Bladder Anomalies in Children

Exstrophy Disorders

The Spectrum of exstrophy disorders ranges from mild to the most severe end of the spectrum.

• The mild version,  epispadias, is seen in which the penial or clitoral part is involved.
•  Bladder exstrophy occurs in the middle part and has modest severity.  It is a common exstrophy disorder. 
• The most severe form is cloacal exstrophy, in which the hindgut is also involved with the urinary tract.

Bladder Exstrophy

• Known as ectopia vesicae.
• A developmental disorder occurs when the urinary bladder is outside the abdominal cavity, and the bladder mucosa is exposed to the environment.
• NO involvement of hindgut-like cloacal exstrophy.
• It is more common in males than females by a 2:1 ratio.
• Incidence -1:35000 live births.

Causes of Bladder Exstrophy Development

• The intestine develops outside the abdominal cavity in the early stages of life (6- 10 weeks). After 10 weeks, it returns to the Abdominal cavity and converses into various folds to form the anterior abdominal wall.
• There are 4 types of embryonal abdominal folds - 

• Cranial abdominal folds and
•  Caudal abdominal folds 
• Right lateral 
• Left lateral abdominal folds.
• Folds converge together and constitute the anterior abdominal wall.

• Defect in the embryonal cranial anterior abdominal fold - sternal absence.
• Defect in the caudal anterior abdominal fold - bladder exstrophy.
• Defect in the right or left lateral anterior abdominal fold- omphalocele 

Features of bladder exstrophy 

• Bladder mucosa exposed to outside results in -
  • Total urinary incontinence
  • Risk of adenocarcinoma of the urinary bladder
• Separation of rectal muscles.
• Slightly downward displacement of the umbilicus. Lie above the bladder mucosa.
• Widely separated pubic rami.
  •  Abnormal Pelvis, Gait abnormality.
• Inguinal hernias.
• Short stature.
• Genitalia abnormalities
  •  In males -total epispadias are associated with the dorsal chordee. A shallow scrotum and short penile length 
  • In Females - bifid clitoris and widely separated labia majora.

Management of bladder exstrophy

• Cover the exposed urinary bladder with Sterile plastic wrap.
• Avoid application of gauze pieces or petroleum jelly, which increases the risk of severe mucosal inflammation.
• Avoid latex-based products.
• SURGERY -Two approaches:-
  • Single-stage reconstruction involves all repairs in a single stage. It is a high risk of injury to penial structures and a risk of incomplete repair. 
  • Staged approaches - in this three surgeries are performed.
    • The first stage of surgery is done within 48 hours or after stabilization between 1 and 2 weeks of postnatal life. The bladder wall is repaired.
    • In the second stage surgery, closure of epispadias at the age of 1-2 years.
    • In the third stage of surgery, the bladder neck is repaired at the age of 3-6. 

• The overall result of the staged approach is slightly better and fewer risks of fertility issues.
• Bilateral iliac osteotomy is needed in all patients.

Cloacal Exstrophy

• The most severe form of exstrophy.
• Involves the exposure of the urinary bladder and cloacal hindgut structures, especially the distal colon and rectum.
• OEIS syndrome
  • O -omphalocele
  •  E - exstrophy
  •  I -imperforate anus
  •  S - spinal defects
• Shows total urinary as well as stool incontinence.

Cloacal exstrophy features:

• Omphalocele is present.
• Urinary bladder is split/ hemi bladder. 
• Prolapsed ileum forms a deformity called elephant trunk deformity.

Bladder Diverticula

• Diverticula - outpouching or herniation of urinary bladder mucosa from defects in the urinary bladder muscles.
• It can be an isolated finding or secondary to other anomalies, such as posterior urethral valve (PUV) and syndromic features.
• The syndromic features are - ehlers danlos syndrome, william syndrome, and menke syndrome.
• The most common site - the ureteropelvic junction.
• Silent if small in size or associated with VUR.
  •   Compromise the flap valve mechanism present at the ureteropelvic junction.

Management of bladder diverticula

• Only observation if the diverticula is small and silent.
• If associated with VUR or significantly large - excisions of bladder diverticula 

Urachal Anomalies

• Urachus is embryonic channel or communication between dome of Urinary bladder and allantoic structure (related to umbilical cord). normally disappears.
• If it persists, it can form various deformities - urachal anomalies.

Types of Urachal Anomalies

• First type- patent urachus, urachal fistula.
  • If entire structure is patent from the umbilical cord to the urinary bladder.
  •  Increased incidence of UTIs and seepage of urine from umbilical regions.
  • Increase risk of VESICOUETERIC REFLEX.
• Second type - Umbilico-urachal sinus.
  •  patency is present in the upper part and ends in a blind track.
  • Asymptomatic, or secondarily infected.
• The third type - vesico-urachal diverticulum.
  • urinary bladder communicates with the lower part of the urachus but not outside.
• Fourth type - urachal cyst
  • The proximal and the distal part of the urachus are obliterated, but the central part persists. urachal cyst.
  • Appear as palpable abdominal mass below the umbilical.
  • May show secondary infections.  

Treatment of urachal anomalies is surgery.

MCU /VCU needed – symptoms of UTI present.

Prune Belly Syndrome

• Prune Belly Syndrome is also known as Eagle Barrett syndrome.
• More common in males than females.
•  A triad of;-
  • Wrinkling and laxity of the anterior abdominal wall.
  • Dilatation and abnormalities of the urinary tract, including the urinary bladder.
  • Undescended testis.

Associations of Prune Belly Syndrome

• High risk of developing pectus excavatum.
• High risk of pulmonary hypoplasia.
• Developmental dysplasia of the hip joint.
• High risk of VUR-Recurrent UTI

Treatment of Prune Belly Syndrome

Surgery

Frequently Asked Questions:

Q: What is the treatment for Cloacal exstrophy?

Answer: Treatment includes surgical reconstructions and a permanent urinary diversion with colostomy.

Q: The defect in the embryonal cranial anterior abdominal fold causes which anomaly in fetus?

Answer: Sternal absence.

Q: Which defect of the abdominal fold causes bladder exstrophy in fetus?

Answer: The Defect in the caudal anterior abdominal fold causes Bladder exstrophy.

Q: What causes the formation of Omphalocele?

Answer: A right or left lateral anterior abdominal fold defect causes omphalocele.