Congenital Nasolacrimal Duct Cysts in Dacryocystocele TRUDI R. GRIN, MD;,2 JANET S. MERTZ, MD/ MERRILL STASS-ISERN, MDt

Abstract: Cystic intranasal masses were found in seven newborn infants with congenital dacryocystocele. The cysts were a direct extension of the nasolac­rimal duct, located beneath the inferior turbinate. Nasal obstruction was present in three infants and was severe enough to cause respiratory distress in two neonates with bilateral cysts. Using fiberoptic nasal endoscopy, treatment con­sisting of probing, irrigation, and marsupialization of the cysts was successful in all infants. The authors believe this rarely reported anomaly is a more common finding in dacryocystocele than previously recognized and advocate careful nasal examination in all infants with congenital dacryocystocele. Early diagnosis and treatment of this condition is critical in the infant with nasal obstruction. Ophthalmology 1991; 98:1238-1242 .

Congenital dacryocystocele is an uncommon variant of congenital nasolacrimal duct obstruction. This con­dition, also known as congenital mucocele, amniotocele, and amniocele is characterized by unilateral or bilateral distention of the nasolacrimal sac with a gray-blue cystic swelling just below the medial canthus. The findings are typically seen at birth or develop within the first few weeks of life. A secondary dacryocystitis frequently develops.

Dacryocystocele is believed to occur as a result of a concomitant upper and lower system obstruction, causing fluid accumulation and distention of the sac. An unusual associated anomaly is cystic nasal distension of the na­solacrimal duct. Only two previous case reports of this complication have been reported in the ophthalmic lit­erature. 1

•2 During a 13-month period, we encountered

seven patients with nasolacrimal duct cysts in association with dacryocystocele. Three of the seven patients had sig­nificant nasal obstruction and respiratory distress occurred in two of the infants with bilateral cysts. The implications of these findings will be discussed.

Originally received: November 13. 1990. Revision accepted: February 28, 1991 .

1 Children's Mercy Hospital, Section of Ophthalmology, Kansas City. 2 University of Missouri-Kansas City, School of Medicine. Department of

Ophthalmology. Kansas City. 3 Children 's Mercy Hospital, Section of Otolaryngology.

Presented in part as a poster at the American Academy of Ophthalmology Annual Meeting. Atlanta. Ocl/Nov 1990.

Reprint requests to Trudi R. Grin. MD. 10550 Quivira Rd, Suite 335, Overland Park. KS 66215

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CASE REPORTS

The finding of a large nasolacrimal duct cyst in a patient with a typical dacryocystocele (case 1), prompted the authors to eval­uate all patients with congenital dacryocystocele for this anomaly. Between May 1989 and June 1990, all infants presenting with typical dacryocystocele or respiratory distress in association with dacryocystocele underwent nasal examination to exclude nasal cyst(s). Except in those cases with respiratory distress, conser­vative treatment was initially tried and consisted of decompres­sion of the sac with massage. If this was unsuccessful and/or dacryocystitis was present, bilateral flexible fiberoptic or rigid nasal endoscopy and probing under general anesthesia were per­formed. Eight infants with dacryocystocele were seen during that time period, and seven of the eight had nasolacrimal duct cysts.

Case 1. A 5-week-old female infant was hospitalized with fever and increasing edema over the right medial canthus. She had a history of a blue nodule over the right medial canthus and mu­copurulent discharge from the right eye during the first week of life, which had cleared with topical sulfacetamide drops and nasolacrimal massage. Examination was consistent with a right dacryocystocele and dacryocystitis.

A computed tomographic (CT) scan demonstrated a soft tissue mass medial to the right orbit, displacing the globe laterally (Fig I). The nasolacrimal duct was enlarged and a right intranasal soft tissue mass was seen in the area of the inferior turbinate (Fig 2). Nasal examination was performed by an otolaryngologist (JSM), and a large firm, yellow-gray mucosal mass was seen bulging beneath the inferior turbinate. The mass almost com­pletely obstructed the right nasal passage.

Despite treatment with intravenous antibiotics and de­compression, swelling over the right medial canthus recurred. Five days after the initial examination, the patient underwent nasal endoscopy and probing under general anesthesia. A 00

GRIN et al • CYSTIC INTRANASAL MASSES

Fig 1. Case 1. CT scan demonstrates soft tissue mass medial to right globe (arrow), displacing globe laterally.

