Duplication of the pituitary gland

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Overview

Duplication of the pituitary gland is an extremely rare congenital condition in which a person is born with two pituitary glands instead of one. The pituitary gland is a small, pea-sized organ at the base of the brain that produces hormones controlling growth, metabolism, reproduction, and many other body functions. When this gland is duplicated, it is almost always part of a broader pattern of developmental abnormalities affecting the face, brain, and other midline structures of the body. Patients with this condition often have distinctive facial features, including a wide or duplicated nose, a cleft lip or palate, and widely spaced eyes. Many also have abnormalities of the brain, such as incomplete separation of the brain hemispheres or other midline brain malformations. Hormonal imbalances may occur because the duplicated glands can produce excess or abnormal amounts of pituitary hormones. Some children may experience seizures, developmental delays, or intellectual disability depending on the severity of associated brain malformations. There is no single cure for this condition. Treatment is tailored to each patient's specific symptoms and may involve hormone management by endocrinologists, surgical correction of facial abnormalities, neurological care for seizures or brain malformations, and developmental support services. Because the condition is so rare, management is best coordinated by a multidisciplinary team at a center experienced with complex congenital anomalies.

Also known as:

Key symptoms:

Duplicated or widened noseCleft lip or cleft palateWidely spaced eyesAbnormal facial features along the midline of the faceHormonal imbalancesSeizuresDevelopmental delaysIntellectual disabilityBrain malformations such as incomplete brain separationAbnormal tooth developmentTongue abnormalities such as a split or enlarged tongueBreathing difficulties in newbornsFeeding difficulties in infancyShort stature or abnormal growth patterns

Clinical phenotype terms (34)— hover any for plain English
Abnormality of the tongueHP:0000157BrachyturricephalyHP:0000244Self-mutilationHP:0000742Abnormal midbrain morphologyHP:0002418Abnormal sella turcica morphologyHP:0002679Thoracic scoliosisHP:0002943Abnormal odontoid process morphologyHP:0003310Abnormality of the cervical spineHP:0003319
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 Duplication of the pituitary gland.

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No actively recruiting trials found for Duplication of the pituitary gland at this time.

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No specialists are currently listed for Duplication of the pituitary gland.

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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 Duplication of the pituitary gland.

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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.What specific brain and facial abnormalities does my child have, and how severe are they?,Are my child's hormone levels normal, and will they need hormone therapy?,What surgeries might be needed, and when should they be done?,What developmental milestones should I watch for, and when should I be concerned?,Should genetic testing be done, and what might it tell us?,What specialists should be part of my child's care team?,What is the long-term outlook for my child's development and independence?

Common questions about Duplication of the pituitary gland

What is Duplication of the pituitary gland?

Duplication of the pituitary gland is an extremely rare congenital condition in which a person is born with two pituitary glands instead of one. The pituitary gland is a small, pea-sized organ at the base of the brain that produces hormones controlling growth, metabolism, reproduction, and many other body functions. When this gland is duplicated, it is almost always part of a broader pattern of developmental abnormalities affecting the face, brain, and other midline structures of the body. Patients with this condition often have distinctive facial features, including a wide or duplicated nose

How is Duplication of the pituitary gland inherited?

Duplication of the pituitary gland follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Duplication of the pituitary gland typically begin?

Typical onset of Duplication of the pituitary gland is neonatal. Age of onset can vary across affected individuals.