Basics
Description
A congenital blockage identified in infants from failure of canalization, most commonly at the distal portion, of the nasolacrimal duct. Epiphora (constant tearing) is the most common presentation followed by discharge unresponsive to treatment. Less commonly, the blockage is an acquired condition.
Epidemiology
- Most common cause of persistent tearing in infants
- True congenital nasolacrimal duct obstruction affects about 6% of newborns.
- Spontaneous resolution occurs in 90% of cases within 1 year.
Risk Factors
- Incidence is higher in infants with craniofacial malformations and Down syndrome.
Pathophysiology
- Congenital obstruction most commonly occurs at the level of the valve of Hasner in the distal portion of the nasolacrimal duct as it enters the nose.
- Types of distal obstruction include an imperforate membrane or cellular debris at the level of the valve, bony obstruction, or narrowing of the inferior meatus.
- Acquired obstructions are rare in children but do occur as a result of chronic inflammation and scar tissue occluding the duct. Causes include infection (i.e., ethmoidal sinusitis), inflammation, malignancy, and trauma.
Etiology
Canalization of the duct is usually complete by the 7th month of gestation, but a persistent membrane can remain and may represent the embryologic basis of lacrimal duct obstruction.
Commonly Associated Conditions
- Dacryocystocele (distention of the lacrimal sac) is a rare variant of lacrimal duct obstruction seen in 0.1% of infants with the disorder. There is the typical distal obstruction as well as a proximal obstruction at the junction of the common canaliculus and the lacrimal sac.
- Acute dacryocystitis is strongly suggestive of the presence of obstruction. Unclear whether obstruction is the primary cause leading to secondary infection from accumulation of tears and cellular debris or that dacryocystitis is the primary event with an acquired obstruction from fibrosis and inflammation
Diagnosis
History
- Congenital obstruction presents in first few weeks of life.
- Symptoms can be either unilateral or bilateral.
- Chronic tearing
- Mucoid discharge
- Crusting on eyelids and eyelashes
- Acquired obstruction is associated wtih chronic eye infections or a history of trauma (i.e., naso-orbito-ethmoidal fractures, lid laceration).
Physical Exam
- Discharge and/or crusting on the eyelashes
- Eyelid excoriation
- Compression of lacrimal sac reveals mucoid discharge and/or tears expressed through the punctum.
- Usually absence of conjunctival injection
- Bluish firm mass below the medial canthus suggests presence of dacryocystocele.
Diagnostic Tests & Interpretation
Usually, patients are treated without imaging and testing. If symptoms are intermittent and diagnosis is unclear, imaging and tests may be done.
Lab
Bacterial cultures are not a reliable indicator of the presence of obstruction or infection thus is not indicated for diagnostic purposes.
Imaging
- Radionuclide dacryocystography (also known as dacryoscintigraphy) helps evaluate the functioning of the lacrimal system by taking pictures as a radioisotope passes through the lacrimal system.
- Technically difficult to perform in children so is rarely done
- Does not allow for visualization of surrounding bony structures
- CT scan is useful for trauma or to assess bony obstruction resulting from craniofacial malformations or presence of dacryocystocele or other masses.
Diagnostic Procedures/Other
- Fluorescein dye disappearance test
- Preferred tool for diagnosis of congenital obstruction if symptoms are intermittent
- 90% sensitive and 100% specific for nasolacrimal duct obstruction
- Fluorescein is placed in the lower conjunctival fornix and the patient is observed for 5 minutes.
- If there is no obstruction, most of the fluorescein drains into the nose and minimal amounts of fluorescein is noted in the eye.
- If there is an obstruction, fluorescein will be seen in the eye with an increased tear meniscus with likely overflow of tears onto the cheeks.
- If dacryocystocele is suspected, nasal endoscopy is recommended. Application of a topical decongestant such as oxymetazoline hydrochloride to the nasal mucous membranes enhances visualization.
