Caf Au Lait Macules With Pulmonary Stenosis C0553586
A condition with light brown skin patches combined with narrowing of a heart valve, part of a genetic syndrome affecting multiple body systems.
Key facts at a glance
- ICD-10
- Q24.9
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Chest discomfort or palpitations
(cardiovascular)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Heart murmur
(cardiovascular)Short stature
(cardiovascular)Pulmonary stenosis symptoms
(cardiovascular)Chest wall deformity
(cardiovascular)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
Cardiac surveillance for pulmonary stenosis
Regular echocardiographic monitoring of pulmonary valve function, right ventricular performance, and hemodynamics to determine disease progression and timing of intervention.
Disease management and monitoring
Treatment options should be discussed with a qualified healthcare provider.
Balloon pulmonary valvuloplasty
Minimally invasive catheter-based procedure to dilate the stenotic pulmonary valve, reducing pressure gradient across the valve and improving right ventricular outflow.
Additional treatment options
Laser therapy for café-au-lait macules
Pulsed dye laser or Q-switched laser treatment to lighten or remove café-au-lait macules for cosmetic concerns, with variable response rates and potential for recurrence.
procedureMedical management of associated hypertension
Antihypertensive medications if systemic hypertension develops as a complication, with regular blood pressure monitoring and cardiovascular risk assessment.
medicationPsychosocial support for cosmetic concerns
Psychological counseling and support groups to address body image concerns, social anxiety, or bullying-related issues particularly relevant for children and adolescents with visible skin manifestations.
psychologicalWhen 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 Caf Au Lait Macules With Pulmonary Stenosis C0553586?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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 27, 2026. · Read our editorial policy