Jeune Thoracic Dystrophy C0265275
A rare genetic disorder affecting chest and bone development, causing a narrow ribcage that can impair breathing. Children are most commonly affected. Treatment includes respiratory support, monitoring, and sometimes surgery to improve chest space.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Progressive renal failure
Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent respiratory infections
(respiratory)Metaphyseal chondrodysplasia
(musculoskeletal)Hepatic fibrosis
(hepatobiliary)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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Vertical expandable prosthetic titanium rib (VEPTR) placement
Implantation of a expandable titanium rib device to gradually expand the thoracic cavity in growing children, allowing for improved lung development and respiratory function.
Respiratory function and growth tracking
Serial pulmonary function testing, polysomnography, and growth monitoring to assess disease progression and timing of surgical intervention.
Additional treatment options
Nocturnal non-invasive ventilation
CPAP or BiPAP support during sleep to maintain alveolar ventilation and prevent nocturnal hypoventilation, often needed as disease progresses.
medicationHigh-calorie nutritional support
Aggressive nutritional supplementation to support the increased metabolic demands and prevent failure to thrive commonly seen with chronic respiratory disease.
dietaryRespiratory muscle training and conditioning
Targeted exercises to strengthen respiratory muscles, improve cough effectiveness, and maintain chest wall mobility to optimize breathing mechanics.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Jeune Thoracic Dystrophy C0265275?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication 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.
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