- Email: [email protected]
Congenital nasolacrimal duct mucocele: a cause of respiratory distress
IHTElWATtONAL JOWLVALOF Rdiitric
ELSEVIER SCIENCE IRELAND
International Journal of Pediatric Otorhinolaryngology 29 (1994) 151-158
Congenital nasolacrimal duct mucocele: a cause of respiratory distress? Suzanne
W. Yee*a, Robert W. Seibert”, Charles Charles M. Glasierb
M. Bower”,
‘Department of Otolaryngology-Head and Neck Surgery, ‘Department of Radiology, Universit_vof Arkansas for Medical Sciences and Arkansas Children’s Hospital, 800 Marshall, Little Rock, AR 72202, USA
Received 22 June 1993; revision received 10 September 1993; accepted 19 September 1993)
Abstract Congenital nasolacrimal duct mucocele is an uncommon condition lapse or expansion of the mucocele into the nose may lead to respiratory
in the newborn. Prodistress and difficulty
in feeding, as newborns are preferential nose breathers. Infants with congenital lacrimal sac distension should be examined for signs of respiratory distress and nasal pathology. In the presence of respiratory distress, nasal examination and imaging studies should be done to ensure the diagnosis of nasolacrimal duct mucocele. If significant respiratory distress exists, endoscopic marsupialization of the nasolacrimal duct mucocele in conjunction with nasolacrimal duct probing by the ophthalmologist and possible insertion of lacrimal drainage tubes should be considered. At our institution the nasolacrimal duct mucocele was expectantly managed in two of three cases. Only one of the three patients manifested a significant degree of respiratory distress and required intervention. Endoscopic marsupialization of the nasolacrimal duct mucocele resulted in complete resolution of the mucocele and improvement in symptoms. Key words: Nasal obstruction;
Nasolacrimal
duct mucocele;
Nasolacrimal
duct cyst
* Corresponding author, Department of Otolaryngology-Head and Neck Surgery University of Arkansas for Medical Sciences, 4301 West Markham/Slot 543, Little Rock, AR 72205, USA. iPresented at the Society for Ear Nose and Throat Advances in Children (SENTAC) Meeting in Toronto, Ontario, December, 1992. 0165-5876/94/$07.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved. SSD1 0165-5876(93)00991-M
152
S. W. Yee et al. /Int. J. Pediatr.
Otorhinolaryngol.
29 (1994) 151-158
1. Introduction
Among the congenital anomalies of the nose two groups are distinguished: first where the malformation is clearly visible immediately after birth and the other where the external aspect of the nose is normal but its patency is reduced or completely obstructed with such clinical consequences as dyspnea and aspiration of fluids during feeding. In the second group of anomalies, choanal atresia is best known. However, a variety of other endonasal lesions may occur which can cause significant respiratory distress in infants. Severe septal deformities, anterior nasal stenosis, nasal gliomas or encephaloceles and nasolacrimal duct mucoceles are all described causes of nasal obstruction in infants. Congenital obstruction of the nasolacrimal duct occurs in approximately 30% of term infants; however, epiphora is quite uncommon 17,101.This obstruction resolves in 85% of cases by the time the child reaches 9 months of age, and therefore surgical intervention is rarely required [9]. Rarely, both distal and proximal obstruction can occur and result in accumulation of fluid in the nasolacrimal system. This may cause dilation of the nasolacrimal duct system manifest by an intranasal inferior meatal mass, a nasolacrimal duct mucocele. If these intranasal masses grow large enough they can potentially occlude the nose and cause significant respiratory distress in neonates who are obligate nasal breathers. Three infants were recently identified at the Children’s Hospital with respiratory distress caused by obstructing nasolacrimal duct mucoceles.
