipation&amp;incontinenceConstipation&amp;incontinence
Constipation&amp;incontinence
 
Acquired intestinal ileus
Acquired intestinal ileusAcquired intestinal ileus
Acquired intestinal ileus
 
Ureterocele,
Ureterocele,Ureterocele,
Ureterocele,
 

Dernier

power point presentation of Clinical evaluation of strabismus
power point presentation of Clinical evaluation  of strabismuspower point presentation of Clinical evaluation  of strabismus
power point presentation of Clinical evaluation of strabismusChandrasekar Reddy
 
DNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptx
DNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptxDNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptx
DNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptxMAsifAhmad
 
QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...
QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...
QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...Ganesan Yogananthem
 
Physiology of Smooth Muscles -Mechanics of contraction and relaxation
Physiology of Smooth Muscles -Mechanics of contraction and relaxationPhysiology of Smooth Muscles -Mechanics of contraction and relaxation
Physiology of Smooth Muscles -Mechanics of contraction and relaxationMedicoseAcademics
 
SGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdf
SGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdfSGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdf
SGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdfHongBiThi1
 
Male Infertility, Antioxidants and Beyond
Male Infertility, Antioxidants and BeyondMale Infertility, Antioxidants and Beyond
Male Infertility, Antioxidants and BeyondSujoy Dasgupta
 
CONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdf
CONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdfCONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdf
CONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdfDolisha Warbi
 
concept of total quality management (TQM).
concept of total quality management (TQM).concept of total quality management (TQM).
concept of total quality management (TQM).kishan singh tomar
 
Pharmacokinetic Models by Dr. Ram D. Bawankar.ppt
Pharmacokinetic Models by Dr. Ram D.  Bawankar.pptPharmacokinetic Models by Dr. Ram D.  Bawankar.ppt
Pharmacokinetic Models by Dr. Ram D. Bawankar.pptRamDBawankar1
 
MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.whalesdesign
 
Female Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before PregnancyFemale Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before PregnancyMedicoseAcademics
 
Role of Soap based and synthetic or syndets bar
Role of  Soap based and synthetic or syndets barRole of  Soap based and synthetic or syndets bar
Role of Soap based and synthetic or syndets barmohitRahangdale
 
Physiotherapy Management of Rheumatoid Arthritis
Physiotherapy Management of Rheumatoid ArthritisPhysiotherapy Management of Rheumatoid Arthritis
Physiotherapy Management of Rheumatoid ArthritisNilofarRasheed1
 
"Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio..."Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio...Sujoy Dasgupta
 
The Importance of Mental Health: Why is Mental Health Important?
The Importance of Mental Health: Why is Mental Health Important?The Importance of Mental Health: Why is Mental Health Important?
The Importance of Mental Health: Why is Mental Health Important?Ryan Addison
 
General_Studies_Presentation_Health_and_Wellbeing
General_Studies_Presentation_Health_and_WellbeingGeneral_Studies_Presentation_Health_and_Wellbeing
General_Studies_Presentation_Health_and_WellbeingAnonymous
 
Basic structure of hair and hair growth cycle.pptx
Basic structure of hair and hair growth cycle.pptxBasic structure of hair and hair growth cycle.pptx
Basic structure of hair and hair growth cycle.pptxkomalt2001
 
BENIGN BREAST DISEASE
BENIGN BREAST DISEASE BENIGN BREAST DISEASE
BENIGN BREAST DISEASE Mamatha Lakka
 
Male Infertility Panel Discussion by Dr Sujoy Dasgupta
Male Infertility Panel Discussion by Dr Sujoy DasguptaMale Infertility Panel Discussion by Dr Sujoy Dasgupta
Male Infertility Panel Discussion by Dr Sujoy DasguptaSujoy Dasgupta
 
SGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdf
SGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdfSGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdf
SGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdfHongBiThi1
 

Dernier (20)

power point presentation of Clinical evaluation of strabismus
power point presentation of Clinical evaluation  of strabismuspower point presentation of Clinical evaluation  of strabismus
power point presentation of Clinical evaluation of strabismus
 
DNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptx
DNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptxDNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptx
DNA nucleotides Blast in NCBI and Phylogeny using MEGA Xi.pptx
 
QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...
QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...
QUESTIONS & ANSWERS FOR QUALITY ASSURANCE, RADIATIONBIOLOGY& RADIATION HAZARD...
 
Physiology of Smooth Muscles -Mechanics of contraction and relaxation
Physiology of Smooth Muscles -Mechanics of contraction and relaxationPhysiology of Smooth Muscles -Mechanics of contraction and relaxation
Physiology of Smooth Muscles -Mechanics of contraction and relaxation
 
SGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdf
SGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdfSGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdf
SGK LEUKEMIA KINH DÒNG BẠCH CÂU HẠT HAY.pdf
 
Male Infertility, Antioxidants and Beyond
Male Infertility, Antioxidants and BeyondMale Infertility, Antioxidants and Beyond
Male Infertility, Antioxidants and Beyond
 
CONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdf
CONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdfCONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdf
CONNECTIVE TISSUE (ANATOMY AND PHYSIOLOGY).pdf
 
concept of total quality management (TQM).
concept of total quality management (TQM).concept of total quality management (TQM).
concept of total quality management (TQM).
 
Pharmacokinetic Models by Dr. Ram D. Bawankar.ppt
Pharmacokinetic Models by Dr. Ram D.  Bawankar.pptPharmacokinetic Models by Dr. Ram D.  Bawankar.ppt
Pharmacokinetic Models by Dr. Ram D. Bawankar.ppt
 
MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.MedMatch: Your Health, Our Mission. Pitch deck.
MedMatch: Your Health, Our Mission. Pitch deck.
 
Female Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before PregnancyFemale Reproductive Physiology Before Pregnancy
Female Reproductive Physiology Before Pregnancy
 
Role of Soap based and synthetic or syndets bar
Role of  Soap based and synthetic or syndets barRole of  Soap based and synthetic or syndets bar
Role of Soap based and synthetic or syndets bar
 
Physiotherapy Management of Rheumatoid Arthritis
Physiotherapy Management of Rheumatoid ArthritisPhysiotherapy Management of Rheumatoid Arthritis
Physiotherapy Management of Rheumatoid Arthritis
 
"Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio..."Radical excision of DIE in subferile women with deep infiltrating endometrio...
"Radical excision of DIE in subferile women with deep infiltrating endometrio...
 
The Importance of Mental Health: Why is Mental Health Important?
The Importance of Mental Health: Why is Mental Health Important?The Importance of Mental Health: Why is Mental Health Important?
The Importance of Mental Health: Why is Mental Health Important?
 
General_Studies_Presentation_Health_and_Wellbeing
General_Studies_Presentation_Health_and_WellbeingGeneral_Studies_Presentation_Health_and_Wellbeing
General_Studies_Presentation_Health_and_Wellbeing
 
Basic structure of hair and hair growth cycle.pptx
Basic structure of hair and hair growth cycle.pptxBasic structure of hair and hair growth cycle.pptx
Basic structure of hair and hair growth cycle.pptx
 
BENIGN BREAST DISEASE
BENIGN BREAST DISEASE BENIGN BREAST DISEASE
BENIGN BREAST DISEASE
 
Male Infertility Panel Discussion by Dr Sujoy Dasgupta
Male Infertility Panel Discussion by Dr Sujoy DasguptaMale Infertility Panel Discussion by Dr Sujoy Dasgupta
Male Infertility Panel Discussion by Dr Sujoy Dasgupta
 
SGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdf
SGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdfSGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdf
SGK RỐI LOẠN TOAN KIỀM ĐHYHN RẤT HAY VÀ ĐẶC SẮC.pdf
 