Differential Diagnosis
- Neonatal conjunctivitis
- Acute dacryocystitis
- Agenesis or imperforation of the lacrimal puncta or canaliculi
- Excess tear production
- Congenital glaucoma
- Corneal abrasion
- Abnormal eyelid position (entropion, epiblepharon)
- Trichiasis
- Foreign body
- Blepharitis
- Keratitis
- Uveitis
Treatment
Medication
Topical ophthalmic antibiotics are used in congenital obstruction when there is an increase in discharge, purulent discharge, or findings consistent with conjunctivitis. Topical antibiotics of choice include erythromycin, tobramycin, sulfacetamide, gentamicin, or fluoroquinolones.
Additional Treatment
General Measures
- Primary treatment for congenital obstruction is lacrimal sac massage in a downward fashion. Topical antibiotics are added as needed.
- Crigler maneuver can be effective in rupturing the membranous obstruction.
- Probing and irrigation are generally recommended if symptoms persist after 6 " 12 months of age.
- Some ophthalmologists prefer to perform the procedure prior to 12 months of age, and some continue with conservative measures until 15 " 18 months of age.
- Failed probing is usually noted within 6 weeks of procedure with return of symptoms.
- Probing may be repeated, although with increasing age, repeated probing may be less successful.
- Additional procedures may be done at the time of repeat probing that may increase the success rate, including the following:
- Balloon catheter dilatation. Some experts advocate that this can be considered as a primary treatment in place of probing.
- Nasal endoscopy allows visualization of the probe entering the nose, helping to avoid false passage.
- Silicone tube intubation helps prevent formation of granulation tissue and can aid in dilating stenotic segments of the lacrimal outflow system. Tube is left in place for 2 " 6 months but success has been seen with removal as early as 6 weeks in patients younger than age 2 years.
Issues for Referral
- Referral to a pediatric ophthalmologist is indicated if symptoms persist beyond 6 " 12 months of age with the application of conservative measures. Depending on severity and preference of the ophthalmologist, probing is done or conservative measures are continued.
- Early referral is warranted in cases of acute dacryocystitis and dacryocystocele.
- Cases of acquired obstruction should be referred for surgical treatment.
Surgery/Other Procedures
- Dacryocystorhinostomy
- Indicated when aforementioned procedures have failed and in cases of chronic dacryocystitis, bony obstruction, or dacryocystocele
- Creates a bypass fistula between the lacrimal sac and the nose to provide an alternate pathway for lacrimal flow
- Primary conjunctivodacryocystorhinostomy
- Creates a direct bypass tract between the medial canthus and the nose
- Limited reports on this technique being used in children
- Surgery is typically performed on patients with acquired obstructions and congenital anomalies of the upper lacrimal system.
Inpatient Considerations
Admission Criteria
- Neonates with dacryocystocele with an associated intranasal cyst obstructing their airway causing significant respiratory distress need to be admitted for airway management.
- Acute dacryocystitis should be admitted for parenteral antibiotic therapy with ophthalmology involved in the plan of care; may need surgery to drain the collection.
Ongoing Care
Follow-up Recommendations
Patient Monitoring
- Conservative measures are appropriate for infants with congenital obstruction, although at 6 " 12 months of age, referral should be considered for possible probing and irrigation.
- Children with a history of lacrimal duct obstruction need close follow-up for anisometropic amblyopia.
Prognosis
- About 90% of cases of congenital obstruction resolve by 12 months of age.
- Success rate of initial probing when performed at 12 months of age is about 80 " 90%.
- Probing is less successful after age 3 years.
