Total colonic aganglionosis
Total colonic aganglionosis
Description, Causes and Risk Factors:
The state of being without ganglia; e.g., absence of ganglion cells from the myenteric plexus as a characteristic of congenital megacolon. Total colonic aganglionosis (TCA) accounts for approximately 3% to 12% of all infants with HD. The prevalence in boys and girls is nearly equal.
Total colonic aganglionosis is a birth defect in which nerve cells known as ganglion cells are lacking in the large intestine. As a result, the large intestine cannot move stool through and becomes blocked, causing the abdomen to swell.
The majority of patients with aganglionosis are diagnosed in the neonatal period. Since it is unusual for a full term and premature neonate not to have a stool within 24 hours and 48 hours, respectively, the first sign may be the failure to pass a first stool without assistance. This is followed by abdominal distension and vomiting.
Sometimes the disease is subtle, and is not suspected until later. In older children, there is almost always a history of never having had a normal bowel movement. Most of these children have a markedly protuberant abdomen. Advanced disease may result in watery diarrhea (as only liquid can get by the obstructed stool), colitis (bacterial overgrowth and inflammation of the colon), and even life-threatening sepsis (blood borne infection).
Total colonic aganglionosis remains a serious surgical challenge. Patients suffering from the condition, have multiple complications, sequelae, and often require re-operations. We found that it is possible to prevent many of these by properly fixing the stoma, avoiding pouch or patch procedures, delaying ileostomy closure, having pathology expertise, and with meticulous surgical technique starting the dissection/anastomosis well above the dentate line.
Symptoms may include:
Abnormal bowel movement.
The customary approach to the diagnosis of total colonic aganglionosisis based on the existence of a single distal aganglionic region extending to the anal margin. Segmental aganglionosis, however, may involve only a limited segment of colon which is interposed between lengths of normal bowel. Awareness of this variant aids in the interpretation of the barium enema in children with signs and symptoms of aganglionosis, especially when the rectal biopsy is normal.
Other tests may include:
Barium enema: Contracted distal colon, abrupt transition to a dilatedproximal colon.
Anorectal manometry: Absence of relaxation of internal anal sphincter inresponse to rectal distention.
Suction rectal biopsy: Absence of ganglion cells. Should be done at 2-4 cmfrom dentate line; must have adequate submucosa.
Abdominal x-ray: Loops of distended intestine, increased air in rectum.
The diagnosis and treatment of TCA are difficult, but histological evaluation has been confirmed in the establishment of the diagnosis in recent years. Several surgical techniques have been utilized for the treatment of TCA. Nevertheless, little information is available for optimal management of recurrent complications caused by failed procedures.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
A new research from the University of Edinburgh, UK, suggests that cheap cilostazol tablets may reduce damage to arteries, which lead to blood clots, resulting in strokes and cognitive decline. The researchers plan to assess the medications’ ability to cut the risk of...
A new study from the US discovers that flavonoids, natural compounds found in fruits and vegetables, may help preserve the lung function, which tends to decline with age. For the study, a team of researchers looked at data from 463 adults from Norway and England whose...
Quiz about this article
0 of 2 questions completed
Please answer on few questions to make our service more useful
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
0 of 2 questions answered correctly
Time has elapsed
You have reached 0 of 0 points, (0)
Question 1 of 2
Was this article useful for you?Correct
Thanks for your feedback!Incorrect
Thanks for your feedback!
Question 2 of 2
What else information about this disease you want to know ?Correct
Thanks for feedback!Incorrect
Thanks for feedback!
Good weather is the best reason to do outdoor sports, which will help not only lose weight, but also will strengthen health. Bicycle The sun dries out the local paths, so you can safely sit on your favorite bike and confidently twist the pedals, where the eyes look....read more
First aid for injuries consists of simple rules that need to be clearly implemented. If this is a closed injury, you need to immobilize the injured limb, otherwise the person may lose consciousness from a painful shock. If you need to get to the emergency room...read more
Many people spontaneously decide starting to do sport, while others weigh all the pros and cons for a log time. But almost all of them make the same mistakes, listening to the advice of non experts. There are 10 anti-tips for those who want to do plan to do some sport...read more