BASICS
DESCRIPTION
• The most common congenital abnormality of the lacrimal drainage system
• The nasolacrimal duct is a bilateral structure that carries tears from the lacrimal sac into the nasal cavity with an opening, the inferior meatus, under the inferior turbinate.
• Congenital nasolacrimal duct obstruction (NLDO) reportedly affects 6–20% of neonates. In 90% of the cases, symptoms spontaneously resolve by 12 months (1)[C].
EPIDEMIOLOGY
Incidence
6–20% of neonates are born with symptoms on NLDO, with the majority having spontaneous resolution (1)[C].
Prevalence
Most common lacrimal system anomaly in childhood.
RISK FACTORS
Patients with some craniofacial malformations, Down syndrome, and a wide variety of other syndromes with craniofacial or oculonasal malformations (e.g., Cornelia de Lange, Johanson-Blizzard) are at increased risk for NLDO as well as other nasolacrimal system anomalies.
Genetics
• There is no known specific causative genetic abnormality but the incidence is higher within families.
– When associated with underlying genetic systemic syndromes, the inheritance pattern of that syndrome applies although variable expression of the nasolacrimal duct anomaly may often be seen.
PATHOPHYSIOLOGY
The nasolacrimal duct canalizes by the eighth month of gestation from the mesoderm centrally and from the surface ectoderm distally. A thin mucosal membrane at the distal end (Valve of Hasner) causes congenital NLDO.
ETIOLOGY
Unknown
COMMONLY ASSOCIATED CONDITIONS
• Down syndrome
• Craniofacial abnormalities
• Preauricular sinus
• Other systemic syndromes characterized by oculofacial involvement
• Majority of cases have no associated systemic disease
DIAGNOSIS
HISTORY
• Parents usually report history of epiphora that may or may not be accompanied by a mucopurulent discharge
• Epiphora may not occur until later in infancy
– Discharge usually mucoid, clinging to lower lashes, not associated with conjunctival injection
– Discharge worse upon wakening from sleep. Eyelashes often stuck together
– May be worse with upper respiratory tract infection or outdoors on windy days
PHYSICAL EXAM
• Eyelashes may appear matted or wet.
• Digital pressure on the lacrimal sac can cause reflux of purulent material by puncta.
• Chronic skin changes of lower lid
• Increased tear lake may be visible on the side that has NLDO. If there is bilateral NLDO, this will be difficult to defect.
• Careful examination of the eyelid, conjunctiva, and cornea should be performed to rule out other causes of epiphora (see Differential Diagnosis).
– Little or no conjunctival injection
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Initial lab tests
None
Follow-up & special considerations
Should erythema and swelling with or without a mass develop in the area of the medial canthus or the inferior medial aspect of the lower lid, suspect dacryocystitis/infected lacrimal sac mucocele and treat urgently with referral to ophthalmology.
Imaging
• In the majority of cases, no imaging is needed.
• Consider CT scan if craniofacial abnormality or trauma (2)[C].
Diagnostic Procedures/Other
Dye Disappearances Test: A drop of fluorescein is placed in the eye and visualized with a cobalt blue light. If pooling persists in the eye after 5 minutes, or significantly longer than the uninvolved eye, the test is positive for an obstruction. When fluorescein is found in the nasal cavity, most commonly by inserting a cotton swab through the nares, then the system is patent.
DIFFERENTIAL DIAGNOSIS
• Dacryocele (lacrimal sac mucocele)
• Congenital entropion/ectropion
• Epiblepharon
• Trichiasis
• Congenital/infantile glaucoma
• Infectious conjunctivitis or keratitis
• Uveitis
• Foreign body
• Other lacrimal system (e.g., anomaly: Punctal/canalicular stenosis/agenesis, lacrimal fistula)
TREATMENT
MEDICATION
First Line
• Massage of lacrimal sac should be the first line of therapy. It is theorized that this will increase the hydrostatic pressure in the lacrimal system allowing the membranous obstruction to rupture (2)[C].
– Caretaker should place forefinger on medial canthal ligament and push posteriorly (towards occiput) to compress underlying lacrimal sac, 3–4 times daily. Often done with feedings.
Second Line
Antibiotics therapy should only be used if there are signs of secondary infection (e.g., conjunctivitis) (2)[C].
ADDITIONAL TREATMENT
Issues for Referral
• A large percentage of babies are born with NLDO and resolve within the first year of life. If by age 12 months tearing persists, then referral to an ophthalmologist is warranted. Severe NLDO with copious discharge may be referred earlier, especially if there is no improvement with massage.
• Concern for dacryocystitis or pre-septal cellulitis should be referred urgently to ophthalmology.
SURGERY/OTHER PROCEDURES
• If symptoms persist past the first year of life, and massage for at least 3 months has failed, surgery may be considered and is highly successful in most cases. Earlier surgery may be considered in severe cases. The use of awake in office (as opposed to general anesthesia) is controversial. Early surgery is also indicated following secondary dacryocystitis/cellulitis.
• Probing: Probing is traditionally seen as first-line treatment (1)[C]. Approximately 78% success rate (3)[B].
• Balloon catheter dilation: Variation of standard probing. More costly. 82% success rate reported in the literature (4)[B]. May not be statistically significant.
• Nasolacrimal duct intubation: A monocanalicular or bicanalicular tube can be left in place for several months at the time of probing. One disadvantage is that the tube does need to be removed and some children may not tolerate this in the office requiring sedation. In a prospective study the success rate of intubation was 91% (5)[B]. Most often this is reserved for older children and children who have failed standard probing once or twice.
• Dacryocystorhinostomy: This is usually a last resort for patients who have had multiple failed procedures.
IN-PATIENT CONSIDERATIONS
Admission Criteria
Admission only necessary if signs of dacryocystitis, pre-septal or orbital cellulitis develop and intravenous antibiotics are needed.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
No follow-up required after NLDO is resolved.
Patient Monitoring
• Minimum massage time is 3 months
• Monitor for signs of cellulitis/dacryocystitis until NLDO resolved
DIET
No dietary recommendations
PATIENT EDUCATION
• Educate parents that this is a common problem that resolves spontaneously in the majority of cases with the assistance of massage. In situations where surgery is needed, outcomes are excellent.
• Patients with craniofacial abnormalities may have lower success rates even with surgery.
PROGNOSIS
Excellent
COMPLICATIONS
• Mild transient nose bleeding after surgery
• Corneal abrasion after surgery
• False passage created with probing
• Extrusion of tube
• Dacryocystitis/cellulitis
• Complications of probing are extremely uncommon
REFERENCES
1. Puvanachandra N, Trikha A, MacEwen CJ, et al. A national survey of the management of congenital nasolacrimal duct obstruction in the United Kingdom. J Pediatr Ophthalmol Strabismus 2010;47(2):76–80.
2. Takahashi Y, Kakizaki H, Chan WO, et al. Management of congential nasolacrimal duct obstruction. Acta Ophthalmol 2010;88(5):506–513.
3. Pediatric Eye Disease Investigator Group. Primary treatment of nasolacrimal duct obstruction with probing in children less than four years old. Ophthalmology 2008;115(3):577–584.
4. Pediatric Eye Disease Investigator Group. Primary treatment of nasolacrimal duct obstruction with balloon catheter dilation in children less than four years old. J AAPOS 2008;12(5):451–445.
5. Pediatric Eye Disease Investigator Group. Primary treatment of nasolacrimal duct obstruction with nasolacrimal duct intubation in children less than four years old. J AAPOS 2008;12(5):445–450.
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