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36 Cards in this Set

  • Front
  • Back
Are the kidneys normal in children with PBS?
spectrum of renal abnormalities extends from normal renal parenchyma to dysplasia

The more severely dysplastic kidneys are generally associated with bladder outlet obstruction in which there has not been decompression through a patent urachus
In children with PBS do they have obstructive hydronephrosis?
Ureteropelvic junction obstruction can occur on a primary or secondary basis; however, nonobstructive hydronephrosis is the rule

It is renal infection rather than obstruction that poses the greatest risk to renal function.
What is the typical morphology of the ureters?
ureters are typically dilated, tortuous, and redundant (Fig. 118-2). The proximal (upper) portions of the ureters are usually less abnormal than the distal segments, although massive dilation and stenosis can occur at all levels. Histologic sectioning demonstrates a lack of smooth muscle cells and an increase in fibrous connective tissue. There generally are more normal-appearing smooth muscle cells in the proximal segments
Do PBS kids have reflux?
Yes 75% of them do
Why do these children get infections that can potentially harm their renal function?
*They have reflux

* large ureters may have ineffective peristalsis because of poor ureteral wall coaptation. The ureteral conduction wave reaches a reduced smooth muscle cell population of poor contractile potential related to reduced myofibrils, often separated by patches of collagen with a resulting bolus of urine reaching more dilated ureteral segments as it progresses toward the bladder (Woodard and Smith, 1998). This can be seen fluoroscopically as ineffective peristalsis, resulting in upper tract stasis, which may lead to infection
Is the urachus ever patent at birth in these children?
The urachus is patent at birth in 25% to 30% of children
What is the trigone like in PBS kids?
trigone is splayed with the ureteral orifices displaced laterally and superiorly, possibly contributing to the high incidence of reflux
What is the ability to void like in kids with PBS?
The ability to empty the bladder is quite variable, with some emptying well and others carrying a significant postvoid residual. This is thought to be due to a relative outlet obstruction and the ability of the bladder to generate sufficient pressure with a detrusor contraction. When the relative outflow resistance prevents effective bladder emptying, the term unbalanced voiding is used (Snyder et al, 1976; Kinahan et al, 1992). Despite these limitations, about 50% of prune belly patients void spontaneously with normal voiding pressures, normal flow rates, and low postvoid residuals
Why do kids with PBS have a dilated prostatic urethra?
The dilation of the posterior urethra is due to prostatic hypoplasia probably related to abnormal mesenchymal-epithelial development (Stephens and Gupta, 1994). Histologically, there are few prostatic cellular elements with a reduction of both epithelial and smooth muscle cells and an increase in connective tissue cells
Are there urethral lesionis associated with PBS?
Various obstructive lesions of the distal posterior urethra have been described, such as urethral atresia, valves, urethral stenosis, urethral membrane, and urethral diverticulum, and are thought to occur in 20% of cases

(Hoaglund and Hutchins, 1987). Stephens (1983) described an angulation of the urethra during voiding that he referred to as type IV valves, which results from lack of prostatic parenchymal tissue. Prostatic hypoplasia, the etiology of which is controversial, is thought to be a factor in the ejaculatory failure of PBS patients
Is urethral atreisa fatal in children with PBS?
Unless it is associated with a patent urachus, urethral atresia is often lethal (see Fig. 118-3). It has been postulated that urethral atresia or microurethra occurs because the urethra is unused rather than malformed. Spontaneous bladder rupture with fistula formation has also been reported
What is megalourethra?
PBS is associated with two variations of megalourethra (Shrom et al, 1981; Mortensen et al, 1985). The fusiform type is a deficiency of the corpus cavernosum as well as the spongiosum, and the scaphoid variety is a deficiency of the spongiosum only with preservation of the glans and corpora cavernosa (Fig. 118-5). With the scaphoid variety the ventral urethra dilates with voiding, whereas with the fusiform variety the entire phallus dilates with voiding
Any gonadal defects in PBS kids?
Yes in the boys.

Bilateral intra-abdominal testes lying over the iliac vessels are the most typical findings
What are extragenitourinary manifestations of PBS?
*Obviously the abdominal wall defects.

