Pediatric urology:Prune Belly Syndrome(PBS)

PRUNE BELLY
SYNDROME
DEPT OF UROLOGY
GOVT ROYAPETTAH HOSPITAL AND KILPAUK MEDICAL COLLEGE
CHENNAI 1

MODERATORS:
Professors:
Prof. Dr. G. Sivasankar, M.S., M.Ch.,
Prof. Dr. A. Senthilvel, M.S., M.Ch.,
Asst Professors:
Dr. J. Sivabalan, M.S., M.Ch.,
Dr. R. Bhargavi, M.S., M.Ch.,
Dr. S. Raju, M.S., M.Ch.,
Dr. K. Muthurathinam, M.S., M.Ch.,
Dr. D. Tamilselvan, M.S., M.Ch.,
Dr. K. Senthilkumar, M.S., M.Ch.
DEPT OF UROLOGY,GRH ANDKMC,CHENNAI. 2

PRUNE
3
DEPT OF UROLOGY,GRH ANDKMC,CHENNAI.

HISTORY
4

ALSO KNOWN AS…
1. Triad syndrome
2. Eagle-Barrett Syndrome
3. Abdominal musculation syndrome
5

EPIDEMIOLOGY
Incidence 1in 29000 to 1 in 40000 live births.
95% in Males
Higher incidence in twins, blacks and children born to younger mothers.
Incidence in twins is discordant.
6

GENETICS
Normal karyotype is the rule.
Association Noted in:
Turner syndrome
Monosomy 16
Trisomy 13
Trisomy 18
Beckwith-Wiedemann Syndrome
7

1. Abdominal musculature deficiency
2. Bilateral intraabdominal testes
3. Anomalous urinary tract
3 MAJOR
FINDINGS
8

PSEUDOPRUNE
Females with prune belly syndrome and
Males who do not have the complete triad of prune belly syndrome.
Hence, males with a lax abdominal wall but without undescended testes or without a
dilated urinary tract and all females are considered pseudoprunes.
9

DIFFERENTIAL DIAGNOSES
Nonimmune ascites,
Posterior urethral valves,
Amniotic band syndrome,
Visceromeglia,
Polycystic kidneys,
Impaired lymphatic drainage in Turner’s syndrome,
Giant liver cyst and
Cystic adenomatoid malformation of the lung.
10

PATHOGENESIS - THEORIES
1. Early in utero posterior urethral obstruction resulting in severe dilatation of urinary
tract and possible fetal ascites and oligohydramnios.
2. Primary defect in the lateral plate mesoderm
3. An intrinsic defect of the urinary tract leading to ureteral dilatation
4. Yolk sac defect
11

URETHRAL OBSTRUCTION THEORY
12

OBSTRUCTION CAUSES
True areas of stenosis, atresia, valves, or pinpoint diaphragms at the junction of the
posterior and membranous urethra.
“Functional obstruction” present in utero, secondary to prostatic hypoplasia, as the
specific etiology of the syndrome.
This “obstruction” is thought to be the result of conformational changes in the prostatic
urethra during voiding, creating a valve-like mechanism.
13

MESENCHYMAL DYSPLASIA
14

RENAL DYSPLASIA – MECHANISMS (STEPHEN)
(1) Defects of the ureteric bud or its branches,
(2) Qualitative or quantitative deficiencies of the nephrogenic mesenchyme, and
(3) Vascular ischemic insults with resultant ureteric obstruction and renal cystic
dysplasia.
(4) Due to a combination of a ureteric bud and metanephric defect.
15

Mesenchymal
Abnormality
Urethral Obstruction
Renal Dysplasia
PCS and ureter
dilatation
Decreased Urine
Output
Oligohydramnios
Urinary Ascites
Abdominal Wall
Defect
Pulmonary
Hypoplasia
Orthopedic
Abnormalities
Patent Urachus
16

1. Renal anomalies
2. Ureteric anomalies
3. Bladder anomalies
4. Prostate and accessory sex organs
5. Urethral anomalies
6. Testicular anomalies
UROGENITAL
ANOMALIES
17

RENAL ANOMALIES
Renal dysplasia present in 50% of cases.
Spectrum varies from normal renal parenchyma to dysplasia.
Potter Type II and Type IV varieties are present.
Renal collecting system is dilated.
Non obstructive hydronephrosis is the rule.
Ureteropelvic junction obstruction can occur in primary or secondary basis.
18

