Initial management of congenital canalicular atresia Mohamed Soliman, MD, and Gregg T. Lueder, MD PURPOSE METHODS
To evaluate the initial management of pediatric patients with lacrimal canalicular atresia (CA) involving the upper or lower eyelid. The medical records of 15 children with symptoms of nasolacrimal duct (NLD) obstruction and CA of either the upper or lower eyelid treated from 1994 to 2014 were retrospectively reviewed. Surgical treatment consisted of nasolacrimal probing through the patent canaliculus. Preoperative findings, treatment, and outcomes were reviewed. Outcomes were considered good if the patients had resolution of signs and symptoms of lacrimal obstruction, fair if the symptoms improved and additional surgery was not required, and poor if additional lacrimal surgery was performed.
RESULTS
CA was found in 19 eyes of 15 patients: 11 eyes had upper CA; 8 eyes, lower. All 11 eyes with upper CA improved after NLD probing through the patent lower canaliculus. Of the 8 eyes with lower CA, 4 (50%) had good outcomes and 4 eyes had poor outcomes after NLD probing through the patent upper canaliculus. All of the latter patients improved after subsequent treatment with balloon catheter dilation (BCD), monocanalicular stent placement, or both.
CONCLUSIONS
If a patient is found to have upper CA during initial surgery for NLD obstruction, NLD probing through the patent lower canaliculus has a good rate of surgical success. For patients with lower CA, the success rate of NLD probing through the upper canaliculus appears to be lower. Additional treatment during the initial surgery with either BCD or monocanalicular stent placement should be considered in these patients. ( J AAPOS 2015;19:220-222)
C
analicular atresia (CA) is an uncommon congenital anomaly affecting the lacrimal drainage system, estimated to occur in approximately 4% to 13% of patients presenting for management of lacrimal disorders.1,2 In children it may be difficult to recognize during office examination. In the authors’ experience, it is most commonly identified during surgery for nasolacrimal duct (NLD) obstruction. Few studies have reported the management and outcomes of patients with CA, particularly if only one eyelid is affected. This study examined our experience treating children with this disorder with the goal of improving management of this abnormality.
Subjects and Methods The Washington University School of Medicine Institutional Review Board approved this study, which was compliant with the US
Author affiliations: Departments of Ophthalmology and Visual Sciences and Pediatrics, St. Louis Children’s Hospital, Washington University School of Medicine, St. Louis, Missouri Presented as a poster at the 39th Annual Meeting American Association for Pediatric Ophthalmology and Strabismus, Boston, Massachusetts, April 3-7, 2013. Submitted October 27, 2014. Revision accepted February 26, 2015. Correspondence: Gregg T. Lueder, MD, St. Louis Children’s Hospital (Rm 2s89), Washington University School of Medicine, One Children’s Place, St. Louis, MO 63110 (email:
[email protected]). Copyright Ó 2015 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2015.02.009
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Health Insurance Portability and Accountability Act of 1996. The medical records of patients treated between 1994 and 2014 for NLD obstruction and who were found to have upper or lower canalicular atresia were retrospectively reviewed. Initial evaluation included a comprehensive medical history and ophthalmologic examination. Patients were excluded if both the upper and lower canaliculi were atretic, if they had systemic disorders associated with lacrimal obstruction (Trisomy-21, ectodermal dysplasia, craniofacial abnormalities), previous NLD surgery, or if no follow-up information was available. Surgery was performed under anesthesia and consisted of dilation of the patent lacrimal puncta, passage of Bowman probes through the patent canaliculus into the distal lacrimal system, and irrigation of fluid following probing. Preoperative symptoms, age at surgery, operative findings, and postoperative outcomes were analyzed. Follow-up was obtained by office evaluation or telephone interviews with the patients’ caregivers. Outcomes were considered good if signs and symptoms of NLD obstruction resolved, fair if there was improvement in symptoms and additional surgery was not necessary, and poor if there was no improvement.
Results A total of 15 patients with lacrimal canalicular atresia who presented for evaluation and management of NLD obstruction were identified. Of these, 10 patients had symptoms of epiphora and recurrent periocular crusting and 5 had primarily epiphora with absent or minimal
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Volume 19 Number 3 / June 2015 periocular crusting. The mean age at the time of surgery was 16.5 months (range, 6-35 months). Eleven eyes had upper CA, and 8 eyes had lower CA. The left eye was affected in 7 patients, the right eye in 4 patients, and both eyes in 4 patients. Probing was performed successfully through the patent canaliculus in all patients. Typical membranous obstruction of the distal duct was palpable and relieved with probing in 18 eyes. Bony stenosis was present in one eye with lower CA. This patient had persistent symptoms and subsequently underwent BCD followed by stent placement. Follow-up duration after surgery ranged from 2 weeks to 84 months. For patients with upper CA, good outcomes occurred in 9 of 11 eyes (82%) and fair outcomes in 2 of 11 eyes (18%). None of these patients underwent additional lacrimal surgery. For patients with lower CA, good outcomes occurred in 4 of 8 eyes (50%) and poor outcomes in 4 of 8 (50%) eyes following probing through the patent upper canaliculus. The persistent symptoms were present immediately after the first surgery. Of the latter 4 eyes with poor outcomes, 2 had good outcomes following BCD through the patent upper canaliculus. The 2 patients with persistent symptoms following BCD were treated with monocanalicular stent placement. One had a good outcome and 1 had a fair outcome. The patient with the fair outcome had bilateral CA. The outcome of the opposite eye, with upper CA, was good following initial NLD probing.
