What is buffalo hump

A buffalo hump also called dorsocervical fat pad, is an area of fat accumulation on the back of the neck between the shoulders. A buffalo hump between the shoulder blades by itself is not a sign of a specific condition, how buffalo hump is commonly seen in patients with Cushing’s syndrome or Cushing’s disease and rarely in HIV patients or some antiretroviral drugs. The exact cause of buffalo hump is unknown. Your health care provider must consider this along with other symptoms and test results.

Your doctor will perform a physical examination and ask about your medical history and symptoms. Tests may be ordered to determine the cause.

Treatment will be aimed at the problem that caused the buffalo hump to develop in the first place.

Buffalo hump causes

Causes of buffalo hump include any of the following:

• Certain medicines used to treat HIV/AIDS
• Long-term use of certain glucocorticoid medicines, including prednisone, cortisone, and hydrocortisone (Cushing’s syndrome)
• Obesity (usually causes more generalized fat deposition)
• High level of the hormone cortisol (caused by Cushing’s disease)
• Certain genetic disorders that cause unusual fat accumulation

A buffalo hump may be due to HIV infection or medicines used to treat HIV. Although more research is needed to prove that there is a link between HIV medicines and buffalo hump, some HIV medicines have been associated with buffalo hump ¹.

Osteoporosis may cause a curvature of the spine in the neck called kyphoscoliosis. This causes an abnormal shape, but does not by itself cause excessive fat in the back of the neck.

Cushing’s syndrome

Cushing’s syndrome occurs when your body is exposed to high levels of the hormone cortisol for a long time. Cushing syndrome, sometimes called hypercortisolism, may be caused by the use of oral corticosteroid medication. The condition can also occur when your body makes too much cortisol on its own. Cushing’s syndrome can be serious if it’s not treated.

Too much cortisol can produce some of the hallmark signs of Cushing syndrome — a buffalo hump between your shoulders, a rounded face, and pink or purple stretch marks on your skin. Cushing syndrome can also result in high blood pressure, bone loss and, on occasion, type 2 diabetes.

Cushing’s syndrome is uncommon. It mostly affects people who have been taking steroid medicine, especially steroid tablets, for a long time. Steroids contain a man-made version of cortisol.

Very rarely, Cushing’s syndrome can be caused by the body producing too much cortisol. This is usually due to:

• a growth (tumor) in the pituitary gland in the brain (Cushing’s disease)
• a tumor in one of the adrenal glands above the kidneys

The tumors are usually non-cancerous (benign). They’re most common in young women.

Treatments for Cushing syndrome can return your body’s cortisol production to normal and noticeably improve your symptoms. The earlier treatment begins, the better your chances for recovery.

Cushing’s syndrome causes

Excess levels of the hormone cortisol are responsible for Cushing syndrome. Cortisol, which is produced in the adrenal glands, plays a variety of roles in your body. For example, cortisol helps regulate your blood pressure and keeps your cardiovascular system functioning normally.

Cortisol also helps your body respond to stress and regulates the way you convert (metabolize) proteins, carbohydrates and fats in your diet into usable energy. However, when the level of cortisol is too high in your body, you may develop Cushing’s syndrome.

The role of corticosteroid medications

Cushing syndrome can develop from a cause outside of your body (exogenous Cushing syndrome). One example is taking oral corticosteroid medications in high doses over an extended period of time. These medications, such as prednisone, have the same effect in the body as does cortisol produced by your body.

Oral corticosteroids may be necessary to treat inflammatory diseases, such as rheumatoid arthritis, lupus and asthma, or to prevent your body from rejecting a transplanted organ. Because the doses required to treat these conditions are often higher than the amount of cortisol your body normally needs each day, side effects from excess cortisol can occur.

It’s also possible to develop Cushing syndrome from injectable corticosteroids — for example, repeated injections for joint pain, bursitis and back pain. Inhaled steroid medicines (taken for asthma) and steroid skin creams (used for skin disorders such as eczema) are generally less likely to cause Cushing syndrome than oral corticosteroids. But, in some individuals, these medications may cause Cushing syndrome, especially if taken in high doses.

