OBJECTIVE. Our goal was to report the presentation, complications, and treatment strategies for infants with congenital dacryocystocele.
PATIENTS AND METHODS. We performed a retrospective chart review of all patients presenting with dacryocystoceles to Children's Hospital Boston between the years of 1997 and 2006. This study was approved by the institutional review board.
RESULTS. Forty-two patients (46 eyes) were identified (18 male, 24 female), presenting at a median age of 7 days of life. Twenty-eight (65%) patients presented with cellulitis or dacryocystitis and required systemic antibiotics. Four (9.5%) patients presented with respiratory compromise. Resolution occurred with conservative treatment for 10 eyes, but 36 (78%) required surgical intervention. Seventeen (37%) eyes received probing in the office or nursery, and 13 (76%) were successful. Probing in the operating room was required for 21 patients, 8 of whom required marsupialization of an intranasal cyst.
CONCLUSIONS. Although congenital dacryocystoceles may resolve with conservative measures, many become infected and require systemic antibiotic treatment, and most require surgical intervention. Referral in the early neonatal period can aid in timely intervention before complications such as infection occur.
Congenital dacryocystoceles (mucocele, amniotocele) are a relatively rare variant of nasolacrimal duct obstruction (NLDO), accounting for only 0.1% of infants with congenital NLDO.1 Infants with any lacrimal anomaly present to the pediatrician first, so an awareness of this condition and its potential complications is important. Typical NLDO presents more often in the first weeks or months of life with symptoms of excess tearing and/or discharge. A bluish, cystic, firm mass below the medial canthus (the angle formed by the union of the upper and lower eyelids medially) presenting shortly after birth is the classic presentation for a dacryocystocele.2–4 The anatomic anomaly responsible for the 2 conditions differ. NLDO is usually a result of a blockage at the distal end of the nasolacrimal system at the valve of Hasner, although blockage may also occur at the level of the puncta. Dacryocystoceles occur when the mesoderm fails to canalize distally, and along with the distal obstruction there is a functional or mechanical proximal obstruction.5 The result is distension of the lacrimal sac below the medial canthal tendon, which may appear as a mass that causes an upward slanting of the palpebral fissure nasally3,6 (Fig 1), or the lacrimal sac contents may cause a cystic expansion into the nose (Fig 2). Encephaloceles, hemangiomas, dermoids, and nasal gliomas can also present similarly and must be considered in the differential diagnosis. Patients with dacryocystoceles can also present with infection, difficulty breathing, or difficulty with breastfeeding on the mother's breast ipsilateral to the dacryocystocele. It is important for the pediatrician to correctly identify congenital dacryocystoceles and appropriately refer the infant to a pediatric ophthalmologist, because life-threatening complications can arise.
Conservative management of dacryocystoceles includes the use of gentle pressure over the lacrimal sac, which can facilitate decompression and drainage of the contents into the nose. Antibiotic drops may also be used prophylactically before infection occurs. The resolution rate after a short course of topical antibiotics, warm compresses, and massage has been reported to be 76%.4,7 However, dacryocystitis can develop within a few days or weeks and requires intravenous antibiotics to prevent life-threatening sepsis. Another serious complication of dacryocystocele is respiratory compromise, which occurs when there is a large intranasal cystic component causing blockage of the nasal cavity.4,8–10 Infants may present with grunting or labored breathing, and radiologic imaging, if performed, will reveal the intranasal cysts.11 Large intranasal cysts can sometimes also be visualized with nasal endoscopy, which is a valuable adjunct to the treatment plan for dacryocystoceles10–12 (Fig 2). In cases of infection or respiratory compromise, probing is necessary.
Probing and irrigation of the nasolacrimal system has been shown to effectively ameliorate the obstructions present in dacryocystoceles, although marsupialization of the cyst wall is often necessary for those with an intranasal component.13 Probing consists of inserting a metal probe via the punctum and canalicular system and passing it down through the nasolacrimal sac past the distal obstruction. Probing can be performed in the office, eliminating the need for general anesthesia,2–4 although for cases complicated by intranasal cysts or infection, the probing is often performed in the operating room.4,5 When dacryocystitis occurs, probing of the nasolacrimal system is often delayed 24 to 48 hours for systemic antibiotic therapy to take effect. Careful probing with a fine (#00-0000) Bowman probe can permit evacuation of the sac with minimal risk of creating a false passage.14
Because significant controversy exists as to the preferred management and timing of intervention for infants with congenital dacryocystoceles, we undertook this study to assess complication rates and the types of intervention required for resolution in our institution.
