ICD-10: Q40.0

Congenital hypertrophic pyloric stenosis

Clinical Information

Inclusion Terms

  • Congenital or infantile hypertrophy
  • Congenital or infantile stricture
  • Congenital or infantile constriction
  • Congenital or infantile stenosis
  • Congenital or infantile spasm

Additional Information

Description

Congenital hypertrophic pyloric stenosis (CHPS) is a condition characterized by the abnormal thickening of the pylorus, the opening from the stomach into the small intestine. This thickening leads to a narrowing of the pyloric channel, which can obstruct the passage of food from the stomach to the intestines. Below is a detailed clinical description and relevant information regarding ICD-10 code Q40.0.

Clinical Description

Definition

Congenital hypertrophic pyloric stenosis is a birth defect that affects the pylorus, the muscular valve that controls the flow of food from the stomach to the duodenum (the first part of the small intestine). The condition is typically diagnosed in infants, usually between 3 to 12 weeks of age, and is more common in males than females.

Symptoms

Infants with CHPS often present with the following symptoms:
- Projectile Vomiting: This is a hallmark symptom, where the infant vomits forcefully after feeding, often several feet away.
- Dehydration: Due to persistent vomiting, infants may show signs of dehydration, including dry mouth, decreased urine output, and lethargy.
- Hunger: Infants may appear hungry shortly after vomiting, as they are unable to retain food.
- Weight Loss or Poor Weight Gain: Due to inadequate nutrition, affected infants may not gain weight appropriately.
- Palpable Olive-like Mass: Upon physical examination, a healthcare provider may feel a firm, olive-shaped mass in the abdomen, which is the hypertrophied pylorus.

Diagnosis

Diagnosis of CHPS typically involves:
- Physical Examination: A thorough examination may reveal the characteristic abdominal mass.
- Ultrasound: Abdominal ultrasound is the preferred imaging modality, as it can visualize the thickened pylorus and confirm the diagnosis.
- Upper Gastrointestinal Series: In some cases, a barium swallow study may be performed to assess the passage of contrast through the gastrointestinal tract.

Treatment

The primary treatment for congenital hypertrophic pyloric stenosis is surgical intervention, specifically a procedure called pyloromyotomy. This surgery involves splitting the thickened pyloric muscle to relieve the obstruction, allowing food to pass from the stomach into the intestines. Postoperative care includes monitoring for complications and ensuring proper hydration and nutrition.

ICD-10 Code Q40.0

Code Details

  • ICD-10 Code: Q40.0
  • Description: Congenital hypertrophic pyloric stenosis
  • Classification: This code falls under the category of congenital malformations of the digestive system, specifically related to the pylorus.

Importance of Coding

Accurate coding is essential for proper diagnosis, treatment, and billing purposes. The use of ICD-10 code Q40.0 allows healthcare providers to document the condition effectively, ensuring that patients receive appropriate care and that healthcare facilities are reimbursed correctly for services rendered.

Conclusion

Congenital hypertrophic pyloric stenosis is a significant condition that requires prompt diagnosis and treatment to prevent complications such as severe dehydration and malnutrition. Understanding the clinical presentation, diagnostic methods, and treatment options is crucial for healthcare providers managing affected infants. The ICD-10 code Q40.0 serves as a vital tool in the medical coding and billing process, facilitating accurate documentation and care delivery.

Clinical Information

Congenital hypertrophic pyloric stenosis (CHPS) is a condition characterized by the abnormal thickening of the pylorus, the opening from the stomach into the small intestine. This thickening leads to gastric outlet obstruction, which can significantly affect an infant's ability to feed and digest food. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with this condition, specifically in relation to the ICD-10 code Q40.0.

Clinical Presentation

Age of Onset

CHPS typically presents in infants, usually between 3 to 12 weeks of age. It is rare for the condition to manifest after 12 weeks, making early diagnosis crucial for effective management[1].

Gender Predilection

The condition shows a notable gender bias, with a higher incidence in males compared to females, with a ratio of approximately 3:1[2].

Signs and Symptoms

Vomiting

One of the hallmark symptoms of CHPS is projectile vomiting, which occurs shortly after feeding. This vomiting is often described as non-bilious, meaning it does not contain bile, and can be forceful enough to project several feet away[3].

Dehydration and Electrolyte Imbalance

Due to persistent vomiting, infants may develop dehydration and electrolyte imbalances. Signs of dehydration include dry mucous membranes, decreased urine output, and lethargy[4].

Hunger and Feeding Behavior

Despite vomiting, infants with CHPS often exhibit signs of hunger and may want to feed again shortly after vomiting. This can lead to a cycle of feeding and vomiting, further exacerbating dehydration[5].

Abdominal Distension

Parents may notice abdominal distension, which can be attributed to the accumulation of gastric contents that cannot pass through the obstructed pylorus[6].

Palpable "Olive" Mass

On physical examination, a characteristic finding is the palpable "olive" mass in the right upper quadrant of the abdomen. This mass represents the hypertrophied pylorus and is often described as firm and mobile[7].