2. Case report 1 A 2636 g white female was a product of a Cesarean section to a 41-year-old white female. The pregnancy was complicated by maternal smoking and only an 11 pound weight gain. Initially the baby did well, but soon developed difficulty feeding. A nasogastric tube was unable to be passed. A local otolaryngologist visualized a partial obstruction of the posterior nares. The patient was transported to Arkansas Children’s Hospital for an evaluation of possible choanal atresia. Physical examination revealed a white female infant resting comfortably. A mild degree of respiratory distress was noted with feeding. Vital signs were recorded: temperature was 36.5”C, respiratory rate of 38 breaths/min and pulse of 138/min. Ninety-eight percent oxygen saturation was noted on room air. The physical examination was essentially normal. An otolaryngology consult was obtained and a CT scan was recommended. The CT scan revealed an obstruction of the left nasolacrimal duct with a small mucocele in the region of the inferior turbinate (Fig. 1). There was minimal narrowing of the left posterior nasal canal. An ophthalmology consultation was obtained and nasolacrimal duct obstruction was diagnosed. No treatment was started. On the fifth day of life the patient was started on feedings with the Haberman feeder and tolerated this well. The patient was then advanced to regular feedings and continued to do well without obstructive symptoms. The patient had a follow-up with ophthalmology in 1 month. No intranasal mass was seen, and the patient was asymptomatic.
S. W. Yee et al. /Int. J. Pediatr. Otorhinolaryngol. 29 (1994)
Fig. I. CT scan revealing a left nasolacrimal
151-158
duct cyst protruding
inferior
153
to the inferior
turbinate.
3. Case report 2 A 3-day-old white female presented with a history of marked bilateral nasal obstruction since birth. Initially the nasal obstruction was significant with airway distress and feeding difficulty but was improved with decongestants and inhalation treatments. The nose was suctioned bilaterally and upon physical examination a prominent mass was noted midway into the left nasal cavity. This mass was compressible and had the appearance of a benign polyp. There was no pulsation visible. Beyond the left nasal mass the nasal airway was patent. The right nasal cavity appeared patent. The external nasal dorsum appeared somewhat broadened and had a bluish discoloration with edema of the left medial canthal region. A CT scan revealed that the nasal passages were occluded bilaterally with nasolacrimal duct mucoceles. There was greater obstruction of the left nasal passage (Fig. 2). The patient continued to improve with nasal decongestants and inhalation treatment and was discharged on day 6 of life with no feeding or breathing difficulty. The
S. W. Yee et al. /Int. 1. Pediatr. Otorhinolaryngol. 29 (1994) 151-158
Fig. 2. CT scan revealing bilateral nasolacrimal duct cyst protruding inferior to the turbinates bilaterally.
patient feeding patient canthal
was seen in follow-up in the ENT Clinic on day 12 of life. She had no further or breathing difficulties and the mass was 213 to l/2 its original size. The had persistent left lacrimal duct obstruction and congestion at the medial tendon area. The remainder of the examination was unremarkable.
4. Case report 3 A premature black male born at 27 weeks gestation was delivered via cesarean section to a 35year-old black female. The birth was complicated by premature rupture of membranes and breech positioning of the neonate. Upon delivery the APGARs were 7 and 6. Initially the neonate did well in the nursery but developed grunting respirations and respiratory distress. The patient was intubated and transferred to Arkansas Children’s Hospital. Approximately 1 month after birth the neonate was successfully extubated. The patient did well but had difficulty feeding with a decrease in oxygen saturations and bradycardia. The patient continued to be fed by oral gastric tube for 1 month. The patient was felt to have an upper airway obstruction and an otolaryngology consult was obtained. Upon examination it was noted that the nasal airway was narrow bilaterally with a septal deviation to the left. There was a polypoid soft tissue lesion inferior to the inferior turbinate on the left side. A nasopharyngoscope could not be passed. A CT scan was obtained which revealed a soft tissue mass protruding from the region of the left inferior meatus into the nasal cavity. The mass was connected with a dilated nasolacrimal duct (Fig. 3). This was felt to be compatible with a nasolacrimal duct mucocele. An ophthalmology consultation was obtained. Due to the patient’s persistent respiratory distress, he was taken
s. w.
Yee et al. / Inr. J. Pediatr. Otorhinolaryngol. 29 (1994)
1.51-158
155
Fig. !1.c T scan revealing a soft tissue mass protruding from the region of the inferior meatus on the left d uct. int 0 the nasal cavity. The mass appears to be connected with a dilated nasolacrimal
Fig. 4. Photograph
of the left nasolacrimal
duct cyst under
the inferior
turbinate.
156
S. W. Yee ef al. /IN
J. Pediatr. Otorhinolaryngol. 29 (1994)
151-158
to the operating room. At the time of the operation the left nasolacrimal duct cyst was endoscopically visualized (Fig. 4) and the cyst was marsupialized using cup forceps. Bilateral nasolacrimal duct probing was performed by the ophthalmology service and a Jones tube was placed in the left nasolacrimal duct system. Postoperatively the patient’s respiratory status and ability to feed improved significantly. On follow-up examinations there has been complete resolution of the mucocele. 5. Discussion It is well known that the full development of the lacrimal apparatus during fetal life is achieved rather late in development. It is not uncommon that the process of canalization is not yet finished at birth. The consequence may be distention of the lacrimal sac and, as an effect of secondary infection, mucopurulent dacryocystitis of the newborn. When the inferior part of the unperforated nasolacrimal duct is distended, a submucous cyst may be formed with obstruction of the nasal fossa [3]. Embryologically, the nasolacrimal passageway arises from a thickening of the ectoderm in the naso-optic fissure. The thickened ectoderm buries itself in the mesenchyme between the lateral nasal process and the maxillary process, detaches itself from the surface, and extends downward. In the early fetal stage there is segmental canalization of the duct. It begins at the upper end and progresses downward. During the fetal period there is gradual extension of the canalization until the entire duct is patent. The ocular lumen connects with the inferior and superior canaliculi and with the surface of the lid borders. At birth the lower end of the lacrimal duct is not usually open to connect with the nasal cavity. The point of coalescence between the nasal end of the nasolacrimal duct and the mucous membrane of the inferior meatus is the last point in the duct system to become patent. In most instances, canalization is deferred until postnatal life [lo]. In an investigation of a number of stillborn infants, 73% of the ducts were not connected with the nose at birth. The lower end of the lacrimal duct, although in close apposition to the mucosa of the inferior meatus, was separated from it by a membrane. The lower end of the duct can be seen distended and ballooned out under the inferior turbinate [lo]. Nasolacrimal duct imperforation is quite common at birth. Most investigators agree that approximately 30% are closed at birth although impatency has been reported as high as 84% and as low as 6% [lo]. Only 2% to 4% of these impatent ducts become symptomatic, usually within the first weeks of life [7]. Fourteen percent have bilateral symptoms. Fortunately, of those that become symptomatic, the vast majority (80%) undergo spontaneous resolution within the first 6 months as the imperforate canal opens. Therefore, conservative therapy is indicated in the uncomplicated dacryostenosis [9]. Encysted mucoceles of the lacrimal sac are rare. A mucocele is a cystic structure lined by ciliated columnar epithelium with goblet cells and is secondarily filled with mucus and epithelial debris. An occluded lacrimal sac is such a structure. Should this closed system develop a secondary infection, it is then called a mucopyocele. A mucocele needs to be further contrasted with amniotocele, which is the same cystic
