What is Spontaneous Intestinal Perforation of the Newborn?
Spontaneous intestinal perforation in newborns, also called focal intestinal perforation or isolated perforation, is a single hole that occurs often at the end of the small intestine, specifically the part known as the terminal ileum. This dangerous condition primarily affects very small newborn babies, specifically those who weigh less than 3.3 pounds (birth weight <1500 g) or particularly underweight babies who weigh less than 2.2 pounds (birth weight <1000 g).
Spontaneous intestinal perforation can lead to serious illness and potentially death. The hole is usually found at one specific spot and typically affects the edge of the terminal ileum that’s not connected to the mesentery, a fold of membrane that attaches the intestine to the abdominal wall. In some cases, these holes can occur in the jejunum (the part of the small intestine that comes after the stomach and before the ileum) and colon as well.
What Causes Spontaneous Intestinal Perforation of the Newborn?
Spontaneous intestinal perforation is a condition where the intestine develops a hole without a clear cause. Being born prematurely is the main risk factor for developing this condition. However, there are several other risk factors that are divided into two categories: antenatal (before birth) and postnatal (after birth).
Antenatal risk factors include severe maternal inflammation of the amniotic fluid and membranes surrounding the baby (chorioamnionitis) accompanied by signs of vascular response in the fetus. This has been identified as a risk factor for this condition. Certain drugs taken during pregnancy, such as glucocorticoids, indomethacin, and magnesium sulfate, have also been linked to spontaneous intestinal perforation in very low birth weight infants, but this topic is still under debate.
Other risk factors include conditions associated with a lack of oxygen in the fetus, such as high blood pressure in the mother during pregnancy (preeclampsia), low amniotic fluid (oligohydramnios), growth restriction in the womb, and low APGAR scores, which measure a newborn’s health at birth, including activity (muscle tone), pulse (heart rate), grimace (reflexes), appearance (skin color), and respiration (breathing rate and effort).
Postnatal risk factors include giving the baby glucocorticoids early after birth. One study found that early postnatal glucocorticoids can lessen chronic lung disease but they also increase the incidence of spontaneous intestinal perforation. However, recent evidence suggests that a lower dose may improve respiratory outcomes without increasing the risk of intestinal perforation.
Early use of indomethacin, a type of medication, together with dexamethasone, another type of medication, has been linked with spontaneous intestinal perforation. But the association of indomethacin alone with spontaneous intestinal perforation remains a topic of debate.
In instances of spontaneous intestinal perforation, two types of bacteria—Candida and coagulase-negative staphylococcus epidermidis—have been found. However, it is unclear whether these bacteria caused the condition or were a result of post-perforation infections, as these are normal types of bacteria found in the gut of infants that are extremely low in weight at birth.
Feeding extremely low birth weight infants early after birth is linked to a lower incidence of spontaneous intestinal perforation. Certain components found in breast milk and colostrum (the first form of milk produced by the mammary glands of mammals immediately following delivery of the newborn) like arginine, glutamine, threonine, and polyunsaturated fatty acids are thought to help improve the intestinal wall’s structure.
Risk Factors and Frequency for Spontaneous Intestinal Perforation of the Newborn
Spontaneous intestinal perforation is a condition that primarily affects infants with extremely low birth weights. For very low birth weight infants (those less than 1500g) the incidence is 1-2%. The incidence increases to 5-8% for extremely low birth weight infants (those less than 1000g).
- Spontaneous intestinal perforation is more common in infants with lower gestational age.
- It’s reported more often in male infants compared to females.
- Though rare, there have been some cases in term infants.
- The median age when the perforation occurs is seven days, but it can happen anytime within the first 15 days of life.
Signs and Symptoms of Spontaneous Intestinal Perforation of the Newborn
Spontaneous intestinal perforation is a condition that can display a wide range of symptoms. Infants with this condition might experience a sudden swelling of their stomachs. Sometimes, their belly might even take on a blue-black color. As the problem gets worse, they may develop a condition called metabolic acidosis, which involves too much acid in the body. They may also exhibit rapid heart rate and low blood pressure as signs of an infection of the lining of the abdomen, known as peritonitis.
Testing for Spontaneous Intestinal Perforation of the Newborn
If there’s a suspicion of spontaneous intestinal perforation, the diagnosis is mainly based on specific signs and symptoms that a person presents with.
Usually, your doctor will also order some blood tests, but the results are not specific and hence, don’t confirm the disease on their own. You may have an increased number of white blood cells (termed leukocytosis), lower levels of red blood cells (anemia), a lower than normal number of platelets (thrombocytopenia), or increased liver function tests. The last ones essentially mean that certain substances, such as alkaline phosphatase or bilirubin, show higher than normal levels in your blood.
The most reliable way to confirm that a person has spontaneous intestinal perforation is through imaging methods that look inside the body. A common sign of this condition is pneumoperitoneum, which means that air has escaped into the space within the abdomen. This can often be seen on different types of X-ray pictures, including supine views (taken when you’re lying on your back) and lateral decubitus or cross-table lateral views (taken from the side). In some cases, an ultrasound of the abdomen can also be helpful. It can show an abnormal fluid that’s highly reflective to ultrasound waves (echogenic free fluid) in the abdominal cavity.
Treatment Options for Spontaneous Intestinal Perforation of the Newborn
Spontaneous intestinal perforation is a serious condition where a hole occurs in the intestine unexpectedly. It has a high death rate, so quick treatment is very important.
In terms of medical treatment, when a patient experiences this condition, their feeding and medications must be stopped temporarily. A tube is placed in either their nose or mouth that reaches to their stomach; this is done to relieve pressure from the abdomen. “Total Parenteral Nutrition”, a special type of nutrition given through a vein, is also started. If the patient’s blood pressure is low, certain medications are given to raise it. The patient will also receive antibiotics to prevent infection.
In terms of surgical treatment, there are typically two approaches: a bedside draining procedure or a surgical operation known as a laparotomy. The drain procedure is often done temporarily in infants who cannot withstand a full operation. In this case, the abdomen is drained of fluid to relieve pressure. On the other hand, a laparotomy involves the removal of the affected part of the intestine, followed by the creation of an artificial opening (stoma) in the abdomen.
Recent studies have looked at the drain procedure as a long-term solution, and found that the overall outcomes were similar to a laparotomy. This included similar time to restart feeding and similar length of hospital stay.
A large detailed study looked at infants with extremely low birth weight that had this condition. They found there was no significant difference in death rates or delayed developmental progress at 18 to 22 months of age between infants who initially had the draining procedure versus those who had a laparotomy.
However, because spontaneous intestinal perforation is rare and requires immediate intervention, research has been limited in assessing differences in outcomes based on the severity of the disease, the diagnosis made during surgery, and how disease progression affects the outcomes.
What else can Spontaneous Intestinal Perforation of the Newborn be?
When considering a diagnosis, it’s important to be aware of similar conditions. For example, spontaneous intestinal perforation, a condition that can occur in infants, can be mistaken for necrotizing enterocolitis, another condition found in infants with extremely low birth weight. Both conditions share similar symptoms, hence they are often studied together. However, it’s crucial to accurately distinguish between the two because they have different causes, risk factors, and outcomes.
To differentiate between the two conditions, doctors will explore several factors:
- Spontaneous intestinal perforation usually appears earlier in the infant’s life compared to necrotizing enterocolitis. On average, a newborn with spontaneous intestinal perforation will show signs at seven days of age, whereas signs of necrotizing enterocolitis typically occur around 14 days.
- Babies with spontaneous intestinal perforation are usually born significantly more prematurely and have a smaller birth weight compared to babies with necrotizing enterocolitis.
- Infections during pregnancy and certain medications can increase the likelihood of spontaneous intestinal perforation. Early feeding can be a risk factor for necrotizing enterocolitis. However, early feeding with specific substances like colostrum and breast milk can actually decrease the possibility of spontaneous intestinal perforation.
