Orbits, eyelids and lacrimal pathways

A wide spectrum of tumors and pseudotumors (inflammatory disorders) involve the orbit. Their approach may require a handful of different medical fields, with Ophthalmology as pivotal speciality. My role as Maxillofacial Surgeon is to perform biopsies, tumor removal, orbital decompression, orbital and eyelid reconstruction.  

Orbital tumors and inflammatory diseases do not have specific symptoms. They may present with proptosis (eye bulgidng), eye redness, eye pain, visual impairment, diplopia (double vision), eyelid ptosis, epiphora (constant basal tearing), headaches and/or nasal obstruction. 

Imaging with CT scan and MRI are key for evaluation. While fine needle biopsy may be faster and cheaper to obtain, and useful for benign/malignant differatiation of orbital masses, a final diagnosis usually requires an open biopsy, in particular for bone, neural tumors and lymphomas.  

The most common orbital tumoral masses include: 

• Vascular malformations are one of the most frequent benign tumors involving the orbital tissues. Surgery is done when the lesion is symptomatic and interventional (neuro)radiology may be called for vascular diagnostic angiography, and in some cases with therapeutic vascular embolization. 
• Mesenchimal tumors encompass wide variety of tumors, usually benign such as lipomas. Within this group, the solitary fibrous tumors, are locally aggressive and therefore should be removed. Distant metastasis are very rare.  
• Shwannoma or neurilemmomas are benign tumors that arise from neural cells. If asymptomatic, can be monitored regularly. Gliomas and meningiomas must be surgically address, with Neurosurgery cooperation. 

• Tumors from the orbital bone, when benign and symptomatic they are surgically removed, trying to preserve the eye structure. When malignant, a prompt and radical excision is needed. 
• Lymphoma (in particular MALT lymphoma) are not infrequent, and required a biopsy for confirmation. Its treatment is based on drugs managed by a Haematologist. 
• Eyelid and facial skin tumors can invade the orbit with and should therefore surgically removed. A new drug, directed to basal cell carcinoma, enlarges the treatment portfolio for selected patients with irresectable or potentially eye losing resection surgery. Its prescription must be approved and directed by an oncology board. Radiotherapy is also frequently prescribed some of orbital invading tumors from the skin. 
• Lesions from the lacrimal sac and nasal sinus can also invade the orbit and should be referred to a surgeon.   
• Lacrimal gland tumors are rare, but must also be surgically removed.  

There are two main categories of inflammatory orbital diseases: 

• Non-specific or Idiopatic, previously known as inflammatory pseudotumor, because its clinical presentation resembles an orbital tumor. The biopsy is fundamental, but even with highly experienced Pathologist, it may be difficult to differentiate it from a lymphoma. Searching for a specific underlying cause may reveal an Ig4 immue-medidated syndromes or other rheumatological diseases such as sarcoidosis or lupus, and must be refered  to and auto-immune diseases specialist. 
• Graves’ disease is an autoimmune disease which deserves a topic on its own. 

It is an autoimmune mediated thyroid hyperfunction. Approximatively 25% to 30% will develop an inflammatory orbit and eye disease, named Graves´ orbitopathy, Graves´ ophtalmopathy or thyroid eye disease. It is classified in three severity categories: mild, moderate and sight-threatening. It impacts negatively the quality of life, even in mild and moderate disease. 

In Graves´orbitopathy there is an abnormal growth of adipose (fat) and muscle inside the orbit, which is a bony non-distensible cavity. Graves`s disease is classified in three severity categories: mild, moderate and sight-threatening. It impacts negatively the quality of life, even in mild and moderate disease. The growing tissues push the eye outward through the orbital natural opening, leading to proptosis (eye bulging), eyelid retraction and incapacity of eyelid closure leading to corneal dryness and consequently a corneal ulcer (sore). Besides changing the light refraction and visual power, it represents an open door for a severe eye infection.  

The impaired muscle activity and unbalanced eye axis may lead to double vision. Optic nerve compressing by the growing surrounding tissues is a sight-threatening event and needs expeditious medical and surgical care. 

Patient will probably present other hyperthyroidism signs such as tachycardia, excessive sweating, diarrhea, enlarged neck. 

General recommendations: 

The first and important active behaviour patient can do on their own: quit smoking and adopt a healthy lifestyle to reduce cholesterol. 

Thyroid hormonal control is of paramount importance, as well eye ointment with artificial tears and gels when dry eye is present, therefore prompt counselling with Endocrinologist and an Ophthalmologist.  

According to your disease activity and severity, the multidisciplinary medical team may decide for corticoid treatment and/or immunomodulatory drugs or in very selected cases radiotherapy. 

In case of sight threatening Graves’ ophthalmopathy, without sufficient response to medication, it must be quickly referred to a surgeon, for a prompt orbital decompression to reduce the pressure on the optic nerve. It is usually done endoscopically through the nose, but in severe cases can also include external approach to the lateral orbital wall.  

In case of inactive or stable disease, rehabilitative surgery may include eyelid surgery and orbital decompression as needed or required. 

If you think you might have Graves´ disease or orbital tumor, please write to us so we can provide your multidisciplinary evaluation.