Figure 1 Typical congenital lacrimal fistula emerging inferomedial to the lower punctum.
Figure 3 Communication between the nasolacrimal system and the fistula demonstrated by syringing of normal saline through the lower canaliculus.
lacrimal fistula seen at The Hospital for Sick Children, Toronto, Canada from 1980-91. Patients with a history of dacryocystitis and subsequent fistula formation were not included. There were eight girls and five boys.
bilateral cases. No patients had preauricular fistulae or other fistulae elsewhere in the body. Six of 13 patients (46%) had additional lacrimal drainage abnormalities. Four of 13 (31%) had nasolacrimal duct obstruction in addition to the fistulae and one of these also had common canalicular obstruction. One patient had an absent upper punctum and another had an absent lower punctum and canaliculus. Other ocular abnormalities were present in five patients (39%). The three patients with Down’s syndrome had typical ocular adnexal features of epicanthic folds, mongoloid slant to the palpebral fissures and blepharitis. The patient with facial clefts had lower eyelid colobomas and esotropia and another patient had caruncular hypoplasia. The origin of the fistula was found to be from the common canaliculus in six patients (46%) and from the lacrimal sac in three cases (23%). In two cases it was impossible to tell whether the fistula
Results The fistula was noticed soon after birth in nine patients, before 12 months in two more and in two others it was not recorded when the fistula was first noticed. Two of our patients were sisters, however there were no other affected siblings and their parents had no nasolacrimal abnormalities. No other patients gave a positive family history. Systemic anomalies occurred in five of 13 patients (38%). Three patients had Down’s syndrome, one had a meningomyelocoele and an umbilical hernia, and another had bilateral Tessier 3-4 facial clefts. The fistula was right sided in seven patients and left sided in the remaining six. There were no
Figure 2 Congenital lacrimal fistula arising from the common canaliculus in an older child. 110
Figure 4 Closed fistula excision: elliptical incision around the fistula mouth following insertion of an ‘0’ Bowman probe into the fistula to trace its course. Australian and New Zealand Journal of Ophthalmology 1992; ZO(2)
Figure 5 Closed fistula excision: fistulous tract traced to its origin.
Figure 7 Closed fistula excision: vertical skin closure of elliptical incision with 6-0 plain catgut.
arose from the common canaliculus or the adjacent sac and in the remaining two cases the origin was not mentioned. All of the patients suffered from epiphora, six from both the eye and the fistula, three from the eye alone, and four from the fistula alone. Four patients had episodes of fistulitis and one experienced recurrent dacryocystitis. Seven patients had probing of the lacrimal drainage apparatus prior to surgical management of their fistulae. Three were probed once, two patients were probed twice and one was probed four times prior to surgery. The remaining six patients were probed when they underwent surgical repair of their fistulae. Five patients underwent silastic intubation of their nasolacrimal drainage apparatus. Three of these were intubated at the time of their closed excision. The reason for intubation was a partial common canalicular obstruction in one case and was not mentioned in the other two cases. Two
other patients who were intubated were treated unsuccesshlly with multiple excisions of the mouth of the fistula and did not undergo formal closed excision. Eleven patients had a formal closed excision of their congenital lacrimal fistula by a technique described below. This was the initial surgical procedure in eight cases. Ten of the 11 patients (9 1%) had complete relief of symptoms, without recurrence of the fistula, with a follaw-up time ranging from 25 to 125 months (mean 74 months). The patient who had fistula recurrence later underwent a dacryocystorhinostomy with open excision of the residual fistulous tract, which was curative. Five patients underwent procedures to close the mouth of the fistula, presumably with the belief that such procedures would be curative. This consisted of circumcising the fistula mouth and suturing the skin defect. The fistula recurred in all of these patients. The patient with the associated meningomyelocoele and umbilical hernia had two such procedures, both of which failed. Her parents were offered definitive treatment of the fistula; however, they felt that given her other problems hrther intervention was not warranted. Another patient, who had been treated with cautery directly to the fistula tract, had the fistula recur, after which the mouth of her fistula was closed, again with prompt recurrence. She was finally cured with a formal closed excision. Her sister also had a congenital lacrimal fistula and an absent lower canaliculus. She was treated with four minor operations to close the mouth of the fistula, but had recurrence of the fistula after each procedure. Her parents refused further treatment. In summary, of the five patients who had procedures to close the mouth of the fistula
Figure 6 Closed fistula excision: 6-0 polyglycolic acid suture around the base of the fistula. The surgical management of congenital lacrimal fistulae
111
only, three went on to have formal closed excisions which cured the problem, and two have declined further treatment despite having symptomatic fistulae.
