eMedicine from WebMD
 

eMedicine Specialties > Ophthalmology > Lacrimal System

Nasolacrimal Duct, Congenital Anomalies

Mounir Bashour, MD, CM, FRCS(C), PhD, FACS, Assistant Professor of Ophthalmology, McGill University; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD

Updated: Jun 8, 2009

Introduction

Background

The congenital problems that can affect the nasolacrimal system are outlined below.

Dacryostenosis

A very common condition in which the extreme end of the nasolacrimal duct underneath the inferior turbinate fails to complete its canalization in the newborn period and may produce clinical symptoms in 2-4% of newborns.

Absence of valves

The fold that is normally present at the end of the nasolacrimal duct or the valve of Hasner may be absent, in which case, pneumatoceles of the sac may occur and nose blowing may cause retrograde passage of air. If the valve of Rosenmüller also is absent, it is possible to blow air from the nose into the eye, and nosebleeds may produce bloody tears.

Anomalies of the sac

Although diverticulum of the lacrimal sac may occur, a congenital fistula of the lacrimal sac, which has been termed lacrimal anlage duct by Jones, is more common.

Anomalies of the puncta

Congenital atresia, supernumerary or double puncta, and congenital slits of the puncta all may occur. Lateral displacement of the puncta may occur in some congenital syndromes, such as blepharophimosis.

Anomalies of the canaliculi

Atresia or failure of canalization of the lacrimal canaliculi may occur in conjunction with punctal atresia.

Pathophysiology

Canalization of the nasolacrimal duct system usually is complete by the eighth month of gestation; problems in this normal developmental process can cause any of the above anomalies.

Frequency

United States

Nasolacrimal obstruction occurs in 2-4% of newborns. Of those patients with serious nasolacrimal obstruction (nonresponsive to 2 or more probing procedures, with or without intubation), 35% have nasolacrimal duct obstruction, 15% have punctal agenesis, 10% have congenital fistulas, and 5% have craniofacial defects.

International

Nasolacrimal obstruction occurs in 2-4% of newborns.

Recent studies show the incidence of nasolacrimal obstruction in children with Down syndrome is likely between 22%1 and 36%.2

Mortality/Morbidity

Congenital nasolacrimal anomalies, particularly obstruction, can lead to various clinical manifestations, to include the following:

  • Amniotocele: This condition occurs in neonates as a distention in the lacrimal sac. Amniotic fluid enters the sac, is retained by a nonpatent nasolacrimal duct, and is trapped in the sac by the valve at the common canaliculus, the valve of Rosenmüller. Probing the nasolacrimal duct as an office procedure usually is curative.
  • Dacryocystitis (acute mucocele or pyocele): This condition exhibits acute distention and inflammation in the lacrimal sac region and may occur in the neonatal period. Probing is necessary in newborns with acute dacryocystitis to establish drainage as soon as possible. This procedure is performed with topical local anesthesia only.
  • Tearing and mattering: Newborns who have congenital dacryostenosis may not develop acute dacryocystitis with a mucocele or pyocele of the sac in the early neonatal period but may simply have tearing with a chronic mucopurulent discharge, which usually manifests at 2 weeks. Topical antibiotics should be administered, and the parents must be instructed in the proper technique of lacrimal sac compression and massage. More than 90% of these cases clear and become asymptomatic with conservative management. Under normal circumstances, these children with mild-to-moderate symptoms of epiphora and lid crusting can be monitored for the first year of life without serious consequence or sequela. It is rarely necessary to make probing mandatory at an early age (eg, before 6 mo). A number of studies have now confirmed that probing or silicone tube intubation in children after 12 months still has a very high success rate. These techniques are discussed in Treatment.

Race

No quoted difference in racial incidence of congenital nasolacrimal system abnormalities exists.

Sex

No sexual difference exists.

Age

Nasolacrimal obstruction occurs in 2-4% of newborns.

Clinical

History

  • Perinatal and pregnancy history
  • Family history of nasolacrimal duct problems, congenital glaucoma, or other congenital anomalies, especially facial (eg, cleft lip/palate)
  • History of other birth defects
  • Pediatric review of systems
  • To rule out congenital glaucoma, ask for history of tearing and photophobia, as well as increasing corneal size and clouding.

Physical

  • A complete ophthalmic assessment must be performed.
    • A dye disappearance test is probably the single most useful test.
    • Place a drop of fluorescein in each eye, and monitor with a cobalt blue light; if a pool is still present after 5 minutes, the test is positive, and the baby likely has some type of obstruction in the nasolacrimal system.
  • Measure corneal diameter, and, if suspicious, consider performing an intraocular pressure measurement. (See Glaucoma, Primary Congenital.)