Prune belly syndrome

  • 1. PRUNE-BELLY SYNDROME Gaurav Nahar DNB Urology Resident MMHRC, Madurai
  • 2. INTRODUCTION • A constellation of anomalies with variable degrees of severity. • Three characteristic findings: 1. a deficiency of abdominal musculature, 2. bilateral intra-abdominal testes, and 3. an anomalous urinary tract. • Single most important determinant of long-term survival- severity of the urinary tract anomaly, in particular, degree of renal dysplasia.
  • 3. Also known as: • Triad syndrome, • Eagle-Barrett syndrome, and • Abdominal musculation syndrome
  • 4. Urinary tract anomalies: variable degrees of • Hydronephrosis, • Renal dysplasia, • Dilated tortuous ureters, • Enlarged bladder, and • Dilated prostatic urethra Additional associated anomalies: • Respiratory tract, • Gastrointestinal tract, • Cardiac system, and • Musculoskeletal system.
  • 5. ...flashback • Frolich(1839) first described the characteristic abdominal wall in PBS. • Parker(1895) described the full triad of anomalies. • Osler(1901) coined the term Prune-belly syndrome d/t characteristic abdominal wall findings.
  • 6. EPIDEMIOLOGY • Incidence: 1 in 29,000 to 1 in 40,000 live births(similar as bladder exstrophy). • 95% cases in males. • PBS females exhibit abdominal wall deficiency and urinary tract dysmorphism without any gonadal anomaly. • Higher incidence noted in twins, blacks, and children born to younger mothers. • Incidence declining in developed countries because of prenatal diagnosis and a decision to terminate the pregnancy.
  • 7. GENETICS • Most cases sporadic with normal karyotype. • Reported association with  Turner syndrome  Monosomy 16  Trisomy 13  Trisomy 18  Beckwith Wiedemann syndrome. Proposed inheritance patterns- X-linked recessive, 2- step autosomal dominant, sex-influenced autosomal recessive & polygenic transmission.
  • 8. EMBRYOLOGY • Exact mechanism unclear. • Four main theories: (1) Early in utero posterior urethral obstruction resulting in severe dilation of urinary tract and possible fetal ascites and oligohydramnios. (2) Primary defect in lateral plate mesoderm (precursor of ureters, bladder, prostate, urethra, and gubernaculum). (3) Intrinsic defect of urinary tract leading to ureteral dilation and fetal ascites (4) A yolk sac defect.
  • 9. CLINICAL FEATURES GENITOURINARY ANOMALIES: Kidneys: • Spectrum of anomalies range from normal renal parenchyma to dysplasia. • Dysplasia in 50%. • Severe degree of renal collecting system dilatation- characteristic. • Calyceal morphology well-preserved.
  • 10. • Severely dysplastic or dilated kidney. • Non-obstructive hydronephrosis. • Primary or secondary UPJO.
  • 11. Ureters: • Dilated, tortuous & redundant. • Distal ureters more severely affected. • Vesicoureteral reflux(VUR) in 75% pts. • Histology- a lack of smooth muscle cells and an increase in fibrous connective tissue. • Ratio of collagen to smooth muscle cells in prune-belly ureters is elevated.
  • 12. • Decreased number of thick and thin myofibrils (ultrastructural examination) contributes to poor peristalsis. • UPJ & UVJ obstruction- uncommon. • Ineffective ureteral peristalsis because of poor ureteral wall coaptation. • Severity of urinary tract abnormalities is not proportional to flaccidity of abdominal wall.
  • 14. • Excretory urogram (A to C) demonstrating the variable degree of HUN in PBS. • Note the preservation of calyceal architecture, despite severe ureteral dilation in C. • D, Dilated tortuous refluxing ureters as seen on a voiding cystourethrogram
  • 15. Bladder: • Massively enlarged + Urachal pseudodiverticulum. • Patent urachus in 25-30%. • Despite being very thick, bladder wall is smooth. • Increased ratio of collagen to muscle fibers in the absence of obstruction. • Delayed first sensation to void and a large capacity.
  • 16. • A significant postvoid residual may result from a relative outlet obstruction and inability of bladder to generate sufficient pressure with a detrusor contraction. • Despite these limitations, 50% of PBS patients void spontaneously with normal voiding pressures, normal flow rates, and low postvoid residuals. • Trigone is splayed with ureteric orifices displaced laterally and superiorly(? cause of VUR).
  • 17. VCUG of a child with PBS demonstrating urethral atresia, urachal diverticulum, and VUR
  • 18. Prostate and Accessory Sex Organs: • Posterior urethral dilatation d/t prostatic hypoplasia, k/a type 4 valve- angulation of urethra during voiding. • Related to abnormal mesenchymal-epithelial development. • Reduction of both epithelial and smooth muscle cells and increase in connective tissue cells. • Various obstructive lesions of distal posterior urethra:- urethral atresia, valves, urethral stenosis, urethral membrane, and urethral diverticulum- occur in 20% of cases.
