Overview
Congenital unilateral pulmonary hypoplasia is a rare condition present from birth in which one lung does not develop fully. 'Unilateral' means only one side is affected, and 'hypoplasia' means the lung tissue is underdeveloped or smaller than normal. This condition may also be called one-sided lung underdevelopment or unilateral lung hypoplasia. The underdeveloped lung has fewer air sacs (alveoli) and smaller airways, which reduces its ability to exchange oxygen and carbon dioxide properly. Babies born with this condition may show signs of breathing difficulty right after birth, including rapid or labored breathing, low oxygen levels, and a bluish tint to the skin. The severity can vary widely — some children have mild symptoms and adapt reasonably well over time, while others face serious breathing challenges that require ongoing medical support. The heart and other chest structures may shift toward the smaller lung, which can sometimes cause additional complications. Treatment focuses on supporting breathing and managing symptoms rather than correcting the underlying lung development. This may include supplemental oxygen, mechanical ventilation in severe cases, and treatment of any related lung infections. As children grow, the remaining healthy lung can sometimes compensate by expanding. A team of specialists typically works together to monitor lung function and overall health throughout childhood and into adulthood.
Key symptoms:
Difficulty breathing, especially shortly after birthRapid or labored breathingBluish color of the skin or lips (cyanosis) due to low oxygenLow oxygen levels in the bloodReduced exercise tolerance or tiring easilyFrequent respiratory infections or pneumoniaChest that appears asymmetrical or unevenReduced breath sounds on the affected sidePersistent coughPoor weight gain or slow growth in infants
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for OBSOLETE: Congenital unilateral pulmonary hypoplasia.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for OBSOLETE: Congenital unilateral pulmonary hypoplasia at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for OBSOLETE: Congenital unilateral pulmonary hypoplasia.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to OBSOLETE: Congenital unilateral pulmonary hypoplasia.
Community
No community posts yet. Be the first to share your experience with OBSOLETE: Congenital unilateral pulmonary hypoplasia.
Start the conversation →Latest news about OBSOLETE: Congenital unilateral pulmonary hypoplasia
No recent news articles for OBSOLETE: Congenital unilateral pulmonary hypoplasia.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How severe is my child's lung underdevelopment, and what does that mean for their long-term health?,What signs should I watch for at home that would mean I need to call for emergency help?,Will my child's healthy lung be able to compensate and grow over time?,Are there any other conditions or birth defects I should be tested for that might be related to this?,What vaccinations are most important to protect my child's lungs?,Are there any restrictions on physical activity, and will those change as my child grows?,What specialists should be part of my child's care team, and how often should we have check-ups?
Common questions about OBSOLETE: Congenital unilateral pulmonary hypoplasia
What is OBSOLETE: Congenital unilateral pulmonary hypoplasia?
Congenital unilateral pulmonary hypoplasia is a rare condition present from birth in which one lung does not develop fully. 'Unilateral' means only one side is affected, and 'hypoplasia' means the lung tissue is underdeveloped or smaller than normal. This condition may also be called one-sided lung underdevelopment or unilateral lung hypoplasia. The underdeveloped lung has fewer air sacs (alveoli) and smaller airways, which reduces its ability to exchange oxygen and carbon dioxide properly. Babies born with this condition may show signs of breathing difficulty right after birth, including rap
How is OBSOLETE: Congenital unilateral pulmonary hypoplasia inherited?
OBSOLETE: Congenital unilateral pulmonary hypoplasia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does OBSOLETE: Congenital unilateral pulmonary hypoplasia typically begin?
Typical onset of OBSOLETE: Congenital unilateral pulmonary hypoplasia is neonatal. Age of onset can vary across affected individuals.