S. W. Yee et al. /Int. J. Pediarr. Otorhinolaryngol.
29 (1994)
151-158
157
structure that is tilled with amniotic fluid at the time of birth rather than with secreted mucus and epithelial debris, which replaces the amniotic fluid after birth. Thus, these congenital lacrimal sac masses do not represent distinct entities, but rather a continuum of the same process: an amniotocele becomes a mucocele, which may become a mucopyocele. Respiratory distress associated with a unilateral congenital nasolacrimal duct mucocele is probably due to a combination of factors. Firstly, the imperforate distal end of the nasolacrimal mucocele balloons or prolapses into a nasal passage leading to obstruction. Secondly, 80% of the population including neonates experience a normal, cyclic vasocongestion of the nasal mucus membranes. This commonly results in alternating obstruction of the nasal passages. Finally, most neonates breath predominately through their nose, except when crying. It is possible, therefore, for the infant to experience acute respiratory distress when one nasal passage is obstructed by the mucocele and the other by the cyclic vascular congestion. The clinical picture of a mass inferior to the medial canthus with a nasal cavity mass may represent a nasolacrimal sac mucocele, but an encephalocele or meningocele should be considered in the differential diagnosis. Other conditions which may cause respiratory distress in infants include a nasal cavity glioma, dermoid, or choanal atresia. Nasal examination and a CT scan should be obtained when a congenital nasolacrimal sac mucocele is associated with respiratory distress [2]. Investigation by CT scan is important as it not only gives the correct anatomic diagnosis but also allows easy differentiation from other masses. The triad of a dilated nasal lacrimal duct in contiguity with an intranasal cyst and a cystic dilatation of the lacrimal sac on CT scan are diagnostic of nasal lacrimal duct mucocele [71. Most investigators believe that early treatment of the mucocele is indicated, as secondary infection is a possible complication. A more important indication for intervention is nasal obstruction manifest by respiratory distress or feeding difficulties. One of our three patients had significant respiratory distress and feeding difficulties which required intervention. The other two patients were able to be treated with more conservative measures such as use of nasal decongestants and a Haberman feeder. Treatments advocated are (1) nasolacrimal duct probing so that to relieve both the distal and proximal obstruction of the nasolacrimal drainage system, (2) puncture and marsupialization of the cyst wall below the inferior turbinate, (3) puncture of the cyst intranasally and (4) intranasal excision with a CO* laser [l-4,9]. When intervention is necessary due to the extent of symptoms, we recommend doing the procedure in conjunction with an ophthalmologist. The ophthalmologist can probe the lacrimal system from above and insert a drainage tube if necessary. We found the use of endoscopic techniques for the intranasal portion of the procedure to be quite helpful. After vasoconstriction, a nasal telescope can provide excellent visualization of the inferior meatus and the mucocele. The mucoceles can be easily opened and marsupialized with a small cup forceps under direct visualization. Bleeding was not a problem in our case. Excellent results should be possible to obtain with direct visualization.
S. W. Yee et al. /Int. J. Pediatr. Otorhinolaryngol. 29 (1994) 151-158
158
6. Acknowledgement Acknowledgement is made to Dr. Monty Barker for assistance in preparing this paper. 7. References I
5 6
Berkowitz, R.G., Grundfast, K.M. and Fitz, C. (1990) Nasal obstruction of the newborn revisited: Cinical and subclinical manifestations of congenital nasolacrimal duct obstruction presenting as a nasal mass. Otolaryngol. Head Neck Surg. 103, 468-471. Cassady, J.V. (1952) Developmental anatomy of nasolacrimal duct. Arch. of Ophthalmol. 47, 141-158. Demaeril, P.H., Casteels, I., Perne, P., Maudgal, PC., Wilms, G., Baert, A.L. and Missotten, L. (1991) Unilateral congenital nasolacrimal mucocele. Pediatr. Radiol. 21, 51 I. Divine, R.D., Anderson, R.L. and Bumsted, R.M. (1983) Bilateral congenital lacrimal sac mucoceles with nasal extension and drainage. Arch. Ophthalmol. 101, 246-248. Edmond, J.C. and Keecho, R.V. (1991) Congenital nasolacrimal sac mucocele associated with respiratory distress. J. Pediatr. C?phthalmol. Strabismus 28, 287-289. Goralowna, M. and Tarantowicz, W. (1979) Imperforation of the nasolacrimal duct as a cause of nasal obstruction in the newborn. Rhinology 17, 174-175. Levy, N.S. (1979) Conservative management of congenital amnionotocele of the nasolacrimal duct. J. Pediatr. Ophthalmol. Strabismus 16, 254-256. Lusk, R.P. and Muntz, H.M. (1987) Nasal obstruction in the neonate secondary to nasolacrimal duct cysts. Int. J. Pediatr. Otorhinolaryngol. 12, 315-322. Petersen, R.A. and Robb, R.M. (1978) The natural course of congenital obstruction of the nasolacrimal duct. J. Pediatr. Ophthalmol. Strabismus 15, 246-250. Sevel, D. (1981) Development and congenital abnormalities of the nasal lacrimal apparatus. J. Pediatr. Ophthalmol. Strabismus 18, 13-19.