Surgical technique The procedure successfully used in 10 patients was performed under general anaesthesia with endotracheal intubation. Prior to the procedure, the nasolacrimal drainage system was probed to assess patency. An '0' Bowman probe was left in the lower canaliculus (when present), lying horizontally. A second probe was then inserted into the fistulous tract to trace its course. Using a number 11 BardParker blade, an incision was made into the skin surrounding the probe, to create an elliptical opening large enough to identify and manipulate the fistulous tract (Figure 4). The fistula was dissected as a small core of tissue through the orbicularis oculi (Figure 5). Below orbicularis the core was traced to its origin, a 6-0 polyglycolic acid suture placed around the base of the fistula (Figure 6), and the fistula was excised. The cut end of the fistula was imbricated with the 6-0 polyglycolic acid suture in a purse-string. The defects in the orbicularis oculi and the skin were closed in layers with 6-0 plain catgut (Figure 7). Silastic intubation of the nasolacrimal apparatus was performed if there were other abnormalities of the nasolacrimal drainage system (common canalicular stenosis, nasolacrimal duct obstruction), or at the operating surgeon's discretion. Pathology was not performed on the excised fistulous tract. Discussion Schirmer, in 1877, cited Rasors' 1675 description of congenital lacrimal fisulae.' Since then many reports have been made in the European and more recently the English literature. Our understanding of congenital lacrimal fistulae assumes a knowledge of the embryology of the nasolacrimal drainage apparatus. Some workers have based their theories of pathogenesis of congenital lacrimal fistulae on embryological beliefs that have been altered by more recent work. Sevel studied the nasolacrimal apparatus in 54 orbits of 27 foetuses ranging from 13.6 mm to term.6 By performing serial sections a composite picture of the development of the human nasolacrimal apparatus was possible. He and others noted that the nasolacrimal apparatus forms from a core of surface epithelium that thickens and invaginates between the maxillary and frontonasal Previous researchers found that this cord became buried, extended caudal and cephalad, 112
and later segmentally canalised to form the canaliculi and the nasolacrimal duct.',' Sevel found that the canaliculi, lacrimal sac and nasolacrimal duct developed contemporaneously from a buried epithelial core and canalised at the same time throughout the length of the drainage apparatm6 This has implications for the pathogenesis of connenital lacrimal fistulae. Several theories of pathogenesis of congenital lacrimal fistulae have been proposed. Levine, and later Werb and Caputo have suggested that congenital lacrimal fistulae form in a manner analogous to fistula formation elsewhere in the body with a failure of the surface ectoderm to fuse fully after invagination has taken p l a ~ e . *Others ~ ~ ~ ' have ~ related them to failure of the facial fissure between the frontonasal and maxillary processes to close fully.1'~'2Another theory suggests that the fistulae arise as an outbudding of the lacrimal duct or an epithelial outgrowth from the tear passage^.'^-'^ Harman also put forward that congenital fistulae represent a rudimentary lacrimal sinus." DukeElder's other theories involved amniotic bands and some form of primary developmental a r r e ~ t . More '~ recently, Welham proposed that the fistulae represent aberrant or extra canaliculi as an outgrowth from the common c a n a l i c ~ l u s This .~~~ is supported by the histological finding that congenital fistulae are lined by stratified squamous epitheli~m.~ While most congenital lacrimal fistulae arise from the common canaliculus, they can also arise from the lacrimal sac, and have been reported to arise from the nasolacrimal duct lateral to the alar n a ~ i . ~ . ' ~ . ' ' We believe that congenital lacrimal fistulae arise when there is interference with the invagination, burial, and later tissue remodelling of the surface ectoderm cord that gives rise to the nasolacrimal apparatus. The nature of the interference is speculative but could involve alterations oC (1) programmed cell death affecting tissue remodelling; (2) local tissue extracellular matrix affecting cellcell adhesiveness, movement and morphogenesis; ( 3 ) local inductive influences or other factors causing an isolated field defect. The cord of surface ectoderm may have its location and orientation altered to explain the site of the fistula and it may undergo unusual branching in cases where there are normal canaliculi and the fistula seems to be a supernumery canaliculus. For some reason this process affects the canalicular portion of the drainage anlage most frequently, although there is no embryological reason why more distal Australian and New Zealand Journal of Ophthalmology 1992; 20(2)