A retrospective chart review of all patients diagnosed with a congenital dacryocystocele between the years of 1997 and 2006 at Children's Hospital Boston and seen by a senior member of the Department of Ophthalmology was conducted. This study was approved by the institutional review board at Children's Hospital Boston. No patients were excluded from the study. The following characteristics were recorded for each patient: age and symptoms at presentation, gender, laterality, age at treatment (if one was initiated), treatment type, and rate of infection or other complication. Treatment modalities used consisted of topical antibiotic, massage, intravenous antibiotic, probing (in clinic or operating room), and marsupialization of an intranasal cyst. The location of treatment was also recorded (clinic or operating room).
Forty-two patients (46 eyes) were identified (18 male, 24 female), presenting at a median age of 7 days of life. Twenty-five patients were referred directly to ophthalmology, 16 were admitted to the hospital and ophthalmology consulted, and 1 was referred initially to otolaryngology. Thirty eyes of 28 patients presented with either cellulitis or dacryocystitis, and these patients required systemic antibiotic treatment. Four patients presented with respiratory compromise (3 of whom had radiologically or endoscopically confirmed intranasal cysts). Two of these patients had bilateral dacryocystoceles, and the other 2 had large unilateral intranasal cysts. An additional 8 patients also had confirmed intranasal cysts yet only presented with signs of infection or were asymptomatic.
Treatment modalities are summarized in Table 1. Resolution occurred without surgical intervention in 10 (22%) eyes. These patients had been scheduled for surgery and their conditions resolved in the interim with massage alone, or they had been started on warm compresses and massage by their pediatricians before referral to our department. Of the 36 eyes that had surgical intervention, 17 eyes received probing in the office, and 13 (76%) were successful. Twenty-three eyes required probing in the operating room (including the 4 that had failed an office probing), and 8 of these underwent marsupialization of an intranasal cyst. None of these patients experienced recurrence. Two patients were diagnosed with dacryocystoceles prenatally (Fig 3), so treatment was provided shortly after birth.
Dacryocystoceles were initially thought to occur as a result of amniotic fluid trapped within the lacrimal system in utero, thus giving rise to the term amniotocele.15 However, amniotic fluid alone cannot account for lacrimal sac distention, especially when it is noted days after birth. Some literature uses the term “mucocele,” implying that mucus created by the lacrimal sac glands is responsible for the accumulation of fluid in the lacrimal sac. It is more likely that a combination of dissolved mesoderm, mucus, amniotic fluid, tears, and bacterial colonization comprise the lacrimal sac contents and cause the distension of the lacrimal system seen in dacryocystoceles.
The course, appropriate timing, and management of congenital dacryocystoceles vary greatly in the ophthalmic and pediatric literature. In 1978, Petersen and Robb16 presented 7 cases of congenital dacryocystoceles within their larger study of 50 patients with NLDOs. Five of the 7 patients underwent probing and irrigation to decompress the lacrimal sac, 3 required repeat probing because of recurrence, and 1 patient who was not initially treated developed an abscess, necessitating systemic antibiotics and incision and drainage of the sac. The authors advocated early surgical decompression of the sac in cases of congenital dacryocystoceles.
In contrast, in a 3½-year prospective study, Schnall and Christian7 concluded that medical management of congenital dacryocystoceles can also be effective. In their series, 17 patients (all <4 weeks of age) with 21 dacryocystoceles were examined. The authors advocated a conservative approach to noninfected dacryocystoceles and found that 16 dacryocystoceles (76%) resolved with medical management consisting of antibiotic drops, warm compresses, and massage 3 times per day. Probing was performed only if medical management failed after 2 weeks of treatment. Infected dacryocystoceles were treated with intravenous antibiotics, warm compresses, and massage. Our study, however, shows that a good number of dacryocystoceles do become infected early, necessitating intervention.
At our center, only 12 patients were asymptomatic at presentation (Fig 4), whereas 26 patients required systemic antibiotics for infection, and 4 had respiratory compromise. The patients with dacryocystitis or cellulitis presented at a mean age of 7 days of life. Ten eyes did resolve with massage and antibiotics, which supports the argument that uncomplicated dacryocystoceles can be treated with conservative measures for 1 to 2 weeks. Still, 8 of these 10 eyes developed signs of infection in the interim and were treated with systemic antibiotics. The higher rate of infection may be a reflection of referral to our large tertiary care center.
