Specialties & Conditions Hypopituitarism
Hypopituitarism
Hypopituitarism (an underactive pituitary gland) is rare in children. When a child has hypopituitarism, the pituitary gland has lost its ability to make one, some or all pituitary hormones. The condition is often permanent, but very treatable.
The pituitary gland — in the middle of the head and brain — is the body’s master gland. The table below describes what each hormone made by the pituitary gland does, what happens when each hormone is missing and medication that can replace each hormone.
What Causes Hypopituitarism?
In children, hypopituitarism is usually caused by something congenital (the child is born with the problem) or by a pituitary tumor. The tumor interferes with the gland. Sometimes, the cause cannot be determined.
Congenital
Sometimes, the cause was present before the child was born. Often, we see that the pituitary gland is under-developed. A genetic error may be the reason the gland doesn’t work well.
Children with septo-optic dysplasia have varying degrees of hypopituitarism. Their vision is usually impaired because the optic nerves are under-developed. The eyes can move irregularly or “wander.” This disorder can affect the pituitary gland and other structures in the brain. Often, these children have diabetes insipidus and not enough growth hormone.
There are other forms of congenital hypopituitarism. Sometimes, the pituitary gland doesn’t make enough growth hormone. Sometimes, the thyroid gland is underactive, or the adrenal gland doesn’t work well. In Kallman syndrome, not enough of the hormones that stimulate the testes or ovaries are made, puberty is late or doesn’t happen, and the sense of smell is affected.
Tumor
A tumor can cause hypopituitarism. The tumor may grow in the pituitary gland or outside the gland, compressing the normal tissue. Parents worry that the tumor may be cancer, but that’s unlikely.
Rare causes
Rarely, one of these conditions leads to this hormone problem:
- An infection in the brain
- A traumatic brain injury
- Radiation to the brain
- Bleeding into the pituitary gland
Long-term & Serious Effects
Some hormone deficiencies cause complications over time. Our experienced doctors help you understand any long-term or serious effects of the missing hormones. For example:
- Immediate risk: Lack of adrenocorticotropic hormone (ACTH) or cortisol (glucocorticoid or stress hormone) can create a life-threatening emergency. Periods of stress or illness need medicine changes, for which you need to call the doctor.
- Long-term concerns: A child lacking growth hormone grows slowly or not at all and has more body fat. Bones and muscles are affected, too. The doctor will answer your questions about issues such as growth and fertility.
What Are the Symptoms of Hypopituitarism?
Signs and symptoms vary, depending on which hormones are lacking and the child’s age.
Common symptoms in newborns:
- Symptoms of low blood sugar, sluggishness, jitteriness, seizures
- A lot of urine
- Penis is small
- Skin is yellow (jaundice)
Common symptoms in older infants and children:
- Stunted growth, short stature (based on growth curve chart)
- Weight gain doesn’t match growth in height
- Slow sexual development; menstrual periods may stop or be irregular
- Tooth development and eruption are delayed
- Thirst and urination are increased
- Fatigue, low tolerance for stress
The symptoms you see may be due to other conditions and medical problems. Always talk to your child’s doctor if you have a concern.
How Is Hypopituitarism Diagnosed?
Our approach to diagnosing hypopituitarism is very thorough. We take one step at a time and minimize invasive procedures.
- History and physical examination: At your first visit, the doctor gets a complete medical history. Next is a head-to-toe physical exam. This includes the genitalia/private areas to determine if puberty is happening. If we need a blood test, you may be asked to return for a morning blood draw.
- Morning blood test: We measure hormone levels in the blood sample. The morning is the best time for this because hormone levels go up and down throughout the day. This blood test may be all that’s needed.
We can use other tests as needed. For example:
- Stimulation: If we need more information on which hormones are lacking, we do stimulation tests. These need careful medical supervision. You come to the infusion center at the hospital, and a child life specialist helps your child through the experience. We usually administer a hormone or other stimulating agent and take blood samples at intervals for testing.
- Imaging: We can use an MRI scan to see inside the head if needed. The scan can show us a tumor or other developmental abnormality in the pituitary gland — without exposing the child to radiation. Medicine lets the child sleep comfortably while we get the pictures.
- Ophthalmology referral/eye exam: We make this referral if we are concerned about the child’s vision or septo-optic dysplasia.
- Water deprivation: This test identifies diabetes insipidus. The child is admitted to the hospital, and under very close supervision, the child has nothing to eat or drink. We collect blood and urine samples at regular intervals for this test.
- Bone age: We can use this type of X-ray (of the hand and wrist) in diagnosis and as we manage the condition. The images tell us about the skeleton’s development.
We know that the weeks of waiting for the full picture can be difficult. Our compassionate team and family-friendly environment support your family while we progress toward the answers and plan the right treatment. As soon as possible, you’ll receive a call from a doctor or nurse about what we’ve found and the next steps to take.
How Is Hypopituitarism Treated?
We treat the cause of the condition and replace the hormones the body isn’t making.
Hormone replacement
Hormone replacement therapy mimics the body’s natural production. The medicines can be continued as long as needed, during childhood and adulthood. These medications are tolerated very well when the right amounts of hormones are replaced. The following are examples of hormone replacement therapy:
- Levothyroxine for hypothyroidism: The child takes a tablet of levothyroxine each day.
- Synthetic growth hormone for growth deficiency: The child needs an injection of synthetic growth hormone every day. The needle is very small.
- Hydrocortisone for adrenal insufficiency: The child takes a tablet two to three times a day. To deal with illness, surgery or dehydration, the child needs a “stress dose” (determined by the doctor) plus the regular daily medicine. The child should wear a medical alert bracelet or necklace. Sometimes prednisone or dexamethasone can be used instead of hydrocortisone.
- Replacements for sex hormones: Boys take testosterone. Girls take estrogen. Testosterone can be injections 1-2 times monthly or topical daily. Estrogen can be oral daily, or a topical patch changed a few times each week. When puberty development is nearly complete, girls may use an estrogen/progesterone combination.
- Replacements for antidiuretic hormone: Nasal spray or tablets of desmopressin/DDAVP that melt in the mouth or can be swallowed work for children. (Some are used once a day, some more often; everyone is different). Infants with diabetes insipidus can receive chlorothiazide; this oral drug helps lower the infant’s large urine output.
Tumor treatment
Some tumors respond to medicine that is swallowed. Other tumors need to be removed with surgery. Usually, the hormone deficiencies remain after a tumor is removed. Hormone therapy works for this.
Ongoing Care
To be effective, hormone replacement must be supported with ongoing care. Throughout childhood, we need to adjust the hormone doses to accommodate the growing child’s needs and changes in symptoms. We evaluate the child’s growth and development frequently and develop a working relationship with parent and child.
Our resources help bring the right specialists into your child’s care to make sure the child gets the best treatment possible. Our endocrinologists and neurosurgeons co-manage patients in our hospital. Tools such as our electronic health record help nurses and doctors throughout the team work closely together.
We care for children with all forms of hypopituitarism. We treat each hormone deficiency to maintain the child’s health and normal development. With the right care plan, children with hypopituitarism usually enjoy a normal life. We help the child to develop normally, interact with peers and feel well.
Resources
Learn more about pituitary disorders by visiting these physician-recommended websites: