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Agenesis of the nasolacrimal duct
International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 269–271
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology Extra journal homepage: www.elsevier.com/locate/ijporl
Case report
Agenesis of the nasolacrimal duct Adva B. Friedman a,*, Patrick D. Munson a, Christopher T. Westfall b, Gresham T. Richter a a b
University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Department of Otolaryngology, Division of Pediatric Otolaryngology, United States Department of Pediatric Ophthalmology, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 July 2010 Received in revised form 29 December 2010 Accepted 30 December 2010 Available online 1 February 2011
Congenital nasolacrimal duct obstruction results from anomalies of the distal and proximal portions of the canalicular system. Patients frequently present with persistent mucoid discharge from the affected eye. Maldevelopment of the nasolacrimal duct is a rare cause of obstruction. We report a case of complete absence of the intraosseus and membranous portions of the nasolacrimal duct. The patient underwent dacrocystorhinostomy and intubation with Crawford tubes which resulted in successful resolution of the obstruction. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Nasolacrimal Duct Agenesis Obstruction Stenosis Absence Congenital Otolaryngology Ophthalmology Dacrocystorhinostomy Endoscopic Sinus
1. Introduction The nasolacrimal system is the drainage terminal of tears and periorbital discharge. This system consists of puncta at the medial aspect of the eyelid margins, their associated canaliculi, and the lacrimal sac into which they drain. The lacrimal sac empties into the nasolacrimal duct that subsequently emerges beneath the inferior turbinate of the nasal cavity. The nasolacrimal duct is composed of an intraosseus portion which is contained within the maxillary bone. As it descends it becomes a membranous channel contained within the mucosa of the nose. Its ostium is located at the inferior meatus. Anomalies of the nasolacrimal system can occur during development. These predominately include those of the proximal system at the puncta, canaliculi, and lacrimal sac. Aberrancies of the nasolacrimal duct also occur. Collectively these anomalies can cause obstruction of the nasolacrimal system with subsequent back-flow and chronic epiphora. Maldevelopment of the nasola-
* Corresponding author at: Arkansas Children’s Hospital, 1 Children’s Way Slot 668, Little Rock, AR 72202, United States. Tel.: +1 501 364 7546; fax: +1 501 364 1935. E-mail address: [email protected] (A.B. Friedman). 1871-4048/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2010.12.001
crimal system can be an isolated finding or part of a syndrome involving a defect in facial development. In this report we describe a rare case of complete intraosseus and membranous nasolacrimal duct agenesis in an otherwise normal child. 2. Case presentation By the request of ophthalmology, a 3 year old healthy male presented to otolaryngology clinic with chronic mucoid discharge of the left eye that had been present since birth. The patient was seen by ophthalmology and diagnosed with left nasolacrimal duct obstruction. Two attempts at probing the canalicular system had been previously performed. The second attempt to probe the duct was performed by a pediatric opthamologist at the same institution. During the procedure, an atypically hard barrier was encountered during the attempted probing of the canalicular system. The patient was referred to otolaryngology for evaluation and possible coordinated endoscopic dacrocystorhinostomy. On examination, the patient was found to have mucoid discharge with compression of the lacrimal sac at the area of the medial canthus. He was also noted to have punctal hypoplasia of the right eye. A thorough nasal examination did not reveal a cyst or other anatomic abnormalities below the inferior turbinate or the pathway of the nasolacrimal system.
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A.B. Friedman et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 269–271
At two weeks follow up, the patient had improvement in symptoms. Crawford tubes were still in place. The patient no longer had epiphora. At one month follow up, the patient presented with displaced Crawford tubes and no longer required stenting of the newly formed duct. At six months, the patient had improvement of symptoms and no further intervention was pursued. 4. Discussion
Fig. 1. Vitreoretinal retinal light probe introduced into the lower canalicular system.
3. Surgical approach The patient was brought to the operative suite for possible endoscopic dacrocystorhinostomy. Under anesthesia, ophthalmology made a single attempt to cannulate the upper and lower canaliculi without success. Nasal endoscopy was subsequently performed. Endoscopic visualization of the inferior meatus demonstrated no apparent lacrimal duct orifice. A mucosal flap was elevated along the left lateral nasal wall anterior to the attachment of the middle turbinate and just superior to the inferior turbinate. The intersection of the maxillary and lacrimal bones was identified. Reduction of the bone using a diamond burr microdrill and osteotome did not reveal a lacrimal duct or cyst. Further dissection demonstrated only bone formation at the normal anatomic site of the lacrimal duct. Canalization of the bone was not present. A vitreoretinal light probe was subsequently introduced by ophthalmology through the canalicular system to transilluminate the termination of the lacrimal canniculus (Fig. 1). Only bone was observed from the intranasal side at the site of illumination (Fig. 2). An endoscopic DCR burr was used to penetrate through the maxillary and lacrimal bone until the light probe was apparent. Connection to the lacrimal apparatus was subsequently made and Crawford tubes were introduced through the upper and lower canaliculi to the nasal cavity.
