Category Archives: Defects Of the Digestive system

Hirschsprung’s Disease (Congenital Megacolon)

Hirschsprung’s disease is a birth defect in which the nerves which regulate the rhythmic intestinal contractions are missing in a portion of the large intestine, affecting the movement of the fecal material towards the anus. This condition, which causes the symptoms usually associated with intestinal obstruction, is also known as congenital megacolon.

  • The large intestine is affected and normal contractions of the intestine are absent in the affected area.
  • Delay in passing the meconium by the newborn infant is the first indication. Abdominal distension, vomiting, and refusal of feeds, are the other symptoms which develop in the infant later on.
  • Biopsy of the rectum, and manometry to measure the rectal pressure, help diagnose this condition.
  • Surgical intervention is necessary, and it is aimed at facilitating the normal passage of fecal material through the large intestine.

The rhythmic movement of the large intestine is controlled by a several nerves in the intestinal wall which brings about the synchronized contractions which move as a wave down the digestive tract, carrying the intestinal contents along. Bowel movements are possible because of these contractions. When Hirschsprung’s disease (congenital megacolon) affects a section of the large intestine, it is unable to contract in the normal way, thus impeding the passage of the stool to the anus.

Normally, meconium, a sticky substance, dark green in color, is passed by the infants soon after birth.  Hirschsprung’s disease is suspected when the passage of this fecal material is delayed. Symptoms of intestinal obstruction such as abdominal distension, vomiting with bile in the vomit, and refusal to feed may appear later on. When only a limited area of the intestine is affected by Hirschsprung’s disease, the associated symptoms also may be mild, often resulting in the condition remaining undiagnosed till much later in childhood. Abdominal distension, passing ribbon-like stools, and the child not gaining weight normally, are also indications of Hirschsprung’s disease. Rarely, the only indication of this condition is chronic constipation.

A potentially fatal condition called toxic enterocolitis may result from Hirschsprung’s disease (congenital megacolon). Fever and abdominal distension develop suddenly, accompanied by explosive diarrhea, which may be bloody in some cases.

Biopsy of the rectum, and measuring the pressure in the rectum known as manometry, are the tests to detect Hirschsprung’s disease accurately. Another test called barium enema also may be helpful. In this test, barium as well as air is introduced into the rectum, and then x-rays are taken.

Toxic enterocolitis may develop if Hirschsprung’s disease (congenital megacolon) is not treated rapidly by removing the abnormal parts of the intestine. The unaffected part of the intestine is then attached to the rectum to facilitate the normal functioning of the digestive system. Occasionally, when an infant is severely ill, an alternate route is created for the passage of stools by a procedure called colostomy. The healthy part of the intestine is temporarily connected to an opening created on the abdomen to which a plastic bag is attached for the collection of stool. The defective, disconnected portion of the intestine remains in the abdominal cavity till the infant recovers, and becomes older and ready for further surgery. In a surgery known as the pull-through procedure, the defective portion is removed and the healthy portion of the intestine is reattached to the rectum for normal functioning. The opening in the abdomen is also closed.

Watch This Explanatory Video Of  Hirschsprung’s Disease

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Congential Diaphragmatic Hernia

A weak spot or hole in the diaphragm allowing the protrusion of certain abdominal organs into the thoracic cavity results in the condition referred to as congenital diaphragmatic hernia.

In 90% cases of diaphragmatic hernia, it is found to be present on the left side of the body.  Abdominal organs such as stomach and intestine may be protruding into the thoracic or chest cavity. Spleen and liver also may be herniated. The lung on the side of the hernia may not be fully developed when the hernia is quite large. Certain heart defects are found to occur in infants who have congenital diaphragmatic hernia.

At the time of birth, as the newborn takes in the first breath and cries, the gastrointestinal tract rapidly enlarges getting filled with air. The herniated digestive organ too becomes large and presses against the vital organs in the chest such as the heart and the lungs. This causes considerable breathing difficulty in the newborn, usually immediately after birth. Diaphragmatic hernia can be detected by an x-ray. Pre-natal ultrasound screening can detect this defect in the fetus. Surgical intervention is necessary to repair the weak spot or hole in the diaphragm. When it is detected before birth, surgery is planned in advance. Supplementary oxygen is given to the infant through a breathing tube, or respiratory assistance is provided by a ventilator, if necessary, to relieve breathing difficulty.

Watch The Video About Congential Diaphragmatic Hernia

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Biliary Atresia in Children

Biliary atresia in children is a condition resulting from the partial or complete destruction of the bile ducts, which prevents the bile from reaching the small intestine.

  • Biliary atresia causes the accumulation of bile in the liver leading to permanent liver damage.
  • Jaundice, characterized by the yellowing of the skin, dark colored urine and pale stools are the usual symptom. The liver is enlarged.
  • Ultrasound scan and blood tests help in diagnosing the condition. The bile ducts as well as the liver can be surgically examined for accurate assessment.
  • Bile ducts are created surgically to facilitate the flow of bile into the intestine.

The functions of the bile prodiced by the liver include digestion of fats and the removal of the waste products formed by the chemical processes taking place in the liver. Bile is collected by the bile ducts in the liver and then carried to the small intestine. Biliary atresia causes the accumulation of the bile within the liver, and some of it gets into the blood, resulting in the characteristic yellowing of the skin. Eventually, the liver becomes permanently damaged, as a condition called biliary cirrhosis develops in the infant, usually within two months of birth.