Your body’s own overproduction

The condition may also be due to your body’s own overproduction of cortisol (endogenous Cushing syndrome). This may occur from excess production by one or both adrenal glands, or overproduction of the adrenocorticotropic hormone (ACTH), which normally regulates cortisol production, In these cases, Cushing syndrome may be related to:

• A pituitary gland tumor (pituitary adenoma). A noncancerous (benign) tumor of the pituitary gland, located at the base of the brain, secretes an excess amount of ACTH, which in turn stimulates the adrenal glands to make more cortisol. When this form of the syndrome develops, it’s called Cushing disease. It occurs much more often in women and is the most common form of endogenous Cushing syndrome.
• An ectopic ACTH-secreting tumor. Rarely, when a tumor develops in an organ that normally does not produce ACTH, the tumor will begin to secrete this hormone in excess, resulting in Cushing syndrome. These tumors, which can be noncancerous (benign) or cancerous (malignant), are usually found in the lungs, pancreas, thyroid or thymus gland.
• A primary adrenal gland disease. In some people, the cause of Cushing syndrome is excess cortisol secretion that doesn’t depend on stimulation from ACTH and is associated with disorders of the adrenal glands. The most common of these disorders is a noncancerous tumor of the adrenal cortex, called an adrenal adenoma.
• Cancerous tumors of the adrenal cortex (adrenocortical carcinomas) are rare, but they can cause Cushing syndrome as well. Occasionally, benign, nodular enlargement of both adrenal glands can result in Cushing syndrome.
• Familial Cushing syndrome. Rarely, people inherit a tendency to develop tumors on one or more of their endocrine glands, affecting cortisol levels and causing Cushing syndrome.

Cushing’s syndrome symptoms

Symptoms of Cushing’s syndrome can start suddenly or gradually. They tend to get slowly worse if not treated.

Common signs and symptoms of Cushing’s syndrome involve progressive obesity and skin changes, such as:

• Weight gain and fatty tissue deposits, particularly around the midsection and upper back, in the face (moon face), and between the shoulders (buffalo hump), increased fat on your chest and tummy, but slim arms and legs
• Pink or purple stretch marks (striae) on the skin of the abdomen, thighs, breasts and arms
• Thinning, fragile skin that bruises easily
• Slow healing of cuts, insect bites and infections
• A red, puffy, rounded face
• Acne

Cushing’s syndrome can also cause high blood pressure, which can be serious if not treated.

Women with Cushing syndrome may experience:

• Thicker or more visible body and facial hair (hirsutism)
• Irregular or absent menstrual periods

Men with Cushing syndrome may experience:

• Decreased libido
• Decreased fertility
• Erectile dysfunction

Other signs and symptoms include:

• Severe fatigue
• Muscle weakness
• Depression, anxiety and irritability
• Loss of emotional control
• Cognitive difficulties
• New or worsened high blood pressure
• Headache
• Bone loss, leading to fractures over time
• In children, impaired growth

Cushing’s syndrome complications

Without treatment, complications of Cushing syndrome may include:

• Bone loss (osteoporosis), which can result in unusual bone fractures, such as rib fractures and fractures of the bones in the feet
• High blood pressure (hypertension)
• Type 2 diabetes
• Frequent or unusual infections
• Loss of muscle mass and strength

Cushing’s syndrome diagnosis

Cushing syndrome can be difficult to diagnose, particularly endogenous Cushing syndrome, because other conditions share the same signs and symptoms. Diagnosing Cushing syndrome can be a long and extensive process. You may not have any firm answers about your condition until you’ve had a series of medical appointments.

Your doctor will conduct a physical exam, looking for signs of Cushing syndrome. He or she may suspect Cushing syndrome if you have signs such as rounding of the face (moon face), a pad of fatty tissue between the shoulders and neck (buffalo hump), and thin skin with bruises and stretch marks.