Patient Characteristics

Family History

There is evidence suggesting a genetic component to CHPS, as it may occur more frequently in families with a history of the condition. A family history of pyloric stenosis can increase the likelihood of occurrence in subsequent generations[8].

Associated Conditions

Infants with CHPS may also have other congenital anomalies, although these are not common. Conditions such as congenital heart defects or other gastrointestinal malformations may be present, necessitating a comprehensive evaluation[9].

Nutritional Status

Infants with CHPS may present with poor weight gain or failure to thrive due to the inability to retain adequate nutrition from feedings. This can lead to significant weight loss if not addressed promptly[10].

Conclusion

Congenital hypertrophic pyloric stenosis is a significant condition that requires timely diagnosis and intervention. The classic presentation includes projectile vomiting, dehydration, and a palpable abdominal mass. Understanding the clinical signs and patient characteristics associated with CHPS is essential for healthcare providers to ensure early recognition and management, ultimately improving outcomes for affected infants. If you suspect CHPS in an infant, immediate medical evaluation is critical to prevent complications related to dehydration and nutritional deficiencies.

Approximate Synonyms

Congenital hypertrophic pyloric stenosis (CHPS) is a condition characterized by the narrowing of the pylorus, the opening from the stomach into the small intestine, due to muscle hypertrophy. This condition primarily affects infants and can lead to severe vomiting and dehydration if not treated. The ICD-10 code for this condition is Q40.0, which falls under the broader category of congenital malformations of the digestive system.

Alternative Names for Congenital Hypertrophic Pyloric Stenosis

  1. Pyloric Stenosis: This is a more general term that refers to the narrowing of the pylorus, which can occur due to various causes, including congenital factors.
  2. Infantile Hypertrophic Pyloric Stenosis: This term emphasizes that the condition typically presents in infants, usually between 3 to 12 weeks of age.
  3. Congenital Pyloric Stenosis: This name highlights the congenital nature of the condition, indicating that it is present at birth.
  4. Hypertrophic Pyloric Stenosis: This term focuses on the hypertrophy (thickening) of the pyloric muscle, which is the primary pathological feature of the condition.
  1. Pyloromyotomy: This is the surgical procedure commonly performed to treat CHPS, where the thickened pyloric muscle is cut to relieve the obstruction.
  2. Gastric Outlet Obstruction: This term describes the blockage at the pylorus, which can be caused by CHPS among other conditions.
  3. Congenital Gastrointestinal Malformations: This broader category includes various congenital defects affecting the gastrointestinal tract, of which CHPS is a specific example.
  4. Non-bilious Vomiting: A common symptom of CHPS, where infants vomit without bile due to the obstruction at the pylorus.

Conclusion

Understanding the alternative names and related terms for ICD-10 code Q40.0 is essential for healthcare professionals involved in diagnosis, treatment, and coding of congenital hypertrophic pyloric stenosis. These terms not only facilitate communication among medical practitioners but also enhance the clarity of medical records and billing processes. If you need further information on treatment options or clinical guidelines related to CHPS, feel free to ask!

Diagnostic Criteria

Congenital hypertrophic pyloric stenosis (CHPS) is a condition that affects infants, characterized by the narrowing of the pylorus, the opening from the stomach into the small intestine. This condition leads to severe vomiting and dehydration, necessitating prompt diagnosis and treatment. The ICD-10 code Q40.0 specifically refers to this condition, and the criteria for its diagnosis typically include a combination of clinical evaluation, imaging studies, and laboratory tests.

Diagnostic Criteria for Congenital Hypertrophic Pyloric Stenosis

1. Clinical Presentation

  • Symptoms: Infants with CHPS often present with projectile non-bilious vomiting, which typically occurs shortly after feeding. Other symptoms may include dehydration, weight loss, and signs of electrolyte imbalance.
  • Physical Examination: A key finding during the physical examination is the presence of an "olive-like" mass in the right upper quadrant of the abdomen, which is the hypertrophied pylorus.

2. Imaging Studies

  • Ultrasound: Abdominal ultrasound is the primary imaging modality used to confirm the diagnosis. It can reveal:
    • Thickening of the pyloric muscle (greater than 4 mm).
    • Elongation of the pylorus (greater than 14-16 mm).
    • Narrowing of the pyloric channel.
  • Upper Gastrointestinal Series: In some cases, an upper GI series may be performed to visualize the passage of contrast through the gastrointestinal tract, showing delayed gastric emptying and the characteristic "string sign" of the narrowed pylorus.

3. Laboratory Tests

  • Electrolyte Panel: Due to the vomiting, infants may present with metabolic alkalosis and electrolyte imbalances, which can be assessed through blood tests. Common findings include hypochloremic metabolic alkalosis and hypokalemia.

4. Exclusion of Other Conditions

  • It is essential to rule out other causes of vomiting in infants, such as gastroesophageal reflux disease (GERD), infections, or other gastrointestinal obstructions. This is typically done through a thorough clinical history and additional diagnostic tests as needed.

