6: Lower urinary tract dysfunction and anomalies in children

Question 1

Which of the following is a potential consequence of constipation on bladder function in children?
a. Increased bladder capacity
b. Decreased risk of UTI
c. Improved control of incontinence
d. Low functional capacity, incontinence, UTI, and triggering or exacerbating vesicoureteral reflux (VUR)

Answer 1

d. Low functional capacity, incontinence, UTI, and triggering or exacerbating vesicoureteral reflux (VUR)
Explanation: Constipation may adversely affect bladder function, leading to low functional capacity, incontinence, urinary tract infection (UTI), and triggering or exacerbating vesicoureteral reflux (VUR).

Question 2

Which gender is daytime incontinence more common in school-age children?
a. Boys
b. Girls
c. Equally common in both boys and girls
d. There is no gender difference in daytime incontinence in school-age children

Answer 2

b. Girls
Explanation: Daytime incontinence varies with both age and gender in school-age children and seems to be more common in girls.

Question 3

What is the most common urinary symptom associated with LUT dysfunction in children?
a. Urgency
b. Hesitancy
c. Weak urine stream
d. Painful urination

Answer 3

a. Urgency
Explanation: The most common urinary symptoms associated with LUT dysfunction in children include holding maneuvers and urgency.

Question 4

What percentage of children will have some degree of nighttime wetting at 5 years of age?
a. 5%
b. 10%
c. 15%
d. 20%

Answer 4

c. 15%
Explanation: Approximately 15% of children will have some degree of nighttime wetting at 5 years of age, with a spontaneous resolution rate of approximately 15% per year, so 15 years of age only 1% to 2% of teenagers will still wet the bed.

Question 5

What is the association between LUT dysfunction and UTI?
a. LUT dysfunction decreases the risk of UTI
b. LUT dysfunction has no impact on the risk of UTI
c. LUT dysfunction is associated with an increased risk of UTI
d. LUT dysfunction is not associated with UTI

Answer 5

c. LUT dysfunction is associated with an increased risk of UTI
Explanation: LUT conditions resulting in urinary stasis are associated with UTI.

Question 6

What is the association between LUT dysfunction and VUR?
a. LUT dysfunction decreases the risk of VUR
b. LUT dysfunction has no impact on the risk of VUR
c. LUT dysfunction is associated with an increased risk of VUR
d. LUT dysfunction is not associated with VUR

Answer 6

c. LUT dysfunction is associated with an increased risk of VUR
Explanation: There is a known association between LUT and VUR, and VUR may be secondary to bladder dysfunction.

Question 7

What information should be included in the history of a child with LUT dysfunction?
a. Evaluation of cardiac symptoms
b. Evaluation of respiratory symptoms
c. Evaluation of urinary symptoms and infections (UTI), diet, bowel function, and developmental milestones
d. Evaluation of musculoskeletal symptoms

Answer 7

c. Evaluation of urinary symptoms and infections (UTI), diet, bowel function, and developmental milestones
Explanation: History includes evaluation of urinary symptoms and infections (UTI), diet, bowel function, and developmental milestones, including toilet training.

Question 8

What should be assessed during the physical exam of a child with LUT dysfunction?
a. Assessment of vision and hearing
b. Assessment of lung sounds
c. Inspection of the back spine for signs of occult spinal dysraphism or tethered cord
d. Assessment of joint range of motion

Answer 8

c. Inspection of the back spine for signs of occult spinal dysraphism or tethered cord
Explanation: Examination should include inspection of the back spine for signs of occult spinal dysraphism or tethered cord such as lipoma, mass, or hair tuft.

Question 9

What diagnostic tools are valuable in diagnosing simple and complex LUT voiding dysfunction?
a. Blood tests
b. Imaging studies
c. Bowel function tests
d. 7-day bowel and bladder diary and 48-hour frequency volume charts

Answer 9

d. 7-day bowel and bladder diary and 48-hour frequency volume charts
Explanation: A 7-day bowel and bladder diary and 48-hour frequency volume charts are invaluable in diagnosing simple and complex LUT voiding dysfunction.

Question 10

FIG. 6.1 Bristol stool chart with visuals and descriptions of different stool types. This scale provides a helpful, objective reference for documenting stool consistency when talking to patients about bowel function. Source: (Modified from Lewis SJ, Heaton KW. Stool form scale as a useful guide to intestinal transit time. Scand J Gastroenterol 1997;32(9):920-924.)

Answer 10

Question 11

Table 6.1

Rome IV Diagnostic Criteria for Functional Constipation

Answer 11

Question 12

What is the first step in managing LUT dysfunction in children with evidence of bowel dysfunction?
a. Initiate pharmacotherapy
b. Initiate physical therapy
c. Initiate a regimen that includes high fiber and increased fluid intake, as well as timed voiding every 2 hours
d. Initiate neuromodulation

Answer 12

c. Initiate a regimen that includes high fiber and increased fluid intake, as well as timed voiding every 2 hours
Explanation: If evidence of bowel dysfunction is present, a regimen that includes high fiber and increased fluid intake is initiated, as well as timed voiding every 2 hours.