Spontaneous intestinal perforation can cause symptoms like a sudden increase in belly size and a darkened color of the abdomen. Necrotizing enterocolitis, on the other hand, tends to cause symptoms such as vomiting, bloody stools, and swelling of the abdomen. When using medical imaging for diagnosis, different things could be seen in each condition.
Finally, necrotizing enterocolitis is a condition that involves varying levels of inflammation and cuts off the blood supply to the intestines. It can lead to the spread of bacteria. Spontaneous intestinal perforation, however, is a condition that involves a specific area of perforation but the rest of the intestines typically appear normal.
What to expect with Spontaneous Intestinal Perforation of the Newborn
Spontaneous intestinal perforation, a condition where a hole develops in the intestine unexpectedly, can be quite dangerous. The risk of death for these cases is around 20 to 40%, and the outcome can depend heavily on how severe and widespread the perforation is. If an infection spread throughout the body (sepsis) is present, the prognosis can be even worse.
However, there have been significant advances in newborn care, including specialized nutritional support and antibiotics, which have significantly improved the survival rates for babies with this condition. However, those who survive often face challenges. These can include the need for surgical procedures, long-term dependence on parenteral nutrition (a method of getting nutrition directly into the bloodstream), extended stays in the hospital, and an increased need for mechanical breathing assistance.
In addition to these challenges, babies with spontaneous intestinal perforation are also at a higher risk for developing a number of medical conditions. These can include bronchopulmonary dysplasia (a type of chronic lung disease), intraventricular hemorrhage (bleeding in the brain), periventricular leukomalacia (damage to the white matter near the ventricles of the brain), and issues with growth. These babies also face an increased risk of neurodevelopmental impairments, meaning they may have difficulties with learning, communication, and other aspects of development.
Possible Complications When Diagnosed with Spontaneous Intestinal Perforation of the Newborn
The problems that may occur after a spontaneous gut rupture can take many forms. These include complications from surgery, having a longer than normal hospital stay, and requiring parenteral nutrition, which is nutrition given through a vein. Surgery may result in adhesions (tissues sticking together) and scarring, possibly leading to narrowing or blockage of the intestines. Other problems could include short bowel syndrome (when part of the small intestine is missing or isn’t working properly), cholestasis (a condition where bile cannot flow from the liver to the small intestine), intestinal failure (when the intestines are not digesting food or absorbing the nutrients effectively), nutritional deficiencies, and failure to thrive (a general term for children who aren’t growing or developing as expected).
Possible Risks:
- Surgery complications
- Long hospital stay
- Parenteral nutrition
- Adhesions and scarring
- Narrowing or blocking of the intestines
- Short bowel syndrome
- Cholestasis
- Intestinal failure
- Nutritional deficiencies
- Failure to thrive
Recovery from Spontaneous Intestinal Perforation of the Newborn
After an operation for a sudden hole in the intestines, babies should be given antibiotics through a vein and full nutrition through an IV drip. The spot where a surgical opening (stoma) was made should be closely watched. Comprehensive healthcare, such as keeping track of fluid intake and output, managing any abnormalities in blood mineral levels, preventing a condition of low red blood cell count (anemia), and so on should be kept up after the operation. Many babies may also need support for breathing. When to start giving normal feedings after the operation should be decided by the main care team and the surgical team, depending on how the baby recovers after the operation.
Preventing Spontaneous Intestinal Perforation of the Newborn
Getting a better knowledge of what causes spontaneous intestinal perforation (a sudden tear in the intestine) could help us develop improved strategies for prevention. Both conditions before and after birth that lead to low oxygen levels in the blood and reduced blood flow to the intestines have been identified as factors that increase the risk of developing this condition.
Medications such as indomethacin (a drug used to reduce inflammation) and glucocorticoids (a type of steroids) may need to be used carefully in newborn babies who have these risk factors. Feeding from the get-go, particularly with breast milk and colostrum (the first form of milk produced by the mammary glands of mammals immediately following delivery of the newborn), should be encouraged. This can assist with the newborn’s health and help in preventing such conditions.