Complications
- Infections such as bacterial conjunctivitis, acute and chronic dacryocystitis, and orbital cellulitis
- Respiratory distress in neonates with dacryocystocele that have an associated intranasal cyst at the valve of Hasner that obstructs their airway
- Anisometropic amblyopia
Additional Reading
- Arora S, Koushan K, Harvey JT. Success rates of primary probing for congenital nasolacrimal obstruction in children. J AAPOS. 2012;16(2):173 " 176. [View Abstract]
- Casady DR, Meyer DR, Simon JW, et al. Stepwise treatment paradigm for congenital nasolacrimal duct obstruction. Ophthal Plast Reconstr Surg. 2006;22(4):243 " 247. [View Abstract]
- Dantas RR. Lacrimal drainage system obstruction. Semin Ophthalmol. 2010;25(3):98 " 103. [View Abstract]
- Goldich Y, Barkana Y, Zadok D, et al. Balloon catheter dilatation versus probing as primary treatment for congenital dacryostenosis. Br J Ophthalmol. 2011;95(5):634 " 636. [View Abstract]
- Kapadia MK, Freitag SK, Woog JJ. Evaluation and management of congenital nasolacrimal duct obstruction. Otolaryngol Clin N Am. 2006;39(5):959 " 977. [View Abstract]
- Schnall BM. Pediatric nasolacrimal duct obstruction. Curr Opin Ophthalmol. 2013;24(5):421 " 424. [View Abstract]
- Takahashi Y, Kakizaki H,, Chan WO, et al. Management of congenital nasolacrimal duct obstruction. Acta Ophthalmol. 2010;88(5):506 " 513. [View Abstract]
- Wong RK, VanderVeen DK. Presentation and management of congenital dacryocystocele. Pediatrics. 2008;122(5):e1108 " e1112. [View Abstract]
Codes
ICD09
- 375.56 Stenosis of nasolacrimal duct, acquired
- 375.55 Obstruction of nasolacrimal duct, neonatal
- 743.65 Specified congenital anomalies of lacrimal passages
- 375.22 Epiphora due to insufficient drainage
ICD10
- H04.559 Acquired stenosis of unspecified nasolacrimal duct
- H04.539 Neonatal obstruction of unspecified nasolacrimal duct
- Q10.6 Other congenital malformations of lacrimal apparatus
- H04.229 Epiphora due to insufficient drainage, unsp lacrimal gland
- H04.551 Acquired stenosis of right nasolacrimal duct
- H04.222 Epiphora due to insufficient drainage, left lacrimal gland
- H04.533 Neonatal obstruction of bilateral nasolacrimal duct
- H04.532 Neonatal obstruction of left nasolacrimal duct
- H04.552 Acquired stenosis of left nasolacrimal duct
- H04.553 Acquired stenosis of bilateral nasolacrimal duct
- H04.223 Epiphora due to insufficient drainage, bi lacrimal glands
- H04.531 Neonatal obstruction of right nasolacrimal duct
- H04.221 Epiphora due to insufficient drainage, right lacrimal gland
SNOMED
- 314022009 obstruction of nasolacrimal duct (disorder)
- 9420008 Neonatal obstruction of nasolacrimal duct
- 271431003 Congenital stenosis of nasolacrimal duct
- 85042000 Epiphora due to insufficient drainage
FAQ
- Q: Why not fix the obstruction upon diagnosis?
- A: Studies have shown that about 90% of cases of congenital obstruction resolve by 12 months of age using conservative measures consisting of massage with occasional addition of topical antibiotics for infection that can occur concurrently with obstruction.
- Q: When is the optimal age for probing and irrigation?
- A: This is controversial, although most studies suggest conservative measures are preferred younger than 12 months of age. After 12 months of age, continued conservative measures or probing are selected based on severity of symptoms. Recent studies indicate that probing becomes less successful as a primary treatment in patients around age 3 years. This may be due to factors other than increasing age, including severe symptoms, canalicular stenosis, and nonmembranous obstruction.
- Q: Does probing and irrigation require general anesthesia?
- A: Sometimes probing is performed as an in-office procedure without general anesthesia if done when patient is young, younger than 12 months of age. When child is older than 12 months of age, probing is ideally performed under general anesthesia to ensure procedure is controlled and safe as well as allowing for direct visualization with endoscopy.