Question 13

What are the main side effects of oxybutynin?
a. Constipation, dry mouth, blurred vision, reduced sweating, flushing, and altered behavior and cognition
b. Diarrhea, nausea, and vomiting
c. Headache and dizziness
d. Muscle weakness and tremors

Answer 13

a. Constipation, dry mouth, blurred vision, reduced sweating, flushing, and altered behavior and cognition
Explanation: The main side effects of oxybutynin include constipation, dry mouth, blurred vision, reduced sweating, flushing, and altered behavior and cognition.

Question 14

FIG. 6.2 Management algorithm for childhood defecation disorders seen in a pediatric urology practice. Lack of improvement or intractable constipation should be diagnosed based on worsening or absence of suboptimal response to adequate medical treatment for at least 3 months. MACE, Malone antegrade continence enema.

Answer 14

Question 15

At what gestational age may prenatal bladder anomalies be detected?
a. 12th week
b. 10th week
c. 8th week
d. 6th week

Answer 15

b. 10th week
Explanation: Prenatal bladder anomalies may be detected as early as the 10th week of gestation.

Question 16

What is the management of an infected urachal cyst or sinus with abscess?
a. Antibiotics only
b. Excision of the bladder cuff
c. Complete excision of the patent urachus with a bladder cuff
d. Observation

Answer 16

c. Complete excision of the patent urachus with a bladder cuff
Explanation: Management of an infected urachal cyst or sinus with abscess includes initial drainage and antibiotics followed by complete excision of the patent urachus with a bladder cuff.

Question 17

FIG. 6.3 Urachal anomalies. (A) Patent urachus. (B) Urachal cyst. (C) Umbilical-urachus sinus. (D) Vesicourachal diverticulum.

Answer 17

Question 18

What is the incidence of posterior urethral valves in boys?
a. 1.6-2.1 per 1,000 births
b. 1.6-2.1 per 10,000 births
c. 1.6-2.1 per 100,000 births
d. 1.6-2.1 per 1,000,000 births

Answer 18

b. 1.6-2.1 per 10,000 births
Explanation: Posterior urethral valves are the most common cause of LUT obstruction in boys with an incidence of 1.6-2.1 per 10,000 births.

Question 19

What is the most common appearance of posterior urethral valves?
a. Sphincteric
b. Annular
c. Leaflets arising from the verumontanum
d. Diaphragmatic

Answer 19

c. Leaflets arising from the verumontanum
Explanation: Most posterior urethral valves appear as leaflets arising from the verumontanum that fuse anteriorly.

Question 20

What is the result of posterior urethral valves during fetal development?
a. Bladder neck hypertrophy
b. Detrusor atrophy
c. Low storage and voiding pressures
d. No effect

Answer 20

a. Bladder neck hypertrophy
Explanation: Posterior urethral valves during fetal development result in detrusor hypertrophy with high storage and voiding pressures and may lead to dilation of the posterior urethra, bladder neck hypertrophy, bladder wall thickening, vesicoureteral reflux, upper tract dilation, and in one third of affected patients, end-stage renal disease.

Question 21

What is the incidence of end-stage renal disease in patients with posterior urethral valves?
a. One fourth of affected patients
b. One third of affected patients
c. One half of affected patients
d. Two thirds of affected patients

Answer 21

b. One third of affected patients
Explanation: Posterior urethral valves may lead to end-stage renal disease in one third of affected patients.

Question 22

What is the most common cause of LUT obstruction in boys?
a. Posterior urethral valves
b. Bladder diverticula
c. Urachal anomalies
d. Urethral strictures

Answer 22

a. Posterior urethral valves
Explanation: Posterior urethral valves are the most common cause of LUT obstruction in boys.

Question 23

FIG. 6.4 (A) Young’s original figures from his 1919 article describing three types of posterior urethral valves. (B) William P. Didusch illustrates the pathognomonic findings associated with posterior urethral valves: the thickened bladder with elevated bladder neck, dilated prostatic urethra, and the valve leaflets commonly ascribed to type 1 valves. The ureters are shown to be dilated. Source: (From Young HH, Frontz WA, Baldwin JC. Congenital obstruction of the posterior urethra. J Urol 1919;3:289.)

Answer 23

Question 24

What is the most common reason for detecting posterior urethral valves in neonates?
a. Thick-walled bladder
b. Oligohydramnios
c. Prenatal hydronephrosis
d. Prenatal UTI

Answer 24

c. Prenatal hydronephrosis
Explanation: Many infants with posterior urethral valves are detected due to prenatal hydronephrosis, oligohydramnios, and/or a thick-walled bladder.