components cannot be affected and give rise to fistulae. Although the incidence of congenital lacrimal fistula is reported to be 1 in 2000, the paucity of reports in the literature and cases seen in clinical practice would suggest the incidence is lower than this. The figure of 1 in 2000 may reflect referral bias.17 We found a family history in two sisters with congenital lacrimal fistula. There were no other affected family members, so mode of inheritance was uncertain. Several reports exist of a positive family history, and in some the inheritance has been autosomal d ~ m i n a n t . ~17-23 Systemic problems were found more frequently than has been previously described. We originally and recently other workers4 have also noted the association between Down's syndrome and congenital lacrimal fistula. The associations of one patient with facial clefts and another with meningomyelocoele and umbilical hernia have interesting pathogenetic implications. Although congenital lacrimal fistulae and other errors of nasolacrimal drainage embryogenesis do occur with craniosynostoses and clefiing syndromes, they more commonly occur in i s ~ l a t i o n . 2'~ 5 26 In some cases, such as ours with meningomyelocoele and umbilical hernia, there may be a generalised defect in the ability of the surface ectoderm to close fully following invagination. The majority of fistulae are unilateral, although bilateral cases have been repOrted.4 11 17 18 22 2 3 27 28 Because we were looking at the surgical management of these patients, all were symptomatic. Studies which have included both surgical and non-surgical patients have found the fistulae to be symptomatic in 929'0, although the true incidence of symptoms may be lower as asymptomatic patients may be unaware of their fistulae. Epiphora is the most common presenting symptom and can occur from the eye or from the fistula, or both. Recurrent fistulitis and dacryocystitis occurs less commonly. The different techniques used in this series for treatment and the long mean follow-up time has allowed us to make several treatment observations. (1) All patiehts who were treated with cautery or simple skin closure of the mouth of the fistula had recurrences, presumably due to incomplete obliteration of the epithelial lining of the fistula. This can be contrasted with acquired lacrimal fistulae with distal obstruction, where the lining is not epithelialised, and in which treatment of the distal obstruction usually results in a cure. (2) 10 of 11 The surgical management of congenital lacrimal fistulae
patients who underwent a formal closed excision of the fistulous tract were cured. The one patient for whom this technique failed to effect a cure was found subsequently to have had an incomplete fistula excision, explaining the failure. ( 3 ) Several of the patients had associated nasolacrimal drainage abnormalities. From these observations, treatment recommendations can be made. (1) Techniques that do not fully remove the fistulous tract (such as cautery or simple skin closure of the fistula mouth) are doomed to fail, and should not be performed. (2) Properly performed closed excision is a safe, effective technique to remove the fistula. With a generous elliptical skin incision, anatomical landmarks can be identified to allow complete excision and there is minimal danger to the common canaliculus for the experienced lacrimal surgeon. ( 3 ) When there are associated nasolacrimal drainage abnormalities, intubation of the lacrimal system should be performed in conjunction with the formal closed excision. Should the additional abnormalities warrant, appropriately timed exploratory dacryocystorhinostomy combined with open fistula excision may be necessary.
