Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome

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Overview

Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome is an extremely rare genetic condition that affects the eyes and surrounding structures. The name describes its three main features: ptosis (drooping of the upper eyelids), limited ability to move the eyes upward, and the absence of the lacrimal punctum, which is the tiny opening in the inner corner of each eyelid that normally drains tears into the nose. Because the tear drainage system is missing or underdeveloped, affected individuals often experience excessive tearing (epiphora), as tears cannot drain properly and instead overflow onto the cheeks. The drooping eyelids can partially block vision, and the restricted upward eye movement may affect how a person sees objects above their line of sight. This syndrome is present from birth and is classified under congenital malformation syndromes that primarily affect the face. Because it is so rare, there is very limited published medical literature about it. Treatment is mainly supportive and surgical. Ptosis can be corrected or improved with eyelid surgery (blepharoplasty or frontalis sling procedures), and tear drainage problems may be addressed with surgical creation of an alternative drainage pathway (dacryocystorhinostomy). Management typically involves a team of eye specialists. There is currently no cure that addresses the underlying cause, but surgical and supportive treatments can significantly improve quality of life and visual function.

Key symptoms:

Drooping of the upper eyelids (ptosis)Difficulty moving the eyes upwardMissing tear drainage openings in the eyelidsExcessive tearing or watery eyesTears overflowing onto the cheeksReduced field of vision due to droopy eyelidsTilting the head backward to see betterPossible eye infections from poor tear drainagePossible amblyopia (lazy eye) if untreated in childhood

Clinical phenotype terms (18)— hover any for plain English
Absent eyelashesHP:0000561Absent lacrimal punctumHP:0001092Biparietal narrowingHP:0004422
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome.

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No actively recruiting trials found for Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome at this time.

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No specialists are currently listed for Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome.

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is the ptosis, and is it affecting my child's vision development?,When is the best time to consider surgery for the drooping eyelids?,What surgical options are available for the tear drainage problem?,Is there a risk of amblyopia, and should we start treatment now?,Should we pursue genetic testing to understand the cause better?,What signs of complications should I watch for at home?,Will my child need multiple surgeries as they grow?

Common questions about Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome

What is Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome?

Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome is an extremely rare genetic condition that affects the eyes and surrounding structures. The name describes its three main features: ptosis (drooping of the upper eyelids), limited ability to move the eyes upward, and the absence of the lacrimal punctum, which is the tiny opening in the inner corner of each eyelid that normally drains tears into the nose. Because the tear drainage system is missing or underdeveloped, affected individuals often experience excessive tearing (epiphora), as tears cannot drain properly and

At what age does Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome typically begin?

Typical onset of Ptosis-upper ocular movement limitation-absence of lacrimal punctum syndrome is neonatal. Age of onset can vary across affected individuals.