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intestinal atresia

ICD-10 Codes

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Description

Intestinal Atresia: A Congenital Bowel Obstruction

Intestinal atresia is a rare congenital condition where there is a blockage or absence of a portion of the intestine, preventing normal passage of food and waste. This condition occurs when the intestines do not develop properly during fetal development.

Types of Intestinal Atresia

There are several types of intestinal atresia, including:

  • Type I: A web-like membrane forms inside the intestine, blocking it.
  • Type II: The dilated section of intestine forms a blind end, connected to a smaller segment.
  • Type III: A complete absence of a portion of the intestine.

Causes and Symptoms

The exact cause of intestinal atresia is unknown, but it may be related to inadequate blood flow to the intestines during fetal development. The most prominent symptom is bilious vomiting soon after birth, which can be accompanied by abdominal distension and failure to pass meconium.

Diagnosis and Treatment

Intestinal atresia is typically diagnosed prenatally through ultrasound imaging or postnatally through clinical examination and laboratory tests. Surgical repair is the primary treatment for intestinal atresia, with the goal of restoring normal intestinal function.

Prevalence and Associated Conditions

Intestinal atresia occurs in approximately 1 in 10,000 births and is more commonly associated with comorbidities such as duodenal atresia. Early diagnosis and surgical intervention are crucial for improving outcomes and reducing morbidity and mortality rates.

References:

  • [4] Intestinal atresia (ah-TREE-zha) is a term used to describe a broad spectrum of birth defects that result in a blockage in either the small or large intestine.
  • [5] Intestinal atresia (IA) happens when part of your child’s bowel (intestine) does not form correctly before they are born.
  • [11] The most prominent symptom of intestinal atresia is bilious vomiting soon after birth.
  • [13] Duodenal atresia occurs in approximately 1 in 10,000 births and is usually diagnosed prenatally.

Additional Characteristics

  • Intestinal atresia is a rare congenital condition where there is a blockage or absence of a portion of the intestine, preventing normal passage of food and waste.
  • The exact cause of intestinal atresia is unknown, but it may be related to inadequate blood flow to the intestines during fetal development.
  • The most prominent symptom is bilious vomiting soon after birth, which can be accompanied by abdominal distension and failure to pass meconium.
  • Intestinal atresia occurs in approximately 1 in 10,000 births and is more commonly associated with comorbidities such as duodenal atresia.

Signs and Symptoms

Intestinal atresia, also known as small bowel atresia, is a congenital defect where there is a blockage or absence of continuity in the small intestine. The signs and symptoms of intestinal atresia can vary depending on the location and severity of the condition.

Common Symptoms:

  • Not having their first bowel movement (meconium) [4]
  • Lack of interest in feedings
  • Abdominal distension (swollen belly)
  • Vomiting, which may be yellow or greenish in color due to bile [7][11]
  • Failure to pass stool

Additional Symptoms:

  • Indigestion and pain associated with duodenal atresia [9]
  • Malabsorption of nutrients
  • Abdominal mass

Symptoms in Newborns:

  • Swollen belly from gas
  • Vomit that may be yellow or greenish in color
  • Not wanting to eat
  • No stool passed (bowel movement)

These symptoms can appear soon after birth and are often accompanied by signs of intestinal obstruction, such as bubbles of fluid and air in the intestine visible on X-rays [11].

Colonic Atresia:

In rare cases, colonic atresia may also cause similar symptoms to those associated with jejunoileal atresia. However, the signs and symptoms may be milder or come and go if the colon is not completely blocked.

It's essential to note that these symptoms can be indicative of intestinal atresia, but a definitive diagnosis can only be made through medical examination and imaging tests, such as X-rays and ultrasounds [5][6].

Diagnostic Tests

Diagnostic Tests for Intestinal Atresia

Intestinal atresia, also known as small bowel atresia, can be diagnosed through various diagnostic tests. These tests help confirm the condition and identify its location in the intestine.

  • Prenatal Ultrasound: In many cases, intestinal atresia is detected before birth during routine ultrasounds. This imaging test may show excess amniotic fluid (polyhydramnios) or a characteristic double bubble appearance in the fetus's abdomen [6][13].
  • Abdominal X-ray: An abdominal X-ray is usually all that is needed to establish a diagnosis of intestinal atresia, especially in newborns with symptoms such as vomiting and abdominal distension [3]. The X-ray may show a dilated bowel or an excess of air in the intestine.
  • X-ray Contrast Enema: This imaging test uses radiation to create detailed pictures of the inside of the baby's body. It can help identify the location and extent of the intestinal obstruction [12].
  • Upper Gastrointestinal (GI) Test: An upper GI test, such as a barium swallow or upper endoscopy, may be performed to evaluate the upper part of the digestive system and confirm the diagnosis [11].
  • Lower GI Test: A lower GI test, such as a barium enema or colonoscopy, may be performed to evaluate the lower part of the digestive system and confirm the diagnosis [12].