Acknowledgements Dr Sullivan's work is supported in part by the RACOlOPSM fellowship. Dr Clarke's work is supported in part by the Frost charitable trust. References 1. Birchansky LD, Nerad JA, Kersten RC, Kulwin DR. Management of congenital lacrimal sac fistula. Arch Ophthalmol 1990;108:388-90. 2. Caputo AR, Smith NH, Cinotti AA, Angiuoli D. Definitive treatment of congenital lacrimal sac fistula. Arch Ophthalmol 1978;96: 1443-4. 3. Welham RAN, Bergin DJ. Congenital lacrimal fistulas. Arch Ophthalmol 1985;103:545-8. 4. Welham RAN, Bates AK, Stasior GO. Congenital lacrimal fistula. Eye 1992; 6 (in press). 5. Schirmer R. Graefe-Saemisch Handbuchder Augenheilkunde. Leipzig: Englemann, 1877: 1-58. 6. Sevel D. Development and congenital abnormalities of the nasolacrimal apparatus. J Pediatr Ophthalmol Strabismus 1981;18:13-9. 7. Schaeffer J, Parson S. The genesis and development of the nasolacrimal passages in man. Am J Anat 1912;13:1-24. 8. Cassady JV. Developmental anatomy of nasolacrimal duct. Arch Ophthalmol 1952;47: 141-58. 9. Levine J. Congenital fistula of the lacrimal sac. Am J Ophthalmol 1929; 12:745-6. 10. Werb A. Surgery of the lacrimal sac. Ann R Coll Surg 1974;54:236-43. 11. Harmen NB. A minimal form of fissura facialis. Trans Ophthalmol SOCUK 1903;23:256-60. 113
12. Pichler A. Trlnennasengang und schrage Gesichtsspalte. Arch f Augenh 1911;68:172-81. 13. Duke-Elder S. System of Ophthalmology. London: Henry Kimpton, 1964; Vol 3: 241-6, 936-7. 14. Monesi L. Die Morphologie der fotalen Tranenwege beim Menschen. Klin Mbl Augenh 1904;42: 1-37. 15. Lohlein W. Ueber angeborene Trlnensackfisteln. Arch f Augenh 1908;61: 185-93. 16. Kubik J. Tri zreidkavi pripady vrodenej anomalie slznjxh ciest. Cesk Oftal 1952;8:222-5. 17. FranGois J, Bacskulin J. External congenital fistulae of the lacrimal sac. Ophthalmologica 1969;159:249-61. 18. Emmert E. Bilateral congenital lachrymal fistulae. Arch Ophthalmol Otolaryn 1877;6:526-7. 19. Caillaud F. Fistule conginital du sac lacrymal. Arch Ophtalmol 1906;26:167-70. 20. Sidan J. Canalicules lacrymaux surnumiraires et fistules conginitales du sac chez cinq membres d’une m8me famille. Ann Oculist 1949;108:855-8.
114
21. Artifoni E. A proposito del carattere ereditario delle fistole congenite del sacco lacrimale. Ann Ottal 1965;91:1103-12. 22. Mukherji R, Mukhopadhay SD. Congenital bilateral lacrimal and pre-auricular fistulas. Am J Ophthalmol 1972;73:595-6. 23. Jones LT, Wobig JL. Surgery of the eyelids and lacrimal system (1st ed.) Birmingham. Aesculapius publishing company, 1976:167-73. 24. Sullivan TJ, Clarke MI’, Braze1 SM, Morin JD, Pashby RC. Congenital lacrimal fistula associated with Down’s syndrome. Am J Ophthalmol 1992;113:215-6. 25. Bartley GB. Lacrimal drainage abnormalities in mandibulofacial dysostosis. Am J Ophthalmol 1990; 109:571-4. 26. Fries I’D, Katowitz JA. Congenital craniofacial anomalies of ophthalmic importance. S w Ophthalmol 1990;35:87-119. 27. Masi AV. Congenital fistula of the lacrimal sac. Arch Ophthalmol 1969;81:701-4. 28. Penzani B. Su di un caso di fistola lacrimale congenita. Ann Ottal 1952;78:255-8.
