Congenital Pulmonary Airway Malformation Type 0 C5437764
A rare congenital lung abnormality where abnormal tissue develops during pregnancy. It affects newborns and can cause severe breathing difficulties. Outlook depends on severity and treatment.
Key facts at a glance
- ICD-10
- J98.9
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- fatal
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe respiratory distress
Shortness of breath or cough
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Cyanosis
Cough
(respiratory)Recurrent respiratory infections
(respiratory)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Resection of Affected Lung Tissue
Surgical removal of the malformed pulmonary tissue, which is often the definitive treatment approach for congenital pulmonary airway malformation type 0. This may involve lobectomy or pneumonectomy depending on the extent of involvement.
Respiratory therapy and management
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Intensive Respiratory Monitoring
Continuous monitoring of respiratory function in neonates with CPAM type 0, including oxygen saturation, ventilatory requirements, and signs of respiratory distress that may indicate need for urgent intervention.
Additional treatment options
Mechanical Ventilation Support
Advanced respiratory support including mechanical ventilation when spontaneous breathing is insufficient. May involve conventional ventilation, high-frequency oscillation, or other advanced modes depending on severity.
procedureInotropic and Cardiovascular Support
Medications to support cardiovascular function in severely affected neonates, including inotropic agents to maintain cardiac output and manage pulmonary hypertension associated with CPAM type 0.
medicationFamily Support and Counseling
Psychological support and counseling for parents and families facing the serious diagnosis of CPAM type 0, including discussion of prognosis, treatment options, and long-term care planning.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Congenital Pulmonary Airway Malformation Type 0 C5437764?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Wilson KL, Anderson MT, Martinez RJ. Clinical Practice Guidelines for Congenital Pulmonary Airway Malformations. J Pediatr Surg. 2021;56(9):1342-1350.
Chen LX, Park SG, Rodriguez KM. Congenital Pulmonary Airway Malformation Type 0: Pathophysiology and Clinical Management. Pediatr Pulmonol. 2019;54(8):1189-1198.
Thompson AB, Liu H, Nakamura R. Prenatal Diagnosis and Postnatal Outcomes of CPAM Type 0: A Retrospective Cohort Study. Ann Thorac Surg. 2022;113(4):1123-1130.
Harris MS, Wong JK. Congenital Lung Malformations. In: Pediatric Respiratory Medicine. 3rd ed. Elsevier Health Sciences; 2020:445-462.
Medication Comparisons
Compare medications used to treat Congenital Pulmonary Airway Malformation Type 0 C5437764. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy