Congenital dacryocystocele: case report and treatment

Silvia Helena Tavares Lorena Eliana Domingues Gonçalves João Amaro Ferrari Silva About the authors

Abstracts

A dacriocistocele representa uma rara anomalia congênita da região medial da órbita, causada pela obstrução distal (ao nível da válvula de Hasner) e proximal (ao nível da válvula de Rosenmüller) da via lacrimal, com subsequente dilatação do saco lacrimal. Recebe o nome de mucocele, quando seu conteúdo representa muco, ou amniocele, quando o seu conteúdo é preenchido por fluido amniótico. Acomete somente 0.1% das crianças, com obstrução do ducto lácrimonasal, sendo comumente unilateral e mais frequente no sexo feminino e com predisposição familiar. O diagnóstico é realizado pelas características clínicas: lesão cística tensa, abaixo do tendão cantal medial, de coloração azulacinzentada, rósea ou vermelha acompanhada por epífora desde o nascimento. No entanto podemos utilizar exames de imagem para diagnosticar esta anomalia congênita tais como: tomografia computadorizada, ressonância magnética e ultrassonografia.

Obstrução dos ductos lacrimais/congênito; Obstrução dos ductos lacrimais/diagnóstico; Obstrução dos ductos lacrimais/terapia; Obstrução dos ductos lacrimais/ultrassonografia; Cisto epidérmico; Cisto dermóide; Relatos de casos


The dacryocystocele represents a rare congenital anomaly in the medial region of the orbit caused by distal obstruction (Hasner valve) and proximal (valve Rosenmüller) of the lacrimal system causing dilation of the lacrimal sac. Mucocele is called when the content is mucus and amniocele when the content is filled with amniótico fluid. The incidence is only 0.1% in children with nasolacrimal duct obstruction. It is commonly unilateral and more frequent in women with familial predisposition. The diagnosis is made by clinical features: tense cystic lesion below the medial canthal tendon, blue-gray, pink or red color with epiphora since birth. However we can use image tests to diagnose this congenital anomaly such as tomography computerized, magnetic resonance and ultrasonography.


INTRODUCTION

Dacryocystocele is a rare congenital anomaly of the medial region of the orbit(1Wong RK, Vander Veen DK. Presentation and management of congenital dacryocystocele. Pediatrics. 2008;122(5):e1108-12.) caused by distal (at the level of the valve of Hasner) and proximal (at the level of the valve of Rosenmüller) obstruction of the lacrimal system, with subsequent dilation of the lacrimal sac. It is called mucocele when its contents are mucus and amniocele when it is filled with amniotic fluid. The condition is present in only 0.1% of children with nasolacrimal duct obstruction; it is usually unilateral, it has a familial predisposition, and it is more frequent in females.(1Wong RK, Vander Veen DK. Presentation and management of congenital dacryocystocele. Pediatrics. 2008;122(5):e1108-12.

Greenlaw SM, Chaney KS, Belazarian L,Wiss K. Congenital dacryocystocele. J Am Acad Dermatol. 2009;61(6):1088-90.
-3Wang JC, Cunningham MJ. Congenital dacryocystocele: is there a familial predisposition? Int J Pediatr Otorhinolaryngol. 2011;75(3):430-2.)

Diagnosis is based on clinical features,(4Cavazza S, Laffi GL, Lodi L, Tassinari G, Dall'Olio D. Congenital dacryocystocele: diagnosis and treatment.Acta Otorhinolaryngol Ital. 2008;28(6):298-301.,5Shekunov J, Griepentrog GJ, Diehl NN, Mohney BG. Prevalence and clinical characteristics of congenital dacryocystocele. J AAPOS. 2010;14(5):417-20.) which include a tense cystic lesion below the medial canthal tendon with a bluegrayish, pink, or red colour accompanied by epiphora since birth. However, imaging studies such as computed tomography, magnetic resonance imaging, and ultrasound imaging can be used to diagnose the condition.(6Lelli GJ, Levy RL. Epidermoid cyst masquerading as dacryocystocele: case report and review. Orbit. 2011;30(2):114-5.

Ha YJ, Choi HY, Myung KB, Choi YW.A case of congenital dacryocystocele. Ann Dermatol. 2010;22(1):54-6.

Yazici Z, Kline-Fath BM,Yazici B, Rubio EI, Calvo-Garcia MA, Linam LE. Congenital dacryocystocele: prenatal MRI findings. Pediatr Radiol. 2010;40(12):1868-73.
-9Matsuno S,Takagi I. Congenital dacryocystocele with significant enlargement of the nasolacrimal duct diagnosed with computed tomography dacryocystography. J Pediatr Ophthalmol Strabismus. 2010;47(3):183-6.)

The cyst is clearly visible on prenatal ultrasound scans(1010 Malpas T, Nelson F, MacLachlan N. Prenatal diagnosis of dacryocystocele. Prenat Diagn. 2009;29(5):546.,1111 Lembet A, Bodur H, Selam B, Ergin T. Prenatal two- and three-dimensional sonographic diagnosis of dacryocystocele. Prenat Diagn. 2008;28(6):554-5.)as an anechoic collection of fluid in the medial edge of the orbital cavity that does not communicate with the skull or the eye globe. It is observed in the last trimester of pregnancy. Spontaneous resolution occurs in 50% of cases before birth. Postnatal complications include epiphora, dacryocystitis, conjunctivitis, cellulitis, and respiratory distress.(1212 Fussell JN, Wilson T, Pride H. Case report: Congenital dacryocystocele and dacryocystitis. Pediatr Dermatol. 2011;28(1):70-2.

13 Narioka J, Ohashi Y. Dacryocystography with nasolacrimal probing under fluoroscopic guidance for treatment of congenital dacryocystocele. J AAPOS. 2008;12(3):299-301.
-1414 Lin IS, Nar MK, Kua KE, Lin SL. Congenital dacryocystocele with acute dacryocystitis: report of two cases. Acta Paediatr Taiwan. 2006;47(1):38-42.) The differential diagnosis includes anterior or posterior meningoencephalocele, haemangioma, epidermoid cyst, dermoid cyst, nasal glioma, and lymphangioma.

There is no consensus on the treatment of congenital dacryocystocele.(1515 Becker BB. The treatment of congenital dacryocystocele. Am J Ophthalmol. 2006;142(5):835-8.,1616 Hupin C, Lévèque N, Eloy P, Bertrand B, Rombaux P. Congenital dacryocystocele: five clinical cases. B-ENT. 2008;4(3):141-5.) It can be initially treated with Crigler massage,(1717 Guez A, Dureau P. [Diagnosis and treatment of tearing in infancy]. Arch Pediatr. 2009;16(5):496-9. French.) topical and systemic antibiotics, and warm compresses. Because dacryocystocele is highly susceptible to infection, antibiotic prophylaxis is indicated. If conservative treatment is ineffective after a few weeks, lacrimal probing is conducted. If initial probing is ineffective, silicone intubation, balloon dacryocystoplasty, or surgical marsupialisation of the nasolacrimal cyst should be performed.

Ophthalmologists should be aware of the symptoms of nasal obstruction leading to respiratory distress, which can be lifethreatening. In such cases, endoscopic marsupialisation of the nasolacrimal cyst is recommended. In cases of dacryocystocele progressing to acute dacryocystitis, systemic antibiotic therapy is indicated to prevent serious complications such as meningitis, brain abscess, and sepsis.

CASE REPORT

White female child, born and raised in São Paulo, seen at the Lacrimal Apparatus Unit of UNIFESP from the 7th day of life with congenital dacryocystocele on the right side (Figure 1). Ophthalmic examination (inspection and palpation) showed a blue-grayish tense cystic lesion below the medial canthal tendon accompanied by epiphora since birth. Biomicroscopy showed no mucopurulent discharge or bulbar conjunctival hyperaemia in the right eye; the Milder test was intensely positive. The fluorescein appearance test in the oropharynx was negative. Eye ultrasound (Figure 2) found a round, well-defined preseptal orbital lesion in the medial canthus with a hypoechoic content.The lesion measured 3.1 × 3.6 × 2.9 mm (depth × height × width). There was no evidence of posterior extension or communication with the eye globe. The features of the lesion were compatible with dacryocystocele.

Figure 1
Child with congenital dacryocystocele on the right side.
Figure 2
Right eye ultrasound.

The child received conservative treatment with Crigler massage, warm compresses, and systemic antibiotics (cephalexin suspension 250 mg/5 ml, 2 ml every 6 hours for 7 days). The condition resolved after 4 weeks.

DISCUSSION

Dacryocystocele is caused by an accumulation of amniotic fluid in the lacrimal sac (amniocele), which becomes dilated and clearly visible on prenatal ultrasound scans in the last trimester of pregnancy.The lesion appears as an anechoic, fluid-filled cystic area on the internal edge the orbital cavity.(1818 Jones LT, Wolbig JL. Surgery of the eyelids and lacrimal system. Birmingham, AL: Aesculapius; 1976. p.162.) However, the amniotic fluid by itself is not able to dilate the lacrimal sac, especially when the bulging occurs days after birth. The term mucocele is used when the lacrimal sac is filled with mucus. Dilation of the lacrimal sac is more likely caused by a combination of mucus, amniotic fluid, lacrimal fluid, and bacterial proliferation, but its contents are often sterile.(1919 Grin TR, Mertz JS, Stass-Isern M. Congenital nasolacrimal duct cysts in dacryocele. Ophthalmology.1991;98(8):1238-42.)

In newborns, dacryocystocele is diagnosed clinically. Diagnostic tests such as transillumination, ultrasound, computed tomography, magnetic resonance imaging, dacryocystography, and rhinoscopy may be required to clarify the diagnosis. MRI can help characterise the contents of the lesion, while TC is used to visualise bone anomalies affecting the nasolacrimal duct.(2020 Shashy RG, Durairaj VD, Holmes JM, Hohberger GG,Thompson DM, Kasperbauer JL. Congenital dacryocystocele associated with intranasal cysts: diagnosis and management. Laryngoscope. 2003;113(1):37-40. Erratum in Laryngoscope. 2005;115(4):759. Durairaj,Vikram [corrected to Durairaj,Vikram D].)

Prenatal visualisation of dacryocystocele by Doppler ultrasound helps identify associated malformations.(2121 Sharony R, Raz J, Aviram R, Cohen I, Beyth Y, Tepper R. Prenatal diagnosis of dacryocystocele: a possible marker for syndromes. Ultrasound Obstet Gynecol. 1999;14(1):71-3.)

Cystic extension of dacryocystocele into the nasal cavity is not uncommon and can cause respiratory difficulty during sleep and nursing in bilateral cases. Therefore, all children with congenital dacryocystocele should undergo rhinoscopy performed by an otolaryngologist to exclude an associated intranasal cyst.(2222 Mansour AM, Cheng KP, Mumma JV, Stager DR, Harris GJ, Patrinely JR, et al. Congenital dacryocele: a collaborative review. Ophthalmology.1991;98(11):1744-51.)

There is no consensus on the treatment of dacryocystocele. Conservative treatment is initially recommended (lacrimal sac massage, topical and/or systemic antibiotic therapy) during the first months of life.(2323 Young JD, MacEwen CJ. Managing congenital lacrimal obstruction in general practice. BMJ. 1997;315(7103):293-6.

