Fistula spied near infant’s eye

Figure

The case

The mother of a healthy 4-week-old boy brings him to the office for evaluation of a small pit on the medial canthus of the left eye, noted since birth. There is constant drainage of tears onto the left side of his face and exudate on the bottom of the left medial canthus.

CONGENITAL LACRIMAL FISTULA

History

A mother brought to the clinician’s attention a small opening located inferonasally from medial canthus during a well-check examination of a 4-week-old male infant. Symptoms included epiphora and exudation from the left eye. There was no inflammation or discharge from that opening, which appeared exactly like preauricular pit/preauricular sinus. The remaining eye examination was normal. The infant’s birth history was normal except for acquired left clavicular fracture because of birth trauma.

Discussion

Congenital lacrimal fistulas are developmental, rudimentary, supernumerary lacrimal canaliculi connecting skin to common canaliculus or lacrimal sac. These fistulas are located inferonasally from the medial canthus and are unilateral, but bilateral cases have been reported. The incidence is 1 in 2000.^1,2 Etiopathogenesis based upon histopathology suggest outgrowth from common canaliculus failing to fuse and aberrant budding.^1-3 The majority of these fistulas are lined with squamous epithelium, but rarely columnar epithelium or cuboidal epithelium have been found.²

The diagnosis of lacrimal fistula is mostly clinical, but irrigation of the lacrimal sac with fluorescein balanced salt solution can be used to detect skin ostium and the patency of the nasolacrimal duct. Dacryoendoscopy, computerized tomography, and polyvinyl siloxane cast also have been used to confirm diagnosis.^1,4

Congenital lacrimal fistulas are mostly asymptomatic; however, there can be complications or undesired symptoms attributed to the fistula. For example, chronic epiphora through the fistula or eye, or mucoid discharge from the fistula, can occur. In addition, coughing and blowing one’s nose may cause clear discharge.¹ There also may be redness of medial canthal angle, or constant tearing during crying. A mucocele can be found if associated with nasolacrimal duct obstruction.

There is no evidence of any systemic associations, but congenital lacrimal fistulas have been found with Down syndrome; naso-orbital meningocele; VACTERL association-like VATER (vertebral defects, anal atresia, tracheoesophageal fistula, esophageal atresia, and radial dysplasia); ectrodactyly-ectodermal dysplasia-clefting syndrome; and CHARGE syndrome (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities and ear abnormalities).¹

The most common differential diagnosis would be congenital nasolacrimal duct obstruction or stenosis attributed to failure of the opening of the lacrimal duct at birth. Dacryostenosis is commonly attributed to an imperforate membrane at the valve of Hasner.⁵ Duct obstruction or stenosis there is typically constant epiphora unrelated to crying, and it typically gets better with time and lacrimal sac massage.

Asymptomatic patients are managed conservatively. Symptomatic fistulas are treated with various surgical procedures such as probing, fistulectomy, and dacryocystorhinostomy.⁶

Patient outcome

In this infant, there was no inflammation or discharge from the opening. The remaining eye examination was normal.

The patient is currently being managed conservatively for epiphora. He is awaiting evaluation by an otorhinolaryngologist for fistulectomy with or without dacryocystorhinostomy.

References:

1. Chaung JQ, Sundar G, Ali MJ. Congenital lacrimal fistula: a major review. Orbit. 2016;35(4):212-220.

2. François J, Bacskulin J. External congenital fistulae of the lacrimal sac. Ophthalmologica. 1969;159(4):249-261.

3. Welham RA, Bergin DJ. Congenital lacrimal fistulas. Arch Ophthalmol. 1985;103(4):545-548.

4. Anderson SR, Wesley RE. CT scan of cutaneous lacrimal (anlage) fistula. Ophthalmic Surg. 1988;19(3):202-203.

5. Örge FH, Boente CS. The lacrimal system. Pediatric Clin North Am. 2014;61(3):529-539.

6. Ugurbas SH, Zilelioglu G. Congenital lacrimal fistula. Euro J Ophthalmol. 2000;10(1):22-26.