BASICS

DESCRIPTION

• Distended lacrimal sac usually in neonates or very early infancy

• With or without associated intranasal cyst

• Also known as dacryocele, lacrimal sac mucocele, dacryocystocele, or amniotocele

EPIDEMIOLOGY

Incidence

Incidence unknown

Prevalence

0.1% of infants with congenital nasolacrimal duct obstruction (NLD) (1)[A]

RISK FACTORS

Unknown

Genetics

• Usually sporadic

• Familial cases have been reported

GENERAL PREVENTION

• Can be detected by prenatal ultrasound (2)

– No preventive measures identified

PATHOPHYSIOLOGY

Congenital distal membranous blockage of nasolacrimal duct at inferior meatus intranasally causing lacrimal sac distension and obstruction of the entrance to the sac by the common canaliculus at valve of Rosenmüller. Antegrade and retrograde discharge of accumulated secretions prevented.

ETIOLOGY

Congenital membrane or valve (Hasner) obstruction related to canalization failure or persistence of embryologic membrane.

COMMONLY ASSOCIATED CONDITIONS (3)[A]

• Dacryocystitis

• Pre-septal cellulitis

• Congenital NLD obstruction

– Cystic bulging of the mucosa of the distal nasolacrimal duct into the nasal cavity

– May cause nasal airway obstruction and respiratory distress

DIAGNOSIS

HISTORY

• Distended mass with bluish discoloration below the medial canthal tendon within the medial aspect of the lower lid, presenting in a neonate unilaterally or bilaterally

• May be history of overlying progressive erythema and swelling (if infected)

• May be history of difficulty breast feeding on mother’s breasts ipsilateral to dacryocele (e.g., right breast of woman feeding child with a right dacryocele) due to compression of contralateral patent nares against nipple

PHYSICAL EXAM

• Palpable mass

– Distended and firm

– May be tender

– Non-moveable

– Medial lower eyelid displaced superiorly

– May be signs of NLD obstruction or dacryocystitis

– Mucoid discharge may be expressed from puncta or nasal cavity on compression of mass

– Conduct full eye examination with attention to possible secondary periorbital/orbital cellulitis, globe displacement, strabismus, amblyopia, or induced astigmatism

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

• CBC with differential and blood culture if dacryocystitis or cellulitis suspected

• Consider additional cultures (rule out sepsis) including urine and cerebrospinal fluid if infection present and child showing systemic signs

Follow-up & special considerations

Monitor for infection

Imaging

Initial approach

• In the presence of signs and symptoms of nasal airway obstruction

– CT or MRI to demonstrate endonasal cyst occasionally indicated (4)[A]

Follow-up & special considerations

Consider otorhinolaryngology consult to evaluate nasal cysts

Diagnostic Procedures/Other

Endoscopic identification of nasal cysts at time of surgery (when indicated)

Pathological Findings

Membranous intranasal cyst wall at inferior meatus with ciliated respiratory epithelium on nasal side and stratified columnar epithelium on side of NLD

DIFFERENTIAL DIAGNOSIS

• Other masses in medial canthal area

– Capillary hemangioma (not present at birth, rubbery and soft to palpation, and possible reddish surface component)

– Dermoid or epidermoid cyst (moveable subcutaneous mass, typically superonasal, no blue discoloration, rarely infected)

– Encephaloceles (typically present above medial canthal ligament, usually with hypertelorism)

TREATMENT

MEDICATION

• Decompression by firm but careful digital massage (if successful, immediate decompression of mass occurs)

– If dacryocystitis and/or cellulitis present, some recommend warm compresses and systemic antibiotics but in this age group risk for systemic infection is high and surgery should be considered

ADDITIONAL TREATMENT

General Measures

Monitor and treat subsequent NLD obstruction if present

Issues for Referral

Consider otorhinolaryngology consult to evaluate nasal cysts

COMPLEMENTARY & ALTERNATIVE THERAPIES

None known

SURGERY/OTHER PROCEDURES

• Probing and irrigation with or without nasal endoscopy if nonresponse to medical therapy, airway obstruction, or infection

– If not infected, gentile probing through puncta into proximal lacrimal sac by common canaliculus in cases resistant to digital message

• Probing under general anesthesia is the mainstay of surgical therapy

– Nasal endoscopy useful to visualize nasal cyst and to guide probe

– Nasal mucocele can be marsupialized under endoscopic guidance (5)[A]

– In office awake nasal endoscopy has been performed but is technically challenging (6)[B]

IN-PATIENT CONSIDERATIONS

Admission Criteria

• Advised if dacryocystitis/periorbital cellulitis especially if signs of systemic infection for intravenous antibiotics prior to surgery

• Some recommend an additional intravenous day of antibiotics after surgery

Discharge Criteria

• Reduction or resolution of cystic mass and infection

– No evidence of nasal airway compromise

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

As needed if cystic mass recurs

Patient Monitoring

• Care givers to observe for recurrence of cystic mass

– Monitor for breathing difficulty

– Monitor for signs of infection

– May have residual NLD obstruction

PATIENT EDUCATION

• Parents instructed to gently digitally massage inferior medial canthal area where cyst was present for a few days to possibly prevent reformation of dacryocele

– Parents educated as to signs of infection and urgent need for returning to care in that circumstance

PROGNOSIS

• 99% complete recovery and resolution of signs and symptoms with surgery including nasal endoscopy

• Success less for probing without endoscopy (approximately 70–80%)

– May develop signs and symptoms to typical nasolacrimal duct obstruction in the future

COMPLICATIONS

• Development of fistulous tract through skin over distended dacryocele (NOTE: Do not drain dacryocele through skin)

• Chronic nasolacrimal duct obstruction

• Pre-septal cellulitis

– Sepsis/meningitis

– May be transient excavation of infraorbital bone that is palpable after cyst decompressed

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