Australian and
New Zealand Journal of Ophthalmology 1992;20(2)
Figure 3 Communication between the nasolacrimal system and the fistula demonstrated by syringing of normal saline through the lower canaliculus.
lacrimal fistula seen at The Hospital for Sick Children, Toronto, Canada from 1980-91. Patients with a history of dacryocystitis and subsequent fistula formation were not included. There were eight girls and five boys.
bilateral cases. No patients had preauricular fistulae or other fistulae elsewhere in the body. Six of 13 patients (46%) had additional lacrimal drainage abnormalities. Four of 13 (31%) had nasolacrimal duct obstruction in addition to the fistulae and one of these also had common canalicular obstruction. One patient had an absent upper punctum and another had an absent lower punctum and canaliculus. Other ocular abnormalities were present in five patients (39%). The three patients with Down’s syndrome had typical ocular adnexal features of epicanthic folds, mongoloid slant to the palpebral fissures and blepharitis. The patient with facial clefts had lower eyelid colobomas and esotropia and another patient had caruncular hypoplasia. The origin of the fistula was found to be from the common canaliculus in six patients (46%) and from the lacrimal sac in three cases (23%). In two cases it was impossible to tell whether the fistula
Results The fistula was noticed soon after birth in nine patients, before 12 months in two more and in two others it was not recorded when the fistula was first noticed. Two of our patients were sisters, however there were no other affected siblings and their parents had no nasolacrimal abnormalities. No other patients gave a positive family history. Systemic anomalies occurred in five of 13 patients (38%). Three patients had Down’s syndrome, one had a meningomyelocoele and an umbilical hernia, and another had bilateral Tessier 3-4 facial clefts. The fistula was right sided in seven patients and left sided in the remaining six. There were no
Figure 2 Congenital lacrimal fistula arising from the common canaliculus in an older child. 110
Figure 4 Closed fistula excision: elliptical incision around the fistula mouth following insertion of an ‘0’ Bowman probe into the fistula to trace its course. Australian and New Zealand Journal of Ophthalmology 1992; ZO(2)
Figure 5 Closed fistula excision: fistulous tract traced to its origin.
Figure 7 Closed fistula excision: vertical skin closure of elliptical incision with 6-0 plain catgut.
arose from the common canaliculus or the adjacent sac and in the remaining two cases the origin was not mentioned. All of the patients suffered from epiphora, six from both the eye and the fistula, three from the eye alone, and four from the fistula alone. Four patients had episodes of fistulitis and one experienced recurrent dacryocystitis. Seven patients had probing of the lacrimal drainage apparatus prior to surgical management of their fistulae. Three were probed once, two patients were probed twice and one was probed four times prior to surgery. The remaining six patients were probed when they underwent surgical repair of their fistulae. Five patients underwent silastic intubation of their nasolacrimal drainage apparatus. Three of these were intubated at the time of their closed excision. The reason for intubation was a partial common canalicular obstruction in one case and was not mentioned in the other two cases. Two
other patients who were intubated were treated unsuccesshlly with multiple excisions of the mouth of the fistula and did not undergo formal closed excision. Eleven patients had a formal closed excision of their congenital lacrimal fistula by a technique described below. This was the initial surgical procedure in eight cases. Ten of the 11 patients (9 1%) had complete relief of symptoms, without recurrence of the fistula, with a follaw-up time ranging from 25 to 125 months (mean 74 months). The patient who had fistula recurrence later underwent a dacryocystorhinostomy with open excision of the residual fistulous tract, which was curative. Five patients underwent procedures to close the mouth of the fistula, presumably with the belief that such procedures would be curative. This consisted of circumcising the fistula mouth and suturing the skin defect. The fistula recurred in all of these patients. The patient with the associated meningomyelocoele and umbilical hernia had two such procedures, both of which failed. Her parents were offered definitive treatment of the fistula; however, they felt that given her other problems hrther intervention was not warranted. Another patient, who had been treated with cautery directly to the fistula tract, had the fistula recur, after which the mouth of her fistula was closed, again with prompt recurrence. She was finally cured with a formal closed excision. Her sister also had a congenital lacrimal fistula and an absent lower canaliculus. She was treated with four minor operations to close the mouth of the fistula, but had recurrence of the fistula after each procedure. Her parents refused further treatment. In summary, of the five patients who had procedures to close the mouth of the fistula
Figure 6 Closed fistula excision: 6-0 polyglycolic acid suture around the base of the fistula. The surgical management of congenital lacrimal fistulae
111
only, three went on to have formal closed excisions which cured the problem, and two have declined further treatment despite having symptomatic fistulae.