Question 25

What symptoms should raise suspicion of posterior urethral valves in boys?
a. Headache and nausea
b. Recurrent abdominal pain
c. Recurrent infections, overflow incontinence, gross hematuria, and/or renal dysfunction
d. Difficulty breathing and wheezing

Answer 25

c. Recurrent infections, overflow incontinence, gross hematuria, and/or renal dysfunction
Explanation: Boys presenting with LUT symptoms such as recurrent infections, overflow incontinence, gross hematuria, and/or renal dysfunction should raise suspicion of posterior urethral valves.

Question 26

What imaging modality is useful in diagnosing posterior urethral valves?
a. X-ray
b. Ultrasound
c. CT scan
d. MRI

Answer 26

b. Ultrasound
Explanation: Ultrasound is a useful imaging modality in diagnosing posterior urethral valves.

Question 27

FIG. 6.5 Note small, irregular bladder, unilateral high grade vesicoureteral reflux and posterior urethral filling defect consistent with posterior urethral valves.

Answer 27

Question 28

What prenatal ultrasound finding has a high sensitivity for posterior urethral valves?
a. Polyhydramnios
b. Dilated anterior urethra
c. Dilated posterior urethra
d. Renal agenesis

Answer 28

c. Dilated posterior urethra
Explanation: Thickened dilated bladder, upper tract dilation, and oligohydramnios have a high sensitivity for posterior urethral valves on prenatal ultrasonography; a dilated posterior urethra results in the “keyhole sign.”

Question 29

What study is the definitive diagnostic test for posterior urethral valves?
a. Renal ultrasound
b. Voiding cystourethrogram (VCUG)
c. Magnetic resonance imaging (MRI)
d. Computed tomography (CT) scan

Answer 29

b. Voiding cystourethrogram (VCUG)
Explanation: VCUG remains the definitive study to confirm posterior urethral valves.

Question 30

What percentage of boys with posterior urethral valves have high-grade vesicoureteral reflux (VUR)?
a. 10%
b. 25%
c. 50%
d. 75%

Answer 30

c. 50%
Explanation: The bladder often appears thickened and trabeculated with multiple diverticuli, and high-grade VUR is seen in approximately 50% of boys.

Question 31

What postnatal biochemical evaluation is important in assessing renal function in boys with posterior urethral valves?
a. Blood glucose
b. Hemoglobin level
c. Electrolytes and creatinine
d. Liver function tests

Answer 31

c. Electrolytes and creatinine
Explanation: Postnatal biochemical evaluation of renal function in boys with posterior urethral valves includes electrolytes and creatinine.

Question 32

What classic ultrasound finding is indicative of posterior urethral valves in the postnatal period?
a. Dilated anterior urethra
b. Renal agenesis
c. Distended bladder with thickened wall
d. Normal bladder and urethral anatomy

Answer 32

c. Distended bladder with thickened wall
Explanation: Much like the antenatal period, classic ultrasound findings in the postnatal period include distended bladder with thickened wall and dilated posterior urethra.

Question 33

What is the suggested cutoff for urinary sodium in a favorable fetal urine sample in a case of posterior urethral valves?
a. <50 mEq/L
b. <75 mEq/L
c. <100 mEq/L
d. <125 mEq/L

Answer 33

c. <100 mEq/L
Explanation: A favorable fetal urine sample obtained after 20 weeks’ gestation is suggested by urinary sodium <100 mEq/L, chloride <90 mEq/L, osmolarity <200 mEq/L, and β2 microglobulin <6 mg/L.

Question 34

What size feeding tube is typically used for postnatal management of posterior urethral valves?
a. 2-Fr
b. 5-Fr
c. 10-Fr
d. 12-Fr

Answer 34

b. 5-Fr
Explanation: A 5- or 8-Fr feeding tube should be inserted.

Question 35

What is the surgical intervention of choice for posterior urethral valves?
a. Vesicostomy
b. Upper urinary tract diversion
c. Cystoscopy with PUV ablation
d. Urethral dilation

Answer 35

c. Cystoscopy with PUV ablation
Explanation: Cystoscopy with PUV ablation is the surgical intervention of choice.

Question 36

Under what circumstances is a vesicostomy reserved for in the treatment of posterior urethral valves?
a. For all cases
b. For very high birth weight infants
c. For very low birth weight infants
d. For cases with no upper tract involvement

Answer 36

c. For very low birth weight infants whose urethras cannot accommodate the scope and/or those with continued impaired renal function, high residuals, and upper tract deterioration
Explanation: Vesicostomy is reserved for very low birth weight infants whose urethras cannot accommodate the scope and/or those with continued impaired renal function, high residuals, and upper tract deterioration.

Question 37

What postoperative procedure should be encouraged in boys undergoing cystoscopy with PUV ablation?
a. Urethral dilation
b. Foley catheter placement
c. Upper urinary tract diversion
d. Circumcision

Answer 37

d. Circumcision
Explanation: Circumcision should be encouraged.

Question 38

Question: What is the lifetime prevalence of end-stage renal disease in children with posterior urethral valves?

Answer choices:
a) Less than 10%
b) Between 10% and 20%
c) Between 20% and 50%
d) More than 50%

Answer 38

c) Between 20% and 50%.

Explanation: The lifetime prevalence of end-stage renal disease (ESRD) in children with posterior urethral valves is between 20% and 50%. Nadir creatinine value less than 0.8 mg/dL appears to indicate lower risk while greater than 1.2 mg/dL at 1 year predicts higher risk for renal failure. The valve bladder evolves through three distinct patterns: detrusor hyperreflexia in infancy and early childhood, decreasing intravesical pressures and improved compliance in childhood, and increased capacity bladder with hypocontractility and atony in adolescence. The focus of vesicoureteral reflux (VUR) management is centered on improving bladder function, and ureteral reimplantation is rarely offered.

Question 39

What is the exstrophy-epispadias complex?

Answer choices:
a) A rare congenital malformation with upper abdominal wall defect and epispadias urethral opening
b) A common congenital malformation with upper abdominal wall defect and normal urethral opening
c) A rare congenital malformation with lower abdominal wall defect exposing an open bladder and urethra, wide diastasis of pubic symphysis, anorectal anomalies, and epispadiac urethral opening
d) A common congenital malformation with lower abdominal wall defect exposing an open bladder and urethra, wide diastasis of pubic symphysis, anorectal anomalies, and normal urethral opening

Answer 39

c) A rare congenital malformation with lower abdominal wall defect exposing an open bladder and urethra, wide diastasis of pubic symphysis, anorectal anomalies, and epispadiac urethral opening.

Explanation: The exstrophy-epispadias complex is a rare congenital malformation with lower abdominal wall defect exposing an open bladder and urethra, wide diastasis of pubic symphysis, anorectal anomalies, and epispadiac urethral opening. It is caused by failure of the anterior abdominal wall and bladder to close during embryonic development, resulting in exposure of the urinary tract to the external environment. It occurs in approximately 1 in 10,000 to 50,000 live births and is more common in males.

Question 40

Image
FIG. 6.6 (A) Newborn female with classic bladder exstrophy: note the bifid clitoral halves, divergent labia and mons, as well as the anteriorly displaced anus and diastasis of pubic symphysis. (B) Newborn male: note the short, exposed urethral plate with dorsal curvature.

Answer 40

Question 41

Image
FIG. 6.7 Pelvic bone abnormalities noted in classic bladder exstrophy. The posterior bone segment is externally rotated (12 degrees mean on each side), but the length is unchanged. The anterior segment is externally rotated (18 degrees mean on each side) and shortened by 30%. The distance between the triradiate cartilage is increased by 31%.

Answer 41

Question 42

What are the diagnostic criteria for bladder exstrophy on prenatal ultrasound?
a. Presence of bladder filling, high-set umbilicus, narrow pubic rami, large genitalia and upper abdominal mass that increases in size as pregnancy progresses
b. Absence of bladder filling, low-set umbilicus, widening pubic rami, diminutive genitalia and lower abdominal mass that increases in size as pregnancy progresses
c. Presence of bladder filling, low-set umbilicus, widening pubic rami, diminutive genitalia and lower abdominal mass that decreases in size as pregnancy progresses
d. Absence of bladder filling, high-set umbilicus, narrow pubic rami, large genitalia and upper abdominal mass that decreases in size as pregnancy progresses

Answer 42

b. Absence of bladder filling, low-set umbilicus, widening pubic rami, diminutive genitalia and lower abdominal mass that increases in size as pregnancy progresses.

Explanation: The diagnostic criteria for bladder exstrophy on prenatal ultrasound include the absence of bladder filling, a low-set umbilicus, widening pubic rami, diminutive genitalia, and a lower abdominal mass that increases in size as the pregnancy progresses.

Question 43

What is the purpose of fetal magnetic resonance imaging (MRI) in prenatal diagnosis?
a. To determine the size and quality of bladder template and urethral plate in males
b. To confirm the diagnosis of bladder exstrophy
c. To obtain pelvic and hip plain films
d. To diagnose a condition after birth

Answer 43

b. To confirm the diagnosis of bladder exstrophy.

Explanation: Fetal magnetic resonance imaging (MRI) can be used to confirm the diagnosis of bladder exstrophy in prenatal diagnosis. This imaging technique can provide detailed images of the fetal anatomy and can be helpful in making a more accurate diagnosis.

Question 44

What is the purpose of postnatal testing for bladder exstrophy?
a. To diagnose the condition
b. To determine the size and quality of bladder template and urethral plate in males
c. To obtain pelvic and hip plain films
d. To determine the quality of the fetal bladder

Answer 44

b. To determine the size and quality of bladder template and urethral plate in males.

