Perinatal meet

Prasanna kumar.M 3rd semester Department of pediatrics 7/11/2013

27 Yrs female, PGR , full term pregnancy Antenatal history: Conceived on drugs USG (34+1 weeker) SLIVF, EFW-2.53 kg AGA,Plancenta upper grade-1, Fetal kidney enlarged, Lt Grade-3 HDN, Rt Grade -4 HDN, fetal ECHO normal Normal uncomplicated vaginal delivery

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 kidney palpable,Scrotum Empty with cryptorchism Systemic examination:- Chest unremarkable Cardiac Normal.

Renal FunctionUrea/ 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)

Diagnosis of Prune belly syndrome is made because of Involvement of abdominal wall , Inomalies in genitourinary tract, Cryptorchidism

Also known as 1. Eagle Barret syndrome 2. Triad syndrome 3. 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.

TRIAD SYNDROME

Abdominal wall defect

Urinary tract abnormality

Cryptorchidism

Urinary Tract Obstruction Primary Mesodermal Developmental Defect Intrinsic defect in urinary tract defect Yolk sac defect

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.
8 8 8

(Wheatley et al. 1996)

2.Primary Mesodermal Developmental Defect.

Primary defect in lateral plate mesoderm ureters,prostate,bladder,urethra,

Abdominal
1.Myopathy 2.Patchy and asymmetrical 3.Involves medial and inferior 4.Initially wrinkled 5.Later pot belly

KIdneys
1.Dysplastic(II,IV), cystic(IV), hypoplastic or grossly hydronephrotic 2.Prognosis depend on degree of kidney damage 3 Renal infecion poses more risk than obstruction.

Genital
1.Cryptorchidism 2.Testicles usually intra-abdominal at the sacroiliac level 3.Complications are infertility and azospermia

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%)

Excretory urography reveals tortuous dilated ureters

Often widely dilated may be an area of abrupt narrowing distal to dilatation. Proximal will be usually less abnormal than distal segments Peristalsis will be ineffective 10% with posterior uretheral valve ( Overlapping syndrome) VUR in 75% of children with PBS. Posterior urethral dilatation is due to prostatic hypoplasia , which leads to angulation of the urethra during voiding.

markedly enlarged may show an "hourglass configuration" on voiding cystogram

lateral displaced ureteral orifices(l/t reflux)

Complete emptying mostly (50%) patent urachus at times Postvoid residues will be present in some(unbalanced voiding)

Antenatal USG : Bw 11-14 weeks Hydroureteronephrosis,distended bladder,irregular abdominal circumferences It is difficult to decide about termination of pregnancy in light of difficulty in determining the etiology of hydrounephrosis.

In utero intervention for relief of urinary tract dilation and oligohydramnios

(Gadziala et al, 1982; Glazer et al, 1982; Nakayama et al, 1984; Scarborough et al, 1988; Estes and Harrison, 1993; Leeners et al, 2000)

Termination of pregnancy
(Pescia et al, 1982).

It is difficult to justify termination 1.Due to our inability to diagnose the etiology of prenatal hydronephrosis 2.Inability to predict postnatal renal function on the basis of the degree of urinary tract dilation.

Antenatal USG Clinical features Post natal USG

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.

CXR and infantogram RFT Voiding cystourethrogram (VCUG)

To differentiate obstrution VS stagnation To Dx VUR

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

Primarily conservative 1.Catheerisation. 2.fluid and electrolyte Prophylactic antibiotics CXR done Normal Usg abdomen and pelvis- Thick irregular trabecular bladder wall,B/L testis could not be localised , B/L gross HDUN wih thinned out cortex with megaloureter.(day 3)

Echo planned Planned for surgery and shifted to pediatric surgery

Dnes FT, Arap MA, Giron AM, et al. Comprehensive surgical treatment of prune belly syndrome: 17 years experience with 32 patients. Urology 2004;64:78994. Fusaro F, Zanon GF, Ferreli AM, et al. Renal transplantation in prune belly syndrome. Transpl Int 2004:17(9):54952. Monfort G, Guys JM, Bocciardi A, et al. A novel technique for reconstruction of the abdominal 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;162:1399401. Reinberg Y, Manivel JC, Fryd D, et al. The outcome of renal transplantation in children with the prune belly syndrome. J Urol 1989;142:1541. Smith CA, Smith EA, Parrott TS, et al. Voiding function in patients with prune belly syndrome after Monfort abdominoplasty. J Urol 1998;159:809. Stephens FD, Gupta D. Pathogenesis of the prune belly syndrome. J Urol 1994;152:232831. Woodard JR, Smith EA. Prune belly syndrome. In: Walsh PC, Retik AB, Vaughan Jr ED, Wind AJ, editors. Campbells urology. Philadelphia: WB Saunders; 1998. p. 191738. Woodhouse CR, Ransley PG, Innes Williams D. Prune belly syndromereport of 47 cases. Arch Dis Child 1982;57:8569.