Willard Bwalya Mumbi1, Brian Ng’andwe2 and Ivan P. Hwang3
1Eye Hospital, University Teaching Hospitals – Lusaka, Zambia.
2Faculty of Ophthalmology, Department of Surgery, School of Medicine, University of Zambia, Lusaka, Zambia
3California Clinic, California, United States of America
Abstract
Congenital lacrimal cutaneous fistula is a rare condition but a significant cause of epiphora in children. Many cases might be asymptomatic and may require no treatment however some might need surgical intervention owing to the high burden of recurrent infections and epiphora associated with the disease. Thus, this article reports the first case of congenital lacrimal cutaneous fistula at the University Teaching Hospitals – Eye Hospital (UTHs–EH) in Lusaka, Zambia and its management in a resource limited setting. A 3-year-old female presented with a two months history of epiphora and an opening on the lower aspect of the medial canthus of the left eye. The rest of the history was otherwise unremarkable. Physical examination revealed a prominent orifice noted on the inferomedial aspect of the medial canthal angle of the left eye and a negative ROPLAS sign. The opening was noted to be discharging a watery discharge. The rest of the anterior and posterior segment examination was normal. Examination under anaesthesia revealed a patent lower canaliculus as well as nasolacrimal pathway. A simple excision(fistulectomy) and closure of the fistula was done successfully. The immediate postoperative period was uneventful. 1-week post-operative the child was noted to have developed an ocular infection which was successfully treated. Asymptomatic cases of lacrimal fistula might be managed conservatively. Simple surgical excision of lacrimal cutaneous fistula may be sufficient to treat symptomatic cases.
Introduction
Congenital lacrimal cutaneous fistula is a rare developmental condition in which a normally epithelized tract connects the skin to the common canaliculus, lacrimal sac or nasolacrimal duct [1].
The incidence of congenital lacrimal fistula is reported globally to occur in one in 2000 births with no sex predilection [2]. Most are unilateral and are located inferonasal to the medial canthus. These fistulae may be asymptomatic and therefore overlooked for some time after birth. In some cases, it may be difficult to ascertain whether a fistula has been present since birth and remained unnoticed or has arisen later as a result of infection or surgical intervention. Symptoms may be of tearing from the ostium of the fistula or from the eye or both depending on whether there is also nasolacrimal obstruction.
This article presents the first case of lacrimal fistula in our setting and we present its management in a resource limited setting like ours.
Case scenario
A 3-year-old female presented to the UTHs–EH in Lusaka, Zambia with a 2 months history of an opening on the nasal side of the left lower eyelid and watering through that same opening. There was no associated history of trauma and past ocular history. Birth history and past medical history were otherwise unremarkable. The visual acuity was 6/6 in both eyes and the fundus was normal.
Adnexa Examination
No facial asymmetry and no dysmorphic features. Fistula noted inferomedially on the left medial canthal angle as shown in fig 1.
Fig 1: arrow showing a depression inferomedially
Management
Intra-operatively, probing and irrigation was done in our patient which demonstrated a patent nasolacrimal pathway. The patient thus received a simple excision and closure of the fistula, fig 2.
Fig 2: Immediate post operatively showing no tearing
On week 1 review, the patient had progressed well and was healing well (Fig 3). The child remains asymptomatic 3 months after the operation and we will keep following up since some literature reports high recurrence rates with simple excision.
Fig 3: One-week post operatively showing complete healing
Discussion
Congenital lacrimal fistulae are thought to arise due to an intrusion with the invagination, burial and subsequent tissue remodelling of the surface ectoderm that gives rise to the nasolacrimal pathway [3]. Most cases of lacrimal fistula are asymptomatic and usually might not need any treatment apart from observation. This case was symptomatic and needed surgical intervention which was conducted successfully. However, when the disease is coupled with obstruction of the nasolacrimal system, complications ensue. Commonly this might lead to epiphora [4]. This patient had no nasolacrimal obstruction which was confirmed during probing and syringing.
The treatment of choice for a symptomatic fistula is surgery, consisting of complete excision of the fistula, sometimes in conjunction with nasolacrimal intubation if there is associated distal nasolacrimal duct obstruction [1]. In the case under review the fistula was cut and sutured and there was no need to do a nasolacrimal intubation.
Establishing the patency of the nasolacrimal apparatus is important and can be done through the dye disappearance test, probing and irrigation or dacryocystogram. This is because excisions done without correcting nasolacrimal obstructions are bound to fail. In this case, probing and irrigation was adequate to demonstrate patency of the nasolacrimal apparatus.
Conclusion
Congenital lacrimal cutaneous fistula can occur anywhere despite it being rare. In longstanding cases surgical intervention may be the management of choice.
Consent
Consent to publish and use the images was obtained from the mother of the patient.
References
- Birchansky, L.D., 1990. Management of Congenital Lacrimal Sac Fistula. Arch. Ophthalmol. 108, 388. https://doi.org/10.1001/archopht.1990.01070050086037
- Chaung, J.Q., Sundar, G., Ali, M.J., 2016. Congenital lacrimal fistula: A major review. Orbit 35, 212–220. https://doi.org/10.1080/01676830.2016.1176052
- Missner, S.C., Kauffman, C.L., 2001. Congenital Lacrimal Sac Fistula: A Case Report and Review.
- Sullivan J Timothy., Clarke P Michael., Morin J Donald., Pashby C Robert., 1992. The surgical management of congenital lacrimal fistulae. Australian and New Zealand Journal of Ophthalmology.