ELSEVIER SCIENCE IRELAND
International Journal of Pediatric Otorhinolaryngology 29 (1994) 151-158
Congenital nasolacrimal duct mucocele: a cause of respiratory distress? Suzanne
W. Yee*a, Robert W. Seibert”, Charles Charles M. Glasierb
M. Bower”,
‘Department of Otolaryngology-Head and Neck Surgery, ‘Department of Radiology, Universit_vof Arkansas for Medical Sciences and Arkansas Children’s Hospital, 800 Marshall, Little Rock, AR 72202, USA
Received 22 June 1993; revision received 10 September 1993; accepted 19 September 1993)
Abstract Congenital nasolacrimal duct mucocele is an uncommon condition lapse or expansion of the mucocele into the nose may lead to respiratory
in the newborn. Prodistress and difficulty
in feeding, as newborns are preferential nose breathers. Infants with congenital lacrimal sac distension should be examined for signs of respiratory distress and nasal pathology. In the presence of respiratory distress, nasal examination and imaging studies should be done to ensure the diagnosis of nasolacrimal duct mucocele. If significant respiratory distress exists, endoscopic marsupialization of the nasolacrimal duct mucocele in conjunction with nasolacrimal duct probing by the ophthalmologist and possible insertion of lacrimal drainage tubes should be considered. At our institution the nasolacrimal duct mucocele was expectantly managed in two of three cases. Only one of the three patients manifested a significant degree of respiratory distress and required intervention. Endoscopic marsupialization of the nasolacrimal duct mucocele resulted in complete resolution of the mucocele and improvement in symptoms. Key words: Nasal obstruction;
Nasolacrimal
duct mucocele;
Nasolacrimal
duct cyst
* Corresponding author, Department of Otolaryngology-Head and Neck Surgery University of Arkansas for Medical Sciences, 4301 West Markham/Slot 543, Little Rock, AR 72205, USA. iPresented at the Society for Ear Nose and Throat Advances in Children (SENTAC) Meeting in Toronto, Ontario, December, 1992. 0165-5876/94/$07.00 0 1994 Elsevier Science Ireland Ltd. All rights reserved. SSD1 0165-5876(93)00991-M
152
S. W. Yee et al. /Int. J. Pediatr.
Otorhinolaryngol.
29 (1994) 151-158
1. Introduction
Among the congenital anomalies of the nose two groups are distinguished: first where the malformation is clearly visible immediately after birth and the other where the external aspect of the nose is normal but its patency is reduced or completely obstructed with such clinical consequences as dyspnea and aspiration of fluids during feeding. In the second group of anomalies, choanal atresia is best known. However, a variety of other endonasal lesions may occur which can cause significant respiratory distress in infants. Severe septal deformities, anterior nasal stenosis, nasal gliomas or encephaloceles and nasolacrimal duct mucoceles are all described causes of nasal obstruction in infants. Congenital obstruction of the nasolacrimal duct occurs in approximately 30% of term infants; however, epiphora is quite uncommon 17,101.This obstruction resolves in 85% of cases by the time the child reaches 9 months of age, and therefore surgical intervention is rarely required [9]. Rarely, both distal and proximal obstruction can occur and result in accumulation of fluid in the nasolacrimal system. This may cause dilation of the nasolacrimal duct system manifest by an intranasal inferior meatal mass, a nasolacrimal duct mucocele. If these intranasal masses grow large enough they can potentially occlude the nose and cause significant respiratory distress in neonates who are obligate nasal breathers. Three infants were recently identified at the Children’s Hospital with respiratory distress caused by obstructing nasolacrimal duct mucoceles.
