Wolfring Gland Ductal Cyst

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Article initiated by:

Mostafa Mohamed Diab, MD

All contributors:

Michael T Yen, MD, Mostafa Mohamed Diab, MD

Assigned editor:

Adam R. Sweeney, MD

Review:

Assigned status Up to Date

 by Michael T Yen, MD on April 28, 2024.

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Contributing Editors:	add

Wolfring gland ductal cysts (WGDCs) are benign cystic lesions of the eyelid. They are not uncommon in countries where trachoma has been endemic. WGDC represents a clinical diagnostic challenge with a high possibility of misdiagnosis. Certain clinical and histologic clues help guide the diagnosis. Complete surgical excision with intact wall remains the recommended treatment modality.

Disease Entity

Disease

Wolfring gland ductal cyst (WGDC) refers to cystic dilatation of the duct of the accessory lacrimal glands of Wolfring. Wolfring glands are located in the deep conjunctival stroma along the proximal tarsal border in the upper eyelid, and to a lesser extent in the lower eyelid. WGDC is also known as Wolfring dacryops, ductal cyst of the accessory lacrimal gland of Wolfring, Wolfring ductal cysts, and accessory lacrimal gland ductal cyst. WGDC usually affects young adults or middle-aged individuals.

Etiology

The exact etiology remains unclear.

Risk Factors

WGDC has been reported to be linked with conditions causing chronic conjunctival inflammation and cicatrization. Most reported case series come from Saudi Arabia and Egypt, showing a significant association with trachomatous scarring (100% in Weatherhead's series and 79.2% in Diab's series). This association might explain why WGDCs are relatively rare in Western countries.

General Pathology

A WGDC is lined by a double-layered nonkeratinizing cuboidal epithelium with a serpiginous cavity.

Grossly, WGDC appears smooth, thin walled with telangiectatic blood vessels on the surface, and often contains clear fluid. Histologically, it is lined by a double-layered nonkeratinizing epithelium with occasional inflammatory and goblet cells, and commonly having a serpinginous cavity. Lacrimal glandular acini are typically absent. This can be attributed to the limited amount of glandular tissue in accessory lacrimal gland lesions compared to main lacrimal gland lesions that can be overlooked without meticulous tissue sampling and sectioning or due to the careful surgical separation of the cyst from surrounding tissues, including lacrimal glandular tissue.

Pathophysiology

The pathogenesis of WGDC is still debatable and likely multifactorial. Conjunctival cicatrization appears to play a significant role in the formation of dacryops. However, the original hypothesis of cicatricial ductular blockage with secondary build up of secretory pressure, as the sole mechanism of dacryops formation has been challenged experimentally. Some authors suggest that there is a concurrent IgA hypersecretion into the cyst by activated plasma cells, leading to an osmotic effect. Others have proposed dysfunction of the periductular neural plexus.

Diagnosis

WGDC can be diagnostically challenging with a high possibility of misdiagnosis due to its different presentations and similarities with other cystic lesions. Diagnosis is typically made on clinical basis alone. Histopathological examination helps differentiate WGDC from other cystic conditions.

History

Patients are usually presenting with slowly progressive painless eyelid mass. Small lesions are often asymptomatic.

Physical examination

Wolfring gland ductal cyst (WGDC) appears as a yellowish grey subconjunctival cyst in the superonasal fornix with surrounding conjunctival scarring. Note the associated pannus siccus, which is pathognomonic for chronic trachoma.

A dense transverse fibrotic band is seen along tarsal border in the area of a WGDC on complete lid eversion.

Exam reveals an immobile bluish grey translucent subconjunctival cyst associated with the proximal tarsal border. A dense fibrotic band along the proximal tarsal border is typically seen in the area of the cyst on complete lid eversion. These lesions more frequently appear in the upper eyelid medially or centromedially, likely because more Wolfring glands exist in this location. The cyst tends to grow insidiously; however, a rapid increase in size may occur and should raise the suspicion of cyst complications such as hemorrhage or infection. Signs of chronic trachoma or other cicatrizing conjunctival diseases may be present.