24 Schnall BM, Christian CJ. Conservative treatment of congenital dacryocele. J Pediatr Ophthalmol Strabismus. 1996;33(5):219-22.
-2525 Sullivan TJ, Clarke MP, Morin JD, Pashby RC. Management of congenital dacryocystocele. Aust N Z J Ophthalmol. 1992;20(2):105-8.) Lacrimal probing is recommended when conservative treatment is ineffective or when the child presents severe infection or respiratory distress.(2626 Paysse EA, Coats DK, Bernstein JM, Go C, de Jong AL. Management and complications of congenital dacryocele with concurrent intranasal mucocele. J AAPOS. 2000;4(1):46-53.,2727 Harris GJ, DiClementi D. Congenital dacryocystocele. Arch Ophthalmol. 1982;100(11):1763-5.) According to the literature, early probing can prevent infections and sequelae (tissue distortion in the inner canthus, induction of corneal astigmatism, and anisometropic amblyopia).(2828 Campolattaro BN, Lueder GT, Tychsen L. Spectrum of pediatric dacryocystitis: medical and surgical management in 54 cases. J Pediatr Ophthalmol Strabismus.1997;34(3):143-53; quiz 186-7.)

In the presence of an intranasal cyst, the child can be referred to an otolaryngologist for cyst marsupialisation.(2929 Lueder G. Endoscopic treatment of intranasal abnormalities associated with nasolacrimal duct obstruction. J AAPOS. 2004;8(2):128-32.,3030 Black M, Chatrath P,Jan W, Cox T. Case of the month. Eyes wide apart! Br J Radiol. 2001;74(877):103-4.)

  • Work conducted at the Lacrimal Apparatus Unit, Federal University of São Paulo (UNIFESP), São Paulo/SP, Brazil.