Cardiac - PDA, VSD, ASD, tetrology of fallot

Pulmonary - pulmonary hypoplasia, pneumothorax, pneumomediastinum

GI - malrotation, intestinal atresia, omphalocele, gastroischesis, Hirschsprung's, Imperforate Anus

Orthopedic - Pectus, scoliosis, sacral agenesis, hip or other

Misc - splenic issues, adrenal issues.
What is the abdominal wall defect in PBS kids?
The appearance of the abdominal wall gives PBS of newborns their most characteristic feature (Fig. 118-6). Although in some cases the musculature of the abdominal wall may be totally absent (Manivel et al, 1989), most commonly there is uneven involvement with the medial and inferior muscular typically most deficient

The most severely affected areas may have skin, subcutaneous fat, and a single fibrous layer on the peritoneum
How often do these kids get cardiac issues?
*10% of time
Why do PBS kids get pulmonary hypoplasia?
Pulmonary hypoplasia can result from severe oligohydramnios related to renal dysplasia or severe bladder outlet obstruction and may result in newborn demise. In addition, pneumothorax and pneumomediastinum can be seen with or without pulmonary hypoplasia
What are some of the clinical concerns for PBS kids in relation to their GI abnormalities?
The majority of the anomalies result from incomplete rotation of the midgut giving way to a wide mesentery, which results in increased bowel mobility with intestinal malrotation, volvulus, atresias, and stenosis

Splenic torsion related to abnormal mesenteric fixation has also been reported

With a limited ability to generate intra-abdominal pressure, constipation becomes a lifelong problem and leads to acquired megacolon
Are Orthopedic complications common or uncommon, why do they occur, and what are they?
Orthopedic abnormalities, ranging in incidence from 30% to 45%, are second in frequency to those of the genitourinary tract and abdominal wall. Many of these abnormalities result from the compressive effects of oligohydramnios. Some think the musculoskeletal defects result from the abnormal mesenchymal development at 6 weeks of gestation (Loder et al, 1992). Green and colleagues (1993), however, pointed out that because many of the deformities are unilateral, oligohydramnios is the most likely etiology. Dimpling of the lateral aspect of the knees is a common finding in oligohydramnios. Oligohydramnios may also result in talipes equinovarus (26%), hip dysplasia (5%), and congenital scoliosis (4%) (Woodard and Smith, 1998). It has been proposed that a distended bladder that may impinge on the external iliac vessels may compromise the blood supply to the lower extremities and in severely affected cases result in lower extremity hypoplasia, absence, or amputation
When a child with PBS is diagnosed in utero and they have severe ureterohydronephrosis is that a bad prognostic sign about their renal function?
It is imperative to remember that the majority of patients with PBS do not have demon-strable urinary obstruction and that the degree of hydroureteronephrosis does not correlate with the postnatal renal function
What is category I PBS and what interventions should they undergo from a urologist?
Category I consists of neonates who have experienced marked oligohydramnios as a result of renal dysplasia or severe bladder outlet obstruction with resultant pulmonary hypoplasia and skeletal abnormalities. Most infants with urethral atresia fall in this category. The exceptions to this are patients with urethral atresia and a patent urachus (Rogers and Ostrow, 1973). Those in this category, who are not stillborn, commonly succumb within a few days of life to pulmonary hypoplasia or later to renal failure. Approximately 20% of newborns with PBS die in the perinatal period (Woodard and Parrott, 1978b; Burbige et al, 1987; Fallat et al, 1989). It would be unusual for any urologic intervention in this category of patients to alter the course of events. Simple catheter drainage is all that is justifiable
What is category II PBS and what interventions should they undergo from a urologist?
Category III consists of patients with mild features of the triad or incomplete forms of PBS. This category comprises the majority of PBS patients in whom hydroureteronephrosis is present to some degree but renal function is well maintained (Woodhouse et al, 1982; Woodard, 1998). There is no evidence of pulmonary insufficiency
What is another name for PBS?
Eagle-Barrett syndrome
What is the incidence of PBS and are males or females more predisposed?
1/29-40,000, males are more predisposed.
Do females with PBS have gonadal anomalies?
No
What demographics have increased predisposition to PBS?
Twins, blacks, children born to younger mothers
What are the typical urodynamic findings in PBS?
Large capacity, normal compliance bladders with delayed first sensation to void.
Do PBS patients have retrograde ejaculation?
Yes because of an incompetent bladder neck.
Have any PBS patients fathered a child without fertilization techniques?
No
What feature does a pseudoprune patient lack?
Abdominal wall musculature deficiency.
What creatinine cutoff does the book give for predicting adequate childhood renal function?
Less than or equal to 0.7ng/dl
When is a DMSA scan most accurate and why?
At 4-6 weeks postnatal because of less transitory neonatal physiology.
If you are going to do surgery on PBS patients, when should you do it?
At 3 months of age to allow for pulmonary maturation.
A PBS child with urethral atresia should have what done?
sp tube, unless they have patent urachus, and really that is it, these are the category I kids and they don't live long.
What is the standard approach for a PBS orchidopexy?
-Bilateral transabdominal approach at 6 months, an inguinal approach doesn’t give you enough

length
What are the two most accepted techniques for abdominal wall reconstruction for PBS?
-The Montfort and Ehrlich.