Explanation: Postnatal testing for bladder exstrophy involves a detailed examination to determine the size and quality of the bladder template and urethral plate in males. Pelvic and hip plain films may also be obtained to further assess the condition. These tests can help with planning for surgical intervention and management of the condition.

Question 45

Image
FIG. 6.8 Prenatal ultrasound scan demonstrating bladder exstrophy. (A) Longitudinal view showing the low-set umbilicus ( cyan arrow, lack of intraabdominal bladder, and lower abdominal mass (red arrow). (B) Transverse view through the plane (X) in A shows presence of the umbilicus (cyan arrow) and the upper edge of the bladder plate that appears hyperechoic (red arrow).

Answer 45

Question 46

What is the purpose of tying the umbilical cord close to the abdominal wall at birth in the postnatal management of bladder exstrophy?
a. To protect the bladder
b. To prevent infection
c. To promote bladder growth
d. To facilitate surgical repair

Answer 46

b. To prevent infection.

Explanation: Tying the umbilical cord close to the abdominal wall with 2-0 silk at birth in the postnatal management of bladder exstrophy is done to prevent infection.

Question 47

What are the goals of surgical repair in bladder exstrophy?
a. To reconstruct the bladder and urethra only
b. To reconstruct the bladder, urethra and genitalia
c. To create functional organs for continence, voiding, and sexual function
d. To prevent tension on closure

Answer 47

c. To create functional organs for continence, voiding, and sexual function.

Explanation: The goals of surgical repair in bladder exstrophy are to close the bladder and urethra, reconstruct the genitalia, and create functional organs for continence, voiding, and sexual function.

Question 48

What is the difference between delayed closure and neonatal closure in bladder exstrophy repair?
a. Delayed closure has higher continence rates
b. Neonatal closure has higher continence rates
c. Delayed closure results in greater ultimate bladder capacity
d. Neonatal closure results in greater ultimate bladder capacity

Answer 48

a. Delayed closure has higher continence rates.

Explanation: Delayed closure and neonatal closure have been used in bladder exstrophy repair. Delayed closure has been found to have equal continence rates and no difference in ultimate bladder capacity compared to neonatal closure. However, delayed closure may be preferred in some cases to allow for growth and development of the bladder.

Question 49

What is the modern staged repair of bladder exstrophy (MSRE)?
a. Combination of bladder closure, bladder neck reconstruction, urethral elongation, and epispadias repair in a single operation
b. Converting bladder exstrophy into complete epispadias to allow time for bladder to cycle and grow
c. Penile reconstruction to correct dorsal chordee, urethral reconstruction, glanular reconstruction, and skin closure
d. Pelvic osteotomy to prevent tension on closure

Answer 49

b. Converting bladder exstrophy into complete epispadias to allow time for bladder to cycle and grow.

Explanation: The modern staged repair of bladder exstrophy (MSRE) involves converting bladder exstrophy into complete epispadias to allow time for bladder to cycle and grow. This is done before a complete primary repair of bladder exstrophy (CPRE), which includes a combination of bladder closure, bladder neck reconstruction, urethral elongation, and epispadias repair in a single operation. Pelvic osteotomy may also be performed to prevent tension on closure.

Question 50

What are the possible outcomes after exstrophy repair?
a. Published continence rates vary from 37% to 90%
b. Erectile function, sensation, and libido are intact for most males
c. Fertility is unimpaired, but prolapse occurs more commonly in females
d. All of the above

Answer 50

d. All of the above.

Explanation: The outcomes after exstrophy repair vary widely, but published continence rates vary from 37% to 90%. Erectile function, sensation, and libido are intact for most males. Fertility is unimpaired, but prolapse occurs more commonly in females because of the lack of pelvic floor support.

Question 51

Image
Image
FIG. 6.9 Robot-assisted laparoscopic appendicovesicostomy in a child with valve bladder syndrome and a history of posterior urethral valves. (A) The detrusor muscle is incised in the posterior bladder wall to allow tunneled anastomosis of the appendix. (B) Anastomosis of the appendix to the bladder with a feeding tube traversing the appendix into a cystotomy along the posterior wall of the bladder. (C) Postoperative appearance with appendicovesicostomy stoma visible just inside the umbilicus.

Answer 51

Question 52

What is Prune Belly Syndrome?

a) A disease that affects the respiratory system
b) A condition characterized by deficient or absent abdominal wall musculature, bilateral intraabdominal cryptorchidism, and urinary tract anomalies
c) A disorder that affects the nervous system
d) An autoimmune disease

Answer 52

b) A condition characterized by deficient or absent abdominal wall musculature, bilateral intraabdominal cryptorchidism, and urinary tract anomalies

Explanation: Prune belly syndrome (PBS) is a rare condition that affects the abdominal wall, urinary tract, and genital organs. It is characterized by a lack of or underdeveloped abdominal muscles, bilateral intraabdominal cryptorchidism (undescended testicles), and various urinary tract anomalies.