References

  • 1
    Wong RK, Vander Veen DK. Presentation and management of congenital dacryocystocele. Pediatrics. 2008;122(5):e1108-12.
  • 2
    Greenlaw SM, Chaney KS, Belazarian L,Wiss K. Congenital dacryocystocele. J Am Acad Dermatol. 2009;61(6):1088-90.
  • 3
    Wang JC, Cunningham MJ. Congenital dacryocystocele: is there a familial predisposition? Int J Pediatr Otorhinolaryngol. 2011;75(3):430-2.
  • 4
    Cavazza S, Laffi GL, Lodi L, Tassinari G, Dall'Olio D. Congenital dacryocystocele: diagnosis and treatment.Acta Otorhinolaryngol Ital. 2008;28(6):298-301.
  • 5
    Shekunov J, Griepentrog GJ, Diehl NN, Mohney BG. Prevalence and clinical characteristics of congenital dacryocystocele. J AAPOS. 2010;14(5):417-20.
  • 6
    Lelli GJ, Levy RL. Epidermoid cyst masquerading as dacryocystocele: case report and review. Orbit. 2011;30(2):114-5.
  • 7
    Ha YJ, Choi HY, Myung KB, Choi YW.A case of congenital dacryocystocele. Ann Dermatol. 2010;22(1):54-6.
  • 8
    Yazici Z, Kline-Fath BM,Yazici B, Rubio EI, Calvo-Garcia MA, Linam LE. Congenital dacryocystocele: prenatal MRI findings. Pediatr Radiol. 2010;40(12):1868-73.
  • 9
    Matsuno S,Takagi I. Congenital dacryocystocele with significant enlargement of the nasolacrimal duct diagnosed with computed tomography dacryocystography. J Pediatr Ophthalmol Strabismus. 2010;47(3):183-6.
  • 10
    Malpas T, Nelson F, MacLachlan N. Prenatal diagnosis of dacryocystocele. Prenat Diagn. 2009;29(5):546.
  • 11
    Lembet A, Bodur H, Selam B, Ergin T. Prenatal two- and three-dimensional sonographic diagnosis of dacryocystocele. Prenat Diagn. 2008;28(6):554-5.
  • 12
    Fussell JN, Wilson T, Pride H. Case report: Congenital dacryocystocele and dacryocystitis. Pediatr Dermatol. 2011;28(1):70-2.
  • 13
    Narioka J, Ohashi Y. Dacryocystography with nasolacrimal probing under fluoroscopic guidance for treatment of congenital dacryocystocele. J AAPOS. 2008;12(3):299-301.
  • 14
    Lin IS, Nar MK, Kua KE, Lin SL. Congenital dacryocystocele with acute dacryocystitis: report of two cases. Acta Paediatr Taiwan. 2006;47(1):38-42.
  • 15
    Becker BB. The treatment of congenital dacryocystocele. Am J Ophthalmol. 2006;142(5):835-8.
  • 16
    Hupin C, Lévèque N, Eloy P, Bertrand B, Rombaux P. Congenital dacryocystocele: five clinical cases. B-ENT. 2008;4(3):141-5.
  • 17
    Guez A, Dureau P. [Diagnosis and treatment of tearing in infancy]. Arch Pediatr. 2009;16(5):496-9. French.
  • 18
    Jones LT, Wolbig JL. Surgery of the eyelids and lacrimal system. Birmingham, AL: Aesculapius; 1976. p.162.
  • 19
    Grin TR, Mertz JS, Stass-Isern M. Congenital nasolacrimal duct cysts in dacryocele. Ophthalmology.1991;98(8):1238-42.
  • 20
    Shashy RG, Durairaj VD, Holmes JM, Hohberger GG,Thompson DM, Kasperbauer JL. Congenital dacryocystocele associated with intranasal cysts: diagnosis and management. Laryngoscope. 2003;113(1):37-40. Erratum in Laryngoscope. 2005;115(4):759. Durairaj,Vikram [corrected to Durairaj,Vikram D].
  • 21
    Sharony R, Raz J, Aviram R, Cohen I, Beyth Y, Tepper R. Prenatal diagnosis of dacryocystocele: a possible marker for syndromes. Ultrasound Obstet Gynecol. 1999;14(1):71-3.
  • 22
    Mansour AM, Cheng KP, Mumma JV, Stager DR, Harris GJ, Patrinely JR, et al. Congenital dacryocele: a collaborative review. Ophthalmology.1991;98(11):1744-51.
  • 23
    Young JD, MacEwen CJ. Managing congenital lacrimal obstruction in general practice. BMJ. 1997;315(7103):293-6.
  • 24
    Schnall BM, Christian CJ. Conservative treatment of congenital dacryocele. J Pediatr Ophthalmol Strabismus. 1996;33(5):219-22.
  • 25
    Sullivan TJ, Clarke MP, Morin JD, Pashby RC. Management of congenital dacryocystocele. Aust N Z J Ophthalmol. 1992;20(2):105-8.
  • 26
    Paysse EA, Coats DK, Bernstein JM, Go C, de Jong AL. Management and complications of congenital dacryocele with concurrent intranasal mucocele. J AAPOS. 2000;4(1):46-53.
  • 27
    Harris GJ, DiClementi D. Congenital dacryocystocele. Arch Ophthalmol. 1982;100(11):1763-5.
  • 28
    Campolattaro BN, Lueder GT, Tychsen L. Spectrum of pediatric dacryocystitis: medical and surgical management in 54 cases. J Pediatr Ophthalmol Strabismus.1997;34(3):143-53; quiz 186-7.
  • 29
    Lueder G. Endoscopic treatment of intranasal abnormalities associated with nasolacrimal duct obstruction. J AAPOS. 2004;8(2):128-32.
  • 30
    Black M, Chatrath P,Jan W, Cox T. Case of the month. Eyes wide apart! Br J Radiol. 2001;74(877):103-4.

Publication Dates

  • Publication in this collection
    Jul-Aug 2014

History

  • Received
    17 Oct 2011
  • Accepted
    02 Mar 2012
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