Congenital nasolacrimal duct obstruction can result from anatomic obstruction or dysgenesis of the nasolacrimal duct system. The incidence of nasolacrimal duct obstruction has been reported to be 1.2–30% in various studies. Anatomic obstruction results from an imperforate membrane at the valve of Hasner, lacrimal fistula, dacrocystocele, lacrimal duct cysts, or other mass lesions [1]. Formation of the nasolacrimal duct begins as a groove between the maxillary and frontonasal prominences at around 30 days of gestation. Ectoderm is buried within this groove and eventually separates to form an epithelial cord. The caudal portion of this cord ultimately becomes the nasolacrimal duct while the cephalic portion of the cord forms the lacrimal sac. Simultaneously cords of epithelium form at the upper and lower lid margins and later regress to form the canaliculi. Canalization occurs from the third to seventh month of gestation leading to formation of the nasolacrimal apparatus. The nasolacrimal duct is covered by the membrane of Hasner. Spontaneous rupture of this membrane leads to the formation of a mucosal fold called the Hasner valve in the first year of life to allow lacrimal drainage into the nose. Maldevelopment of the nasolacrimal system has been described in the literature. Ahn Yuen et al. reported their experience over a 10 year period with dysgenesis of the nasolacrimal system [2]. They found proximal anomalies to be more common. The majority of the group who displayed distal anomalies had a systemic syndrome or dysmorphism and also displayed concomitant proximal anomalies. Complete absence of the nasolacrimal duct is rare and there have been only a few reports of this finding. McNabb reported three cases of agenesis of the nasolacrimal duct [3]. Two of the patients were found to have bony obstruction at the midlevel of the nasolacrimal duct. The third was found to have complete absence of the bony portion of the nasolacrimal duct. Prabhakaran et al. reported absence of the membranous nasolacrimal duct in association with Velocardiofacial syndrome [4]. Similarly to the third patient in McNabb’s case report, our patient had complete absence of his nasolacrimal duct. In addition, he had punctual hypoplasia of his contralateral eye consistent with the impression that these cases often have simultaneous anomalies of the nasolacrimal system due to an insult at the time of development. Endoscopic dacryocystorhinostomy (DCR) is a proven surgical technique in patients with primary low mechanical obstruction persistent after previous lacrimal probings [5]. Endoscopic visualization and powered instrumentation aid in achieving the surgical goals, as in the present case. Endoscopic DCR is a safe and effective procedure, offering results comparable to external DCR. 5. Conclusion
Fig. 2. Raised mucosal flap revealing absence of intraosseus duct and proposed area of drilling.
Congenital nasolacrimal duct obstruction can be a consequence of failure of canalization during embryological development. Failed resolution of obstruction by a young age prompts an attempt at probing of the lacrimal system. In cases where probing has proven unsuccessful, otolaryngologists are often asked to perform dacrocystorhinostomy to relieve the distal obstruction. This is best managed as a collaborative effort between the otolaryngologist and the ophthalmologist. Endoscopic dacrocystorhinostomy has proven to be a safe and effective method of approach for the
A.B. Friedman et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 269–271
otolaryngologist. This case report describes the rare finding of complete agenesis of the nasolacrimal duct in an otherwise healthy child and its successful management with endoscopic DCR. References [1] M.K. Kapadia, S.K. Freitag, J.J. Woog, Evaluation and management of congenital nasolacrimal duct obstruction, Otolaryngol. Clin. N. Am. 39 (2006) 959–977.