Discussion The lacrimal drainage system derives embryologically from invagination of a solid core of ectodermal tissue into the nasolacrimal groove during the fifth week of gestation. This tissue normally canalizes to form the lacrimal canaliculi, lacrimal sac, and nasolacrimal duct. Incomplete canalization of the distal NLD is a common finding in normal newborns, resulting in symptoms of NLD obstruction, which usually spontaneously resolve during the first year of life. If NLD obstruction does not improve, surgical probing of the NLD may be necessary. Lack of formation of the canalicular system is an uncommon abnormality characterized by absence of a normal punctum and canalicular tissue. Because of the difficulty involved in examining young children and the small size of the eyelids, punctal and canalicular atresia may not be identified during an office evaluation. It is often not recognized until the time of surgery for NLD obstruction. Few studies have evaluated the initial management of children with canalicular atresia, particularly if only the upper or lower eyelid is involved. Canalicular stenosis may develop as an acquired condition secondary to trauma, topical medication, and inflammatory disorders. Previous reports describing the management of canalicular stenosis include patients with acquired stenosis and patients with congenital atresia of both the upper and lower eyelid as well as patients with single eyelid involvement. Atresia of both the upper and lower eyelids is rare, usually requiring treatment with dacryocys-
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torhinostomy (DCR) and placement of Jones tubes or lacrimal stents.3-5 The current study does not include patients with atresia of both the upper and lower eyelids, nor patients with acquired canalicular stenosis. Previous recommendations for treatment for CA of only one eyelid have included complicated procedures such as DCR with retrograde probing of the canaliculus through an opening in the lacrimal sac.6 Beard7 described attempts to locate patent canalicular tissue by making incisions at the eyelid margin between the normal location of the punctum and the medial canthus but indicated that this was almost never successful. In a group of children with canalicular atresia who had previous NLD probing, Cahill and Burns8 recommended injection of methylene blue dye or placement of a pigtail probe through the patent canaliculi to identify canalicular tissue on the involved eyelid, with opening of the lacrimal sac and retrograde probing and stent placement if the initial steps were unsuccessful. In the largest study identified in a literature review, Lyons and colleagues9 reported 94 eyes of 57 patients with punctal agenesis. Both puncta were absent in 53 eyes and one punctum was absent in 41 eyes. Sixteen of these patients were \5 years of age, similar to the number of patients in our study. The children with single punctum involvement were treated with NLD probing if distal obstruction was identified and DCR if probes could not be passed. However, the outcomes of surgery were not described by age in this report. To our knowledge, treatment outcomes for children with congenital CA treated primarily with NLD probing through the patent canaliculus have not been previously reported. It is important to distinguish true canalicular atresia from an obstructive membrane overlying a normal punctum. If an otherwise normal-appearing punctum is covered by a membrane, opening of the membrane with an instrument, such as a punctal dilator, is usually curative, unless distal obstruction of the NLD is also present. If no punctal tissue is visible (Figure 1), true canalicular atresia is almost always present. In Lyons and colleauges’ study,9 dacryocystography was performed on all children with punctal atresia of a single eyelid, all of whom were found to have agenesis of the canaliculi. This finding explains why attempts to locate canalicular tissue by incising the eyelid margin are almost never successful.7 The type of symptoms associated with lacrimal disorders may provide clues to their cause. Patients with NLD obstruction present with recurrent periocular discharge due to bacterial infection in the lacrimal sac, epiphora due to mechanical blockage of the distal NLD, or both. Patients with atresia of both the upper and lower canaliculi present with symptoms of epiphora only, because bacteria do not have access to the lacrimal sac. Therefore, the presence of epiphora alone should raise the clinician’s suspicion for the presence of CA. However, because NLD obstruction is very common and CA is rare, most children with symptoms of epiphora alone have typical NLD obstruction rather than CA. On the other hand, the presence of recurrent periocular discharge indicates that there is a connection between
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FIG 1. Upper punctal atresia, surgeon’s view, left upper eyelid. Note absence of any normal-appearing punctal tissue.
