Posts Tagged omphalocele

Short Bowel Syndrome

Posted by on Monday, 4 October, 2010

What is Short Bowel Syndrome?

Short bowel syndrome (SBS) occurs after congenital (before birth) or postnatal loss of at least half of the small intestine, with or without loss of a large part of the intense carmine. The loss of the bowel may be due to disease or surgical removal. Short Bowel Syndrome (SBS) produces malabsorption, a condition where the body can not break and / or absorb sugars, proteins or fats.

short bowel syndrome

The extent of the problems associated with SBS depends mostly articles from the small intestine are affected. The small intestine normal height ranges between 10 and 28 meters and is divided into three sections.

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS).

Short bowel syndrome is the result of the alteration of intestinal digestion and absorption that occurs following extensive bowel resection. It represents a complex disorder that affects normal intestinal physiology with nutritional, metabolic, and infectious consequences.

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS).
The small intestine of the newborn is about 250 cm in length. In adulthood, small intestine, grows to about 750 cm. Consequently, infants and toddlers have a favorable long-term prognosis compared with an adult in terms of growth potential of the intestine after intestinal resection. Intestinal adaptation can take weeks or months to achieve, in the meantime, children who underwent intestinal resection require nutritional support through a variety of therapies, including parenteral nutrition. The duodenum and jejunum are responsible for the absorption of most dietary components with the exception of vitamin B-12 and bile acids.

Jejunum is characterized by long and wild large absorbent surface. Tight junctions are relatively large, so that the epithelium of larger molecules to pass more, and the free circulation and quick water and electrolytes. Comparison of the ileum is less wild, and the absorption capacity of surfaces unless the jejunum. In addition, tight connections are more stringent, reducing the flow of water and electrolytes in the vascular space into the intestinal lumen and, consequently, the ileum is a more efficient absorption into the water. Although nutrients are not as well absorbed in the small intestine, which has specific receptors on the site, the absorption of bile acid and vitamin B12. In addition, many hormones that affect the gastrointestinal tract, intestine, including enteroglucagon and peptide YY, produced in the ileum. 

Sites of nutrient absorption:

  • Duodenum – Iron
  • Jejunum – Carbohydrates, proteins, fat, vitamins
  • Ileum – Bile acids, vitamin B-12

In general, virtually all digestion and absorption is completed within the first 100-150 cm of jejunum in a healthy individual. In the absence of an intact colon, the minimum length of healthy bowel necessary to avoid parenteral nutrition is approximately 100 cm. Patients who have less than 100 cm of jejunum exhibit significant malabsorption. Although the ileum is limited in its capacity to form chylomicrons compared to the jejunum, studies have shown that the ileum has greater adaptive function as far as improving its absorptive function in the presence of short bowel syndrome. Similarly, studies in animals have shown that intestinal transit time is more likely to improve (ie, increase) in patients with proximal small-bowel resection as opposed to patients with distal small-bowel resection.

Since the jejunum can not develop site-specific carriers for the transport of vitamin B12 and bile salts, and therefore poorly absorbs permanently in patients after ileal resection. In addition, the loss of enteroglucagon and peptide YY can not be stressed in the regulation of bowel motility.

The leading causes of death among infants with short bowel syndrome who are treated with parenteral nutrition include central line sepsis and liver failure with prolonged use of parenteral nutrition.

The history of patients with short bowel syndrome (SBS) is usually a child born with a congenital anomaly, such as omphalocele, gastroschisis, intestinal atresia or, requiring small bowel resection. In addition, premature infants with necrotizing enterocolitis and require extensive bowel resection, with or without loss of the ileocecal junction also contribute to this patient population.

Other patients have a history of malrotation and volvulus, ischemic bowel, which had to undergo a bowel resection. Congenital short bowel syndrome is a rare cause of short bowel syndrome.

Children with short bowel syndrome may have various medical problems, according to the degree of intestinal resection and the level of medical complexity. The history should consider all potential ramifications of clinical case management of short bowel syndrome, including:

Parenteral nutrition

  • The degree of home nutrition support necessary in the management of a child on total parenteral nutrition (TPN) is noteworthy.
  • Guidelines for the safe use of parenteral nutrition have been established by the American Society for Parenteral and Enteral Nutrition.
  • Patients may present with issues entirely separate from the medical problems related to short bowel syndrome, including problems associated with intravenous access, infection, and signs and clinical symptoms associated with TPN-related liver disease.

Enteral Feeding

  • Quickly initiate enteral feeding for all children with short bowel syndrome.
  • Once again, patients can present a full history of medical problems associated with short bowel syndrome, including questions gastrostomy tube or nasogastric tube. For example, gastrostomy tubes may fall accidentally. In these patients, the immediate replacement of these tubes is important to maintain patency of the tube entry site.
  • Although complications are rare, be aware of the potential of gastric ulcer migration, and tube gastrostomy, intestinal obstruction, which may be associated with bilious vomiting and the risk of pancreatitis.

Nutrition

  • Supervise all children in enteral and parenteral long-term specific nutritional problems.
  • Patients may present with various symptoms related to specific nutritional deficiencies, including vitamins specific (or mineral) deficiencies and associated signs, symptoms and electrolyte disturbances and possible complications.

