“A persistent cloaca is defined as a confluence of the rectum, vagina, and urethra into a single common channel. This defect is one of the most formidable technical challenges in pediatric surgery. The goals of treatment include an anatomic reconstruction and achieving bowel and urinary control, as well as normal sexual function. Cloacal anomalies occur in 1 per 20,000 live births. They occur exclusively in girls and comprise the most complex defect in the spectrum of anorectal malformations. “
“Persistent cloaca is an uncommon (1 per 50,000 live births) congenital anomaly in which there is a single perineal opening for the urinary, gastrointestinal, and reproductive tracts.1 More common in multifetal gestations, persistent cloaca results from abnormal development of the cloacal membrane before 50 days postfertilization. In addition to a single perineal opening, secondary urogenital or gastrointestinal anomalies are often associated with persistent cloaca. Moreover, anomalies of other organ systems such as cardiac or skeletal malformations are known to coexist. “
Pull-Through Procedure (PSARP)
Founding Director Alberto Peña, MD, revolutionized the treatment of pediatric colorectal patients in 1980 when he introduced a surgical procedure called the posterior sagittal anorectoplasty (PSARP), or pull-through procedure.
The pull-through surgery technique provides greater surgical accuracy in repositioning the anus, minimizes damage to surrounding anatomical structures, reduces post-surgical pain and improves outcomes.
Ano-Rectal Malformation (ARM) / Imperforate anus
An imperforate anus or anorectal malformations (ARMs) are birth defects in which the rectum is malformed. ARMs are a spectrum of different congenital anomalies in males and females, that varies from fairly minor lesions to complex anomalies. The cause of ARMs is unknown; the genetic basis of these anomalies is very complex because of their anatomical variability. In 8% of patients genetic factors are clearly associated with ARMs. Anorectal malformation in Currarino syndrome represents the only association for which the gene HLXB9 has been identified.
Ano-Rectal Malformation (ARM)
Anorectal malformations are defects that occur during the fifth to seventh weeks of fetal development.
With these defects, the anus (opening at the end of the large intestine through which stool passes) and the rectum (area of the large intestine just above the anus) do not develop properly.
More info on:
What is an Ano-Rectal Malformation (ARM)? Ano-rectal is a word which describes the last bit of the bowel: the anus and the rectum. The anus is the opening in your bottom, through which poo’s are pushed out. The rectum is the bit of bowel before the anus. The cause of ARMs is not known and families need to be reassured that there is nothing they could have done to either cause or prevent the problem from occurring. Ano rectal malformations affect 1:3300 to 1:5000 live births.
More info at:
Imperforate Anus in Girls
The internal anatomy of girls with imperforate anus usually can be predicted from a careful study of the visible openings in the perineum, the area around her bottom. Three openings typically are seen. Usually the urethral (where urine comes out) and vaginal openings are normal, and the third opening represents a fistula (an abnormal opening) either at the level of the normal anus or more commonly as an opening at an abnormal site in front of where the anus should be. This third opening may not be obvious and can be difficult to see.
BOWEL CONTROL PROGRAME
Bowel training refers to behavioral programs designed to help people with bowel disorders establish or reestablish control. Individuals with symptoms of inability to control bowel movements, incomplete emptying, or chronic constipation may benefit from these programs. Bowel retraining works by teaching new skills or strategies to develop a routine and predictable schedule for evacuation. This prevents constipation and decreases unpredictable elimination.
Bowel training programs generally take these three basic principles into account:
- Improve consistency of stool.
- Establish a regular time for elimination.
- Stimulate emptying on a routine basis.
Understanding bowel training for children with Hirschsprung’s Disease and other Ano-Rectal Malformations :
Vocabulary – list of conditions:
UTI – A urinary tract infection
Urinary tract infections (UTIs) are a common type of infection caused by bacteria (most often E. coli) that travel up the urethra to the bladder. A bladder infection is called cystitis. If bacterial infection spreads to the kidneys and ureters, the condition is called pyelonephritis. Cystitis is considered a lower urinary tract infection. Pyelonephritis is an upper urinary tract infection and is much more serious.
Most informative ( in my opinion ) website that cover all UTI’s – link:
Symptoms of UTIs in Infants and Toddlers
UTIs in infants and preschool children tend to be more serious because they are more likely to involve the kidneys. (Older children are more likely to have lower urinary tract infections and standard symptoms.) Infants and young children should always be checked for UTIs if the following symptoms are present:
- A persistent high fever of otherwise unknown cause, particularly if it is accompanied by signs of feeding problems, listlessness and fatigue.
- Painful, frequent, and foul smelling urine. (Parents are generally unable to identify a UTI just by the smell of their child’s urine. Medical tests are needed.)
- Cloudy urine. (If the urine is clear, the child most likely has some other ailment, although it is not absolute proof that the child is UTI-free.)
- A recurrence of bedwetting or poor urine control during the day in the child who had previously achieved control.
- Abdominal and low back pain may be present.
- Vomiting and abdominal pain (usually in infants)
CIC – Clean Intermittent Catheterisation
is a clean (not sterile), way to insert a catheter (hollow tube) into the bladder trough the urinary opening ( urethra) to release urine and empty the blader.
bacteria normally live in the intestines of healthy people and animals. Infections due to Escherichia coli bacteria can cause severe, bloody diarrhea. Some cases can result in kidney failure or other serious complications. Fortunately, most healthy kids who get the infection recover on their own without the need for treatment.
Klebsiella is a ‘superbug’ that causes a range of diseases, depending on which part of the body it infects.
Klebsiella pneumoniae is a bacterium that normally lives inside human intestines, where it doesn’t cause disease.
However, if K. pneumoniae gets into other areas of the body, it can cause a range of different illnesses.
- Bloodstream infections
- Wound infections
- Surgical site infections
- Urinary tract infections (UTI)
When a person has no symptoms of infection but significant numbers of bacteria have colonized the urinary tract, the condition is called asymptomatic UTI (also calledasymptomatic bacteriuria). The condition is harmless in most people and rarely persists, although it does increase the risk for developing symptomatic UTIs.
DMSA renal scan
VUR : Vesicoureteral reflux
Reflux is a disorder in which urine backs up (refluxes) from the bladder to the kidney.
“In normal kidney-bladder function, urine flows from the kidneys to the bladder. In children with vesicoureteral reflux (VUR), the urine flows backwards from the bladder up toward the kidneys. As a result, children with VUR are at risk for kidney infections and may develop kidney damage “
Cystoscopy, or cystourethroscopy, is a procedure that lets a urologist view the inside of the bladder and urethra in detail.
A cystoscope is a thin tube with a camera and light on the end. During a cystoscopy, a doctor inserts this tube through your urethra (the tube that carries urine out of your bladder) and into your bladder so they can visualize the inside of your bladder. Magnified images from the camera are displayed on a screen where your doctor can see them.
Picture is from : https://www.urologyhealth.or
General anesthesia means you’ll be unconscious during the cystoscopy. With general anesthesia, you may need to fast for several hours ahead of time.