  • 19. • Prostatic hypoplasia may cause ejaculatory failure. • Vas deferens and seminal vesicles are atretic; may be dilated or thickened. • Epididymis may be poorly attached to the testis (as is seen commonly in abdominal undescended testes). • Lack of continuity between efferent ductules and rete testis. • Retrograde ejaculation because of an incompetent bladder neck.
  • 20. Anterior urethra: • Usually normal. • Most common anomalies: urethral atresia or hypoplasia and megalourethra. • Unless associated with a patent urachus, urethral atresia is lethal. • Spontaneous bladder rupture with fistula formation also may occur.
  • 21. In PBS, two types of megalourethra seen. Fusiform type: • a deficiency of corpus cavernosum + spongiosum. • Entire phallus dilates with voiding. • results from a mesenchymal deficiency of urethral folds. Scaphoid variety: • a deficiency of spongiosum only with preservation of glans and corpora cavernosa. • Ventral urethra dilates with voiding. • results from a mesenchymal deficiency of urethral supportive tissues.
  • 22. • Megalourethra is more commonly seen in PBS than any other syndrome. • Transient in utero obstruction of junction between glanular & penile urethra- proposed cause of megalourethra.
  • 23. Testes: • Bilateral intra-abdominal testes lying over iliac vessels and adjacent to dilated ureters- most typical findings. • Some authors found no difference in germ cell counts, Ad spermatogonia, and Leydig cells between PBS testes and non-PBS intra- abdominal testes; others found decreased numbers.
  • 25. • Infertility caused by a combination of testicular histologic abnormalities, structural defects of the ducts, and prostatic abnormalities. • No PBS pts.have fathered a child. More recently, paternity achieved by sperm retrieval techniques and intracytoplasmic sperm injection(ICSI). • Normal pregnancy with assisted vaginal delivery- described in a female PBS patient.
  • 26. EXTRA GENITOURINARY ABNORMALITIES: 75% have non–urinary tract abnormalities
  • 27. Abdominal wall defect: • Most characteristic feature in newborn- appearance of abdominal wall. • M.c.- uneven involvement, medial and inferior musculature most deficient. • Totally absent abdominal wall musculature, in some cases. • Appearance at birth is that of wrinkled, redundant skin with bulging flanks. Intra- abdominal organs can be discerned through the thinned abdominal wall.
  • 28. • The most severely affected areas may have skin, subcutaneous fat, and a single fibrous layer on the peritoneum. • More vulnerable to respiratory illness because their cough effectiveness is compromised • Good wound healing. • "Pot-belly" appearance in adults.
  • 29. Wrinkled abdomen in PBS newborns
  • 30. Cardiac Anomalies: • Occur in 10% of children with PBS • Patent ductus arteriosus, • Atrial septal defect, • Ventricular septal defect, and • Tetralogy of Fallot.
  • 31. Pulmonary: • Pulmonary hypoplasia (sec. to severe oligohydramnios related to renal dysplasia or severe bladder outlet obstruction) • Pneumothorax and pneumomediastinum can be seen • Pneumonia and lobar atelectasis( ineff. cough)
  • 32. Gastrointestinal Abnormalities • 30% of cases. • Result from incomplete rotation of midgut giving way to a wide mesentery • Int. malrotation, volvulus, atresia, stenosis • Splenic torsion • Omphalocele, gastroschisis, and anorectal abnormalities • Chr.constipation and acquired megacolon( sec. to dec. intra abd. pressure)
  • 33. Orthopedic: • 30% to 45%, • Result from compressive effects of oligohydramnios • Dimpling of lateral aspect of the knees is a common finding. • Talipes equinovarus (26%), hip dysplasia (5%), and congenital scoliosis(4%).
  • 34. PRENATAL DIAGNOSIS & MANAGEMENT • Prenatal ultrasonography- major role • Fetal hydronephrosis, • Distended bladder, • Irregular abdominal circumference. classic findings 30 weeks
  • 35. Massively dilated bladder filling most of the abdominal cavity. Note the lack of amniotic fluid.
  • 36. A dilated bladder with a urachal diverticulum(arrow) and an elongated and dilated posterior urethra
  • 38. • Cardiac or pulmonary often should take precedence over the urinary tract( in the absence of BOO) Category 1 Category 2 Category 3 Oligohydramnios Marked Moderate to severe mild/nil BOO severe( uret. atresia) nil Pulm Hypoplasia severe mild nil Prognosis succumb within few days / still born variable good Urologic intervention Not usual- cath. drain Mx controversy nil or if rec. UTI/VUR Early reconstr. - for VUR/ red. cystoplasty (after 3 mths age) cons.mx/limited sx
  • 39. Adult Presentation • Incomplete forms of PBS- present into adulthood. • Symptoms of renal failure and hypertension. Female Syndrome- 5% • BOO with anorectal anomalies similar to males
