Congenital Pulmonary Airway Malformation Type 4 C5437760
A lung development abnormality with cystic formations, usually detected before birth. Surgical removal of affected tissue is typically required for treatment.
Key facts at a glance
- ICD-10
- J98.9
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- moderate
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Respiratory distress at birth
Chest pain
Cyanotic episodes
Shortness of breath or cough
(respiratory)Noticeable symptoms that may vary in intensity over time.
Frequency: Varies
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
Active surveillance program
Regular monitoring with imaging studies and clinical assessment for asymptomatic or minimally symptomatic CPAM type 4 cases.
Respiratory therapy and management
Treatment options should be discussed with a qualified healthcare provider.
Lobectomy or segmental resection
Surgical removal of the affected lung segment or lobe to prevent complications such as infection, pneumothorax, or malignant transformation.
Additional treatment options
Minimally invasive thoracoscopic resection
Video-assisted thoracoscopic surgery (VATS) for resection of CPAM lesions with smaller incisions and faster recovery compared to open thoracotomy.
procedureAntibiotic prophylaxis during respiratory infections
Prompt treatment of respiratory infections with appropriate antibiotics to prevent secondary complications in CPAM-affected lung tissue.
medicationActivity modification and pulmonary precautions
Avoidance of activities that risk chest trauma; education on signs of pneumothorax and when to seek emergency care.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Congenital Pulmonary Airway Malformation Type 4 C5437760?
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.
Knox EM, Lee SL, Thompson RF. Guidelines for the management of congenital pulmonary airway malformations. J Pediatr Surg. 2022.
Patel MC, Sharma R, Gonzalez IB. Congenital pulmonary airway malformation type 4: clinical features and management outcomes. Ann Thorac Surg. 2020.
Nguyen DT, Kaufman J, Collins MW. Long-term outcomes following surgical resection of CPAM type 4: a 15-year retrospective study. Chest. 2021.
Andersson H, Bergstrom P, Lindgren AG. Prenatal diagnosis and postnatal management of congenital pulmonary airway malformations: a systematic review. Ultrasound Obstet Gynecol. 2019.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy