Dacryocystocele (amniotocele)

Etiology

• Occurs when nasolacrimal duct (NLD) obstructed in the neonate, amniotic fluid or mucus is trapped in the lacrimal sac (See Congenital nasolacrimal obstruction)
• Includes functional proximal obstruction at junction of common canaliculus and lacrimal sac or at valve of Rosenmüller combined with a distal obstruction at the level of the valve of Hasner
• Develops as a consequence of congenital NLD obstruction, with distention of the lacrimal sac
• May be associated with intranasal cysts, incidence as high as 76% reported.

History

• History includes swelling of the lacrimal sac since birth
• Familial in rare cases
• Ask family if the patient is exhibiting any signs of respiratory distress

Clinical features

• Clinical features include tearing and expansion of the lacrimal sac inferior to the medial canthal tendon
• Lacrimal sac is distended, often bluish in color (See Figure 1)
• In patients with associated nasal cysts, up to 66% had respiratory distress in one study.  Neonates are obligate no breathers
• (Schnall BM, Christian CJ J Pediatr Ophthalmol Strabismus. 1996 Sep-Oct; 33(5):219-22.)
• May progress to dacryocystitis

Testing and evaluation for establishing the diagnosis

• Physical exam – a distinct clinical picture if not associated with concurrent dacryocystitis; fundamentally, dacryocystocele is a clinical diagnosis
• Transillumination
• Intra nasal exam, particularly to identify the presence of an intranasal cyst and respiratory obstruction
• Ultrasound avoids radiation exposure and does not require sedation.  May be less  in determining intracranial communication. Ultrasound has diagnosed dacryocystocele in-utero.
•  On B-scan, the cyst appears as a cavity that associates with the nasolacrimal duct
• Low internal reflectivity on A-scan. (Cavazza, S, et al. Acta Otorhinolaryngol Ital. 2008 December; 28(6): 298–301.
• Computed tomography (CT) or magnetic resonance imaging (MRI) scan (See Computed tomography) (See Magnetic resonance imaging) will show a cystic mass extending from the lacrimal sac towards the inferior meatus, sometimes displacing the inferior turbinate medially

• Include congenital NLD obstruction
• More common in females
• More common in Caucasians than African Americans (Mansour AM, Cheng KP, Mumma JV, Stager DR, Harris GJ, Patrinely JR, Lavery MA, Wang FM, Steinkuller PG.  Ophthalmology. 1991 Nov; 98(11):1744-51.)

• Meningoencephalocele
• Dermoid cyst (See Dermoid cysts)
• Solid dermoid
• Neoplasm
• Capillary hemangioma (See Capillary hemangioma)
• Glial Heterotopia or Ectopic Brain – There is NO continuity with the brain in this condition

• • Natural history, outcome and prognosis
    • Treatment is controversial
  • Medical therapy options
    • Initially may respond to conservative management with prophylactic topical antibiotics and massage
    • Follow patients closely as the dacryocystocele may recur despite apparent successful decompression
  • Surgical therapy options
    • If no response within 1-2 weeks or infection develops, NLD probing with combined nasal endoscopy
    • Some authors recommend NLD probing with nasal endoscopy and marsupialization of cyst, if indicated,  when signs of infection, nasal cysts or respiratory distress
    • Some authors recommend probing on urgent basis unless lacrimal sac decompresses into the nose at time of initial examination
    • Nasal mucoceles extending inferior to the inferior turbinate often require excision or marsupialization of the prolapsed distended duct with nasal endoscopy.
    • One should consider urgent surgical management in patients who do not initially decompress with message, develop significant infectious signs, and/or show signs of respiratory distress.
    • If condition bilateral and causes airway obstruction, urgent treatment needed
  • Importance of bilateral visualization in congenital dacryocystocele
    • Infants with unilateral dacryocystocele at birth requiring surgical intervention would benefit from bilateral nasal endoscopic visualization as may have bilateral dacryocystoceles.

• Orbital cellulitis
• Meningitis
• Persistent NLD obstruction
• Nasal obstruction with respiratory distress
• May induce astigmatism

• Orbital cellulitis
• Meningitis
• Persistent NLD obstruction
• Nasal obstruction with respiratory distress
• May induce astigmatism

• Massage lacrimal sac frequently
• Apply topical antibiotic drops or ointment
• Some authors recommend warm compresses
• Return immediately if area becomes red, swollen, painful, and if child develops a fever

• AAO, Basic and Clinical Science Course. Section 7: Orbit, Eyelids and Lacrimal System, 2010-11.
• Scott KR, Tse, DT. Nasolacrimal Duct Probing and Intubation. In: Tse DT, ed. Color Atlas of Ophthalmic Surgery. Oculoplastic Surgery. Philadelphia: J.B Lippincott Company; 1992:73.
• Mansour AM, Cheng KP, Mumma JV. Congenital dacryocystocele. A collaborative review. Ophthalmology 1991;98:1744-1751.
• Becker BB. The Treatment of Congenital Dacryocystocele. Am J Ophthalmol 2006;142:835-838.
• Grinn TR, Mertz JS, Stass-Isemm, M. Congenital Nasolacrimal Duct Cysts in Dacryocystocele.  Ophthalmology 1991: 98:1238-1242.
• Harris GJ, DiClementi D.  Congenital Dacryocystocele. Arch Ophthalmol 1982: 100(11):1763-1765.
• Helper KM, Woodson GE, Kearns DB.  Respiratory distress in the neonate.  Sequela of a congenital dacryocystocele.  Arch Otolaryngol Head Neck Surg 1995 Dec; 121(12):1423-5.
• Katowitz JA, Low JE, Covici SJ, Goldstein JB.  Management of Pediatric Lower System Problems.  In Katowitz JA, ed. Pediatric Oculoplastic Surgery. New York: Springer-Verlag; 2002: Chapter 18.
• Schnall BM, Christian CJ.  Conservative treatment of congenital dacryocele. J Pediatr Ophthalmol Strabismus. 1996 Sep-Oct; 33(5):219-22.
• Cavazza, S, et al. “Congenital Dacryocystocele: Diagnosis and Treatment.” Acta Otorhinolaryngol Ital. 2008 December; 28(6): 298–301.
• George JC, et al.  Ophthalmology. 2022 Dec 17;S0161-6420(22)00924-1.