OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome

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Overview

Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome is an extremely rare condition that has been described in medical literature but is now classified as obsolete in disease databases, meaning it may have been reclassified or merged with another condition over time. The syndrome was originally described as a combination of limb malformations (phocomelia, where the hands or feet attach close to the body with shortened or absent long bones of the limbs, and ectrodactyly, where fingers or toes are missing or fused giving a split-hand or split-foot appearance), hearing loss (deafness), and an irregular heart rhythm known as sinus arrhythmia. These features are present from birth. Because this condition is so rare and now considered obsolete in classification systems, very little is known about its underlying cause, and there is no established standard treatment. Management would typically focus on addressing each symptom individually — for example, hearing aids or cochlear implants for deafness, orthopedic interventions for limb differences, and cardiac monitoring for the heart rhythm abnormality. Patients and families should work closely with a team of specialists to manage the various aspects of this syndrome.

Also known as:

Key symptoms:

Shortened or absent arm or leg bones (phocomelia)Missing or fused fingers or toes (split hand or split foot)Hearing loss or deafnessIrregular heart rhythm (sinus arrhythmia)Limb length differencesDifficulty with fine motor tasks due to hand differencesPossible difficulty with balance or coordination

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 OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome.

View clinical trials →

No actively recruiting trials found for OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome community →

No specialists are currently listed for OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia 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 OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome.

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Community

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Latest news about OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What is the severity of my child's limb differences, and what surgical or prosthetic options are available?,What type of hearing loss does my child have, and what is the best hearing device option?,How serious is the sinus arrhythmia, and how often should the heart be monitored?,Should we pursue genetic testing to look for an underlying cause?,What early intervention therapies should we start, and how soon?,Are there any activity restrictions due to the heart rhythm abnormality?,Is there a risk of this condition occurring again in future pregnancies?

Common questions about OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome

What is OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome?

Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome is an extremely rare condition that has been described in medical literature but is now classified as obsolete in disease databases, meaning it may have been reclassified or merged with another condition over time. The syndrome was originally described as a combination of limb malformations (phocomelia, where the hands or feet attach close to the body with shortened or absent long bones of the limbs, and ectrodactyly, where fingers or toes are missing or fused giving a split-hand or split-foot appearance), hearing loss (deafness), and

At what age does OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome typically begin?

Typical onset of OBSOLETE: Phocomelia-ectrodactyly-deafness-sinus arrhythmia syndrome is neonatal. Age of onset can vary across affected individuals.