If you’ve been taking a corticosteroid medication for a long time, your doctor may suspect that you’ve developed Cushing syndrome as a result of this medication. If you haven’t been using a corticosteroid medication, these diagnostic tests may help pinpoint the cause:

• Urine and blood tests. These tests measure hormone levels in your urine and blood and show whether your body is producing excessive cortisol. For the urine test, you may be asked to collect your urine over a 24-hour period. Both the urine and blood samples will be sent to a laboratory to be analyzed for cortisol levels. Your doctor might also recommend other specialized tests that evaluate the blood and urine to help determine if Cushing syndrome is present and to help identify the underlying source of any excess production. These tests often involve measuring cortisol levels before and after stimulation or suppression with other hormone medications.
• Saliva test. Cortisol levels normally rise and fall throughout the day. In people without Cushing syndrome, levels of cortisol drop significantly in the evening. By analyzing cortisol levels from a small sample of saliva collected late at night, doctors can see if cortisol levels are too high, suggesting a diagnosis of Cushing syndrome.
• Imaging tests. Computerized tomography scans or magnetic resonance imaging scans can provide images of your pituitary and adrenal glands to detect abnormalities, such as tumors.
• Petrosal sinus sampling. This test can help determine whether the cause of endogenous Cushing syndrome is rooted in the pituitary or somewhere else. For the test, blood samples are taken from the petrosal sinuses — veins that drain the pituitary glands. A thin tube is inserted into your upper thigh or groin area while you’re sedated, and threaded to the petrosal sinuses. Levels of ACTH are measured from the petrosal sinuses, and from a blood sample taken from the forearm. If ACTH is higher in the sinus sample, the problem stems from the pituitary. If the ACTH levels are similar between the sinus and forearm, the root of the problem lies outside of the pituitary gland.

These tests don’t only help your doctor diagnose Cushing syndrome, they may also help rule out other medical conditions with similar signs and symptoms. For example, polycystic ovary syndrome — a hormone disorder in women with enlarged ovaries — shares some of the same signs and symptoms as Cushing has, such as excessive hair growth and irregular menstrual periods. Depression, eating disorders and alcoholism also can partially mimic Cushing syndrome.

Cushing’s syndrome treatment

Treatments for Cushing syndrome are designed to lower the high level of cortisol in your body. The best treatment for you depends on the cause of the syndrome. Treatment options include:

• Reducing corticosteroid use. If the cause of Cushing syndrome is long-term use of corticosteroid medications, your doctor may be able to keep your Cushing signs and symptoms under control by reducing the dosage of the drug over a period of time, while still adequately managing your asthma, arthritis or other condition. For many of these medical problems, your doctor can prescribe noncorticosteroid drugs, which will allow him or her to reduce the dosage or eliminate the use of corticosteroids altogether. Don’t reduce the dose of corticosteroid drugs or stop taking them on your own. Do so only under your doctor’s supervision. Abruptly discontinuing these medications could lead to deficient cortisol levels. Slowly tapering off corticosteroid drugs allows your body to resume normal cortisol production.
• Surgery. If the cause of Cushing syndrome is a tumor, your doctor may recommend complete surgical removal. Pituitary tumors are typically removed by a neurosurgeon, who may perform the procedure through your nose. If a tumor is present in the adrenal glands, lungs or pancreas, the surgeon can remove it through a standard operation or in some cases by using minimally invasive surgical techniques, with smaller incisions. After the operation, you’ll need to take cortisol replacement medications to provide your body with the correct amount of cortisol. In most cases, you’ll eventually experience a return of normal adrenal hormone production, and your doctor can taper off the replacement drugs. However, this process can take up to a year or longer. In some instances, people with Cushing syndrome never experience a resumption of normal adrenal function; they then need lifelong replacement therapy.
• Radiation therapy. If the surgeon can’t totally remove a pituitary tumor, he or she will usually prescribe radiation therapy to be used in conjunction with the operation. Additionally, radiation may be used for people who aren’t suitable candidates for surgery. Radiation can be given in small doses over a six-week period, or with a technique called stereotactic radiosurgery (Gamma Knife surgery). In the latter procedure, administered as a single treatment, a large dose of radiation is delivered to the tumor, and the radiation exposure to surrounding tissues is minimized.
• Medications. Medications can be used to control cortisol production when surgery and radiation don’t work. Medications may also be used before surgery in people who have become very sick with Cushing syndrome. Doctors may recommend drug therapy before surgery to improve signs and symptoms and minimize surgical risk. Medications to control excessive production of cortisol at the adrenal gland include ketoconazole (Nizoral), mitotane (Lysodren) and metyrapone (Metopirone). Mifepristone (Korlym) is approved for people with Cushing syndrome who have type 2 diabetes or glucose intolerance. Mifepristone does not decrease cortisol production, but it blocks the effect of cortisol on your tissues. Side effects from these medications may include fatigue, nausea, vomiting, headaches, muscle aches, high blood pressure, low potassium and swelling. Some have more serious side effects, such as neurological side effects and liver toxicity. The newest medication for Cushing’s is pasireotide (Signifor), and it works by decreasing ACTH production from a pituitary tumor. This medication is given as an injection twice daily. It’s recommended if pituitary surgery is unsuccessful or cannot be done. Side effects are fairly common, and may include diarrhea, nausea, high blood sugar, headache, abdominal pain and fatigue. In some cases, the tumor or its treatment will cause other hormones produced by the pituitary or adrenal gland to become deficient and your doctor will recommend hormone replacement medications.

If none of these treatment options are appropriate or effective, your doctor may recommend surgical removal of your adrenal glands (bilateral adrenalectomy). This procedure will cure excess production of cortisol, but will require lifelong replacement medications.

Lifestyle and home remedies

The length of your recovery from Cushing syndrome will depend on the severity and cause of your condition. Remember to be patient. You didn’t develop Cushing syndrome overnight, and your symptoms won’t disappear overnight, either. In the meantime, these tips may help you on your journey back to health.

• Increase activities slowly. You may be in such a hurry to get your old self back that you push yourself too hard too fast, but your weakened muscles need a slower approach. Work up to a reasonable level of exercise or activity that feels comfortable without overdoing it. You’ll improve little by little, and your persistence will be rewarded.
• Eat sensibly. Nutritious, wholesome foods provide a good source of fuel for your recovering body and can help you lose the extra pounds that you gained from Cushing syndrome. Make sure you’re getting enough calcium and vitamin D. Taken together, they help your body absorb calcium, which can help strengthen your bones, counteracting the bone density loss that often occurs with Cushing syndrome.
• Monitor your mental health. Depression can be a side effect of Cushing syndrome, but it can also persist or develop after treatment begins. Don’t ignore your depression or wait it out. Seek help promptly from your doctor or a therapist if you’re depressed, overwhelmed or having difficulty coping during your recovery.
• Gently soothe aches and pains. Hot baths, massages and low-impact exercises, such as water aerobics and tai chi, can help alleviate some of the muscle and joint pain that accompanies Cushing syndrome recovery.

Cushing’s disease

Cushing disease is a rare disorder (only affecting 10 to 15 people per million every year) characterized by increased adrenocorticotropic hormone (ACTH) production from the anterior pituitary, leading to excess cortisol release from the adrenal glands ². Most often, Cushing disease caused by a pituitary adenoma or as the result of excess production of corticotropin-releasing hormone (CRH) from the hypothalamus ³. Symptoms include generalized weakness, high blood pressure, diabetes mellitus, menstrual disorders or psychiatric changes ². Physical manifestations of excess cortisol levels include moon facies, buffalo hump, bruising, abdominal striae, obesity, facial plethora, and hirsutism ³.

Cushing’s disease is more common in women and occurs most often in people between the ages of 20 and 50.

Pituitary adenoma is the most common cause of Cushing’s disease. Adenoma is a pituitary tumor that is almost always benign. Pituitary adenomas can be hard to diagnose, because they are quite small, so many people who have Cushing’s disease have a delayed diagnosis. Having an endocrinologist help with the diagnostic process can improve the chances of a prompt, successful diagnosis for patients who have symptoms of Cushing’s syndrome and Cushing’s disease.

Cushing’s disease was first described by Harvey Cushing in 1912 after he was presented with a unique case in 1910 ³. Cushing hypothesized that excess basophil pituitary cells were responsible for his patients presenting symptoms of obesity, amenorrhea, abnormal hair growth, underdevelopment of sexual characteristics, hydrocephalus, and cerebral tension ³.

Cushing’s disease causes

Pituitary adenomas are responsible for nearly 80% of the cases of Cushing disease ³. On the other hand, Cushing syndrome refers to the general state of hypercortisolemia which can be caused by a variety of mechanisms including exogenous steroid use, adrenal tumors, ectopic-ACTH production, or high estrogen levels ³. Cushing disease is specific to the endogenous production of ACTH that leads to secondary hypercortisolism ³. Both Cushing’s syndrome and Cushing’s disease cause the same symptoms, including red lines on the abdomen, abdominal weight gain, buffalo hump and roundness to the face.

