Download - Prune Belly

8/13/2019 Prune Belly

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Perinatal meet

Prasanna kumar.M

3rd

semesterDepartment of pediatrics

7/11/2013


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27 Yrs female, PGR , full term pregnancy

Antenatal history:Conceived on drugsUSG (34+1 weeker)SLIVF, EFW-2.53 kgAGA,Plancenta upper grade-1,Fetal kidney enlargedLt Grade-3 HDNRt Grade -4 HDNfetal ECHO normal

.

Normal uncomplicated vaginal delivery


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Child birth - apgar score 8/9 ,no cyanosis,normal cry , birth weight of 3398 grams, AFD

External apperanace Abdomen distended,loose skin, bowel loops visible, Rt kidneypalpable,Scrotum Empty with cryptorchism

Systemic examination:- Chest unremarkableCardiac Normal.


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Renal Function

Urea/ Creatine-39/2.6(D1),137/4.1(D10)

USG abdomen Thick irregular trabecular bladder

wall,B/L testis could not be localised , B/L gross

HDUN wih thinned out cortex with

megaloureter. (day 3)


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Diagnosis of Prune belly syndrome is madebecause ofInvolvement of abdominal wall ,

Inomalies in genitourinary tract, Cryptorchidism


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Also known as1. Eagle Barret syndrome2. Triad syndrome3. Abdominal musculature deficiency syndrome

First described in 1839.

1 in 29,000-40,000 live births

almost exclusively in males; less than 3% of cases in females

2 times higher if pregnancy is less than 25 yrs old.


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TRIADSYNDROME

Abdominalwall defect

Urinary tractabnormality Cryptorchidism


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Urinary Tract Obstruction

Primary Mesodermal Developmental Defect

Intrinsic defect in urinary tract defect

Yolk sac defect


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hypo plastic or dysplastic prostate

obstruction of the urethra

overdistension of the bladder and theupper urinary tract

stretches the abdominal wall

damage to the abdominal musculature andinterferes with the descent of the testicles .(Wheatley et al. 1996)

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2.Primary Mesodermal Developmental Defect .

Primary defect in lateral plate mesodermureters,prostate,bladder,urethra,


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1.Myopathy2.Patchy andasymmetrical

3.Involves medialand inferior

4.Initiallywrinkled

5.Later pot belly

1.Dysplastic(II,IV),cystic(IV), hypoplasticor grosslyhydronephrotic

2.Prognosis dependon degree of kidney

damage

3 Renal infecion posesmore risk thanobstruction.

1.Cryptorchidism2.Testicles usuallyintra-abdominalat the sacroiliaclevel

3.Complicationsare infertility andazospermia

Abdominal KIdneys Genital


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hydro- or megalo-ureter with characteristicmarked dilatation, tortuous, and elongated

distal ureter most severely affected

Congenital ureterovesical junction obstruction -10%

vesicoureteral reflux present in most (>50%)


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Excretory urography reveals tortuous

dilated ureters


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Often widely dilated may be an area of abrupt narrowingdistal to dilatation.

Proximal will be usually less abnormal than distalsegments

Peristalsis will be ineffective

10% with posterior uretheral valve ( Overlappingsyndrome)

VUR in 75% of children with PBS.Posterior urethral dilatation is due to prostatic hypoplasia ,which leads to angulation of the urethra during voiding.


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markedly enlarged

may show an "hourglass configuration" on voidingcystogram

lateral displaced ureteral orifices(l/t reflux)

Complete emptying mostly (50%)

patent urachus at timesPostvoid residues will be present insome(unbalanced voiding)


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Antenatal USG :-Bw 11-14 weeksHydroureteronephrosis,distended bladder,irregularabdominal circumferencesIt is difficult to decide about termination of pregnancy inlight of difficulty in determining the etiology ofhydrounephrosis.


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It is difficult to justify termination1.Due to our inability to diagnose the

etiology of prenatal hydronephrosis2.Inability to predict postnatal renal

function on the basis of the degree of urinarytract dilation.


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Antenatal USGClinical featuresPost natal USG


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20% of patients are stillborn

30% die of renal failure or urosepsis withinthe first two years of life

remaining 50% have varying degrees ofurinary pathology.


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CXR and infantogramRFTVoiding cystourethrogram (VCUG)

To differentiate obstrution VS stagnationTo Dx VUR

USG abdomen with pelvisDMSA(4-6 weeks).MAG 3ECHO


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Primarily conservative1.Catheerisation.2.fluid and electrolyte

Prophylactic antibiotics

CXR done NormalUsg abdomen and pelvis- Thick irregular

trabecular bladder wall,B/L testis could not belocalised , B/L gross HDUN wih thinned outcortex with megaloureter. (day 3)


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Echo plannedPlanned for surgery and shifted to pediatricsurgery


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Dnes FT, Arap MA, Giron AM, et al. Comprehensive surgical treatment of prunebelly syndrome: 17 years experience with 32 patients. Urology 2004;64:789 94.Fusaro F, Zanon GF, Ferreli AM, et al. Renal transplantation in prune bellysyndrome. Transpl Int 2004:17(9):549 52.Monfort G, Guys JM, Bocciardi A, et al. A novel technique for reconstruction of theabdominal wall in the prune belly syndrome. J Urol 1991;146:639.Noh PH, Cooper CS, Zderic SA, et al. Prognostic factors in patients with prune belly

syndrome. J Urol 1999;162:1399 401.Reinberg Y, Manivel JC, Fryd D, et al. The outcome of renal transplantation inchildren with the prune belly syndrome. J Urol 1989;142:1541.Smith CA, Smith EA, Parrott TS, et al. Voiding function in patients with prune bellysyndrome after Monfort abdominoplasty. J Urol 1998;159:80 9.Stephens FD, Gupta D. Pathogenesis of the prune belly syndrome. J Urol1994;152:2328 31.

Woodard JR, Smith EA. Prune belly syndrome. In: Walsh PC, Retik AB, Vaughan JrED, Wind AJ, editors. Campbells urology. Philadelphia: WB Saunders; 1998. p.1917 38.Woodhouse CR, Ransley PG, Innes Williams D. Prune belly syndrome report of 47cases. Arch Dis Child 1982;57:856 9.


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