Patent urachus

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ORPHA:431341Q64.4
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Overview

Patent urachus (also called a urachal fistula or open urachus) is a rare birth defect where a small tube-like structure called the urachus fails to close properly before birth. During fetal development, the urachus connects the bladder to the belly button (umbilicus) and normally seals shut before a baby is born. When it stays open, urine can leak from the bladder all the way up to the belly button. This condition is present from birth, though it is sometimes not noticed until early infancy. The most noticeable sign is wetness or urine draining from the belly button, which can look like a constantly moist or weeping navel. The area around the belly button may become red, irritated, or infected. Some babies and children may also have a higher risk of urinary tract infections because bacteria can travel through the open channel. Treatment usually involves surgery to close or remove the open urachus. When caught early and treated with surgery, most children recover fully and go on to live completely normal, healthy lives. The outlook after successful surgery is excellent, and long-term complications are uncommon.

Key symptoms:

Urine leaking from the belly buttonConstantly wet or moist belly buttonRedness or irritation around the belly buttonFoul smell coming from the belly buttonRecurrent urinary tract infectionsSwelling or tenderness near the belly buttonDischarge or crusting at the navelAbdominal discomfort or pain in some cases

Clinical phenotype terms (10)— hover any for plain English
Patent urachusHP:0010479Abnormality of the umbilical cordHP:0010881Urachal cystHP:0012618Pelvic painHP:0034267CystoceleHP:0100645Recurrent gram-negative bacterial infectionsHP:0005420Congenital posterior urethral valveHP:0010957Neonatal omphalitisHP:0032435
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Patent urachus.

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No actively recruiting trials found for Patent urachus at this time.

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No specialists are currently listed for Patent urachus.

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 Patent urachus.

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Community

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Latest news about Patent urachus

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Questions for your doctor

Bring these to your next appointment

  • Q1.What type of surgery is recommended for my child, and what does the recovery look like?,Is there an infection present right now, and does it need to be treated before surgery?,How soon does surgery need to happen, or is it safe to wait?,What are the risks of the surgery, and what is the chance the urachus could reopen?,Will this condition affect my child's bladder or kidney function in the future?,What signs of complications should I watch for after surgery?,How often will follow-up visits be needed after the procedure?

Common questions about Patent urachus

What is Patent urachus?

Patent urachus (also called a urachal fistula or open urachus) is a rare birth defect where a small tube-like structure called the urachus fails to close properly before birth. During fetal development, the urachus connects the bladder to the belly button (umbilicus) and normally seals shut before a baby is born. When it stays open, urine can leak from the bladder all the way up to the belly button. This condition is present from birth, though it is sometimes not noticed until early infancy. The most noticeable sign is wetness or urine draining from the belly button, which can look like a con

How is Patent urachus inherited?

Patent urachus follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Patent urachus typically begin?

Typical onset of Patent urachus is neonatal. Age of onset can vary across affected individuals.