Surgical technique The procedure successfully used in 10 patients was performed under general anaesthesia with endotracheal intubation. Prior to the procedure, the nasolacrimal drainage system was probed to assess patency. An '0' Bowman probe was left in the lower canaliculus (when present), lying horizontally. A second probe was then inserted into the fistulous tract to trace its course. Using a number 11 BardParker blade, an incision was made into the skin surrounding the probe, to create an elliptical opening large enough to identify and manipulate the fistulous tract (Figure 4). The fistula was dissected as a small core of tissue through the orbicularis oculi (Figure 5). Below orbicularis the core was traced to its origin, a 6-0 polyglycolic acid suture placed around the base of the fistula (Figure 6), and the fistula was excised. The cut end of the fistula was imbricated with the 6-0 polyglycolic acid suture in a purse-string. The defects in the orbicularis oculi and the skin were closed in layers with 6-0 plain catgut (Figure 7). Silastic intubation of the nasolacrimal apparatus was performed if there were other abnormalities of the nasolacrimal drainage system (common canalicular stenosis, nasolacrimal duct obstruction), or at the operating surgeon's discretion. Pathology was not performed on the excised fistulous tract. Discussion Schirmer, in 1877, cited Rasors' 1675 description of congenital lacrimal fisulae.' Since then many reports have been made in the European and more recently the English literature. Our understanding of congenital lacrimal fistulae assumes a knowledge of the embryology of the nasolacrimal drainage apparatus. Some workers have based their theories of pathogenesis of congenital lacrimal fistulae on embryological beliefs that have been altered by more recent work. Sevel studied the nasolacrimal apparatus in 54 orbits of 27 foetuses ranging from 13.6 mm to term.6 By performing serial sections a composite picture of the development of the human nasolacrimal apparatus was possible. He and others noted that the nasolacrimal apparatus forms from a core of surface epithelium that thickens and invaginates between the maxillary and frontonasal Previous researchers found that this cord became buried, extended caudal and cephalad, 112
and later segmentally canalised to form the canaliculi and the nasolacrimal duct.',' Sevel found that the canaliculi, lacrimal sac and nasolacrimal duct developed contemporaneously from a buried epithelial core and canalised at the same time throughout the length of the drainage apparatm6 This has implications for the pathogenesis of connenital lacrimal fistulae. Several theories of pathogenesis of congenital lacrimal fistulae have been proposed. Levine, and later Werb and Caputo have suggested that congenital lacrimal fistulae form in a manner analogous to fistula formation elsewhere in the body with a failure of the surface ectoderm to fuse fully after invagination has taken p l a ~ e . *Others ~ ~ ~ ' have ~ related them to failure of the facial fissure between the frontonasal and maxillary processes to close fully.1'~'2Another theory suggests that the fistulae arise as an outbudding of the lacrimal duct or an epithelial outgrowth from the tear passage^.'^-'^ Harman also put forward that congenital fistulae represent a rudimentary lacrimal sinus." DukeElder's other theories involved amniotic bands and some form of primary developmental a r r e ~ t . More '~ recently, Welham proposed that the fistulae represent aberrant or extra canaliculi as an outgrowth from the common c a n a l i c ~ l u s This .~~~ is supported by the histological finding that congenital fistulae are lined by stratified squamous epitheli~m.~ While most congenital lacrimal fistulae arise from the common canaliculus, they can also arise from the lacrimal sac, and have been reported to arise from the nasolacrimal duct lateral to the alar n a ~ i . ~ . ' ~ . ' ' We believe that congenital lacrimal fistulae arise when there is interference with the invagination, burial, and later tissue remodelling of the surface ectoderm cord that gives rise to the nasolacrimal apparatus. The nature of the interference is speculative but could involve alterations oC (1) programmed cell death affecting tissue remodelling; (2) local tissue extracellular matrix affecting cellcell adhesiveness, movement and morphogenesis; ( 3 ) local inductive influences or other factors causing an isolated field defect. The cord of surface ectoderm may have its location and orientation altered to explain the site of the fistula and it may undergo unusual branching in cases where there are normal canaliculi and the fistula seems to be a supernumery canaliculus. For some reason this process affects the canalicular portion of the drainage anlage most frequently, although there is no embryological reason why more distal Australian and New Zealand Journal of Ophthalmology 1992; 20(2)