the eyelids and lacrimal sac, and further indicates that there is obstruction of the distal NLD beyond the lacrimal sac. Therefore, if symptoms of recurrent discharge are present in a patient with CA, this implies that the patient also has obstruction of the distal duct. The findings of this study indicate that probing of the distal NLD obstruction through the patent canaliculus is an appropriate first treatment for children with symptoms of NLD obstruction who are found to have CA during surgery. Although the number of patients is small, we found a difference in success between patients with upper and lower obstruction. The success rate in patients with upper CA was 82%, which is in the same range as that for NLD probing in children without CA.10 This suggests that upper CA does not contribute to the symptoms of epiphora. However, the success rate of surgery was only 50% (4/8) in patients with lower CA when NLD probing was performed through the upper canaliculus. Of note, 1 patient had upper CA in one eye and lower CA in the other and had a good outcome following initial probing of the eye with upper CA but only a fair outcome following BCD and monocanalicular stent placement in the eye with lower CA. The other 3 patients with lower CA and persistent symptoms after NLD probing had good outcomes following additional procedures through the upper canaliculus (BCD and monocanalicular stents), indicating that distal NLD obstruction was still the primary cause of the symptoms. The primary limitations of the study are the small number of patients and the retrospective design. A collaborative study could provide more data, but would likely be difficult given the rarity of this abnormality. It is not clear why there was a difference in outcomes between patients with upper and lower CA. In a study by Linberg and Moore,11 normal volunteers reported symptoms of epiphora after temporary occlusion of either the upper or lower puncta, and there was no difference between the two groups.11 Similarly, Daubert and colleagues12 found no difference in tear flow studied by lacrimal scintillography after occlusion of either the upper or lower punctum. Because there appears to be no difference in tear flow between the upper and lower systems, this suggests that there
Volume 19 Number 3 / June 2015 may an anatomic reason for the different outcomes between upper and lower CA. Sevel13 described the embryologic development of the lacrimal apparatus and found that canalization occurs throughout the entire system at the same time. It is possible that a disturbance of this canalization process is more severe if it includes the lower canaliculus, although this cannot be determined in the present study. In conclusion, if a child with NLD obstruction is found to have CA during initial NLD surgery, probing through the patent canaliculus is recommended as a first step. More involved procedures, such as attempts to localize canalicular tissue by incisions along the lid margin or DCR, are not indicated at the time of the first surgery. If the child has upper CA, probing alone through the patent lower system has a high probability of success. Because our findings suggest that the prognosis is poorer for patients with lower CA, additional procedures such as BCD or monocanalicular stent placement through the patent upper canaliculus should be considered during the initial procedure.
Literature Search A MEDLINE search was performed using the terms canalicular atresia, punctal atresia, canalicular agenesis, and punctal agenesis. References 1. Welham RAN, Hughes SM. The Results of Pediatric Lacrimal Surgery. In: Bosniak SL, Smith BC, eds. Advances in Ophthalmic Plastic and Reconstructive Surgery. Vol. 3. The Lacrimal System. New York: Pergamon Press; 1984:87-92. 2. Nordlow W, Vennerholm I. Congenital atresia of the lacrimal passages: their occurence and treatment. Acta Ophthalmol 1953;31:367-71. 3. Jones LT. An anatomical approach to problems of the eyelids and lacrimal apparatus. Arch Ophthalmol 1961;66:111-24. 4. Katowitz JA. Silicone tubing in canalicular obstructions. A preliminary report. Arch Ophthalmol 1974;91:459-62. 5. LaPiana FG. Management of occult atretic lacrimal puncta. Am J Ophthalmol 1972;74:332-3. 6. Jones LT. The cure of epiphora due to canalicular disorders, trauma and surgical failures on the lacrimal passages. Trans Am Acad Ophthalmol Otolaryngol 1962;66:506-24. 7. Beard C. Congenital and hereditary abnormalities of the eyelids, lacrimal system and orbit. Symposium on Surgical and Medical Management of Congenital Anomalies of the Eye: Transactions of the New Orleans Academy of Ophthalmology. St. Louis: C.V. Mosby; 1968:377-421. 8. Cahill KV, Burns JA. Management of epiphora in the presence of congenital punctal and canalicular atresia. Ophthal Plast Reconstr Surg 1991;7:167-72. 9. Lyons CJ, Rosser PM, Welham RAN. The management of punctal agenesis. Ophthalmology 1993;100:1851-5. 10. Pediatric Eye Disease Investigator Group. A randomized trial comparing the cost-effectiveness of 2 approaches for treating unilateral nasolacrimal duct obstruction. Arch Ophthalmol 2012;130: 1525-33. 11. Linberg JV, Moore CA. Symptoms of canalicular obstruction. Ophthalmology 1988;95:1077-9. 12. Daubert J, Nik N, Chandeyssoun PA, el-Choufi L. Tear flow analysis through the upper and lower systems. Ophthal Plast Reconstr Surg 1990;6:193-6. 13. Sevel D. Development and congenital abnormalities of the nasolacrimal apparatus. J Pediatr Ophthalmol Strabismus 1981;18:13-19.
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