URETERAL ANOMALIES
Dilated, tortuous and redundant ureters.
Proximal ureters are less abnormal than distal ureters.
Massive dilation and stenosis can occur at all levels.
VUR present in 75% of children.
Ratio of collagen to smooth muscle is elevated in refluxing ureters.
19

Infection
Stasis
Ineffective
Peristalsis
Dilated
Ureters
20

BLADDER ANOMALIES
Enlarged with pseudodiverticulum at the urachus.
Patent urachus in 25% to 30%.
Ratio of collagen to smooth muscle is increased without obstruction.
Smooth muscle hypertrophy seen in obstructed prune bladder.
21

Dilated
ureter and
PCS
Dilated
bladder
Urethral
obstruction
22

Ureteric
Reflux
Displaced
Ureteric
Orifices
Splaying
of
Trigone
Dilated
Bladder
23

UNBALANCED
VOIDING
Dilated
Bladder
Delayed First
Sensation
Ineffective
Contraction
Increased PVR
24

PROSTATIC ANOMALIES
Dilated posterior urethra
Obstructive lesions – Urethral atresia, urethral stenosis, urethral membrane and
Urethral diverticulum (20%)
Type IV valve – Angulation of the urethra during voiding
25

Dilated
Posterior
urethra
Prostatic
Hypoplasia
Abnormal
Mesenchymal
Development
26

Retrograde
Ejaculation
Incompetent
Bladder
Neck
Dilated
Prostatic
Urethra
27

ACCESSORY SEXUAL ORGANS ANOMALIES
Atretic vas and seminal vesicles
Poor attachment of epididymis to the testis
Lack of continuity between efferent ductules and rete testis.
Infertility is a normal in Prune Belly syndrome.
28

URETHRAL ANOMALIES
Urethral atresia/Hypoplasia (Associated with Patent urachus)
Megalourethra – Transient in utero obstruction of junction between glanular and
penile urethra
Two types:
1. Scaphoid variety – Deficiency of spongiosum only
2. Fusiform variety - Deficiency of spongiosum and cavernosum.
29

MEGALOURETHRA
A newborn with prune belly syndrome
with evidence of megalourethra and
penoscrotal transposition.
30

MEGALOURETHRA
(a) A newborn with prune belly
syndrome and megalourethra.
(b) Voiding cystourethrogram in this
patient demonstrates a large dilated
bladder, open bladder neck, dilated
posterior urethra, and wide, patent
megalourethra.
31

ABDOMINAL WALL DEFECT
Medial and inferior muscular segments
are typically involved.
The order of severity of involvement
from most to least involved is:
Transversus abdominis,
Rectus abdominis below the umbilicus,
Internal oblique,
External oblique, and
Rectus abdominis above the umbilicus.
32

ABDOMINAL WALL DEFECT
Most severely affected areas may have
skin, subcutaneous fat and a single
fibrous layer on the peritoneum.
Some patients show improvement in
muscle tonus as they grow.
Poor support of lower chest wall.
Vulnerable to respiratory infections.
33

EXTRA GENITOURINARY ABNORMALITIES
Cardiopulmonary (49%)
Orthopedic (65%)
Gastrointestinal (65%)
34

Orthopedic (65%)
Pulmonary hypoplasia
Pneumothorax
Pneumomediastinum
Lobar atelectasis
Pneumonia
Chronic bronchitis
Patent ductus arteriosus
Atrial septal defect
Ventricular septal defect
Tetralogy of Fallot
35

Orthopedic (65%)
Congenital dislocation of hips
Chest wall deformity: Pectus excavatum/
carinatum
Scoliosis
Genu valgum
Talipes equinovarus
Severe leg maldevelopment: Arthrogryposis
Knee Dimple
36

ORTHOPEDIC ANOMALIES
Pectus excavatum, scoliosis noted in a
older patient with Prune belly syndrome
37

KNEE DIMPLE
Lateral dimple of the knee is a mild
manifestation of the compression effects
of intrauterine oligohydramnios.
38