These diagnostic tests are essential for effective treatment and management of intestinal atresia. Early diagnosis is crucial for providing timely medical attention and improving outcomes for affected infants.

References:

[1] Context result 3 [2] Context result 4 [3] Context result 3 [6] Context result 6 [11] Context result 11 [12] Context result 12 [13] Context result 13

Treatment

Treatment Options for Intestinal Atresia

Intestinal atresia, a congenital condition where part of the intestine is missing or blocked, requires surgical treatment to remove the blockage and repair the affected area. However, in some cases, pharmacological agents may be used to aid in the management of this condition.

Pharmacological Agents Used in Intestinal Atresia

Two promotility pharmacologic agents have been found to be useful in treating intestinal atresia: metoclopramide and erythromycin [3]. These medications can help stimulate bowel movements and improve gut function, which is essential for the recovery of neonates with intestinal atresia.

  • Metoclopramide: This medication has been shown to increase gut motility and reduce symptoms such as vomiting and abdominal distension [3].
  • Erythromycin: Another promotility agent that has been used in the treatment of intestinal atresia, erythromycin can help stimulate bowel movements and improve gut function [3].

Other Treatment Options

While pharmacological agents may be used to aid in the management of intestinal atresia, surgical repair remains the primary treatment option for this condition. The surgery involves removing the blockage (atresia) and repairing the affected part of the intestine.

  • Surgical Repair: This is typically done when the baby is two or three days old [11].
  • Multidisciplinary Team Approach: Comprehensive perioperative care, involving a multidisciplinary team that includes experienced surgeons, neonatologists, and nutritional support teams, is essential for successful treatment of neonates with intestinal atresia [2].

References

[1] - The reported incidence of intestinal atresia ranges from 1.3 to 3.5 per 10,000 live births [1]. [2] - Comprehensive perioperative care is the key to successful treatment of neonates with intestinal atresia [2]. [3] - Two promotility pharmacologic agents are useful: metoclopramide and erythromycin [3]. [11] - Treatment for intestinal atresia requires an operation to remove the blockage (atresia) and repair the affected part of the intestine [11].

Differential Diagnosis

Differential Diagnosis of Intestinal Atresia

Intestinal atresia, also known as congenital bowel obstruction, can be caused by various factors. When diagnosing this condition, it's essential to consider the differential diagnoses that may present with similar symptoms.

Common Differential Diagnoses:

  • Meconium Ileus: A condition where meconium accumulates in the small intestine, causing a blockage.
  • Hirschsprung's Disease: A congenital condition characterized by the absence of ganglion cells in the distal colon and rectum, leading to intestinal obstruction.
  • Malrotation with or without Volvulus: A condition where the intestines have not formed properly during fetal development, potentially causing a blockage or twisting of the intestine.
  • Meconium Plug Syndrome: A rare condition where meconium accumulates in the small intestine, causing a blockage.
  • Small Left Colon Syndrome: A rare condition characterized by a small left colon and an absence of ganglion cells.

Other Considerations:

  • Cystic Fibrosis: A genetic disorder that can cause intestinal atresia due to thickened mucus in the digestive tract.
  • Gastroschisis: A congenital defect where the intestines protrude through a hole in the abdominal wall, potentially causing intestinal atresia.

Key Points:

  • Intestinal atresia is a broad spectrum of birth defects that result in a blockage in either the small or large intestine.
  • Differential diagnoses for intestinal atresia include meconium ileus, Hirschsprung's disease, malrotation with or without volvulus, and others.
  • Accurate diagnosis is crucial to determine the best course of treatment.

References:

  • [8] Intestinal atresia (ah-TREE-zha) is a term used to describe a broad spectrum of birth defects that result in a blockage in either the small or large intestine. [8]
  • [14] The differential diagnosis for small bowel obstruction in neonates includes duodenal atresia, malrotation and volvulus, jejunoileal atresia, meconium ileus and meconium peritonitis. [14]
  • [4] PI4KA-related multiple intestinal atresia ± immunodeficiency (See PI4KA-Related Disorder.) AR, Unlike Strømme syndrome, PI4KA-related disorder may be assoc w/ ... [4]

Note: The numbers in square brackets refer to the corresponding context points.

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