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multiple intestinal atresia
Description
Multiple intestinal atresia is a rare form of intestinal atresia characterized by the presence of numerous atresic segments in the small bowel (duodenum) or large bowel [10][12][14]. This condition leads to symptoms of intestinal obstruction, including vomiting, abdominal bloating, and inability to pass meconium in newborns [10].
Multiple intestinal atresia is a congenital defect that occurs when there are multiple blockages or obstructions in the intestine, resulting in a short length of useful intestine [4]. This condition can be associated with other anomalies and is usually familial [4].
The diagnosis of multiple intestinal atresia is typically confirmed by abdominal radiograph, which shows the presence of multiple atretic segments in the small or large bowel [4].
Treatment for multiple intestinal atresia usually involves surgical correction, including multiple anastomoses, followed by total parenteral nutrition over the long term or permanently [15].
Additional Characteristics
- Vomiting
- Abdominal bloating
- Intestinal obstruction
- Surgical correction
- Total parenteral nutrition
- Inability to pass meconium
- Multiple blockages or obstructions in the intestine
- Short length of useful intestine
Signs and Symptoms
Multiple intestinal atresia, also known as hereditary multiple intestinal atresia (HMIA), is a rare congenital disorder characterized by the presence of multiple blockages in the small or large intestine. The symptoms of this condition can vary depending on the location and number of affected segments, but common signs and symptoms include:
- Vomiting: Infants with HMIA often experience vomiting, which may be bilious (containing bile) or non-bilious, depending on the location of the obstruction.
- Abdominal bloating: The accumulation of gas in the intestines can cause abdominal distension, making the abdomen appear swollen and tender to touch.
- Inability to pass meconium: Newborns with HMIA may have difficulty passing their first stool (meconium), which is a normal process that occurs within the first 24-48 hours after birth.
- Feeding intolerance: Infants with HMIA may show signs of feeding intolerance, such as refusal to feed or showing discomfort while eating.
These symptoms can occur due to the blockages in the intestine, which prevent food and waste from passing through normally. The severity and duration of these symptoms can vary depending on the individual case and the extent of the intestinal atresia.
According to [10] and [14], multiple intestinal atresia is characterized by the presence of numerous atresic segments in the small bowel (duodenum) or large bowel, leading to symptoms of intestinal obstruction. Similarly, [15] states that disease definition of Multiple intestinal atresia is a rare form of intestinal atresia characterized by the presence of numerous atresic segments in the small bowel (duodenum) or large bowel and leading to symptoms of intestinal obstruction: vomiting, abdominal bloating and inability to pass meconium in newborns.
References: [10] - Multiple intestinal atresia is a rare form of intestinal atresia It is characterized by the presence of numerous atresic segments in the small bowel (duodenum) or large bowel and leading to symptoms of intestinal obstruction: vomiting, abdominal bloating and inability to pass meconium in newborns [14] - Intestinal Atresia Multiple (Multiple Intestinal Atresia) is a rare form of intestinal atresia It is characterized by the presence of numerous atresic segments in the small bowel (duodenum) or large bowel and leading to symptoms of intestinal obstruction: vomiting, abdominal bloating and inability to pass meconium in newborns [15] - Disease definition. Multiple intestinal atresia is a rare form of intestinal atresia characterized by the presence of numerous atresic segments in the small bowel (duodenum) or large bowel and leading to symptoms of intestinal obstruction: vomiting, abdominal bloating and inability to pass meconium in newborns.
Additional Symptoms
- Abdominal bloating
- Feeding intolerance
- Inability to pass meconium
- vomiting
Diagnostic Tests
Multiple intestinal atresia, also known as familial intestinal polyatresia syndrome, can be diagnosed through various diagnostic tests.
- Prenatal Ultrasound: A prenatal ultrasound may show signs of intestinal obstruction, such as a dilated intestine or the presence of excess amniotic fluid (polyhydramnios) [9][10].
- Abdominal X-rays and Contrast Enema: After birth, an abdominal X-ray along with an X-ray contrast enema can confirm the diagnosis of multiple intestinal atresia [1].
- Endoscopy: Endoscopy may be used to visualize the inside of the intestine and identify areas of obstruction [5].
- Rectal Biopsy: A rectal biopsy may be performed to examine the tissue for any abnormalities [5].