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[2] S.J. Ahn Yuen, C. Oley, T.J. Sullivan, Lacrimal outflow dysgenesis, Ophthalmology 111 (2004) 1782–1790. [3] A.A. McNabb, Congenital absence of the nasolacrimal duct, J. Pediatr. Ophthalmol. Strabismus 35 (1998) 294–295. [4] V.C. Prabhakaran, G. Davis, P.J. Wormald, D. Selva, Congenital absence of the nasolacrimal duct in velocardiofacial syndrome, J. Am. Assoc. Pediatr. Ophthalmol. Strabismus 12 (2008) 85–86. [5] P. Eloy, E. Leruth, A. Cailliau, S. Collet, B. Bertrand, P. Rombaux, Pediatric endonasal endoscopic dacryocystorhinostomy, Int. J. Pediatr. Otorhinolaryngol. 73 (2009) 867–871.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology Extra journal homepage: www.elsevier.com/locate/ijporl
Case report
Agenesis of the nasolacrimal duct Adva B. Friedman a,*, Patrick D. Munson a, Christopher T. Westfall b, Gresham T. Richter a a b
University of Arkansas for Medical Sciences, Arkansas Children’s Hospital, Department of Otolaryngology, Division of Pediatric Otolaryngology, United States Department of Pediatric Ophthalmology, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 July 2010 Received in revised form 29 December 2010 Accepted 30 December 2010 Available online 1 February 2011
Congenital nasolacrimal duct obstruction results from anomalies of the distal and proximal portions of the canalicular system. Patients frequently present with persistent mucoid discharge from the affected eye. Maldevelopment of the nasolacrimal duct is a rare cause of obstruction. We report a case of complete absence of the intraosseus and membranous portions of the nasolacrimal duct. The patient underwent dacrocystorhinostomy and intubation with Crawford tubes which resulted in successful resolution of the obstruction. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Nasolacrimal Duct Agenesis Obstruction Stenosis Absence Congenital Otolaryngology Ophthalmology Dacrocystorhinostomy Endoscopic Sinus
1. Introduction The nasolacrimal system is the drainage terminal of tears and periorbital discharge. This system consists of puncta at the medial aspect of the eyelid margins, their associated canaliculi, and the lacrimal sac into which they drain. The lacrimal sac empties into the nasolacrimal duct that subsequently emerges beneath the inferior turbinate of the nasal cavity. The nasolacrimal duct is composed of an intraosseus portion which is contained within the maxillary bone. As it descends it becomes a membranous channel contained within the mucosa of the nose. Its ostium is located at the inferior meatus. Anomalies of the nasolacrimal system can occur during development. These predominately include those of the proximal system at the puncta, canaliculi, and lacrimal sac. Aberrancies of the nasolacrimal duct also occur. Collectively these anomalies can cause obstruction of the nasolacrimal system with subsequent back-flow and chronic epiphora. Maldevelopment of the nasola-
* Corresponding author at: Arkansas Children’s Hospital, 1 Children’s Way Slot 668, Little Rock, AR 72202, United States. Tel.: +1 501 364 7546; fax: +1 501 364 1935. E-mail address: [email protected] (A.B. Friedman). 1871-4048/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2010.12.001
crimal system can be an isolated finding or part of a syndrome involving a defect in facial development. In this report we describe a rare case of complete intraosseus and membranous nasolacrimal duct agenesis in an otherwise normal child. 2. Case presentation By the request of ophthalmology, a 3 year old healthy male presented to otolaryngology clinic with chronic mucoid discharge of the left eye that had been present since birth. The patient was seen by ophthalmology and diagnosed with left nasolacrimal duct obstruction. Two attempts at probing the canalicular system had been previously performed. The second attempt to probe the duct was performed by a pediatric opthamologist at the same institution. During the procedure, an atypically hard barrier was encountered during the attempted probing of the canalicular system. The patient was referred to otolaryngology for evaluation and possible coordinated endoscopic dacrocystorhinostomy. On examination, the patient was found to have mucoid discharge with compression of the lacrimal sac at the area of the medial canthus. He was also noted to have punctal hypoplasia of the right eye. A thorough nasal examination did not reveal a cyst or other anatomic abnormalities below the inferior turbinate or the pathway of the nasolacrimal system.
270
A.B. Friedman et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 269–271
At two weeks follow up, the patient had improvement in symptoms. Crawford tubes were still in place. The patient no longer had epiphora. At one month follow up, the patient presented with displaced Crawford tubes and no longer required stenting of the newly formed duct. At six months, the patient had improvement of symptoms and no further intervention was pursued. 4. Discussion
Fig. 1. Vitreoretinal retinal light probe introduced into the lower canalicular system.
3. Surgical approach The patient was brought to the operative suite for possible endoscopic dacrocystorhinostomy. Under anesthesia, ophthalmology made a single attempt to cannulate the upper and lower canaliculi without success. Nasal endoscopy was subsequently performed. Endoscopic visualization of the inferior meatus demonstrated no apparent lacrimal duct orifice. A mucosal flap was elevated along the left lateral nasal wall anterior to the attachment of the middle turbinate and just superior to the inferior turbinate. The intersection of the maxillary and lacrimal bones was identified. Reduction of the bone using a diamond burr microdrill and osteotome did not reveal a lacrimal duct or cyst. Further dissection demonstrated only bone formation at the normal anatomic site of the lacrimal duct. Canalization of the bone was not present. A vitreoretinal light probe was subsequently introduced by ophthalmology through the canalicular system to transilluminate the termination of the lacrimal canniculus (Fig. 1). Only bone was observed from the intranasal side at the site of illumination (Fig. 2). An endoscopic DCR burr was used to penetrate through the maxillary and lacrimal bone until the light probe was apparent. Connection to the lacrimal apparatus was subsequently made and Crawford tubes were introduced through the upper and lower canaliculi to the nasal cavity.