Causes

  • Usually, these anomalies are sporadic, but genetics, prematurity, and maternal drug use can be possible influencing factors.
  • Ocular abnormalities are present in 20% of patients, and systemic abnormalities are present in almost 25% of patients with serious congenital nasolacrimal duct anomalies.

Differential Diagnoses

Albinism
Down Syndrome
Aniridia
Glaucoma, Primary Congenital
Aniridia in the Newborn
Conjunctivitis, Neonatal
Corneal Abrasion

Other Problems to Be Considered

Facial clefts
Cleft palate
Treacher Collins syndrome

Workup

Imaging Studies

  • Intubation dacryocystography
  • Scintillography

Other Tests

  • Fluorescein dye disappearance test - Grade as good, fair, or poor clearance
  • Jones dye tests - Not as useful in practice with small children

Histologic Findings

Punctal agenesis is usually associated with the absence of underlying canalicular tissue in 86% of cases in one series.

Treatment

Medical Care

Congenital nasolacrimal duct obstructions spontaneously resolve in 90% of cases during the first year of life. Some physicians have advocated massage with digital pressure as an aid to speeding up this natural resolution. Other than massage, topical antibiotics are useful for mucopurulent discharge, but the only treatment of efficacy for those patients who do not resolve spontaneously is surgery.

Surgical Care

  • Probing: Probing cures 95% of congenital nasolacrimal obstructions. Prognosis for probing decreases exponentially with the increasing number of probings and the age of the patient. Rarely, it is successful after the third time or after 3 years.
  • Nasolacrimal intubation: It has been advocated as an alternate procedure to dacryocystorhinostomy (DCR) in children who have failed probing.
    • Success rates of 80-95% have been reported, but most patients have only been probed twice or less and are younger than 2 years. Prognosis is poor for those patients with previous dacryocystitis and for those patients in which an obstruction is encountered during the procedure.
    • Lim et al noted that increasing the duration of intubation was not associated with increasing the chance of success but with a significantly higher risk of failure if longer than 18 months (P=0.03).3 The retention of stents for longer than 12 months was associated with a significantly lower success rate (67%). The presence of Down syndrome, older age at the time of surgery, and gender of the patient were not predictive factors for treatment failure. The unplanned removal of the tubes because of dislodgement was the most common complication, occurring in 25% of eyes, but did not affect the functional outcome.
  • Balloon catheter dilatation of the nasolacrimal system with or without silicone tubing: This procedure has slightly better results than intubation alone. Most probing failures occur as a result of upper sac or mid duct obstructions and are not amenable to cure by instrumentation. Repeat probing procedures and intubation can cause serious complications, including false passages, canalicular scarring, and stenosis.
  • DCR: This treatment is the criterion standard when a patent canalicular system is present. See Nasolacrimal Duct, Obstruction.
  • Conjunctival dacryocystorhinostomy: If the upper system is scarred or otherwise not amenable to opening, then it can be bypassed using a prosthesis, such as a Lester-Jones tube. This procedure probably should be avoided until the child is older than 10 years because the prosthesis does require care from the patient and often has minor complications and revisions. In punctal agenesis where no canalicular tissue can be identified, the insertion of a Lester-Jones tube is necessary.

Consultations

Pediatrician, genetics counselor, or maxillofacial surgeon

Follow-up

Further Outpatient Care

  • Patients should receive follow-up care as needed.

Complications

  • Bleeding: Serious bleeding is rare, occurring in only 1-2% of surgeries or postoperatively.
  • Surgical failures: In these complicated conditions, a 10% rate of failure occurs.
  • Wound infections: These occur in 5-10% of patients, usually as wound abscesses on the fourth postoperative day.
  • Silicone or polyethylene tubing complications: These complications occur in about 15% of cases and include the following: corneal abrasion, pyogenic granuloma, low-grade infection, chronic nasal irritation and congestion, epistaxis, sinusitis, and pharyngitis.
  • Bypass tube complications: These frequently occur in at least 40% of patients postoperatively and include tube loss or migration and tube obstruction.
  • Anesthesia complications: In children, these complications are more frequent due to drugs, blood loss, malignant hyperthermia, and pseudocholinesterase deficiency.

Prognosis

  • Overall prognosis is excellent. More than 90% of children with these developmental anomalies have improved subjective tearing postoperatively.