- Abdominal Ultrasound and Plain Abdominal X-rays: These imaging tests can aid in confirming the diagnosis by showing signs such as the "double-bubble" sign or "string of pearls" appearance [7][8].
It's worth noting that a combination of these diagnostic tests may be used to confirm the diagnosis of multiple intestinal atresia. Additionally, genetic testing may also be recommended to rule out any underlying chromosomal abnormalities [8].
Treatment
Unfortunately, there is no specific drug treatment for multiple intestinal atresia (MIA). However, I found some relevant information in the search results that may be helpful.
According to [1], MIA is a rare form of intestinal atresia characterized by the presence of numerous atresic segments in the small bowel or large bowel. While there is no specific drug treatment for MIA, close management and monitoring of the pregnancy plays an important role in the ability to offer highly specialized postnatal care and improve outcomes for affected children [3].
In terms of medical management, it's essential to note that treatment for small bowel atresia, including MIA, typically involves surgical intervention to remove the blockage and reconnect the intestine [7]. However, I couldn't find any specific information on drug treatments that are commonly used in conjunction with surgery.
It's worth noting that some studies have explored the use of certain medications, such as corticosteroids, to manage symptoms associated with intestinal atresia. However, these findings are not specifically related to MIA [4].
In summary, while there is no specific drug treatment for multiple intestinal atresia, close monitoring and management of the pregnancy, along with surgical intervention, are essential in improving outcomes for affected children.
References:
[1] Multiple intestinal atresia (HMIA) is a rare form of intestinal atresia that affects the gastrointestinal tract from the pylorus to the rectum. A few cases have been reported in the literature. [3] Treatment for small bowel atresia. There is currently no prenatal treatment option available for small bowel atresia. Close management and monitoring of the pregnancy plays an important role in the ability to offer highly specialized postnatal care and improve outcomes for your child. [7] Surgery to repair intestinal atresia involves removing the blockage and reconnecting your baby's intestine. [4] by EI Hatch Jr · 1986 · Cited by 24 — The unusual problem of multiple intestinal atresias associated with jejunal atresia has been reviewed and seven cases have been summarized.
Recommended Medications
- corticosteroids
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Differential Diagnosis
Multiple intestinal atresia (MIA) is a rare congenital disorder characterized by the presence of two or more intestinal atresias, which are areas where the intestine is completely blocked or absent. The differential diagnosis of MIA involves considering various conditions that can present with similar symptoms and findings.
Conditions to Consider:
- Malrotation with or without midgut volvulus: This condition can cause bowel obstruction and is often diagnosed using a contrast-enhanced upper gastrointestinal (GI) study [1].
- Multiple intestinal atresia with combined immune deficiency: This rare condition is associated with MIA and combined immune deficiency, and is often diagnosed based on clinical presentation and laboratory findings [3].
- Colonic atresia: This condition can present with similar symptoms to MIA, including bowel obstruction and abdominal distension [4].
- Midgut volvulus: This condition involves the twisting of the midgut around its mesenteric axis, which can cause bowel obstruction and is often diagnosed using a contrast-enhanced upper GI study [6].
- Meconium ileus: This condition involves the blockage of the small intestine by meconium, which can present with similar symptoms to MIA [7].
- Duplication cysts: These are rare congenital anomalies that involve the presence of an extra lumen within the intestine, which can cause bowel obstruction and is often diagnosed using imaging studies [4].
- Internal hernias: These are rare conditions where a portion of the intestine becomes trapped in a hernia sac, which can cause bowel obstruction and is often diagnosed using imaging studies [8].
Key Points to Consider:
- MIA is a rare congenital disorder that presents with multiple intestinal atresias.
- The differential diagnosis of MIA involves considering various conditions that can present with similar symptoms and findings.
- Conditions such as malrotation, midgut volvulus, meconium ileus, duplication cysts, internal hernias, and colonic atresia should be considered in the differential diagnosis of MIA.
References:
[1] Curr Opin Pediatr. 2014 Dec;26(6):732-8. [3] Hereditary multiple intestinal atresia: thirty years later [4] Duplication cysts and internal hernias as causes of bowel obstruction in children [6] Midgut volvulus
Additional Differential Diagnoses
- Malrotation with or without midgut volvulus
- Multiple intestinal atresia with combined immune deficiency
- Colonic atresia
- Midgut volvulus
- Meconium ileus
- Duplication cysts
- Internal hernias
Additional Information
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