Congenital nasolacrimal duct obstruction can result from anatomic obstruction or dysgenesis of the nasolacrimal duct system. The incidence of nasolacrimal duct obstruction has been reported to be 1.2–30% in various studies. Anatomic obstruction results from an imperforate membrane at the valve of Hasner, lacrimal fistula, dacrocystocele, lacrimal duct cysts, or other mass lesions [1]. Formation of the nasolacrimal duct begins as a groove between the maxillary and frontonasal prominences at around 30 days of gestation. Ectoderm is buried within this groove and eventually separates to form an epithelial cord. The caudal portion of this cord ultimately becomes the nasolacrimal duct while the cephalic portion of the cord forms the lacrimal sac. Simultaneously cords of epithelium form at the upper and lower lid margins and later regress to form the canaliculi. Canalization occurs from the third to seventh month of gestation leading to formation of the nasolacrimal apparatus. The nasolacrimal duct is covered by the membrane of Hasner. Spontaneous rupture of this membrane leads to the formation of a mucosal fold called the Hasner valve in the first year of life to allow lacrimal drainage into the nose. Maldevelopment of the nasolacrimal system has been described in the literature. Ahn Yuen et al. reported their experience over a 10 year period with dysgenesis of the nasolacrimal system [2]. They found proximal anomalies to be more common. The majority of the group who displayed distal anomalies had a systemic syndrome or dysmorphism and also displayed concomitant proximal anomalies. Complete absence of the nasolacrimal duct is rare and there have been only a few reports of this finding. McNabb reported three cases of agenesis of the nasolacrimal duct [3]. Two of the patients were found to have bony obstruction at the midlevel of the nasolacrimal duct. The third was found to have complete absence of the bony portion of the nasolacrimal duct. Prabhakaran et al. reported absence of the membranous nasolacrimal duct in association with Velocardiofacial syndrome [4]. Similarly to the third patient in McNabb’s case report, our patient had complete absence of his nasolacrimal duct. In addition, he had punctual hypoplasia of his contralateral eye consistent with the impression that these cases often have simultaneous anomalies of the nasolacrimal system due to an insult at the time of development. Endoscopic dacryocystorhinostomy (DCR) is a proven surgical technique in patients with primary low mechanical obstruction persistent after previous lacrimal probings [5]. Endoscopic visualization and powered instrumentation aid in achieving the surgical goals, as in the present case. Endoscopic DCR is a safe and effective procedure, offering results comparable to external DCR. 5. Conclusion
Fig. 2. Raised mucosal flap revealing absence of intraosseus duct and proposed area of drilling.
Congenital nasolacrimal duct obstruction can be a consequence of failure of canalization during embryological development. Failed resolution of obstruction by a young age prompts an attempt at probing of the lacrimal system. In cases where probing has proven unsuccessful, otolaryngologists are often asked to perform dacrocystorhinostomy to relieve the distal obstruction. This is best managed as a collaborative effort between the otolaryngologist and the ophthalmologist. Endoscopic dacrocystorhinostomy has proven to be a safe and effective method of approach for the
A.B. Friedman et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 269–271
otolaryngologist. This case report describes the rare finding of complete agenesis of the nasolacrimal duct in an otherwise healthy child and its successful management with endoscopic DCR. References [1] M.K. Kapadia, S.K. Freitag, J.J. Woog, Evaluation and management of congenital nasolacrimal duct obstruction, Otolaryngol. Clin. N. Am. 39 (2006) 959–977.
271
[2] S.J. Ahn Yuen, C. Oley, T.J. Sullivan, Lacrimal outflow dysgenesis, Ophthalmology 111 (2004) 1782–1790. [3] A.A. McNabb, Congenital absence of the nasolacrimal duct, J. Pediatr. Ophthalmol. Strabismus 35 (1998) 294–295. [4] V.C. Prabhakaran, G. Davis, P.J. Wormald, D. Selva, Congenital absence of the nasolacrimal duct in velocardiofacial syndrome, J. Am. Assoc. Pediatr. Ophthalmol. Strabismus 12 (2008) 85–86. [5] P. Eloy, E. Leruth, A. Cailliau, S. Collet, B. Bertrand, P. Rombaux, Pediatric endonasal endoscopic dacryocystorhinostomy, Int. J. Pediatr. Otorhinolaryngol. 73 (2009) 867–871.