components cannot be affected and give rise to fistulae. Although the incidence of congenital lacrimal fistula is reported to be 1 in 2000, the paucity of reports in the literature and cases seen in clinical practice would suggest the incidence is lower than this. The figure of 1 in 2000 may reflect referral bias.17 We found a family history in two sisters with congenital lacrimal fistula. There were no other affected family members, so mode of inheritance was uncertain. Several reports exist of a positive family history, and in some the inheritance has been autosomal d ~ m i n a n t . ~17-23 Systemic problems were found more frequently than has been previously described. We originally and recently other workers4 have also noted the association between Down's syndrome and congenital lacrimal fistula. The associations of one patient with facial clefts and another with meningomyelocoele and umbilical hernia have interesting pathogenetic implications. Although congenital lacrimal fistulae and other errors of nasolacrimal drainage embryogenesis do occur with craniosynostoses and clefiing syndromes, they more commonly occur in i s ~ l a t i o n . 2'~ 5 26 In some cases, such as ours with meningomyelocoele and umbilical hernia, there may be a generalised defect in the ability of the surface ectoderm to close fully following invagination. The majority of fistulae are unilateral, although bilateral cases have been repOrted.4 11 17 18 22 2 3 27 28 Because we were looking at the surgical management of these patients, all were symptomatic. Studies which have included both surgical and non-surgical patients have found the fistulae to be symptomatic in 929'0, although the true incidence of symptoms may be lower as asymptomatic patients may be unaware of their fistulae. Epiphora is the most common presenting symptom and can occur from the eye or from the fistula, or both. Recurrent fistulitis and dacryocystitis occurs less commonly. The different techniques used in this series for treatment and the long mean follow-up time has allowed us to make several treatment observations. (1) All patiehts who were treated with cautery or simple skin closure of the mouth of the fistula had recurrences, presumably due to incomplete obliteration of the epithelial lining of the fistula. This can be contrasted with acquired lacrimal fistulae with distal obstruction, where the lining is not epithelialised, and in which treatment of the distal obstruction usually results in a cure. (2) 10 of 11 The surgical management of congenital lacrimal fistulae
patients who underwent a formal closed excision of the fistulous tract were cured. The one patient for whom this technique failed to effect a cure was found subsequently to have had an incomplete fistula excision, explaining the failure. ( 3 ) Several of the patients had associated nasolacrimal drainage abnormalities. From these observations, treatment recommendations can be made. (1) Techniques that do not fully remove the fistulous tract (such as cautery or simple skin closure of the fistula mouth) are doomed to fail, and should not be performed. (2) Properly performed closed excision is a safe, effective technique to remove the fistula. With a generous elliptical skin incision, anatomical landmarks can be identified to allow complete excision and there is minimal danger to the common canaliculus for the experienced lacrimal surgeon. ( 3 ) When there are associated nasolacrimal drainage abnormalities, intubation of the lacrimal system should be performed in conjunction with the formal closed excision. Should the additional abnormalities warrant, appropriately timed exploratory dacryocystorhinostomy combined with open fistula excision may be necessary.
