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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 57  |  Issue : 3  |  Page : 243-244

A Case of bilateral agenesis of nasolacrimal ducts with posterior lenticonus


Department of Orbit, Oculoplasty, Reconstructive and Aesthetics, Sankara Nethralaya, Tamil Nadu, India

Date of Submission10-Aug-2019
Date of Decision17-Sep-2019
Date of Acceptance17-Sep-2019
Date of Web Publication11-Nov-2019

Correspondence Address:
Dr. Bipasha Mukherjee
18 College Road, Chennai - 600 006, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_71_19

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  Abstract 


Congenital absence of nasolacrimal ducts is a rare presentation of nasolacrimal obstruction in children, but a frequent cause of failure of probing, especially if endoscopic examination is not undertaken. We report a rare association of bilateral absence of nasolacrimal ducts along with bilateral visually significant developmental cataracts with posterior lenticonus in a 1-year-old female child with dysmorphic features.

Keywords: Agenesis of nasolacrimal duct, pediatric dacryocystorhinostomy, posterior lenticonus


How to cite this article:
Nivean PD, Mukherjee B. A Case of bilateral agenesis of nasolacrimal ducts with posterior lenticonus. TNOA J Ophthalmic Sci Res 2019;57:243-4

How to cite this URL:
Nivean PD, Mukherjee B. A Case of bilateral agenesis of nasolacrimal ducts with posterior lenticonus. TNOA J Ophthalmic Sci Res [serial online] 2019 [cited 2019 Dec 12];57:243-4. Available from: http://www.tnoajosr.com/text.asp?2019/57/3/243/270696




  Introduction Top


Primary nasolacrimal duct obstruction is the most common congenital abnormality of the lacrimal system. This usually happens due to the failure of the membranous valve of Hasner to open normally after birth. It may be associated with other systemic abnormalities such as Down's syndrome, craniosynostosis, Goldenhar syndrome, clefting syndromes, hemifacial microsomia, or midline facial anomalies. Complete absence of the nasolacrimal duct, on the other hand, is relatively rare. We report a case of bilateral absent nasolacrimal duct associated with bilateral cataract and facial dysmorphism.


  Case Report Top


Parents of a 1-year-old girl reported to us with complaints of poor vision and white reflex in both her eyes since birth. In addition, the parents had also noticed watering with discharge in both the eyes for the same duration. The child had undergone probing twice elsewhere without any relief in her symptoms. On examination, the child had dysmorphic facies with hooked nose and midfacial flattening [Figure 1]a and [Figure 1]b. She could fix and follow light. There was horizontal jerk nystagmus of moderate amplitude and frequency. Anterior segment examination showed bilateral dense cataracts [Figure 1]c. Examination with handheld slitlamp revealed posterior lenticonus with posterior capsular dehiscence. Regurgitation on pressure over the lacrimal sac was positive in both the eyes. Fundus examination was not possible due to dense media opacity. Ultrasound B scan showed anechoic vitreous cavity with attached retina. In view of visually significant cataracts with clinical diagnosis of congenital nasolacrimal duct obstruction (CNLDO), probing of the nasolacrimal passages was scheduled under general anesthesia. The probe met with bilateral bony obstructions. Nasal endoscopic examination was done in the same sitting which revealed absence of nasolacrimal duct in both sides; hence, bilateral endoscopic dacryocystorhinostomy (DCR) was done before cataract surgery. Following the procedure, syringing was patent on both sides. Subsequently, the patient underwent uneventful lens aspiration, primary posterior capsulotomy, anterior vitrectomy, and posterior-chamber intraocular lens insertion in both the eyes sequentially.
Figure 1: (a) Clinical picture of patient showing discharge and matting of eyelashes (OS >OD). (b) Dysmorphic features with beaked nose and flattening of the malar prominence is seen. (c) Bilateral visually significant cataracts

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On follow-up, syringing was freely patent in both the eyes. She was prescribed glasses and advised part time occlusion of the right eye. In view of dysmorphism, she also underwent evaluation by dysmorphologist and karyotyping which did not reveal any abnormal pattern.