Most ophthalmologists recommend early surgical intervention in cases of respiratory compromise, dacryocystitis, cellulitis, large dacryocystoceles inducing large amounts of astigmatism, or recurrent dacryocystoceles and for cases in which conservative measures have failed.17,18 A retrospective review of the management and natural history of congenital dacryocystoceles gathered from 7 tertiary care medical centers reported that surgical intervention was performed in 83% of the patients, whereas spontaneous resolution occurred before surgery in 17% of them.3 However, a majority of the physicians who participated in the study opted to treat dacryocystoceles as a surgical disorder, probing the lacrimal system at or shortly after presentation. This particular study found nasal cysts definitively in 6 of 54 cases.3 In addition, a large female preponderance was noted and thought to be secondary to the more narrow nasolacrimal duct in girls than boys.3,19 We also found that more girls than boys were affected.
The general approach in our department, after attempts at massage and manual decompression, is to plan treatment to relieve the blockage as soon as the dacryocystocele is identified to prevent future complications (Fig 5). All cases of infection were treated medically before surgical intervention. Although some staff prefer to probe in the office or nursery, some prefer planned probing in the operating room. Thirty-six eyes required surgical intervention, and the mean timing of the probing was 11 days after presentation. No surgical complications were noted. The success rate of probings for dacryocystoceles not complicated by intranasal cysts was 100% for both the office and operating room procedures. The only “failed” operating room/office probings were a result of undiagnosed intranasal cysts. Six eyes were referred by outside pediatric ophthalmologists who had unsuccessfully repeated probing without radiologic imaging. Careful diagnosis and appropriate imaging could have prevented unnecessary probings for these infants and can direct care toward a more definitive cure (marsupialization). One patient, who had partial resolution with conservative treatment, later developed recurrent dacryocystitis and had probe with silicone intubation of the lacrimal system at 2 years of age.
An intranasal cyst can complicate dacryocystoceles, both in presentation and treatment. Sometimes symptoms of respiratory distress during feeding and sleeping secondary to the nasal obstruction are noted.3,11 These patients occasionally do not show the typical external enlargement of the lacrimal sac, because most of the distension is in the nasal cavity. If the intranasal component of the cyst is not recognized, the probing will fail if the probe is not passed beyond the wall of the cyst. Although a simple probing can open and collapse a small or thin-walled cyst, in our series, marsupialization of an intranasal cyst was necessary for 8 patients in the operating room. Each of these patients underwent multiple (1–3) unsuccessful attempts at probing before a computed tomography scan or endoscopy identified the intranasal cyst, which lead to the more definitive treatment of removal of the cyst wall. Earlier identification and appropriate radiologic imaging of patients who present with symptoms of respiratory compromise or recurrence of dacryocystoceles after probing could limit unsuccessful interventions.
Although some studies have reported a high resolution rate of dacryocystoceles with medical management, we found a significant infection rate (65%) and that respiratory compromise (9.5%) is not uncommon. Pediatricians should be aware of these complications and make an early referral to an ophthalmologist for timely treatment. We advocate early surgical intervention for all dacryocystoceles and nasal endoscopy and/or imaging in cases that are complicated by respiratory compromise.
- Accepted July 24, 2008.
- Address correspondence to Rupa Krishnamurthy Wong, MD, Children's Hospital Boston, Department of Ophthalmology, Fegan 4, 300 Longwood Ave, Boston, MA 02115. E-mail: or
The authors have indicated they have no financial relationships relevant to this article to disclose.
What's Known on This Subject
Currently, there is still debate about the best management of congenital dacryocystoceles.
What This Study Adds
Our study demonstrates that surgical intervention, not medical management, is the best treatment option for patients with congenital dacryocystoceles.
- ↵Katowitz JA, Low JE, Covici SJ, Goldstein JB. Ch. 18: Management of pediatric lower system problems: proving and silastic intubation. In: Katowitz W, ed. Pediatric Oculoplastic Surgery. New York, NY: Springer; 2002:309– 324
- ↵Jones LT, Wolbig JL. In: Surgery of the Eyelids and Lacrimal System. Birmingham, AL: Aesculapius; 1976:162
- ↵Duke-Elder SS, ed. System of Ophthalmology. Vol III: Normal and Abnormal Development. Pt 2: Congenital Deformities. St Louis, MO: CV Mosby; 1964:934– 941
- Copyright © 2008 by the American Academy of Pediatrics