Question 53

Which gender is primarily affected by Prune Belly Syndrome?

a) Boys
b) Girls
c) Both boys and girls equally
d) It is not gender-specific

Answer 53

a) Boys

Explanation: Prune belly syndrome primarily affects boys. In fact, by strict definition, the condition includes cryptorchidism (undescended testicles), which only affects males.

Question 54

What is the contemporary incidence of Prune Belly Syndrome in live births?

a) 1 in 2,000 live births
b) 1 in 10,000–20,000 live births
c) 1 in 29,000–40,000 live births
d) 1 in 100,000 live births

Answer 54

c) 1 in 29,000–40,000 live births

Explanation: Prune belly syndrome is a rare condition, with a contemporary incidence of 1 in 29,000–40,000 live births.

Question 55

What type of urinary tract anomalies are associated with Prune Belly Syndrome?

a) Megacolon and megalourethra
b) Hypospadias and hydroureteronephrosis
c) Polycystic kidneys and bladder exstrophy
d) Megacystis and hydroureteronephrosis

Answer 55

d) Megacystis and hydroureteronephrosis

Explanation: Prune belly syndrome is associated with several urinary tract anomalies, including megacystis (enlarged bladder) and hydroureteronephrosis (dilation of the ureters and kidneys).

Question 56

Image
FIG. 6.10 Appearance of a newborn with prune belly syndrome: wrinkled, redundant skin with bulging at the flanks due to deficient of abdominal wall musculature and massively distended bladder.

Answer 56

Question 57

What is the significance of neonatal abdominal wall appearance in PBS?

a) It can indicate the severity of pulmonary hypoplasia
b) It is not related to PBS
c) It is a sign of gastrointestinal issues
d) It indicates renal dysplasia

Answer 57

a) It can indicate the severity of pulmonary hypoplasia

Explanation: The appearance of the neonatal abdominal wall in a newborn with PBS can provide important information about the severity of the condition. Specifically, the initial postnatal course is dictated by the severity of pulmonary hypoplasia, which can be suggested by the appearance of the abdominal wall.

Question 58

What are some other clinical presentations of PBS besides the abdominal wall appearance?

a) Hearing loss and vision problems
b) Joint pain and stiffness
c) Genital ulcers and lesions
d) Urinary tract anomalies and cryptorchidism

Answer 58

d) Urinary tract anomalies and cryptorchidism

Explanation: While the neonatal abdominal wall appearance is a prominent feature of PBS, the condition is characterized by a range of clinical presentations, including urinary tract anomalies and cryptorchidism (undescended testicles). Many patients also have other complications related to the gastrointestinal, musculoskeletal, and cardiopulmonary systems.

Question 59

Table 6.2

Spectrum of Prune Belly Syndrome

Answer 59

Question 60

What prenatal findings may appear similar to bladder outlet obstruction in PBS?

a) Irregular abdominal circumference
b) Hydroureteronephrosis
c) Severe pulmonary hypoplasia
d) Both a) and b)

Answer 60

d) Both a) and b)

Explanation: Prenatal findings in PBS may appear similar to bladder outlet obstruction, with irregular abdominal circumference and hydroureteronephrosis being characteristic. However, these classic findings may not be consistently seen even at 30 weeks of gestation.

Question 61

How many categories of neonatal presentation are there in PBS?

a) One
b) Two
c) Three
d) Four

Answer 61

c) Three

Explanation: There are three major categories of neonatal presentation in PBS, as outlined in Table 6.2. These categories are based on the severity of the condition and the extent of renal and pulmonary involvement.

Question 62

What does Category I neonatal presentation in PBS include?

a) Pronounced oligohydramnios with severe pulmonary hypoplasia and skeletal abnormalities
b) Moderate renal insufficiency and moderate-severe hydroureteronephrosis
c) Mild features, with normal or mildly impaired renal function and no pulmonary insufficiency
d) None of the above

Answer 62

a) Pronounced oligohydramnios with severe pulmonary hypoplasia and skeletal abnormalities

Explanation: Category I neonatal presentation in PBS includes pronounced oligohydramnios with severe pulmonary hypoplasia and skeletal abnormalities. This is the most severe category of presentation, with a poor prognosis.

Question 63

What postnatal testing is recommended for baseline assessment of renal function in PBS?

a) Ultrasound only
b) Blood urea nitrogen (BUN) and creatinine only
c) Electrolytes only
d) Ultrasound, BUN, creatinine, and electrolytes

Answer 63

d) Ultrasound, BUN, creatinine, and electrolytes

Explanation: Baseline assessment of renal function in PBS should include ultrasound, blood urea nitrogen (BUN), creatinine, and electrolytes. While a voiding cystourethrogram (VCUG) may be necessary to assess bladder outlet and emptying, unnecessary catheterization should be avoided.

