OBSOLETE: Tracheo-esophageal fistula-hypospadias syndrome

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Overview

Tracheo-esophageal fistula-hypospadias syndrome is an extremely rare condition that has been described in medical literature but is now considered an obsolete diagnostic category. This means that the medical community no longer recognizes it as a distinct syndrome, and affected individuals may now be classified under other diagnoses. The condition was originally described as a combination of two birth defects: a tracheo-esophageal fistula (an abnormal connection between the windpipe and the food pipe) and hypospadias (a condition in boys where the opening of the urethra is on the underside of the penis rather than at the tip). A tracheo-esophageal fistula can cause serious problems with feeding and breathing in newborns, as food or saliva may enter the lungs. Hypospadias can affect urination and, later in life, reproductive function. Because this syndrome is now obsolete, patients who were previously given this diagnosis may benefit from a re-evaluation by a clinical geneticist to determine whether a more current diagnosis applies. Treatment has typically focused on surgical correction of both the fistula and the hypospadias, and outcomes depend on the severity of each defect and whether other abnormalities are present.

Key symptoms:

Abnormal connection between the windpipe and food pipeOpening of the urethra on the underside of the penisDifficulty feeding in newbornsChoking or coughing during feedingSaliva or food entering the lungsRecurrent lung infectionsDifficulty breathingAbnormal urine streamExcessive drooling in newbornsBloating of the stomach with air

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: Tracheo-esophageal fistula-hypospadias syndrome.

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No actively recruiting trials found for OBSOLETE: Tracheo-esophageal fistula-hypospadias syndrome at this time.

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No specialists are currently listed for OBSOLETE: Tracheo-esophageal fistula-hypospadias 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: Tracheo-esophageal fistula-hypospadias 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.Is there an underlying genetic cause for my child's combination of birth defects?,Should we pursue genetic testing such as chromosomal microarray or whole exome sequencing?,What is the timeline for surgical repairs, and what are the risks?,What long-term complications should we watch for after surgery?,Will my child need any special feeding support or dietary changes?,Are there any implications for future pregnancies in our family?,Since this syndrome is now considered obsolete, does my child's condition fit a more current diagnosis?

Common questions about OBSOLETE: Tracheo-esophageal fistula-hypospadias syndrome

What is OBSOLETE: Tracheo-esophageal fistula-hypospadias syndrome?

Tracheo-esophageal fistula-hypospadias syndrome is an extremely rare condition that has been described in medical literature but is now considered an obsolete diagnostic category. This means that the medical community no longer recognizes it as a distinct syndrome, and affected individuals may now be classified under other diagnoses. The condition was originally described as a combination of two birth defects: a tracheo-esophageal fistula (an abnormal connection between the windpipe and the food pipe) and hypospadias (a condition in boys where the opening of the urethra is on the underside of

At what age does OBSOLETE: Tracheo-esophageal fistula-hypospadias syndrome typically begin?

Typical onset of OBSOLETE: Tracheo-esophageal fistula-hypospadias syndrome is neonatal. Age of onset can vary across affected individuals.