Fig 2. Case I. Right intranasal soft tissue mass (arrow) adjacent to inferior turbinate.

Bowman probe was easily passed through the nasolacrimal sys­tem into the nasal cyst. The cyst could not be punctured by the probe tip. Under direct visualization, the cyst was incised with a blade and marsupialized with small front biting forceps. After

Fig 3. Case 1. Specimen from cyst wall demonstrates mucous membrane covered with pseudostratified epithelium. The submucosa is fibrous and infiltrated with mononuclear leukocytes (hematoxylin-eosin; original magnification, XI 00).

excision of the cyst, the probe tip was visualized at the opening of the nasolacrimal duct.

Results of pathologic examination of the cyst showed mucous membrane covered by pseudostratified ciliated columnar epi­thelium and chronic inflammation within the submucosa (Fig 3). Postoperatively, the swelling completely resolved and there has been no recurrence of the dacryocystocele or nasal cyst.

Case 2. An 8-day-old female infant presented with increasing erythema and induration of a left dacryocystocele. The CT scan showed a 1 cm left intranasal mass displacing the inferior tur­binate medially and extending to the hard palate. The infant was treated with intravenous antibiotics and the dacryocystocele was decompressed with massage, but it recurred. The child was taken to surgery and underwent nasal endoscopy, probing, and marsupialization of the cyst as in case 1. After surgery, her symptoms completely resolved.

Case 3. A 3-day-old male infant was transferred to Children's Mercy Hospital for evaluation of upper airway obstruction and a right medial canthal mass. Tachypnea, snorting respirations, and feeding difficulty were present since birth. On examination, a 1 cm in diameter blue nodule inferior to the right medial canthus was noted (Fig 4). Clear mucoid material was expressed with pressure on the nodule. Results of endoscopy of the na­sopharynx showed a large nasal cyst in both nares beneath the inferior turbinate. Bilateral probing and marsupialization of the cysts were performed and, postoperatively, his respiratory symptoms and feeding difficulty completely resolved.

Case 4. A 3-week-old male infant was hospitalized with group B Streptococcal bacteremia. He had a history of epiphora, mu­copurulent drainage from the right eye, and stuffiness of the right nares since birth. An ophthalmologic consultation was ob­tained because of increasing edema and erythema overthe right nasolacrimal sac. Examination was consistent with a right dac­ryocystocele and dacryocystitis. The sac was decompressed with massage but the swelling recurred. The child was taken to surgery where nasal endoscopy confirmed a right nasal mucosal cyst. Probing and marsupialization were performed followed by complete resolution of the infant's symptoms.

Case 5. A 7-day-old female infant was referred to Children's Mercy Hospital with upper airway obstruction. Snorting respi­rations and feeding difficulty had been present since birth. A dacryocystocele was clinically evident on the right side only. Results of endoscopy of the nasopharynx and oropharynx

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showed bilateral nasal mucosal cysts under the inferior turbinates (Fig 5). Bilateral nasolacrimal duct probing and marsupialization of the cysts were performed, and, postoperatively, her symptoms completely resolved.

Case 6. A 7-day-old female presented with a right dacryocys­tocele, fever, and purulent discharge from the right eye. She was hospitalized and treated with intravenous antibiotics. At 10 days of age, she underwent nasal endoscopy and probing. A right nasolacrimal duct cyst was found beneath the inferior turbinate and was widely marsupialized. After treatment, the discharge and swelling resolved.

Case 7. An 8-day-old female twin infant presented with a right dacryocystocele and dacryocystitis. The patient was placed on oral cefaclor and tobramycin drops. The following day, nasal endoscopy and nasolacrimal duct probing were performed. A large right nasal cyst was seen emerging beneath the inferior turbinate and was marsupialized. The infant has remained asymptomatic since treatment.

RESULTS

A summary of the case reports is presented in Table 1.

DISCUSSION

Only two previous cases of nasal cysts in association with dacryocystocele have been published in the ophthalmic literature. In 1982, Raflo et al2 reported on a 2-week-old female with a right dacryocystocele and a large intranasal cyst totally occluding the right nares. The find­ings on CT scan were almost identical to those in case 1. The cyst was collapsed in the nose with a single incision in the cyst wall but the mucocele recurred and wide mar­supialization of the cyst was required.