Question 64

What are Potter features?

Answer 64

Potter sequence, also known as Potter syndrome or Potter’s sequence, refers to a constellation of physical findings in a newborn that result from a lack of amniotic fluid during fetal development. The absence of amniotic fluid, also known as oligohydramnios, can be caused by various factors, including renal agenesis, obstructive uropathy, and premature rupture of membranes. The physical findings associated with Potter sequence are commonly referred to as Potter features, which include flattened facial features, wide-set eyes, low-set ears, a beaked nose, and limb deformities. These physical characteristics result from the pressure of the uterine wall on the developing fetus in the absence of amniotic fluid. In addition to the physical features, babies with Potter sequence may have pulmonary hypoplasia, which is a severe underdevelopment of the lungs that can result in life-threatening respiratory distress after birth. The condition is usually fatal unless aggressive neonatal care, including mechanical ventilation, is initiated.

Question 65

Image
FIG. 6.12 Surgical correction of megalourethra in a patient with prune belly syndrome. (A) Penile deglovement. (B) Exposure of the scaphoid megalourethra. (C and D) Tailoring of the dilated urethral segment. (E) Completed urethroplasty. (F) Presentation of excessive preputial skin. (G) Completed procedure after urethroplasty and circumcision (note the empty scrotum).

Answer 65

Question 66

Image
FIG. 6.13 Surgical technique for Monfort abdominoplasty and concomitant reconstruction of prune belly uropathy. (A) Delineation of redundancy by tenting up abdominal wall. (B) Skin incisions are outlined with a separate circumscribing incision to isolate the umbilicus. (C) Skin (epidermis and dermis only) is excised with electrocautery. (D) Abdominal wall central plate is incised at the lateral border of the rectus muscle on either side, from the superior epigastric to the inferior epigastric vessels, creating a central musculofascial plate. (E) Adequate exposure is provided for concomitant transperitoneal genitourinary procedures. B, Bladder; T, testis; U, ureter. (F) Only the more normal proximal ureter is preserved for vesicoureteral reimplantation, and the urachal diverticulum is excised. (G) Transtrigonal ureteral reimplantation is performed with or without ureteral tapering as needed. The bladder is closed in two layers, and ureteral stents (not shown) and a cystostomy tube are used. (H) Completion of abdominoplasty by scoring of the parietal peritoneum overlying the lateral abdominal wall musculature with electrocautery. (I) The edges of the central plate are sutured to the lateral abdominal wall musculature along the scored line. (J) Lateral flaps are brought together in the midline, with closed suction drains placed between the lateral flaps and the central plate. Skin is brought together in the midline, enveloping the previously isolated umbilicus. Source: (From Woodard JR, Perez LM. Prune-belly syndrome. In: Marshall FF, ed. Operative urology. Philadelphia, PA: Saunders, 1996.)

Answer 66

Question 67

When is surgical repair recommended for Prune Belly Syndrome?

a) Immediately after birth
b) Around 6 months of age
c) During adolescence
d) In adulthood

Answer 67

b) Around 6 months of age

Explanation: Transabdominal or laparoscopic bilateral orchidopexy around 6 months of age is recommended for children with Prune Belly Syndrome. The extent and timing of urinary tract reconstruction is tailored to a given child’s bladder dynamics while taking into consideration respiratory status.

Question 68

When is ureteral reconstruction considered in Prune Belly Syndrome?

a) With repeated UTIs
b) With progressive anatomic or functional upper tract deterioration
c) Both a) and b)
d) Neither a) nor b)

Answer 68

c) Both a) and b)

Explanation: Ureteral reconstruction is considered in Prune Belly Syndrome with repeated UTIs or progressive anatomic or functional upper tract deterioration. Reduction cystoplasty may also be considered in cases of large urachal diverticulum or as part of a more extensive reconstruction, including an appendicovesicostomy to afford better long-term bladder emptying.

Question 69

What is the prognosis for Prune Belly Syndrome?

a) It is uniformly fatal
b) It results in chronic renal insufficiency in all cases
c) It has a high mortality rate in the perinatal period, and significant pulmonary difficulties and chronic renal insufficiency in survivors
d) It has no long-term effects on health

Answer: c) It has a high mortality rate in the perinatal period, and significant pulmonary difficulties and chronic renal insufficiency in survivors

Answer 69

c) It has a high mortality rate in the perinatal period, and significant pulmonary difficulties and chronic renal insufficiency in survivors

Explanation: Prune Belly Syndrome has a high mortality rate in the perinatal period due to severe pulmonary hypoplasia, and significant pulmonary difficulties have been reported in 55% of survivors. Up to one-third of survivors develop chronic renal insufficiency. However, adequate bladder emptying can help reduce the risk of UTIs and upper tract deterioration, and a normal pattern of sexual development is expected after orchidopexies.