Acknowledgements Dr Sullivan's work is supported in part by the RACOlOPSM fellowship. Dr Clarke's work is supported in part by the Frost charitable trust. References 1. Birchansky LD, Nerad JA, Kersten RC, Kulwin DR. Management of congenital lacrimal sac fistula. Arch Ophthalmol 1990;108:388-90. 2. Caputo AR, Smith NH, Cinotti AA, Angiuoli D. Definitive treatment of congenital lacrimal sac fistula. Arch Ophthalmol 1978;96: 1443-4. 3. Welham RAN, Bergin DJ. Congenital lacrimal fistulas. Arch Ophthalmol 1985;103:545-8. 4. Welham RAN, Bates AK, Stasior GO. Congenital lacrimal fistula. Eye 1992; 6 (in press). 5. Schirmer R. Graefe-Saemisch Handbuchder Augenheilkunde. Leipzig: Englemann, 1877: 1-58. 6. Sevel D. Development and congenital abnormalities of the nasolacrimal apparatus. J Pediatr Ophthalmol Strabismus 1981;18:13-9. 7. Schaeffer J, Parson S. The genesis and development of the nasolacrimal passages in man. Am J Anat 1912;13:1-24. 8. Cassady JV. Developmental anatomy of nasolacrimal duct. Arch Ophthalmol 1952;47: 141-58. 9. Levine J. Congenital fistula of the lacrimal sac. Am J Ophthalmol 1929; 12:745-6. 10. Werb A. Surgery of the lacrimal sac. Ann R Coll Surg 1974;54:236-43. 11. Harmen NB. A minimal form of fissura facialis. Trans Ophthalmol SOCUK 1903;23:256-60. 113
12. Pichler A. Trlnennasengang und schrage Gesichtsspalte. Arch f Augenh 1911;68:172-81. 13. Duke-Elder S. System of Ophthalmology. London: Henry Kimpton, 1964; Vol 3: 241-6, 936-7. 14. Monesi L. Die Morphologie der fotalen Tranenwege beim Menschen. Klin Mbl Augenh 1904;42: 1-37. 15. Lohlein W. Ueber angeborene Trlnensackfisteln. Arch f Augenh 1908;61: 185-93. 16. Kubik J. Tri zreidkavi pripady vrodenej anomalie slznjxh ciest. Cesk Oftal 1952;8:222-5. 17. FranGois J, Bacskulin J. External congenital fistulae of the lacrimal sac. Ophthalmologica 1969;159:249-61. 18. Emmert E. Bilateral congenital lachrymal fistulae. Arch Ophthalmol Otolaryn 1877;6:526-7. 19. Caillaud F. Fistule conginital du sac lacrymal. Arch Ophtalmol 1906;26:167-70. 20. Sidan J. Canalicules lacrymaux surnumiraires et fistules conginitales du sac chez cinq membres d’une m8me famille. Ann Oculist 1949;108:855-8.
114
21. Artifoni E. A proposito del carattere ereditario delle fistole congenite del sacco lacrimale. Ann Ottal 1965;91:1103-12. 22. Mukherji R, Mukhopadhay SD. Congenital bilateral lacrimal and pre-auricular fistulas. Am J Ophthalmol 1972;73:595-6. 23. Jones LT, Wobig JL. Surgery of the eyelids and lacrimal system (1st ed.) Birmingham. Aesculapius publishing company, 1976:167-73. 24. Sullivan TJ, Clarke MI’, Braze1 SM, Morin JD, Pashby RC. Congenital lacrimal fistula associated with Down’s syndrome. Am J Ophthalmol 1992;113:215-6. 25. Bartley GB. Lacrimal drainage abnormalities in mandibulofacial dysostosis. Am J Ophthalmol 1990; 109:571-4. 26. Fries I’D, Katowitz JA. Congenital craniofacial anomalies of ophthalmic importance. S w Ophthalmol 1990;35:87-119. 27. Masi AV. Congenital fistula of the lacrimal sac. Arch Ophthalmol 1969;81:701-4. 28. Penzani B. Su di un caso di fistola lacrimale congenita. Ann Ottal 1952;78:255-8.
Australian and
New Zealand Journal of Ophthalmology 1992;20(2)
