Isolated congenital laryngeal web

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ORPHA:2374OMIM:150360Q31.0
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Overview

Isolated congenital laryngeal web is a rare congenital malformation of the larynx in which a thin membrane (web) of tissue spans part or all of the glottis, the area between the vocal cords. This condition results from incomplete recanalization of the laryngeal lumen during embryonic development, typically between the 8th and 10th weeks of gestation. The web most commonly forms at the level of the true vocal cords (glottic web), though subglottic and supraglottic webs can also occur. The term "isolated" distinguishes this condition from laryngeal webs that occur as part of broader syndromes, such as velocardiofacial syndrome (22q11.2 deletion syndrome). The severity of symptoms depends on the thickness and extent of the web. Key clinical features include an abnormal cry (weak, hoarse, or absent) at birth, stridor (noisy breathing), varying degrees of airway obstruction, and dysphonia (voice abnormality). In mild cases, the web may be thin and involve only the anterior portion of the glottis, causing primarily voice changes. In severe cases, a thick web may obstruct a significant portion of the airway, leading to respiratory distress that requires urgent intervention in the neonatal period. Treatment depends on the severity of the web. Thin, small webs may be managed endoscopically using cold steel instruments or laser division, sometimes with placement of a keel to prevent re-formation of the web. More extensive or thicker webs may require open surgical approaches, such as laryngofissure with keel placement, or tracheotomy to secure the airway while staged reconstructive procedures are performed. Long-term outcomes are generally favorable for mild to moderate webs, though voice quality may remain affected. Regular follow-up with otolaryngology is important to monitor for web recurrence and to optimize voice and airway outcomes.

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Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Isolated congenital laryngeal web.

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No actively recruiting trials found for Isolated congenital laryngeal web at this time.

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No specialists are currently listed for Isolated congenital laryngeal web.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Isolated congenital laryngeal web.

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Common questions about Isolated congenital laryngeal web

What is Isolated congenital laryngeal web?

Isolated congenital laryngeal web is a rare congenital malformation of the larynx in which a thin membrane (web) of tissue spans part or all of the glottis, the area between the vocal cords. This condition results from incomplete recanalization of the laryngeal lumen during embryonic development, typically between the 8th and 10th weeks of gestation. The web most commonly forms at the level of the true vocal cords (glottic web), though subglottic and supraglottic webs can also occur. The term "isolated" distinguishes this condition from laryngeal webs that occur as part of broader syndromes, s

How is Isolated congenital laryngeal web inherited?

Isolated congenital laryngeal web follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Isolated congenital laryngeal web typically begin?

Typical onset of Isolated congenital laryngeal web is neonatal. Age of onset can vary across affected individuals.