Ocular Paget’s disease, a variant of Paget’s disease of the bone, is a chronic disorder characterized by abnormal bone remodeling. It primarily affects bones such as the skull, pelvis, spine, and legs, with potential implications for the bones surrounding the eyes (orbit). This can lead to various ocular manifestations due to bone enlargement and compression of nearby structures.

Paget’s disease is characterized by an imbalance between bone resorption by osteoclasts and bone formation by osteoblasts. Initially, there is increased osteoclastic activity, causing bone resorption. Subsequently, there is disorganized and excessive bone formation by osteoblasts, resulting in enlarged, weak, and structurally abnormal bones. In ocular Paget’s disease, this process affects the bones of the orbit, altering their shape and size, which can lead to complications involving the eye and surrounding structures.

1. Orbital Involvement: The orbit consists of several bones, including the frontal, maxillary, zygomatic, sphenoid, ethmoid, and lacrimal bones. In Paget’s disease, any of these bones can be affected, leading to thickening and enlargement. This can cause proptosis (bulging of the eye) as the orbit expands outward, affecting the positioning of the eyeball within the socket. Proptosis can be asymmetric and may cause aesthetic concerns or functional issues such as exposure keratitis due to inadequate eyelid closure.
2. Optic Nerve Compression: Enlargement of the bones surrounding the orbit can compress the optic nerve, which transmits visual information from the retina to the brain. Optic nerve compression can result in symptoms such as gradual visual impairment, including blurred vision, decreased visual acuity, and in severe cases, optic neuropathy characterized by visual field defects and color vision abnormalities. Early detection and management are crucial to prevent irreversible optic nerve damage.
3. Cranial Nerve Involvement: Besides the optic nerve, other cranial nerves passing through the orbit may also be affected. These include the oculomotor (III), trochlear (IV), and abducens (VI) nerves, which control eye movements, as well as the trigeminal nerve (V), responsible for facial sensation. Compression of these nerves can lead to double vision (diplopia), ptosis (drooping eyelid), facial numbness, or tingling sensations, depending on the nerve affected and the degree of compression.

• Proptosis: Gradual or sudden protrusion of one or both eyes due to orbital bone enlargement.
• Visual Changes: Variable degrees of visual impairment, ranging from mild blurring to significant loss of visual acuity.
• Eye Movement Disorders: Limited range of eye movements due to mechanical obstruction from enlarged bones.
• Facial Asymmetry: Uneven appearance of the face due to asymmetric bone growth around the orbit.

• Clinical Evaluation: A thorough physical examination may reveal palpable bony enlargement around the orbit, changes in eye position, or signs of optic nerve compression such as reduced visual acuity or color vision defects.
• Imaging Studies: X-rays, computed tomography (CT), and magnetic resonance imaging (MRI) are essential for assessing bone changes, determining the extent of orbital involvement, and identifying compression of surrounding structures, including the optic nerve and other cranial nerves.

• Medical Therapy: Bisphosphonates, such as alendronate or pamidronate, are the mainstay of medical treatment for Paget’s disease. They inhibit osteoclast activity, thereby reducing bone turnover and slowing disease progression. Calcitonin may also be used in some cases to help regulate bone metabolism.
• Surgical Intervention: Surgical options may be considered in cases of severe optic nerve compression or significant proptosis that does not respond to medical therapy. Surgical decompression aims to alleviate pressure on the optic nerve and improve visual function.
• Symptomatic Treatment: Management of specific symptoms, such as dry eye syndrome due to proptosis, and supportive care to enhance quality of life.

• The prognosis of ocular Paget’s disease depends on various factors, including the extent of bone involvement, early diagnosis, and prompt initiation of treatment.
• With timely intervention and appropriate management, including regular monitoring of disease progression and response to therapy, complications such as optic nerve damage can be minimized, and visual function can be preserved.

Ocular Paget’s disease presents unique challenges due to its potential impact on vision and facial aesthetics. Early recognition, thorough evaluation, and a multidisciplinary approach involving ophthalmologists, rheumatologists, and orthopedic surgeons are essential for optimal management. Continued research into the pathophysiology and treatment of Paget’s disease aims to improve outcomes and quality of life for affected individuals, emphasizing the importance of comprehensive care tailored to individual patient needs.

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