Guidebook Home

Table of Contents

Preface

Introductory Comments

General Questions About FOP

Genetics of FOP

How FOP Affects the Body

Care and Treatment

Activities

Feelings About FOP

Helpful Addresses

Family Resources

Ideas for Independence

Medical Articles

Acknowledgments and Contributions

Surgery and FOP: When to Say "Know"

Frederick S. Kaplan, M.D., Michael S. Zasloff, M.D., Ph.D.,
Randolph Cohen, M.D. and Jeffrey Tabas, M.D.

Patients with FOP are appropriately concerned about the potential danger of surgical procedures. We will try to clarify these concerns by outlining the facts about surgery and FOP. We will then propose a set of guidelines for making surgical decisions. Commonly asked questions include: When may surgery be performed? When must it be avoided? What are the risks and potential complications? What precautions should be taken if surgery must be performed?

The natural history of FOP confronts us with an undeniable fact: Injury (trauma) in any form to the musculoskeletal system is likely to trigger an intense and often prolonged course of painful new bone formation (heterotopic ossification). This trauma may come in the form of a slight bump or bruise, or it may be more severe. Surgical incisions are a form of trauma (tissue injury) and often precipitate a worsening of FOP. Unlike in other conditions, when FOP flares up, it leaves residual bone and resultant decreased range of motion, deformity, and disability.

Patients with FOP often consult orthopaedic surgeons and pose the question: "Is it possible to do surgery to remove the extra bone and free up the joint so that it can move?" The technical answer is surprisingly "yes," but that does not tell the whole story. Bone can be removed surgically so that the fused joint may temporarily be more mobile, but we emphasize temporarily. The bone is virtually guaranteed to reform, and often more abundantly than the original condition. The problem, therefore, is not a technical one, but rather a biological one. Although technically bone can be removed, the active removing of the bone stimulates more bone, thus leading to a worsening of the condition. Therefore, elective surgery on the musculoskeletal system should be avoided, as it will be ineffective and may trigger a new bout of bone formation worse than the original condition. Preliminary evaluation of a survey of FOP patients indicates overwhelmingly that patients who had surgery to remove heterotopic bone have almost universally been plagued with a worsening of their condition.

Another commonly asked question is: "Even if the bone reforms, can the joint be placed in a better functional position?" Again the answer is technically "yes." But there is a high risk of complications such as infection and phlebitis, or inflammation of a vein. This is especially true with surgery of the lower limbs. Such surgical intervention often ends in failure, as it is difficult to reposition one set of joints in the lower limbs without affecting posture and balance. The general rule still holds true. Avoid surgery of the musculoskeletal system if at all possible. It is not likely to improve the condition, and, in fact, is likely to worsen it.

As with anyone, patients may develop problems unrelated to FOP. It is important for patients, families, and treating physicians to realize this important fact so that symptoms unrelated to FOP do not go unattended. An attack of appendicitis, for example, might go unrecognized if one attributed the abdominal symptoms to FOP.

It is not uncommon, for example, to see gallbladder disease in patients with FOP, just as one might see it in the general population. Rapid identification of the problem, along with early surgical intervention if necessary, could be life-saving. However, surgical intervention in areas unrelated to the musculoskeletal system often pose a different problem. One of the first areas of the body to be affected with FOP is the neck and back region. Immobility of the neck may complicate anesthetic management during surgery. Thus, patients with FOP should be evaluated by a physician familiar with cardiorespiratory problems, and perhaps even an anesthesiologist, so that a plan could be developed for anesthetic management of potential surgical emergencies. Several patients with FOP have found it helpful to seek such advice on an elective (voluntary) basis so that a plan for anesthetic management of surgical emergencies would be available should the need arise.

One question that often arises relates to injections. Although it is certainly essential to avoid deep intramuscular injections (injections into the muscle), subcutaneous injections (under the skin) and blood test evaluations (obtained through a peripheral vein under the skin) can often be performed without any difficulty or substantial risk of triggering new bone formation.

The following guidelines may be helpful in dealing with surgery and FOP:

• Be aware of the risks of new bone formation following injury to, or surgery on the musculoskeletal system
• Avoid injury to the musculoskeletal system.
• Avoid elective surgery on the musculoskeletal system. Although the bone can be removed, it will often grow back and lead to problems worse than the original condition. Surgical procedures to increase mobility do not work.
• Ask your family physician or internist to schedule a consultation for you with an anesthesiologist in your local area who can work out a safe plan for administering a general anesthetic should an emergency arise. Such a plan could be filed in your chart, and a copy should be made available to you, particularly when you are out of town. Such a plan could be helpful in the event of an emergency.
• If emergency surgery is needed, careful planning and management of airway problems should be recognized and implemented.
• Avoid intramuscular injections. Subcutaneous injections and routine blood drawing can be accomplished with much greater safety and much less risk of precipitating new bone formation.