2. Case report 1 A 2636 g white female was a product of a Cesarean section to a 41-year-old white female. The pregnancy was complicated by maternal smoking and only an 11 pound weight gain. Initially the baby did well, but soon developed difficulty feeding. A nasogastric tube was unable to be passed. A local otolaryngologist visualized a partial obstruction of the posterior nares. The patient was transported to Arkansas Children’s Hospital for an evaluation of possible choanal atresia. Physical examination revealed a white female infant resting comfortably. A mild degree of respiratory distress was noted with feeding. Vital signs were recorded: temperature was 36.5”C, respiratory rate of 38 breaths/min and pulse of 138/min. Ninety-eight percent oxygen saturation was noted on room air. The physical examination was essentially normal. An otolaryngology consult was obtained and a CT scan was recommended. The CT scan revealed an obstruction of the left nasolacrimal duct with a small mucocele in the region of the inferior turbinate (Fig. 1). There was minimal narrowing of the left posterior nasal canal. An ophthalmology consultation was obtained and nasolacrimal duct obstruction was diagnosed. No treatment was started. On the fifth day of life the patient was started on feedings with the Haberman feeder and tolerated this well. The patient was then advanced to regular feedings and continued to do well without obstructive symptoms. The patient had a follow-up with ophthalmology in 1 month. No intranasal mass was seen, and the patient was asymptomatic.
S. W. Yee et al. /Int. J. Pediatr. Otorhinolaryngol. 29 (1994)
Fig. I. CT scan revealing a left nasolacrimal
151-158
duct cyst protruding
inferior
153
to the inferior
turbinate.
3. Case report 2 A 3-day-old white female presented with a history of marked bilateral nasal obstruction since birth. Initially the nasal obstruction was significant with airway distress and feeding difficulty but was improved with decongestants and inhalation treatments. The nose was suctioned bilaterally and upon physical examination a prominent mass was noted midway into the left nasal cavity. This mass was compressible and had the appearance of a benign polyp. There was no pulsation visible. Beyond the left nasal mass the nasal airway was patent. The right nasal cavity appeared patent. The external nasal dorsum appeared somewhat broadened and had a bluish discoloration with edema of the left medial canthal region. A CT scan revealed that the nasal passages were occluded bilaterally with nasolacrimal duct mucoceles. There was greater obstruction of the left nasal passage (Fig. 2). The patient continued to improve with nasal decongestants and inhalation treatment and was discharged on day 6 of life with no feeding or breathing difficulty. The
S. W. Yee et al. /Int. 1. Pediatr. Otorhinolaryngol. 29 (1994) 151-158
Fig. 2. CT scan revealing bilateral nasolacrimal duct cyst protruding inferior to the turbinates bilaterally.
patient feeding patient canthal
was seen in follow-up in the ENT Clinic on day 12 of life. She had no further or breathing difficulties and the mass was 213 to l/2 its original size. The had persistent left lacrimal duct obstruction and congestion at the medial tendon area. The remainder of the examination was unremarkable.
4. Case report 3 A premature black male born at 27 weeks gestation was delivered via cesarean section to a 35year-old black female. The birth was complicated by premature rupture of membranes and breech positioning of the neonate. Upon delivery the APGARs were 7 and 6. Initially the neonate did well in the nursery but developed grunting respirations and respiratory distress. The patient was intubated and transferred to Arkansas Children’s Hospital. Approximately 1 month after birth the neonate was successfully extubated. The patient did well but had difficulty feeding with a decrease in oxygen saturations and bradycardia. The patient continued to be fed by oral gastric tube for 1 month. The patient was felt to have an upper airway obstruction and an otolaryngology consult was obtained. Upon examination it was noted that the nasal airway was narrow bilaterally with a septal deviation to the left. There was a polypoid soft tissue lesion inferior to the inferior turbinate on the left side. A nasopharyngoscope could not be passed. A CT scan was obtained which revealed a soft tissue mass protruding from the region of the left inferior meatus into the nasal cavity. The mass was connected with a dilated nasolacrimal duct (Fig. 3). This was felt to be compatible with a nasolacrimal duct mucocele. An ophthalmology consultation was obtained. Due to the patient’s persistent respiratory distress, he was taken
s. w.
Yee et al. / Inr. J. Pediatr. Otorhinolaryngol. 29 (1994)
1.51-158
155
Fig. !1.c T scan revealing a soft tissue mass protruding from the region of the inferior meatus on the left d uct. int 0 the nasal cavity. The mass appears to be connected with a dilated nasolacrimal
Fig. 4. Photograph
of the left nasolacrimal
duct cyst under
the inferior
turbinate.