A case of bilateral mucoceles with bilateral nasal ex­tension in a healthy female newborn was reported by Di­vine et al. I The infant had no apparent respiratory distress according to the case report. Two probings were unsuc­cessful in eliminating her mucocele, and nasal examina­tion after the second probing disclosed bilateral nasal cysts. The patient was treated with intranasal drainage of the mucocele by needle aspiration followed by wide marsu­pialization of the nasal masses.

In our series of eight infants with dacryocystocele ex­amined during a 13-month period, nasal cysts were dis­covered by nasal endoscopy in seven patients. This leads the authors to conclude that cystic distention of the na­solacrimal duct is a more common finding in dacryocys­tocele than previously recognized. One may postulate that cases of recurrent dacryocystocele reported in the literature may have had unrecognized nasolacrimal duct cysts.

Recurrence rates of treated dacryocystocele are high when compared with the high rate of success of treated nasolacrimal duct obstruction. Weinstein et at3 reported on seven patients with congenital lacrimal sac mucocele. Six of the seven required probing, and a recurrence de­veloped in two patients (33%), necessitating additional

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Fig 4. Case 3. Tense, blue-gray swelling of right lacrimal sac.

Fig S. Case 5. Right nasolacrimal duct cyst (arrow) beneath inferior turbinate as seen on nasal endoscopy. Asterisk marks inferior turbinate.

probing. Petersen and Robb4 probed five of seven un­complicated mucoceles, but three (43%) required addi­tional probing because of recurrence. Two of seven (29%) lacrimal abscesses reported by Ffooks5 also recurred and additional probing was needed. In these cases, if an un­recognized cyst was present, the first probing might have perforated the cyst wall and achieved temporary drainage but the opening sealed, causing a recurrence of the dac­ryocystocele. In our series, all seven patients with dacryo­cystocele were successfully treated with a single probing and marsupiaiization of the nasal cyst(s).

Fiberoptic nasal endoscopy proved to be a valuable di­agnostic and therapeutic aid in our series of patients pre­sented. Although nasal endoscopy was probably first per­formed by Hirschmann in 1901 using a cystoscope, it was not until 1978 that Messerklinger documented his en­doscopic nasal findings in the English-language literature.6

GRIN et al • CYSTIC INTRANASAL MASSES

Table 1. Summary of Case Reports

Case Unilateral/Bilateral Unilateral/Bilateral Respiratory Results of Follow-up No. Sex Dacyrocystocele Nasal Cysts Distress Treatment (mos)

1 F Unilateral Unilateral No Cured* 19 2 F Unilateral Unilateral No Cured 15 3 M Unilateral Bilateral Yes Cured 14 4 M Unilateral Unilateral No Cured 14 5 F Unilateral Bilateral Yes Cured 10 6 F Unilateral Unilateral No Cured 7 7 F Unilateral Unilateral No Cured 7

* Cured is defined as complete resolution without recurrence of nasolacrimal sac swelling, erythema, epiphora, and respiratory distress (when present).

Nasal Septum

Fig 6. Surgical technique of marsupialization of nasal cysts using fiber­optic nasal endoscopy.

The nasal endoscope, a slender lightweight fiberoptic tele­scope, has recently revised the classic procedure of nasal endoscopy, enhancing visualization and making the nasal anatomy more surgically accessible.

In our series, endoscopy was used concomitantly with nasolacrimal duct probing during treatment. The cysts could be visualized directly in the location of the naso­lacrimal duct, under the inferior turbinate (Fig 6). Break­ing the cyst wall with the probe tip was attempted in the first two cases but was unsuccessful, probably due to size and location of the cyst at the nasal floor. With endoscopy, movement of the cyst by the probe tip could be appre­ciated but the nasal floor limited puncture by the probe. After injection of fluorescein solution into the upper puncta, ballooning and discoloration of the cyst wall was seen, confirming that the cysts were located at the distal end of the nasolacrimal system. Additionally, pathologic examination of the cysts performed in all patients dem­onstrated respiratory mucosa. These findings indicate that the cysts most likely originate as a congenital cystic dis-

~ Nasolacrimal Duct

.. Nasolacrimal Duct Cyst

Fig 7. Schematic representation of nasolacrimal duct cyst at distal end of nasolacrimal duct.

tention of the membranous obstruction at the distal na­solacrimal duct (Fig 7). Fluid accumulation with second­ary dacryocystitis may result in increasing size of the cyst and nasal obstruction.