Question 70

Table 6.3

Spinal Level of Myelomeningocele

Answer 70

Question 71

What is the most common cause of neurogenic bladder in children?
A) Cerebral Palsy
B) Neurologic Lesions
C) Meningomyelocele
D) Urinary tract infections

Answer 71

C) Meningomyelocele
Explanation: Meningomyelocele, which is a type of neural tube defect, is the most common cause of neurogenic bladder in children. It is caused by incomplete closure of the neural tube during fetal development, which results in the spinal cord and surrounding membranes protruding through an opening in the back. This condition can cause a range of neurologic abnormalities, including dysfunction of the lower urinary tract.

Question 72

What is the relationship between folic acid and neural tube defects?
A) High levels of folic acid during early pregnancy increase the risk of NTDs.
B) Low levels of folic acid during early pregnancy increase the risk of NTDs.
C) Folic acid has no effect on the risk of NTDs.
D) The relationship between folic acid and NTDs is not yet understood.

Answer 72

B) Low levels of folic acid during early pregnancy increase the risk of NTDs.
Explanation: Folic acid is important for proper development of the neural tube in early pregnancy. Women with low levels of folic acid during early pregnancy are at increased risk of having a fetus with a neural tube defect, including meningomyelocele.

Question 73

Which of the following is associated with almost all infants with spina bifida?
A) Cerebral Palsy
B) Hydrocephalus
C) Muscular Dystrophy
D) Down Syndrome

Answer 73

B) Hydrocephalus
Explanation: Almost all infants with spina bifida have Arnold-Chiari malformation, which is associated with hydrocephalus and developmental brain abnormalities. Arnold-Chiari malformation is a condition in which the lower part of the brain (cerebellum) extends down into the spinal canal. This can block the flow of cerebrospinal fluid, leading to hydrocephalus, which is an accumulation of fluid in the brain.

Question 74

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FIG. 6.14 Oxybutynin is a potent anticholinergic agent that dramatically delays detrusor contractions and lowers contraction pressure, as demonstrated on these two graphs. U.C., Uninhibited contraction.

Answer 74

Question 75

What is the recommended timing of prenatal closure for optimal neuromotor function and decreased need for ventriculo-peritoneal shunting?
A) Before 20 weeks of gestation
B) Before 26 weeks of gestation
C) Before 30 weeks of gestation
D) Before 34 weeks of gestation

Answer 75

B) Before 26 weeks of gestation
Explanation: Prenatal closure before 26 weeks of gestation has been shown to improve neuromotor function and decrease the need for ventriculo-peritoneal shunting. However, there is a significant risk of fetal demise, maternal morbidity, and preterm labor with prenatal intervention.

Question 76

What are the three categories of lower urinary tract dynamics that may be detected in postnatal testing?
A) Synergic, dyssynergic, and incontinent
B) Synergic, dyssynergic with poor detrusor compliance, and complete denervation
C) Overactive, underactive, and normal
D) High pressure, low pressure, and normal

Answer 76

B) Synergic, dyssynergic with poor detrusor compliance, and complete denervation
Explanation: Postnatal testing for lower urinary tract dysfunction involves renal-bladder ultrasound as early as possible after birth, baseline urodynamics, and serum creatinine obtained at 3 months. Three categories of lower urinary tract dynamics may be detected: synergic (26%), dyssynergic with and without poor detrusor compliance (37%), and complete denervation (36%).

Question 77

What is the primary goal of postnatal management for children with lower urinary tract dysfunction?
A) Prevention of bowel dysfunction
B) Preservation of renal function
C) Improvement of bladder control
D) Correction of hydrocephalus

Answer 77

B) Preservation of renal function
Explanation: The primary goal of postnatal management for children with lower urinary tract dysfunction is preservation of renal function by maintaining low bladder pressures and actively managing symptomatic UTI/VUR. Clean intermittent catheterization (CIC) is implemented if postvoid residuals are elevated. Early intervention with CIC and antimuscarinics to keep filling pressures less than 30 cm H2O improves the rate of UTI, VUR, and upper urinary tract deterioration, as well as the incidence of chronic kidney disease (CKD).

Question 78

What are the risk factors for renal dysfunction in children with lower urinary tract dysfunction?
A) Clean intermittent catheterization and antimuscarinics
B) Neurogenic bowel dysfunction and bladder control
C) High detrusor pressures, detrusor overactivity, febrile UTI, and VUR
D) Poor compliance and incomplete denervation

Answer 78

C) High detrusor pressures, detrusor overactivity, febrile UTI, and VUR
Explanation: Risk factors for renal dysfunction in children with lower urinary tract dysfunction include detrusor sphincter dyssynergia (DSD), high detrusor pressures, detrusor overactivity, febrile UTI, and vesicoureteral reflux (VUR). Management of neurogenic bowel dysfunction may also improve bladder function.