Orthopedic (65%)
Malrotation
Intestinal atresia
Intestinal stenosis
Volvulus
Anorectal agenesis
Imperforate anus
Omphalocele
Gastroschisis
Hepatobiliary anomalies
Acquired megacolon
VACTERL anomalies
39

PBS WITH IMPERFORATE ANUS
Newborn with prune-belly syndrome and
imperforate anus
40

PRENATAL DIAGNOSIS
USG is similar to other causes of congenital bladder outlet obstruction.
Diagnosis can be made as early as 11 to 14 weeks.
Classical finding: HUN, Distended bladder and irregular abdominal wall
circumference.
But not consistently present even at 30 weeks.
41

PRENATAL USG
(A) Massively dilated bladder that fills most of the abdominal cavity. Note the lack of amniotic fluid.
(B) The cephalic portion of the bladder reaching the level of both kidneys with hydronephrosis and renal
parenchyma noted.
(C) A dilated bladderwith a urachal diverticulum (arrow) and an elongated and dilated posterior urethra.
42

PRENATAL MANAGEMENT
Some have recommended in utero intervention for relief of urinary tract dilatation
and oligohydramnios.
Prenatal intervention has no benefit in terms of postnatal renal function.
Urethral atresia with progressive oligohydramnios- Only circumstance in which
prenatal intervention is justified.
43

NEONATAL PRESENTATION
Appearance of the abdominal wall immediately suggests the diagnosis of PBS.
Cardiac and pulmonary abnormalities take precedence over urogenital abnormalities
in the absence of true bladder outlet obstruction.
44

WOODARD’S CLASSIFICATION
45

MULTIDISCIPLINARY EVALUATION
Neonatologist
Nephrologist
Urologist
Cardiologist
Orthopedician
46

INITIAL EVALUATION
Sr. Creatinine and electrolytes – rule out systemic acidosis and electrolyte imbalances.
Baseline creatinine of < 0.7 mg/dl – Adequate renal function.
In the presence of renal insufficiency, VCUG is warrented.
47

VCUG
Voiding cystourethrogram of a 1-year-
old child with prune belly syndrome
exhibiting the classic elongates, tortuous,
and dilated ureter representing one of
the radiographic hallmarks of the
syndrome.
The large, distended bladder is noted.
48

VCUG
Voiding cystourethrogram details the
grossly enlarged, but non-trabeculated
bladder.
The bladder neck is widely patent, with
hypoplastic urethra.
49

VCUG
Voiding cystourethrogram of a 1-year-
old child demonstrating the classic
radiographic appearance of the
posterior urethra in prune belly
syndrome.
The open bladder neck and dilated
prostatic/posterior urethra is evident.
No urethral obstruction is present.
50

CATEGORY I
Prognosis is bad.
Most of succumb to the disease in the newborn period.
Supportive care
Simple bladder drainage
51

CATEGORY II
Individualised evaluation and treatment
Evaluation of renal function and drainage is required
IVU provide dramatic images; no sufficient information on comparative function.
52

EVALUATION – CATEGORY II
Parenchymal function – DMSA at 4 to 6 weeks of age
Renal outflow obstruction – MAG3
In case of poor renal function – Selective use of Whitaker test
53

TREATMENT – CATEGORY II
Aggressive interventions recommended.
Infection and progressive renal insufficiency – Greatest threat to quality of life.
Early reconstruction of the urinary system to reduce stasis and infection.
Reconstruction delayed upto 3 months to allow for pulmonary maturation.
Can be combined with circumcision, orchiopexy and abdominoplasty.
54

CATEGORY II- ALTERNATIVE APPROACH
Limited surgical intervention
Close surveillance with medical management of bacteriuria.
Surgical intervention only in patients with proven obstruction or intractable infection.
55

CATEGORY II
Progressive renal function detoriatiation is common in prune belly syndrome.
Many patients ultimately require renal transplantation.
56

CATEGORY III
Good renal function
Rarely require early urological intervention
Regular monitoring of urinary tract dilatation using USG and renal function using
serum creatinine.
Regular monitoring for UTI.
Correct cryptorchidism in 1st year of life.
Abdominoplasty may be needed.
Reflux correction may be needed in mid to long term follow up.
57

SURGICAL MANAGEMENT
Urinary tract procedures
Orchiopexy
Abdominal wall reconstruction
58

