OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome

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ORPHA:1381
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Overview

Cataract-intellectual disability-anal atresia-urinary defects syndrome is an extremely rare condition that has been described in medical literature under this name, though it is now considered an obsolete or outdated classification (meaning the medical community may have reclassified it or merged it with another recognized condition). As the name suggests, this syndrome was characterized by a combination of cataracts (clouding of the lens in the eye), intellectual disability, anal atresia (a birth defect where the opening to the anus is missing or blocked), and urinary tract abnormalities. These features are present from birth or early infancy. Because this condition is so rare and the classification is now obsolete, very limited information is available about its exact genetic cause, prevalence, or optimal treatment. Patients who were historically diagnosed with this syndrome may now fall under a different diagnostic category based on updated genetic testing and clinical understanding. Management has typically been supportive and symptom-based, involving surgical correction of anal atresia, cataract surgery, urological interventions, and educational support for intellectual disability. Families affected by this combination of symptoms should work closely with a clinical geneticist to obtain an updated diagnosis using modern genetic testing methods.

Also known as:

Key symptoms:

Cataracts (cloudy lenses in the eyes)Intellectual disabilityAnal atresia (blocked or absent anal opening at birth)Urinary tract abnormalitiesPossible kidney malformationsDevelopmental delaysVision problemsDifficulty with learning and reasoningPossible feeding difficulties in infancy

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome.

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No actively recruiting trials found for OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome at this time.

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No specialists are currently listed for OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome.

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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 OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Has modern genetic testing been done to confirm or update this diagnosis?,What is the best timing for cataract surgery and what visual outcomes can we expect?,What type of surgical repair is recommended for the anal atresia, and what is the long-term bowel function outlook?,How severe are the urinary tract abnormalities, and will surgery be needed?,What early intervention and developmental support services should we start?,Are there other conditions this could be reclassified as based on current medical knowledge?,Should other family members be tested genetically?

Common questions about OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome

What is OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome?

Cataract-intellectual disability-anal atresia-urinary defects syndrome is an extremely rare condition that has been described in medical literature under this name, though it is now considered an obsolete or outdated classification (meaning the medical community may have reclassified it or merged it with another recognized condition). As the name suggests, this syndrome was characterized by a combination of cataracts (clouding of the lens in the eye), intellectual disability, anal atresia (a birth defect where the opening to the anus is missing or blocked), and urinary tract abnormalities. The

At what age does OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome typically begin?

Typical onset of OBSOLETE: Cataract-intellectual disability-anal atresia-urinary defects syndrome is neonatal. Age of onset can vary across affected individuals.