156
S. W. Yee ef al. /IN
J. Pediatr. Otorhinolaryngol. 29 (1994)
151-158
to the operating room. At the time of the operation the left nasolacrimal duct cyst was endoscopically visualized (Fig. 4) and the cyst was marsupialized using cup forceps. Bilateral nasolacrimal duct probing was performed by the ophthalmology service and a Jones tube was placed in the left nasolacrimal duct system. Postoperatively the patient’s respiratory status and ability to feed improved significantly. On follow-up examinations there has been complete resolution of the mucocele. 5. Discussion It is well known that the full development of the lacrimal apparatus during fetal life is achieved rather late in development. It is not uncommon that the process of canalization is not yet finished at birth. The consequence may be distention of the lacrimal sac and, as an effect of secondary infection, mucopurulent dacryocystitis of the newborn. When the inferior part of the unperforated nasolacrimal duct is distended, a submucous cyst may be formed with obstruction of the nasal fossa [3]. Embryologically, the nasolacrimal passageway arises from a thickening of the ectoderm in the naso-optic fissure. The thickened ectoderm buries itself in the mesenchyme between the lateral nasal process and the maxillary process, detaches itself from the surface, and extends downward. In the early fetal stage there is segmental canalization of the duct. It begins at the upper end and progresses downward. During the fetal period there is gradual extension of the canalization until the entire duct is patent. The ocular lumen connects with the inferior and superior canaliculi and with the surface of the lid borders. At birth the lower end of the lacrimal duct is not usually open to connect with the nasal cavity. The point of coalescence between the nasal end of the nasolacrimal duct and the mucous membrane of the inferior meatus is the last point in the duct system to become patent. In most instances, canalization is deferred until postnatal life [lo]. In an investigation of a number of stillborn infants, 73% of the ducts were not connected with the nose at birth. The lower end of the lacrimal duct, although in close apposition to the mucosa of the inferior meatus, was separated from it by a membrane. The lower end of the duct can be seen distended and ballooned out under the inferior turbinate [lo]. Nasolacrimal duct imperforation is quite common at birth. Most investigators agree that approximately 30% are closed at birth although impatency has been reported as high as 84% and as low as 6% [lo]. Only 2% to 4% of these impatent ducts become symptomatic, usually within the first weeks of life [7]. Fourteen percent have bilateral symptoms. Fortunately, of those that become symptomatic, the vast majority (80%) undergo spontaneous resolution within the first 6 months as the imperforate canal opens. Therefore, conservative therapy is indicated in the uncomplicated dacryostenosis [9]. Encysted mucoceles of the lacrimal sac are rare. A mucocele is a cystic structure lined by ciliated columnar epithelium with goblet cells and is secondarily filled with mucus and epithelial debris. An occluded lacrimal sac is such a structure. Should this closed system develop a secondary infection, it is then called a mucopyocele. A mucocele needs to be further contrasted with amniotocele, which is the same cystic
