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Pyloric Atresia: Symptoms, Diagnosis, and Treatments

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Pyloric atresia is a congenital condition with a blockage or narrowing of the pylorus. Read the article to know more.

Medically reviewed by

Dr. Vasavada Bhavin Bhupendra

Published At September 25, 2023
Reviewed AtFebruary 14, 2024

Introduction

Pyloric atresia (PA) is uncommon in one in 100,000 live births. It has a poor prognosis, especially when associated with other abnormalities, the most common of which is epidermolysis bullosa (EB). The cause has yet to be discovered. Pyloric atresia is usually diagnosed shortly after birth, and quick surgical action is required to avoid more complications. This article will discuss the causes and treatment of pyloric atresia.

What Is Pyloric Atresia?

Pyloric atresia is a congenital condition with a blockage or narrowing of the pylorus, which connects the small intestine to the stomach. This occurs because the muscles in the pyloric region do not develop properly during fetal development, leading to a blockage of the opening. As a result, food and fluids cannot pass from the stomach into the small intestine, leading to vomiting and other symptoms. Pyloric atresia is a rare condition that occurs in approximately one in 5,000 live births. It may occur as an isolated defect or as part of a more complex set of birth defects. The condition is typically diagnosed shortly after birth, based on the existence of signs and symptoms like vomiting, distended abdomen, and failure to pass meconium. Treatment involves surgery to remove the blockage and repair the pylorus, which is generally successful in restoring normal gastrointestinal function.

What Are the Symptoms of Pyloric Atresia?

The disorder known as pyloric stenosis affects the pylorus, which is the entrance between the stomach and the small intestine, and becomes narrow, making it difficult for food to pass from the stomach into the intestine. This can cause a variety of symptoms, including:

  • Vomiting.

  • Upper abdomen distension.

  • Blistering on the skin.

  • Hypovolemia (low blood volume).

  • Newborns' poor growth or failure to thrive.

  • Dehydration and electrolyte imbalances can cause lethargy, weakness, and other symptoms.

  • A swollen or bloated abdomen may feel hard or tender to the touch.

  • Infrequent or absent bowel movements, as food, cannot pass through the digestive system normally.

  • Stomach contents flow backward into the esophagus, which may cause heartburn or discomfort.

These symptoms develop gradually over time as the pylorus becomes increasingly narrow. They may sometimes be mistaken for other conditions, such as gastroesophageal reflux disease (GERD) or a milk allergy. If someone may be experiencing pyloric distension, it is crucial to quickly get medical help to obtain an accurate diagnosis and appropriate treatment.

How to Diagnose Pyloric Atresia?

Diagnosing pyloric atresia typically involves a combination of medical history, physical examination, and diagnostic tests. Here are some of the most common methods used to diagnose pyloric distension:

  • Medical History: The physician will inquire about the signs and symptoms, past health, and any family history of digestive problems.

  • Physical Examination: During the physical exam, the doctor will check for signs of dehydration, abdominal swelling, and other symptoms. They may also feel the child's abdomen to see if the pylorus feels thickened or enlarged.

  • Ultrasound: An ultrasound can provide a clear picture of the pylorus and help confirm a diagnosis of pyloric stenosis. A technician will produce photos during the ultrasound using a transducer, a portable gadget, the pylorus, and surrounding structures.

  • Upper Gastrointestinal (GI) Series: This X-ray type uses a special dye to visualize the pylorus and other structures in the upper digestive tract.

  • Blood Tests: Blood tests can help identify dehydration, electrolyte imbalances, and other complications that may arise from pyloric stenosis.

What Are the Treatment Options for Pyloric Atresia?

The treatment of pyloric atresia is surgical. The treatment of choice for the pyloric diaphragm is excision of the diaphragm. The treatments of EB–PA (epidermolysis bullosa with pyloric atresia) syndrome are symptomatic, including conservative management such as appropriate dressing, infection control, and nutritional supplements. Despite surgical treatment of concomitant PA, the prognosis is generally poor due to nutritional disturbance and sepsis in many cases. Most newborns benefit from quick diagnosis and adequate treatment. Patients with pyloric atresia can recover and go on to lead healthy lives. However, the outcome depends on the severity of the condition and any additional health problems that may be present. The following are the treatment options for pyloric atresia:

1. The main treatment for pyloric atresia is surgery. The surgery removes the blockage and creates a pathway for food and fluids to transit the small intestine after leaving the stomach.

  • The specific type of surgery used depends on the severity and location of the blockage. In most cases, surgery involves making a small incision in the abdomen and carefully cutting through the blocked area of the pylorus to create a passage for food and fluids.

  • The surgeon may then stitch the opening to prevent it from narrowing again. Sometimes, the blockage may be too severe to allow for a straightforward surgery.

  • In these cases, the surgeon may use more complex techniques, such as creating a bypass or rerouting the digestive system.

  • After surgery, infants may need to be monitored closely to ensure that they can tolerate feedings and that there are no complications.

2. Patients must be minimally touched and handled, even for vascular access.3. Adhesive dressing or tapes should never be used.4. A probe of the 'clip-on' variety placed on a digit or the ear lobe is ideal for monitoring oxygen saturation.5. Care for the skin is also important. New blisters are drained and dressed with three layers (first non-adhesive paraffin gauze; a second layer of gamgee for stability and protection; covered with Elastoplast). Skin is protected from shearing forces.

How do Pyloric Stenosis and Duodenal Atresia Differ From Each other?

  • Pyloric Stenosis: It is the partial or incomplete blockage of the central part of the intestinal opening (pylorus) that becomes narrowed.

  • Duodenal Atresia: It is the completed closure or blockage of the intestine (duodenum, the first part of the intestine).

Conclusion

Pyloric atresia is a rare congenital condition where the section of the small intestine that connects the stomach is blocked due to the absence or narrowing of the pylorus. Dehydration, malnutrition, and vomiting are severe complications of pyloric atresia. With advances in medical technology, the prognosis for infants with pyloric atresia has greatly improved, and many can lead a normal, healthy life with appropriate treatment and follow-up care. However, ongoing research is needed to understand the causes of this condition better and to develop new treatments and preventive strategies.

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Dr. Vasavada Bhavin Bhupendra
Dr. Vasavada Bhavin Bhupendra

Surgical Gastroenterology

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