When infants have biliary atresia, they pass dark colored urine and very pale stools. The discoloration of the skin also progresses. In about two weeks of birth, a physical examination of the infant may help the doctor feel the enlarged liver which feels firm to touch. Within two or three months, infants with this condition may develop severe growth retardation. Itching and irritability are usually present. Large, visible veins on the abdomen and enlargement of the spleen are also common symptoms.

If biliary atresia is diagnosed within the first two months of life, biliary cirrhosis can be prevented. Several blood tests and ultrasound scans may be required to make an accurate diagnosis. If biliary atresia cannot be confirmed by these tests, the bile ducts as well as the liver is surgically examined to reach a conclusive diagnosis. A biopsy of the liver may be done too.

A permanent drainage for the bile needs to be surgically created. In about 50% of cases, bile ducts connecting to the small intestine can be constructed surgically and these replacement bile ducts facilitate the normal functioning. Infants, in whom surgical construction of the replacement bile ducts could not be done at birth, often need liver transplantation in about two years.

Watch This Video About Biliary Atresia in Children:

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Intestinal Malrotation

A potentially fatal birth defect known as intestinal malrotation results from abnormal or incomplete rotation of the intestines during the development of the fetus.

Twisting of the intestine, termed volvulus is often caused by intestinal malrotation. Infants who have malrotation can suddenly develop a volvulus which cuts off the blood supply to specific areas of the intestine. Midgut volvulus develops when the middle potion of the intestine is completely deprived of blood supply. Sudden swelling of the abdomen as well as diarrhea and vomiting may indicate this condition. The vomit may contain yellow or green colored bile or it may be rust-colored. Abdominal distension soon develops. A barium x-ray may help diagnose this condition. In this test, barium enema is administered before taking the x-rays so that abnormalities in the intestines can be clearly visible on the x-ray image.

Immediate surgical intervention, usually within hours of birth, may be required to save the infants who have this condition. Fluids are administered intravenously as part of the treatment. Intestinal tissue loss or even death may result if emergency surgery is not performed to correct the defect.

Watch This Video About Intestinal Malrotation (Volvulus):

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Anal Atresia

Narrowing of the anus or an obstruction occurring in the anal canal is referred to as anal atresia

Anal atresia impedes the passage of stool out of the body. Alternate channels called fistula are often present along with anal atresia. These fistulas may connect the anus to the urethra, or the urinary bladder, or to the vagina in female infants. Sometimes the fistula may open to the outside in the area between the anus and the urethra known as perineum.

When anal atresia is present, the newborn is unable to have normal bowel movements after birth. As the fecal matter accumulates in the digestive tract, intestinal obstruction develops. But, anal atresia is often detected during the physical examination of the infant immediately after birth, and usually well before the symptoms begin to appear.

Anal atresia is a birth defect which requires emergency surgical intervention. X-rays can show the abnormal channels or fistulas, if any. Anal obstructions are opened up during the surgery and the fistulas are closed at the same time. Sometimes, the stool is rerouted to a temporary opening created on the abdominal wall and attaching the colon to it. This surgical procedure is known as colostomy. The stool is collected in a plastic bag attached to the opening till another surgery is performed to reattach the colon to the anus.

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Esophageal Atresia And Tracheoesophageal Fistula

Narrowing of the esophagus is termed esophageal atresia. Sometimes, esophagus may end blindly. In tracheoesophageal fistula, an abnormal channel exists between the trachea and the esophagus.

The esophagus, or the food pipe, is an elongated, tube-like structure connecting the mouth and the stomach. When esophageal atresia is present, the passage of food into the stomach becomes restricted or even completely stopped. If tracheoesophageal fistula occurs, it can allow the food particles and the saliva to pass into the lungs through the fistula. It may precipitate potentially fatal situations such as serious infections and pneumonia in the infants. Food particles and fluid in the lungs decrease the ability of the lungs to oxygenate the blood, resulting in cyanosis or a bluish coloration of the skin. Coughing when trying to swallow, and drooling, are characteristic symptoms of esophageal atresia. Infants who have esophageal atresia as well as tracheoesophageal fistula are often found to be having congenital heart defects also.

 X-rays taken while a tube is being inserted into the esophagus help detect the blockage. Oral feedings are stopped immediately and fluids are administered intravenously. A suction tube is inserted into the upper part of the esophagus to remove the saliva before it reaches the lungs. Surgical correction of the abnormalities is performed as soon as possible. The connection of the esophagus to the trachea is closed and a connection from the esophagus to the stomach is opened up for the normal movement of the food.

Watch This Video About Esophageal Atresia:

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Overview of Digestive Tract Defects

Abnormalities occurring in the structure, position and function of the different parts of the digestive tract are referred to as digestive tract defects.

  • Abnormal positioning and incomplete development of the different digestive organs may result in blocks; defects in the nerves and muscles associated with the digestive tract may interfere with the proper functioning of the digestive system.
  • Abdominal distension and pain in the abdomen are the usual symptoms. Vomiting may be present.
  • Physical examination followed by x-rays help diagnose digestive tract defects.
  • Most birth defects of the digestive tract may require surgical correction.

Congenital digestive tract defects may occur in any part of the digestive tract, from the esophagus to the rectum. Often, the incomplete development may cause obstructions in the digestive tract at places where it is narrowed. Abnormal position of the organs also results in blockages.   Weakness of the muscular walls of the abdominal cavity may cause holes to develop. Congenital megacolon or Hirschsprung’s disease may occur due to the improper development of the nerves associated with the intestines Obstructions in the movement of fecal matter can result in the distension of the abdomen, severe pain and vomiting.

Birth defects of the digestive tract almost always require corrective surgery. Obstructions have to be opened up by surgery. If there are weak spots or holes in the muscular wall, they have to be repaired surgically.

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