Miscellaneous

Medicolegal Pitfalls

  • Missing serious associated anomalies, especially congenital glaucoma

Special Concerns

  • Patients with congenital punctal agenesis of both puncti almost always (>90%) have associated canalicular agenesis and, as a result, require conjunctival dacryocystorhinostomy usually delayed until 10 years.

References

  1. Fimiani F, Iovine A, Carelli R, Pansini M, Sebastio G, Magli A. Incidence of ocular pathologies in Italian children with Down syndrome. Eur J Ophthalmol. Sep-Oct 2007;17(5):817-22. [Medline].

  2. Stephen E, Dickson J, Kindley AD, Scott CC, Charleton PM. Surveillance of vision and ocular disorders in children with Down syndrome. Dev Med Child Neurol. Jul 2007;49(7):513-5. [Medline].

  3. Lim CS, Martin F, Beckenham T. Nasolacrimal duct obstruction in children: outcome of intubation. J AAPOS. Oct 2004;8(5):466-72. [Medline].

  4. Bowling BS, Chandna A. Superior lacrimal canalicular atresia and nasolacrimal duct obstruction in the CHARGE association. J Pediatr Ophthalmol Strabismus. Sep-Oct 1994;31(5):336-7. [Medline].

  5. Busse H. [Connatal dacryostenoses. Clinical picture and treatment]. Ophthalmologe. Sep 2004;101(9):945-54; quiz 955-6. [Medline].

  6. Cahill KV, Burns JA. Management of epiphora in the presence of congenital punctal and canalicular atresia. Ophthal Plast Reconstr Surg. 1991;7(3):167-72. [Medline].

  7. Chronister CL, Lee A, Kaiser H. Rarely reported cases of congenital atresia of nasolacrimal puncta. Optometry. Apr 2002;73(4):237-42. [Medline].

  8. Foster JA, Katowitz JA, Heyman S. Results of dacryoscintigraphy in massage of the congenitally blocked nasolacrimal duct. Ophthal Plast Reconstr Surg. Mar 1996;12(1):32-7. [Medline].

  9. Holzberg N, Ward RF. Bilateral congenital dacrocystoceles. Otolaryngol Head Neck Surg. Dec 1993;109(6):1074-7. [Medline].

  10. Ingels K, Kestelyn P, Meire F, Ingels G, Van Weissenbruch R. The endoscopic approach for congenital nasolacrimal duct obstruction. Clin Otolaryngol Allied Sci. Apr 1997;22(2):96-9. [Medline].

  11. Lyon DB, Dortzbach RK, Lemke BN, Gonnering RS. Canalicular stenosis following probing for congenital nasolacrimal duct obstruction. Ophthalmic Surg. Apr 1991;22(4):228-32. [Medline].

  12. Lyons CJ, Rosser PM, Welham RA. The management of punctal agenesis. Ophthalmology. Dec 1993;100(12):1851-5. [Medline].

  13. McNab AA. Congenital absence of the nasolacrimal duct. J Pediatr Ophthalmol Strabismus. Sep-Oct 1998;35(5):294-5. [Medline].

  14. Paul TO, Shepherd R. Congenital nasolacrimal duct obstruction: natural history and the timing of optimal intervention. J Pediatr Ophthalmol Strabismus. Nov-Dec 1994;31(6):362-7. [Medline].

  15. Sevel D. Development and congenital abnormalities of the nasolacrimal apparatus. J Pediatr Ophthalmol Strabismus. Sep-Oct 1981;18(5):13-9. [Medline].

  16. Ugurbas SH, Zilelioglu G. Congenital lacrimal fistula. Eur J Ophthalmol. Jan-Mar 2000;10(1):22-6. [Medline].

  17. Welham RA, Hughes SM. Lacrimal surgery in children. Am J Ophthalmol. Jan 15 1985;99(1):27-34. [Medline].

Keywords

nasolacrimal duct obstruction, nasolacrimal duct system, congenital nasolacrimal duct anomalies, nasolacrimal duct system abnormalities

Contributor Information and Disclosures

Author

Mounir Bashour, MD, CM, FRCS(C), PhD, FACS, Assistant Professor of Ophthalmology, McGill University; Clinical Assistant Professor of Ophthalmology, Sherbrooke University; Medical Director, Cornea Laser and Lasik MD
Mounir Bashour, MD, CM, FRCS(C), PhD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American College of International Physicians, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Mechanical Engineers, American Society of Ophthalmic Plastic and Reconstructive Surgery, Biomedical Engineering Society, Canadian Medical Association, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, International College of Surgeons US Section, Ontario Medical Association, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

Further Reading

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)