Signs

Eyelid mass

Subconjunctival cyst

Conjunctival scarring

Transverse fibrotic band along the proximal tarsal border

A 36-year-old male patient presents with left upper lid ptosis due to a superomedial WGDC.

An elderly woman presents with a recurrent large mass in the right upper lid with complete ptosis. The patient underwent  repeated needle aspiration of the swelling over a couple of years and the cyst refills rapidly after being drained.

Mechanical ptosis, globe dystopia

Symptoms

Painless eyelid swelling

Droopy eyelid

Chronic eye irritation

Diagnostic clues

Certain clinical and histologic clues help differentiate WGDCs from other similar conditions.

• Subconjunctival location in the central or centromedial fornix.
• Attachment to the nonmarginal border of the tarsus.
• Transverse fibrotic band along the tarsal border in the area of the cyst.
• Lack of tenderness.
• The double-layered epithelial lining of the cyst with a serpiginous cavity.

Differential diagnosis

Simple dacryops (palpebral lobe dacryops)- the swelling is mobile and located in the superolateral fornix with a possibility of spontaneous and intermittent disappearance.

Krause gland ductal cysts- anatomically located within the fornices and lack fixation to the tarsal plate.

Periorbital dermoid cysts- painless subcutaneous mass along the suture lines, located in the superotemporal or superonasal orbit in a younger age group, not visible through the conjunctiva, commonly associated with underlying bony changes, lined by a keratinizing epithelium with adnexal structures, its cavity is filled with keratin and sebaceous fluid.

Subconjunctival cysticercosis- painful, yellowish, nodular subconjunctival mass with surrounding conjunctival injection. Orbital cysticercosis- frequently occurs in relation to extraocular muscles with possibility of ocular motility restriction. The cyst has two-layer lining with a scolex can be identified inside the cyst. B scan ultrasonography, CT scan, and MRI are helpful in confirming the diagnosis.

Intratarsal keratinous cyst- firm, well-circumscribed and non-tender, protrudes visibly under the tarsal conjunctiva with anterior tarsal fixation, and lined by stratified squamous epithelium with an inner eosinophilic undulating cuticle and filled with keratin.

Conjunctival inclusion cyst- results from the entrapment of conjunctival epithelial tissue within the substantia propria, there may be a history of trauma or surgery, lack of fixation to the tarsal plate, and lined by multilaminar nonkeratinizing squamous epithelium.

Management

Management options include either observation or surgical intervention, depending on the size of the cyst and the patient's complaints. Small asymptomatic lesions can be safely monitored with serial eye exams.

Surgery

Different treatment modalities, such as excision, simple aspiration, marsupialization, or needling, have been described for WGDCs.

A WGDC is removed through the conjunctiva of the upper eyelid with an intact wall leaving a large conjunctival defect.

A WGDC is removed through a lid crease incision.

A WGDC of the left lower lid is removed through an infraciliary incision.

1- Complete surgical removal with an intact wall remains the mainstay treatment and can be performed either through the conjunctiva or skin. A recent retrospective study has shown more favorable outcomes with the transcutaneous approach.

2- Simple needle aspiration is generally not recommended as cysts readily refill after the procedure.

3- Marsupialization entails excising a part of the cyst roof through a conjunctival incision and leaving the wound to heal spontaneously. The procedure does not require meticulous dissection of the cyst wall, however the risk of recurrence remains a concern.

Complications

An infected WGDC with whitish opaque material inside the cyst and surrounding conjunctival injection.

Bleeding in a WGDC secondary to periocular trauma.

Cysts can develop chronic inflammation or infection with abscess formation or fistulization. Patients having infected cysts often present with painful eyelid swelling and redness. The surrounding conjunctiva shows injection and chemosis with the presence of whitish opacification inside the cyst. Infections require management with systemic antibiotics prior to surgical treatment. Bleeding into a cyst can occur and the cyst becomes chocolate colored with rapid increase in size. A large WGDC in a child can induce  significant refractive error or cause progressive significant ptosis with a potential risk of amblyopia.

A huge WGDC of the right upper eyelid is removed through an lid crease incision.

Prognosis

The prognosis is excellent.