5. Age of Onset

  • CHPS typically presents in infants between 3 to 12 weeks of age, with a peak incidence around 6 weeks. The timing of symptom onset can be a critical factor in diagnosis.

Conclusion

The diagnosis of congenital hypertrophic pyloric stenosis (ICD-10 code Q40.0) relies on a combination of clinical symptoms, physical examination findings, imaging studies, and laboratory tests. Early recognition and diagnosis are crucial for effective management, which often involves surgical intervention to relieve the obstruction. If you suspect CHPS in an infant, it is vital to seek medical evaluation promptly to ensure appropriate care.

Treatment Guidelines

Congenital hypertrophic pyloric stenosis (ICD-10 code Q40.0) is a condition characterized by the abnormal thickening of the pylorus, the opening from the stomach into the small intestine. This thickening leads to gastric outlet obstruction, which can cause severe vomiting and dehydration in infants. The standard treatment approaches for this condition primarily involve surgical intervention, along with preoperative and postoperative care.

Surgical Treatment: Pyloromyotomy

1. Laparoscopic Pyloromyotomy

Laparoscopic pyloromyotomy is the most common surgical procedure for treating congenital hypertrophic pyloric stenosis. This minimally invasive technique involves making small incisions in the abdomen and using a camera and specialized instruments to cut the thickened pyloric muscle, allowing food to pass from the stomach into the intestine more easily. The benefits of laparoscopic surgery include reduced postoperative pain, shorter recovery times, and minimal scarring compared to open surgery[1][6].

2. Open Pyloromyotomy

In some cases, an open pyloromyotomy may be performed, especially if laparoscopic access is not feasible due to the infant's anatomy or other complications. This traditional approach involves a larger incision in the abdomen to access the pylorus directly. While effective, it typically results in a longer recovery period and more postoperative discomfort compared to the laparoscopic method[1][8].

Preoperative Management

1. Fluid and Electrolyte Resuscitation

Before surgery, it is crucial to stabilize the infant's condition. This often involves correcting dehydration and electrolyte imbalances caused by persistent vomiting. Intravenous (IV) fluids are administered to restore hydration and balance electrolytes, which is essential for the infant's safety during surgery[2][8].

2. Nutritional Support

In cases where the infant is unable to tolerate oral feeds due to vomiting, nutritional support may be provided through IV fluids or, in some cases, feeding via a nasogastric tube until surgery can be performed[3][4].

Postoperative Care

1. Monitoring and Recovery

Post-surgery, infants are closely monitored for complications such as infection, bleeding, or recurrence of symptoms. Most infants can resume feeding within 24 hours after surgery, starting with clear liquids and gradually progressing to formula or breast milk as tolerated[5][8].

2. Pain Management

Effective pain management is essential for recovery. Pediatric analgesics are often used to ensure the infant is comfortable during the postoperative period[6][8].

Conclusion

Congenital hypertrophic pyloric stenosis is primarily treated through surgical intervention, with laparoscopic pyloromyotomy being the preferred method due to its advantages in recovery and reduced complications. Preoperative management focuses on stabilizing the infant's condition through fluid resuscitation and nutritional support, while postoperative care emphasizes monitoring and pain management. With timely and appropriate treatment, the prognosis for infants with this condition is generally excellent, allowing for a return to normal feeding and growth patterns shortly after surgery[1][2][5][6].

Related Information

Description

  • Abnormal thickening of pyloric muscle
  • Narrowing of pyloric channel
  • Obstruction of food passage
  • Projectile vomiting
  • Dehydration due to persistent vomiting
  • Hunger shortly after vomiting
  • Weight loss or poor weight gain

Clinical Information

  • Typically presents in infants between 3-12 weeks
  • More common in males than females with 3:1 ratio
  • Projectile non-bilious vomiting occurs shortly after feeding
  • Dehydration and electrolyte imbalance due to persistent vomiting
  • Infants exhibit signs of hunger despite vomiting
  • Abdominal distension due to accumulation of gastric contents
  • Palpable olive mass in right upper quadrant of abdomen
  • Genetic component with increased likelihood in families
  • May have associated congenital anomalies or poor weight gain

Approximate Synonyms

  • Pyloric Stenosis
  • Infantile Hypertrophic Pyloric Stenosis
  • Congenital Pyloric Stenosis
  • Hypertrophic Pyloric Stenosis

Diagnostic Criteria

  • Projectile non-bilious vomiting
  • Dehydration and weight loss
  • Electrolyte imbalance signs
  • Olive-like mass in right upper quadrant
  • Thickened pyloric muscle (>4mm)
  • Elongated pylorus (>14-16mm)
  • Narrowed pyloric channel
  • Delayed gastric emptying
  • String sign of narrowed pylorus
  • Metabolic alkalosis and hypokalemia

Treatment Guidelines

  • Laparoscopic pyloromyotomy preferred method
  • Open pyloromyotomy in complex cases
  • Fluid resuscitation preoperatively
  • Electrolyte balance correction preoperatively
  • Nutritional support via IV fluids or NG tube
  • Monitoring and recovery post-surgery
  • Pain management with pediatric analgesics

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