The most important finding in this study was that re­spiratory distress may occur in patients with dacryocys­tocele who have bilateral nasolacrimal duct cysts. In the two patients in our series with respiratory distress, both had unilateral dacryocystocele. Neonates are obligate nasal breathers and nasal obstruction from any cause results in respiratory distress during feeding and sleeping. The dis­tress usually resolves during crying. In our patients, the obstruction completely resolved after excision of the cysts. To the best of our knowledge, this finding has not been previously reported in the ophthalmic literature. Two re­ports of nasal obstruction and nasolacrimal duct cysts have been published in the otolaryngologic literature. Lusk and Muntz 7 reported on two female infants with neonatal up­per airway obstruction. Bilateral dilated lacrimal sacs, dacryocystitis, and nasal cysts were present in one patient,

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and, in the other case, the findings were unilateral. The cyst wall was removed with a CO2 laser in both cases. Four cases of endonasal cysts {;ausing nasal obstruction in newborns were reported by Goralowna and Taranto­wicz.8 Dacryocystitis was present in three of these infants. Probing was performed in one patient and three were treated with nasal puncture of the cyst. In the above re­ports, the terms dacryocystocele or mucocele were not used to describe the eye findings, but review of details in the case reports indicates most of the infants affected probably had congenital dacryocystocele.

Based on our findings, we recommend that nasal ex­amination be performed in all cases of congenital dac­ryocystocele to exclude the coexistence of nasolacrimal duct cysts. If the ophthalmologist does not have adequate clinical expertise with intranasal examination or nasal en­doscopy, an otolaryngologic consultation should be ob­tained. Combined treatment by an ophthalmologist and otolaryngologist may be necessary in these cases. Based on previously reported cases and the results of our study, we believe that probing alone may not be sufficient in the treatment of dacryocystocele associated with nasal cysts; we recommend probing combined with wide marsupial­ization of the cyst to prevent recurrence.

In addition, the ophthalmologist must be aware that nasal obstruction and respiratory distress can occur in newborn infants with bilateral nasal cysts. Parents of in­fants with dacryocystocele should be carefully questioned

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regarding symptoms of nasal congestion, snorting respi­rations, tachypnea, and breathing difficulty with feeding. Unilateral dacryocystocele does not exclude the possibility of bilateral nasal cysts as demonstrated in two of our pa­tients. In those cases with respiratory distress, excision of the cysts and probing should be performed promptly.

REFERENCES

1. Divine RD, Anderson RL, Bumstead RM. Bilateral congenital lacrimal sac mucoceles with nasal extension and drainage. Arch Ophthalmol 1983; 101 :246-8.

2. Ratio GT, Horton JA, Sprinkle PM. An unusual intranasal anomaly of the lacrimal drainage system. Ophthalmic Surg 1982; 13:741-4.

3. Weinstein GS, Biglan AW, Patterson JH. Congenital lacrimal sac mu· coceles. Am J Ophthalmol1982; 94:106-10.

4. Petersen RA, Robb RM. The natural course of congenital obstruction 01 the nasolacrimal duct. J Pediatr Ophthalmol Strabismus 1978; 15: 246-50.

5. Ffooks 00. Lacrimal abscess in the newborn. A report of seven cases. Br J Ophthalmol1961; 45:562-5.

6. Oral W. Endoscopy of the Paranasal Sinuses: Technique, Typical Findings, Therapeutic Possibilities. Berlin: Springer·Verlag, 1983.

7. Lusk RP, Muntz HM. Nasal obstruction in the neonate secondary to nasolacrimal duct cysts. Int J Pediatr Otorhinolaryngol1987; 13:315-22.

8. Goralowna H, Tarantowicz W. Imperforation 01 the nasolacrimal duct as a cause of nasal obstruction in the newborn. Rhinology 1979; 17: 173-5.