Cushing’s disease symptoms

Because the symptoms of Cushing’s disease are the primary reason a doctor will order testing, patients need to be aware of the common signs that point to this condition. Unfortunately, they are sometimes hard to notice because of the nature of the symptoms. Cushing’s disease symptoms often come on slowly and can be attributed to lifestyle changes or even the aging process, and this can make diagnosis difficult. However, if you notice a combination of these Cushing’s symptoms and do not feel that changes in your lifestyle are the cause, talk to your doctor about getting tested for Cushing’s disease.

Changes to the body

Some of the most common symptoms people notice when they have Cushing’s disease are physical changes to the body. Weight gain is one of these, but the fat accumulation is specific to the abdominal area and the back of the neck. Patients with Cushing’s disease may develop a fatty lump on the back of the neck sometimes called a buffalo hump. Patients may also become very round in the abdomen, while retaining thin arms and legs.

As the patient’s weight increases, the face gains fullness and a distinctive round look. This may be accompanied by redness to the cheeks, known as plethora.

Changes to the skin may also occur. Patients with Cushing’s disease symptoms may find that they bruise more easily and their wounds do not heal quickly. Purple stretch marks, called abdominal striae, on the abdomen, chest and arms are another common body change.

Changes to vision

Vision changes are also sometimes associated with Cushing’s disease. These Cushing’s symptoms occur because the tumors in the pituitary gland grow to the point that they place pressure on the optic chiasm. This can cause loss of overall vision, loss of peripheral vision, blurred vision and inability to see colors brightly.

Other changes to the patient’s health

Changes to the body are the first signs many people notice, but patients may experience other health changes, including:

• Decreased sex drive
• Loss of menstrual periods
• Infertility in women
• High blood pressure that is not easily treated
• Diabetes mellitus
• Psychiatric disorders
• Mood disorders
• Behavior disorders
• Wasting of muscles in the legs
• Fatigue and weakness
• Elevated prolactin levels in the blood
• Increased hair on the body and face

These symptoms do not always mean that a patient has Cushing’s disease, but when combined, they are a strong indicator of the condition. If you are noticing any of these symptoms, talk to your doctor. Finding the cause is the first step toward feeling better.

Cushing’s disease diagnosis

Diagnosing Cushing’s disease can be difficult because symptoms develop slowly. Also, the elevated cortisol levels can happen in cycles, so the levels may not be elevated at the time of testing.

Testing begins with hormone blood tests to determine if blood cortisol levels are too high. This may require more than one test to establish a state of excessive blood cortisol. If the patient is not taking cortisol medications, the doctor will schedule an MRI to pinpoint the location of any tumors.

If you are struggling with symptoms of elevated cortisol levels, schedule an appointment with your endocrinologist to rule out Cushing’s disease.

Cushing’s disease treatment

If you have been diagnosed with Cushing’s disease, your doctor will help you choose the right course of treatment for your condition. Treatment for Cushing’s disease focuses on stabilizing hormone levels and removing the tumors to prevent future problems. Here are the most common Cushing’s disease treatment options doctors will choose for their patients.

Surgical removal of the pituitary tumors

The only true cure of Cushing’s disease is to surgically remove the tumors that are causing the problem. However, because these tumors are quite small and because they are located in the nervous system, the surgery is not 100 percent effective. For small tumors that have not spread outside of the pituitary gland, the cure rate is 80 to 85 percent. For larger tumors, the cure rate is only 50 to 55 percent.

Surgery to treat Cushing’s disease is performed by neurosurgeons who work carefully to remove the tumor in one piece. The surgery is performed through the nasal passages, so it is a minimally invasive procedure that requires minimal hospital time and creates little discomfort.

Medication to manage Cushing’s disease

For patients who are not candidates for surgery, medication can help control the symptoms of Cushing’s disease by inhibiting the adrenal gland’s production of cortisol. While this is not a cure and does not shrink the tumors, it can, for some patients, significantly reduce their symptoms.