  Discussion Top


CNLDO is the most common cause for epiphora in young children.[1] In 88%–96% of these infants, spontaneous resolution occurs before the age of 1 year.[2] Patients with Down's syndrome are commonly associated with CNLDO (22%–36%). Secondary causes of NLDO include trauma, lacrimal sac tumors, and craniofacial anomalies.

Abnormalities in the normal embryological development of the lacrimal system are responsible for CNLDO. The lacrimal system arises from first pharyngeal pouch; hence, CNLDO may be associated with ear, uvula and palate abnormalities. In a study by Uǧurbaş et al., otitis media with uveal effusion was found to be the most common association.[3] They also reported external ear abnormalities, uvula bifida, and palatal abnormalities in the same cohort of patients.

Velocardiofacial syndrome has been associated with absent nasolacrimal duct (NLD).[4] This syndrome is due to deletion of the short arm of 22nd chromosome. Patients have typical facies, cardiac, palatal, and speech abnormalities along with learning disabilities. Ophthalmic abnormalities have been reported in 70% of patients which include bilateral cataract, small optic discs, and tortuous vessels.

Our patient had dysmorphic facies with absent nasolacrimal ducts and congenital cataracts. Velocardiofacial syndrome was considered as a differential diagnosis. However, karyotyping did not reveal any abnormality and the child was free from any cardiac complications. The child had elevated antibody titers for toxoplasma, cytomegalovirus, and rubella virus. Literature search did not reveal any association between these elevated titers and congenital absence of NLD.

Very few infants with CNLDO require surgery beyond probing. Nasolacrimal duct agenesis should be considered in patients with CNLDO and unsuccessful repetitive probing.[5] DCR may be considered if there is a need for urgent intraocular surgery or a history of acute attack of dacryocystitis. External DCR is technically difficult in young children because lacrimal sac fossa and anterior lacrimal crest are poorly defined. In our case, surgical intervention was inevitable in view of bilateral significant cataracts and bony block in the lacrimal system. The success rates of pediatric external DCR range from 89% to 97.5% while that of pediatric endoscopic DCR ranges from 58% to 100%.[6]

High index of suspicion should be maintained for absent or abnormal nasolacrimal duct in children with dysmorphic features and craniofacial anomalies. DCR may be considered as the first line of management in patients with craniofacial anomalies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bhandari S, Sengupta S, Yadalla D, Rajagopalan J, Velis GB, Talele D, et al. Factors affecting treatment outcome in congenital nasolacrimal duct obstruction: A retrospective analysis from South India. Indian J Ophthalmol 2015;63:759-62.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Yagci A, Karci B, Ergezen F. Probing and bicanalicular silicone tube intubation under nasal endoscopy in congenital nasolacrimal duct obstruction. Ophthalmic Plast Reconstr Surg 2000;16:58-61.  Back to cited text no. 2
    
3.
Uǧurbaş SH, Zilelioǧlu G, Saatçi M. Otolaryngological findings in congenital nasolacrimal duct obstruction and implications for prognosis. Br J Ophthalmol 2000;84:917-8.  Back to cited text no. 3
    
4.
Prabhakaran VC, Davis G, Wormald PJ, Selva D. Congenital absence of the nasolacrimal duct in velocardiofacial syndrome. J AAPOS 2008;12:85-6.  Back to cited text no. 4
    
5.
Coban DT, Beden U, Sonmez B, Elmali M, Erkan D. Unsuccessful probing and nasolacrimal canal agenesis in congenital epiphora. Orbit 2010;29:363-6.  Back to cited text no. 5
    
6.
Ali MJ. Pediatric dacryocystorhinostomy. Indian J Ophthalmol 2017;65:1008-9.  Back to cited text no. 6
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