S. W. Yee et al. /Int. J. Pediarr. Otorhinolaryngol.
29 (1994)
151-158
157
structure that is tilled with amniotic fluid at the time of birth rather than with secreted mucus and epithelial debris, which replaces the amniotic fluid after birth. Thus, these congenital lacrimal sac masses do not represent distinct entities, but rather a continuum of the same process: an amniotocele becomes a mucocele, which may become a mucopyocele. Respiratory distress associated with a unilateral congenital nasolacrimal duct mucocele is probably due to a combination of factors. Firstly, the imperforate distal end of the nasolacrimal mucocele balloons or prolapses into a nasal passage leading to obstruction. Secondly, 80% of the population including neonates experience a normal, cyclic vasocongestion of the nasal mucus membranes. This commonly results in alternating obstruction of the nasal passages. Finally, most neonates breath predominately through their nose, except when crying. It is possible, therefore, for the infant to experience acute respiratory distress when one nasal passage is obstructed by the mucocele and the other by the cyclic vascular congestion. The clinical picture of a mass inferior to the medial canthus with a nasal cavity mass may represent a nasolacrimal sac mucocele, but an encephalocele or meningocele should be considered in the differential diagnosis. Other conditions which may cause respiratory distress in infants include a nasal cavity glioma, dermoid, or choanal atresia. Nasal examination and a CT scan should be obtained when a congenital nasolacrimal sac mucocele is associated with respiratory distress [2]. Investigation by CT scan is important as it not only gives the correct anatomic diagnosis but also allows easy differentiation from other masses. The triad of a dilated nasal lacrimal duct in contiguity with an intranasal cyst and a cystic dilatation of the lacrimal sac on CT scan are diagnostic of nasal lacrimal duct mucocele [71. Most investigators believe that early treatment of the mucocele is indicated, as secondary infection is a possible complication. A more important indication for intervention is nasal obstruction manifest by respiratory distress or feeding difficulties. One of our three patients had significant respiratory distress and feeding difficulties which required intervention. The other two patients were able to be treated with more conservative measures such as use of nasal decongestants and a Haberman feeder. Treatments advocated are (1) nasolacrimal duct probing so that to relieve both the distal and proximal obstruction of the nasolacrimal drainage system, (2) puncture and marsupialization of the cyst wall below the inferior turbinate, (3) puncture of the cyst intranasally and (4) intranasal excision with a CO* laser [l-4,9]. When intervention is necessary due to the extent of symptoms, we recommend doing the procedure in conjunction with an ophthalmologist. The ophthalmologist can probe the lacrimal system from above and insert a drainage tube if necessary. We found the use of endoscopic techniques for the intranasal portion of the procedure to be quite helpful. After vasoconstriction, a nasal telescope can provide excellent visualization of the inferior meatus and the mucocele. The mucoceles can be easily opened and marsupialized with a small cup forceps under direct visualization. Bleeding was not a problem in our case. Excellent results should be possible to obtain with direct visualization.
S. W. Yee et al. /Int. J. Pediatr. Otorhinolaryngol. 29 (1994) 151-158
158
6. Acknowledgement Acknowledgement is made to Dr. Monty Barker for assistance in preparing this paper. 7. References I
5 6
Berkowitz, R.G., Grundfast, K.M. and Fitz, C. (1990) Nasal obstruction of the newborn revisited: Cinical and subclinical manifestations of congenital nasolacrimal duct obstruction presenting as a nasal mass. Otolaryngol. Head Neck Surg. 103, 468-471. Cassady, J.V. (1952) Developmental anatomy of nasolacrimal duct. Arch. of Ophthalmol. 47, 141-158. Demaeril, P.H., Casteels, I., Perne, P., Maudgal, PC., Wilms, G., Baert, A.L. and Missotten, L. (1991) Unilateral congenital nasolacrimal mucocele. Pediatr. Radiol. 21, 51 I. Divine, R.D., Anderson, R.L. and Bumsted, R.M. (1983) Bilateral congenital lacrimal sac mucoceles with nasal extension and drainage. Arch. Ophthalmol. 101, 246-248. Edmond, J.C. and Keecho, R.V. (1991) Congenital nasolacrimal sac mucocele associated with respiratory distress. J. Pediatr. C?phthalmol. Strabismus 28, 287-289. Goralowna, M. and Tarantowicz, W. (1979) Imperforation of the nasolacrimal duct as a cause of nasal obstruction in the newborn. Rhinology 17, 174-175. Levy, N.S. (1979) Conservative management of congenital amnionotocele of the nasolacrimal duct. J. Pediatr. Ophthalmol. Strabismus 16, 254-256. Lusk, R.P. and Muntz, H.M. (1987) Nasal obstruction in the neonate secondary to nasolacrimal duct cysts. Int. J. Pediatr. Otorhinolaryngol. 12, 315-322. Petersen, R.A. and Robb, R.M. (1978) The natural course of congenital obstruction of the nasolacrimal duct. J. Pediatr. Ophthalmol. Strabismus 15, 246-250. Sevel, D. (1981) Development and congenital abnormalities of the nasal lacrimal apparatus. J. Pediatr. Ophthalmol. Strabismus 18, 13-19.