Radiation treatment for Cushing’s disease

Sometimes the surgical removal of the tumor is impossible. When that happens, radiation can help control tumor growth. This Cushing’s treatment is not ideal, because it does allow radiation to pass through the brain, but the beam is carefully targeted at the tumor and typically passes through with minimal damage to the surrounding tissue.

Stereotactic radiosurgery is the technique used to focus radiation directly into the tumor. The pituitary gland may be damaged during this treatment, resulting in delayed pituitary failure. This occurs when the pituitary gland fails years after treatment. Because of this risk, patients who undergo radiation therapy for Cushing’s disease need to continue follow-up care with an endocrinologist. Some patients may need hormone replacement.

Bilateral adrenalectomy

Finally, if the tumors do not respond to radiation or medication, and the patient is not a candidate for surgery, a surgical removal of the adrenal glands may be the best option. This will require hormone replacement after surgery, and patients will need to wear a medical alert bracelet or necklace. However, this may limit the symptoms of the disease.

If you have been diagnosed with Cushing’s disease, discuss with your doctor which treatment for Cushing’s disease is best for your specific situation. Schedule an appointment today, so you can begin to find relief from your symptoms.

Buffalo hump treatment

Treatment will be aimed at the problem that caused the buffalo hump to develop in the first place.

A healthy diet and daily exercise may help to build muscle and reduce buffalo hump. Practice guidelines recommend that patients adhere to a balanced diet, including ~ 50 to 60% carbohydrates, 20–30% fat, and 20% protein ⁴. Because hypoleptinemia (absolute or relative) stimulates appetite and food consumption, energy restriction is usually recommended to reduce ectopic fat storage and improve metabolic abnormalities, although it may be difficult to achieve, especially in childhood and adolescence. Of note, growth assessment may be challenging because of the abnormal body composition and the underlying primary disease that may affect linear growth independent of lipodystrophy. Very low–fat diets should be used in patients with acute pancreatitis. Physical exercise should be advised, if not contraindicated by concurrent diseases, but limitations may intervene because of musculoskeletal pain, fatigue, or psychological distress ⁵.

Liposuction (surgical removal of fat) and injectable facial fillers are sometimes used to treat buffalo hump. There are also medicines that may help lessen the effects of buffalo hump. Talk to your health care provider to discuss your treatment options. Metreleptin therapy is effective for metabolic complications in hypoleptinemic patients with generalized lipodystrophy and selected patients with partial lipodystrophy ⁶. Other treatments not specific for lipodystrophy may be helpful as well (e.g., metformin for diabetes, and statins or fibrates for hyperlipidemia) ⁶.

References

1. Ceccarelli G, d’Ettorre G, Marchetti F, et al. Development of Buffalo Hump in the course of antiretroviral therapy including raltegravir and unboosted atazanavir: a case report and review of the literature. J Med Case Rep. 2011;5:70. Published 2011 Feb 17. doi:10.1186/1752-1947-5-70 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3047431
2. Hine J, Schwell A, Kairys N. An Unlikely Cause of Hypokalemia. J Emerg Med. 2017 May;52(5):e187-e191
3. Buliman A, Tataranu LG, Paun DL, Mirica A, Dumitrache C. Cushing’s disease: a multidisciplinary overview of the clinical features, diagnosis, and treatment. J Med Life. 2016 Jan-Mar;9(1):12-18
4. Araújo-Vilar D, Santini F. Diagnosis and treatment of lipodystrophy: a step-by-step approach. J Endocrinol Invest. 2018;42(1):61–73. doi:10.1007/s40618-018-0887-z https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6304182
5. The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline. Brown RJ, Araujo-Vilar D, Cheung PT, Dunger D, Garg A, Jack M, Mungai L, Oral EA, Patni N, Rother KI, von Schnurbein J, Sorkina E, Stanley T, Vigouroux C, Wabitsch M, Williams R, Yorifuji T. J Clin Endocrinol Metab. 2016 Dec; 101(12):4500-4511.
6. Brown RJ, Araujo-Vilar D, Cheung PT, et al. The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline. J Clin Endocrinol Metab. 2016;101(12):4500–4511. doi:10.1210/jc.2016-2466 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5155679