Medical and surgical histories: get a detailed account of past medical and surgical patient.

  • Pathology that leads to surgical resection
  • The extent and location of intestinal resection, the presence or absence of ileocecal valve.
  • Medical complications
  • RPT dependence
  • Enteral nutrition
  • enteral access
  • Type nutritional formula used
  • Dietary Supplements
  • Drugs
  • Allergies

History of complications associated with short bowel syndrome

  • Malabsorptive diarrhea
  • Dehydration
  • Vomiting
  • Bloating
  • Gastroesophageal reflux
  • Failure to thrive
  • Drug toxicities

Omphalocele

Posted by on Sunday, 9 May, 2010

Is an omphalocele is a birth defect, which is an abnormality that occurs before birth as a fetus is forming in its mother’s uterus. Some of the abdominal organs protrude through an opening in the abdominal muscles in the area of the umbilical cord. A translucent membrane covers the protruding organs.

The omphalocele may be small, with only a portion of the intestine protruding outside the abdominal cavity, or large, with most of the abdominal organs (including intestine, liver, and spleen) present outside the abdominal cavity. Further, the abdominal cavity itself may be small due to underdevelopment during pregnancy.

omphalocele

As a fetus is growing in the mother’s uterus before birth, different organ systems are developing and maturing. Between the 6th and the 10th weeks of pregnancy, the intestines actually project into the umbilical cord as they are growing. By the 11th week of development, the intestines should return to the abdomen. When the fetus is growing and developing during pregnancy, there is a small opening in the abdominal muscles that the umbilical cord can pass through, connecting the mother to the fetus. As the fetus matures, the abdominal muscles should meet in the middle and grow together, closing off this opening. An omphalocele occurs when the abdominal organs do not return to the abdominal cavity as they should.
It is not known what causes omphalocele. Steps that normally happen in the development of the abdominal organs and muscles simply did not happen properly. It is not known to be caused by anything the mother did during pregnancy.

When an omphalocele is isolated (no other birth defects are present), the risk for it to happen in a future pregnancy is one percent or one in 100. There are some families that have been reported to have an omphalocele inherited as an autosomal dominant or X-linked recessive trait. In these cases, the chance for reoccurrence would be higher.

Many babies born with an omphalocele also have other abnormalities. The chance for reoccurrence depends upon the underlying disorder. Thirty percent have a chromosomal (genetic) abnormality, most commonly Trisomy 13, Trisomy 18, Trisomy 21, Turner syndrome, or triploidy.

Some infants with omphalocele have a syndrome known as Beckwith-Wiedemann syndrome.
More than half of babies with omphalocele have abnormalities of other organs or body parts, most commonly the spine, digestive system, heart, urinary system, and limbs.

A “small” type omphalocele (involving protrusion of a small portion of the intestine only) occurs in one out of every 5,000 live births. A “large” type omphalocele (involving protrusion of the intestines, liver, and other organs) occurs in one out of every 10,000 live births. More boys than girls are affected with omphalocele.
Since some or all of the abdominal organs are outside the body, infection is a concern, especially if the protective membrane around the organs breaks. Also, an organ may lose its blood supply if it becomes pinched or twisted. A loss of blood flow can damage the affected organ.

Omphalocele can often be detected on fetal ultrasound in the second and third trimesters of pregnancy. A fetal echocardiogram (ultrasound of the heart) may also be done to check for heart abnormalities before the baby is born. After birth, the omphalocele can be noted by the physician during the physical examination. X-rays (diagnostic tests which use invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film) may also be done after birth to evaluate abnormalities of other organs or body parts.

Specific treatment for an omphalocele will be determined by your baby’s physician based on the following:
your baby’s gestational age, overall health, and medical history the extent of the condition
your baby’s tolerance for specific medications, procedures, or therapies expectations for the course of the condition your opinion and preference.,/p>

For a “small” omphalocele (only a portion of the intestine protruding outside the abdominal cavity), shortly after birth, an operation is done to return the organs to the abdomen and close the opening in the abdominal wall.

For a “large” omphalocele (most of the abdominal organs, including intestine, liver, and spleen are present outside the abdominal cavity), the repair is done in “stages” and may include the following:
Initially, sterile, protective sheeting is placed over the abdominal organs. Because the abdomen may be small and underdeveloped, it may not be able to hold all of the organs at once. Therefore, the exposed organs are gradually moved back into the abdomen over several days or weeks. The abdominal wall is closed surgically once the organs have been returned to the abdominal cavity. Also because of underdevelopment and small size of the abdominal cavity the organs may be swollen, a baby with an omphalocele may have breathing difficulties as the organs are returned to the abdomen. Your baby may need help from a breathing machine called a mechanical ventilator while the swelling is decreasing and the size of the abdominal cavity is increasing.

Problems in the future often depend on:

  • The size of the omphalocele.
  • If there was a loss of blood flow to part of the intestine or other organs.

the extent of other abnormalities.

Babies who have damage to the intestines or other abdominal organs may have long-term problems with digestion, elimination, and infection.