  • 40. EVALUATION & MANAGEMENT • Requires a team consisting of a neonatologist, a nephrologist, and a urologist. • Major initial concern is that of management of cardiac and respiratory issues- do CXR. • Pts.with BOO- SPC initially. • BUN,creat,electrolytes - assess renal insuff, met. acidosis. • S.Creat <0.7 % predictive of adeq.renal function.
  • 41. • Circumcision is advisable- reduce risk of infant urinary tract infections. • Prophylactic antibiotic therapy is recommended- before VCUG. • VCUG- assess Bladder emptying, outlet( esp. in renal insuff) • Avoid early VCUG - in normal renal function, patent urachus. • DTPA/MAG3 to assess outflow obstruction in massive HN & stasis(4-6 weeks of age).
  • 42. SURGICAL MANAGEMENT Three components: • Urinary tract reconstruction, • Abdominal wall reconstruction, and • Orchidopexy Supravesical Urinary Diversion • Indications: Repeated upper tract infections or deterioration of renal function. • Cut. Pyeloplasty/ureterostomy( UPJO/UVJO)
  • 43. Cutaneous Vesicostomy • Indications: Acute renal failure, urinary sepsis, or bladder outlet obstruction from urethral atresia. • OR Excise large urachal diverticulum Internal Urethrotomy • True anatomic obstruction- rare in PBS • Used in "Unbalanced Urethrovesical function"- with large PVR. • Does not result in incontinence.
  • 44. Reduction Cystoplasty: • Poor bladder contractibility leads to incomplete and infrequent emptying. • Remodeling into a more spherical shape to better direct the contractible forces. • Simple excision of urachal diverticulum or excision of redundant mucosa with overlaping between flaps.
  • 45. Anterior Urethral Reconstruction: • Urethral atesia/hypoplasia- progresive UD not successful. • Urethroplasty with skin flaps / grafts • Megalourethra- redundant urethra excised, and reconstructed over catheter.
  • 47. Ureteral Reconstruction: • Indications: Repeated nonsuppressible UTI or with progressive upper tract deterioration. • Ureteral reimplantation may be technically challenging- abnormal bladder.
  • 48. Orchidopexy: • Timing of orchidopexy- early: to preserve normal hormonal function. • Transabdominal Orchidopexy- at 6 months current approach of choice. • If adequate mobilisation not possible - • Fowler-Stephens orchidopexy(Single or multi-staged) • Microvascular autotransplantation.
  • 49. Operative photograph showing increased vacularity along vas deferens 4 months after first-stage Fowler-Stephens orchidopexy
  • 50. Reconstruction of Abdominal Wall: • Mild degree- may show improvement with age. • Benefits- improved bladder emptying, more effective cough and improvement in defecation. • Timing - can be combined with other urinary tract reconstructions, even at 6 months.
  • 51. Prune-belly syndrome patient demonstrating preoperative appearance of abdominal wall (A), estimated extent of abdominal wall resection (B), and immediate postoperative appearance (C).
  • 52. A and B, Anterior and lateral views of the abdomen of a 14-year-old boy who underwent major surgical remodeling of the urinary tract during early infancy with good results. Note typical abdominal configuration. C and D, Anterior and lateral views of the same boy 1 month after undergoing abdominoplasty with the technique described by Monfort
  • 53. Techniques: Randolph Technique- • Transverse incision from 12th rib to pubic symphysis to opposite 12th rib with full- thickness removal of skin, lower abdominal musculature, and peritoneum. • Healthy fascia is then approximated to anterior iliac spines, pubic tubercle, and inferior fascia. • Disadv.- Lateral abdominal bulge persists.
  • 54. Ehrlich Technique- • Vertical midline incision, preservation of umbilicus on a vascular pedicle from inferior epigastric artery. • Skin and subcutaneous tissues are elevated off the muscle and fascial layers, and • an overlapping, vest-over-pants advancement of each side to contralateral flank is performed, preserving the less affected lateral muscles and fascia.
  • 55. Monfort Technique- • An elliptically oriented incision isolates the redundant skin, extending from the tip of xiphoid to pubis. A second incision is made around umbilicus to preserve it in situ. • Skin and subcutaneous tissue are dissected off the attenuated fascia and muscle with dissection extending laterally to anterior axillary line. • Vertical fascial incisions are made lateral to superior epigastric arteries, leaving a central fascial bridge. • If intraabdominal surgery is necessary, excellent exposure to urinary tract or abdominal testes is afforded through these lateral fascial incisions. • The lateral fascia is then advanced over central fascial bridge from both sides, alleviating redundancy and increasing thickness of abdominal wall.
  • 58. Modification of Ehrlich & Monfort technique
  • 60. LONG-TERM OUTLOOK • If the nadir value is less than 0.7 mg/dL, renal function tends to be stable • 30% of patients- with impaired renal function - develop ESRD at adolscence - need renal transplant.