Slide 1: CASE REPORT
RUPESH GAUTAM ,MBBS
FIRST YR RESIDENT
JRRMMC,DEPATRMENT OF RADIOLOGY History : History 17 Yrs female, primi , full term pregnanc y
checkup done –no records of antenatal ultrasound,
no history of medicine intake during antenatal period except for multivitamins.
Normal uncomplicated vaginal delivery Birth History : Birth History Child birth - apgar score 8/10 ,no cyanosis, wt -3.1kg, height -42cm ,normal cry
External apperanace – Bulging flanks , thin flabby wrinkled skin ,bilateral testis non palpable within the scrotum.
Systemic examn, Chest –unremarkable
Renal Function-Creatinine 107.84(27-70) External experience : External experience Babygram : Babygram Voiding Cysto Uretherogram : Voiding Cysto Uretherogram VCUG contd… : VCUG contd… VCUG contd….. : VCUG contd….. Post voiding Normal urethra : Normal urethra Ultrasound image : Ultrasound image Ultrasound image contdd… : Ultrasound image contdd… Ultrasound image contdd… : Ultrasound image contdd… Ultrasound image contdd… : Ultrasound image contdd… Discussion : Discussion Diagnosis
Why Prune belly Syndrome ?
Short discussion on prune belly syndrome
Management Prune Belly syndrome : Prune Belly syndrome Also known as Eagle –Barret syndrome /triad syndrome /abdominal musculature deficiency syndrome first described in 1839.
1 in 35,000-50,000 live births
almost exclusively in males; less than 3% of cases in females
4% of all cases are products of twin pregnancies with 4 times higher incidence than singeleton.
2 times higher if pregnancy is less than 25 yrs old. Introduction contd… : Introduction contd… 20% of patients are stillborn
30% die of renal failure or urosepsis within the first two years of life
remaining 50% have varying degrees of urinary pathology. Slide 19: TRIAD SYNDROME Abdominal wall defect Urinary tract abnormality Cyrptorchidism Pathogenesis : Pathogenesis Urinary Tract Obstruction
Primary Mesodermal Developmental Defect
Teratogenic effect Urinary Tract Obstruction : Urinary Tract Obstruction hypo plastic or dysplastic prostate
obstruction of the urethra
overdistension of the bladder and the upper urinary tract
stretches the abdominal wall
damage to the abdominal musculature and interferes with the descent of the testicles. (Wheatley et al. 1996) Pathogenesis contd… : Pathogenesis contd… 2.Primary Mesodermal Developmental Defect.
3.Teratogenic effect: Diagnostic imaging (4 groups) : Diagnostic imaging (4 groups) Group 1 :potters syndrome
Very severe,still birth (pulmonary dysplasia).
Kidneys severly dysplastic or absent
complete uretheral obstruction
may be associated with talipes equinovarus.
Group 2 : Born with severe urinary tract abnormalities
abdomina wall defects(+)
Without pulmonary hypoplasia Radiological types contd… : Radiological types contd… Group 3 :mild involvement of abdominal wall and urinary tract .
Group 4 :
Pseudo prune belly : Normal abdominal wall, absent or incomplete cryptorchidism, urinary tract like that seen with prune-belly syndrome Abdominal Musculature: : Abdominal Musculature: Myopathy ,
patchy and asymmetrical lower ,lateral rectus involvement .
Appearance initially wrinkled
later "pot belly" appearance Kidneys : Kidneys may be dysplastic, cystic, hypoplastic or grossly hydronephrotic
prognosis may depend on degree of kidney damage
chronic UTI and obstructive uropathy may lead to ESRF Ultrasound image of Kidneys : Ultrasound image of Kidneys Ureters : Ureters hydro- or megalo-ureter with characteristic marked dilatation, tortuous, and elongated
distal ureter most severely affected
Congenital ureterovesical junction obstruction -10%
vesicoureteral reflux present in most (>50%) Slide 29: Excretory urography reveals tortuous dilated ureters Urethra and prostate : Urethra and prostate often widely dilated may be an area of abrupt narrowing distal to dilatation.
10% with posterior uretheral valve ( Overlapping syndrome)
lack of prostate differentiation / hypoplastic Bladder : Bladder markedly enlarged
may show an "hourglass configuration" on voiding cystogram
lateral displaced ureteral orifices
Complete emptying mostly
patent urachus at times Normal Vs Prunbelly : Normal Vs Prunbelly Gonads : Gonads Cryptorchidism
testicles usually intra-abdominal at the sacroiliac level
complications are infertility and azospermia
also at risk for testicular malignancy Musculoskeletal : Musculoskeletal 20% to 50% of cases
talipes equinovarus (club foot)congenital dislocation of the hipspectus excavatum or carinatum
Others: polydactyly, scoliosis,, torticollis Respiratory : Respiratory Classified into 2 groups:
Pulmonary Hypoplasia :
associated with pneumothorax and; pneumomediastinum
Recurrent Lobar Atelectasis and Pneumonia
characteristic thoracic pectus deformity with rib flaring
decreased AP diameter of chest Other system involvement : Other system involvement Cardiovascular- 10% cases
Other Gastrointestinal – gastroschisis imperforate anus malrotation with volvulus Differential diagnosis : Differential diagnosis Posterior uretheral valve
Megacystis Microcolon Intestinal Hypoperistalsis syndrome
Severe primary vesico ureteral reflux Posterior uretheral valve : Posterior uretheral valve most common cause of bladder outlet obstruction in male children, 1 in 5000 live births
Anatomy: anterior urethra (bulbous and penile urethra)
posterior urethra (membranous and prostatic urethra)
abnormally anterior insertion of the mesonephric (wolffian) duct on the cloaca before its division into the urogenital sinus and the anorectal canal.
3 types (young classification)
Type 1(95%) ,bicuspid valve that radiates distally from the posterior edge of the verumontanum to the anterior proximal membranous urethra
Hydronephrosis,vesicoureteral reflux,bladder dysfunction PATHOLOGICAL ANATOMY : PATHOLOGICAL ANATOMY Posterior uretheral valve vs PBS images : Posterior uretheral valve vs PBS images Posterior uretheral valve Prune belly Megacystis Microcolon Intestinal Hypoperistalsis syndrome : Megacystis Microcolon Intestinal Hypoperistalsis syndrome predominantly in females ,4:1
1.abdominal mass, absent bowel sounds, and feeding intolerance with bilious emesis
2.Cryptorchidism(-) unlike in Prunebelly syndrome.
Etiology :1.Extensive intramural fibrosis leads to destruction of intestinal intraneural network.
2.Enlarged bladder prevents normal intestinal rotation.
1. Enlarged Urinary Bladder (88%) with no obstruction
2. Hydronephrosis( 53%) with hydroureter
3. Dilated stomach with malroataion/vovlulus Megacystis Microcolon Intestinal Hypoperistalsis syndro me images : Megacystis Microcolon Intestinal Hypoperistalsis syndro me images Management : Management Primarily conservative
Relive obstruction-pyelostomies or vesicostomies,
Treatment of UTI
Anterior abdominal wall repair – to prevent respiratory,intestinal and urinary complications.
Orchidopexy- increased risk of malignany with undescended testis References : References Clinical urography –pollack,Mc clennan
Prune Belly Syndrome: Clinicopathologic Study of 29 Cases
Authors: J. Carlos Manivel a; Guido Pettinato b; Yuri Reinberg c; Ricardo Gonzalez c; Barbara Burke a; Louis P. Dehner a
Prune belly syndrome:T E Herman1 and M J Siegel1
1Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Children's Hospital, Washington University School of Medicine, St Louis, MO, USA
From the Departments of Pediatric Radiology (T.B., A.A., J.G.) and Pediatric Urology (P.L.P.), Hospital Infantil La Paz, Paseo de la Castellana 261, Madrid 28046, Spain. Recipient of a Certificate of Merit award for an education exhibit at the 2000 RSNA scientific assembly.
Prune belly syndrome associated with exomphalos and anorectal agenesis.Walker J, Prokurat AI, Irving IM.
Urtila R, Popa I, Berinde L, Tanase D, Pop L, Raica A. Pediatrie (Bucur). 1992 Jul-Sep;41(3):61-5. Review. Romanian. PMID: 1327234 [PubMed - indexed for MEDLINE
Crompton CH, MacLusky IB, Geary DF.Department of Pediatrics, Hospital for Sick Children, Toronto, Canada PMID: 17039740 [PubMed - indexed for MEDLINE] References : References Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287, USA- abnormal development of the prostate in PBS may be explained as a pressure-induced dysplasia rather than a primary maldevelopment
Journal of Indian association of paediatric surgeon Vol 9 (oct-dec 2004). Slide 46: THANK YOU