URINARY TRACT PROCEDURES
Reserved for children with:
Progressive or severe HUN,
High grade VUR,
Recurrent infection,
True obstructive uropathy,
Progressive renal failure.
59

EXTENSIVE
RECONSTRUCTION
The operation is performed
transperitoneally.
No attempt is made to straighten the
lower ureter.
The ureter may require tapering.
The dome of the bladder is excised
60

SUPRAVESICAL URINARY DIVERSION
Repeated upper tract infections or deterioration of renal function
Cutaneous vesicostomy is usually enough.
Cutaneous pyelostomy/ distal ureterostomy in the presence of PUJ or UVJ obstruction
respectively.
Urachal diverticulum if present can be excised during cutaneous vesicostomy.
61

MEGALOURETHRA RECONSTRUCTION
A, The prepuce is reduced, and a
circumcising incision is formed,
preserving a mucosal collar.
B, With a catheter in place to assist with
identification of the urethra, the penis is
degloved along the subdartos plane.
C, The involved segment of the urethra is
opened longitudinally, and the
redundant urethral wall is excised to
allow tapering of the urethra over a
catheter of approximate size.
62

MEGALOURETHRA RECONSTRUCTION
D, The urethra is closed with absorbable
running sutures and is bolstered with a
second layer of sutures placed in an
interrupted fashion if possible.
E, The penile skin is brought forward,
and the excess foreskin is removed with
a second circumferential incision.
F, The penile shaft skin is approximated
to the mucocutaneous border.
63

UNDESCENDED TESTES - ORCHIDOPEXY
Transabdominal bilateral orchidopexy at 6 months of age is the approach of choice.
Done in conjuction with other abdominal surgeries.
In the absence of need for other abdominal surgeries, it can be done
laparoscopically.
64

UNDESCENDED TESTES
Operative photograph showing the ease
with which
the testes reach the scrotum after
neonatal transabdominal mobilization of
the spermatic cords
65

ABDOMINAL WALL RECONSTRUCTION
Indicated in moderate to severe degree of abdominal wall laxity.
Can be done as early as 6 months of age.
Can be combined with orchidopexy.
Improve cosmesis.
Improvement in bowel, bladder and pulmonary function is controversial.
66

TECHNIQUES
1. Randolph technique
2. Ehrlich technique
3. Monfort technique
4. Denes technique
5. Furness technique
67

RANDOLPH ABDOMINOPLASTY
A curvilinear incision extends from the tip
of the 12th rib, along the anterior
superior iliac spine to the pubic tubercle
and onto the opposite 12th rib.
68

A parallel incision is made, avoiding the
removal of too much abdominal wall.
69

Rhomboid-like pieces of abdominal wall
are removed.
70

Critical non-absorbable sutures are
placed at the anterior iliac spines and
pubic tubercle. These are placed through
full thickness of the abdominal wall
including the periosteum.
71

EHRLICH ABDOMINOPLASTY
(a) Sharp dissection separates the skin the
subcutaneous tissue from the
musculofascial layer.
(b–d) A vest–over-pants closure of the
musculofascial layers. The umbilicus can
be saved on a separate pedicle.
(e) The excess abdominal skin is removed.
72

MONFORT ABDOMINOPLASTY
(a) An almond-shaped incision is made
on the abdomen from the xyphoid to
the pubic symphysis.
(b) The full thickness of skin is removed,
sparing the umbilicus.
73

(c) The peritoneum is opened lateral to
the musculofascial later. Intra-abdominal
surgery can be easily performed through
these incisions.
(d) The parietal peritoneum is incised at
a level to achieve a normal-appearing
waistline.
74

(e) The edges of the musculofascial plate
are sewn to the peritoneal incisions.
(f) The lateral skin flaps are trimmed
and sutured together in the midline.
75

DENES TECHNIQUE
76

DENES TECHNIQUE
77

78

Pediatric urology:Prune Belly Syndrome(PBS)

Recommended

Recommended

More Related Content

What's hot

What's hot (20)

Similar to Pediatric urology:Prune Belly Syndrome(PBS)

Similar to Pediatric urology:Prune Belly Syndrome(PBS) (20)

More from GovtRoyapettahHospit

More from GovtRoyapettahHospit (20)

Recently uploaded

Recently uploaded (20)

Pediatric urology:Prune Belly Syndrome(PBS)