Eric Chien-Wei Liao, MD, PhD, is a pediatric plastic surgeon-scientist and the founding Director of the Center for Craniofacial Innovation at Children's Hospital of Philadelphia (CHOP). Here, Dr. Liao answers questions that cover how clinicians can recognize the signs of fibrous dysplasia and when to refer the patient to a specialist.
Q. What is fibrous dysplasia, and how does it typically present in children?
A. Fibrous dysplasia is a bone disease that replaces normal bone with fibrous-type tissue. This tissue is not as hard as normal bone, and because it is soft and stringy, the bone is more fragile and prone to breaking. This condition can affect facial bones and long bones, such as those in the legs. In the face, the fibrous growths can cause pain and movement of teeth. When it affects the orbit of the eye, it can lead to blindness.
Q. What are the early signs of fibrous dysplasia that primary care providers or pediatric specialists should watch for?
A. Early signs include facial asymmetry; persistent pain in the affected bone; and visual and hearing problems. When fibrous dysplasia occurs as part of McCune-Albright syndrome, it can affect the endocrine system and cause skin pigmentation lesions called café-au-lait spots.
Q. When should a child with suspected fibrous dysplasia be referred to a plastic and reconstructive surgery team?
A. As soon as a child exhibits any signs and symptoms, they should be referred to a plastic surgery specialist.
Q. Why is referral to CHOP’s Plastic, Reconstructive and Oral Surgery team important?
A. CHOP’s team is made up of experts in the care of facial and orthopedic fibrous dysplasia. Because fibrous dysplasia affects people in different ways, we will perform a comprehensive evaluation and create a coordinated care plan specific to the patient’s situation.
Q. What evaluations help guide care for children with suspected fibrous dysplasia?
A. The CHOP team may recommend or interpret certain assessments to understand the extent of the disease and its impact on a child’s growth and function. These evaluations, coordinated through CHOP’s multidisciplinary craniofacial team, help determine the best timing for intervention and long-term management to preserve both function and facial growth. Our evaluation may involve a craniofacial CT scan or x-ray of the affected limb.
Q. How is fibrous dysplasia managed once a child is referred to CHOP?
A. We will assess the condition with CT scan. If necessary, we will decide with the family whether and when to perform surgery, to address functional problems (occlusion, vision) or appearance (overgrowth, asymmetry), or whether some aspects can be treated without surgery.
Q. What features indicate the need for expedited referral?
A. Features that indicate need for expedited referral are functional problems involving vision changes, hearing loss, increasing pain, problems with jaw movement, and problems with breathing.
Q. What are the benefits of early identification and referral?
A. The benefit of early identification and referral is to address any threats to function (vision and hearing) that, if detected too late, could cause irreversible damage.
Q. Are other specialists involved in the care of a child with fibrous dysplasia?
A. An endocrinologist is needed to manage McCune-Albright syndrome, which along with fibrous dysplasia causes café-au-lait skin pigments and precocious puberty.
Q. What is the length of care for fibrous dysplasia patients?
A. A person with fibrous dysplasia will need lifelong care. At CHOP, we facilitate a patient's transition to adult care at the appropriate time.
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Eric Chien-Wei Liao, MD, PhD, is a pediatric plastic surgeon-scientist and the founding Director of the Center for Craniofacial Innovation at Children's Hospital of Philadelphia (CHOP). Here, Dr. Liao answers questions that cover how clinicians can recognize the signs of fibrous dysplasia and when to refer the patient to a specialist.
Q. What is fibrous dysplasia, and how does it typically present in children?
A. Fibrous dysplasia is a bone disease that replaces normal bone with fibrous-type tissue. This tissue is not as hard as normal bone, and because it is soft and stringy, the bone is more fragile and prone to breaking. This condition can affect facial bones and long bones, such as those in the legs. In the face, the fibrous growths can cause pain and movement of teeth. When it affects the orbit of the eye, it can lead to blindness.
Q. What are the early signs of fibrous dysplasia that primary care providers or pediatric specialists should watch for?
A. Early signs include facial asymmetry; persistent pain in the affected bone; and visual and hearing problems. When fibrous dysplasia occurs as part of McCune-Albright syndrome, it can affect the endocrine system and cause skin pigmentation lesions called café-au-lait spots.
Q. When should a child with suspected fibrous dysplasia be referred to a plastic and reconstructive surgery team?
A. As soon as a child exhibits any signs and symptoms, they should be referred to a plastic surgery specialist.
Q. Why is referral to CHOP’s Plastic, Reconstructive and Oral Surgery team important?
A. CHOP’s team is made up of experts in the care of facial and orthopedic fibrous dysplasia. Because fibrous dysplasia affects people in different ways, we will perform a comprehensive evaluation and create a coordinated care plan specific to the patient’s situation.
Q. What evaluations help guide care for children with suspected fibrous dysplasia?
A. The CHOP team may recommend or interpret certain assessments to understand the extent of the disease and its impact on a child’s growth and function. These evaluations, coordinated through CHOP’s multidisciplinary craniofacial team, help determine the best timing for intervention and long-term management to preserve both function and facial growth. Our evaluation may involve a craniofacial CT scan or x-ray of the affected limb.
Q. How is fibrous dysplasia managed once a child is referred to CHOP?
A. We will assess the condition with CT scan. If necessary, we will decide with the family whether and when to perform surgery, to address functional problems (occlusion, vision) or appearance (overgrowth, asymmetry), or whether some aspects can be treated without surgery.
Q. What features indicate the need for expedited referral?
A. Features that indicate need for expedited referral are functional problems involving vision changes, hearing loss, increasing pain, problems with jaw movement, and problems with breathing.
Q. What are the benefits of early identification and referral?
A. The benefit of early identification and referral is to address any threats to function (vision and hearing) that, if detected too late, could cause irreversible damage.
Q. Are other specialists involved in the care of a child with fibrous dysplasia?
A. An endocrinologist is needed to manage McCune-Albright syndrome, which along with fibrous dysplasia causes café-au-lait skin pigments and precocious puberty.
Q. What is the length of care for fibrous dysplasia patients?
A. A person with fibrous dysplasia will need lifelong care. At CHOP, we facilitate a patient's transition to adult care at the appropriate time.
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