Lacrimal Drainage Surgery in Wegener's Granulomatosis

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Br J Ophthalmol 2000;84:329–331 329 Lacrimal drainage surgery in Wegener’s Br J Ophthalmol: first published as 10.1136/bjo.84.3.329 on 1 March 2000. Downloaded from granulomatosis

AnthonySLKwan,GeoVrey E Rose

Abstract A small number of surgeons were involved, Aim—To examine the results of open all using the following technique. A linear lacrimal surgery in patients with Wegen- paranasal incision was followed by complete er’s granulomatosis. release of medial canthal tendon and a large Methods—A retrospective review of pa- rhinostomy was created, together with partial tients with Wegener’s granulomatosis who anterior ethmoidectomy. Anterior and poste- underwent lacrimal surgery over a 17 year rior ﬂaps were formed from both nasal and lac- period. rimal sac mucosa and the anastomoses united Results—11 patients were identiﬁed and a with multiple 6/0 Vicryl (Ethicon) sutures. The total of 14 primary dacryocystorhinosto- medial canthal tendon, orbicularis, and skin mies (DCR) and one revisional DCR were were closed in layers and silicone stents used in performed; symptomatic relief was most cases. achieved in 13/14 operations and one patient required revisional surgery for persistent symptoms. There were no in- Results traoperative and few postoperative com- Eleven patients (eight males, three females) plications. are included in the study, aged between 34 Conclusions—In contrast with some pre- to 63 years (mean 52 years) at presen- vious reports, open DCR appears to be a tation. Wegener’s disease had been safe procedure and it is recommended as a present for an average of 9.8 years (range treatment for lacrimal obstruction in 4–22) and 9/11 patients were maintained on patients with Wegener’s granulomatosis, systemic immunosuppression before surgery. but an increase of perioperative immuno- Fourteen primary DCRs were performed, suppression is recommended in certain with silicone intubation in 11, and one patient cases. underwent revisional DCR (with intubation) ( 2000;84:329–331) Br J Ophthalmol after having had multiple endonasal lacrimal procedures before referral. Extra immunosup- Wegener’s granulomatosis is generally a pressants were used during the perioperative

multisystem inflammatory disease of unknown period in half of the cases. Postoperative http://bjo.bmj.com/ aetiology. Ophthalmic involvement, which complications included membrane formation includes conjunctivitis, episcleritis, scleritis, at the common internal punctum (one case), corneal ulceration, uveitis, retinal vasculitis, punctal granuloma (one case), extension of optic neuropathy, and diVuse orbital inflam- granulomatous disease (one case) and the mation, is seen in 29% of patients and reformation of a large endonasal cyst nasolacrimal duct obstruction has been re- previously excised at the time of revisional ported in 7% of patients with this disease.1 surgery; this latter patient required further on October 1, 2021 by guest. Protected copyright. More limited forms of the disease, often surgery to remove the reformed cyst. Symp- antinuclear cytoplasmic antibody (ANCA) toms were cured in 94% (14/15) cases at a negative, have been recognised in recent mean follow up of 28 months (range 3–96) years. The presentation, operative outcome (Table 1). and complications for patients with Wegener’s granulomatosis who had undergone open dacryocystorhinostomies (DCR) Illustrative case reports are reviewed and suggestions for the PATIENT 3 management of this diYcult problem are pre- A 56 year old man was referred with bilateral sented. epiphora for 1 year and dacryocystography revealed bilateral mucocoeles. He had Wegen- er’s disease for 4 years that had been controlled Moorfields Eye Hospital, London Methods for 1 year on a daily maintenance dose of 5 mg EC1V 2PD Clinical notes were reviewed for patients with prednisolone. Bilateral dacryocystorhinosto- ASLKwan pathologically confirmed Wegener’s granu- mies were performed, with silicone intubation G E Rose loma, who underwent lacrimal drainage sur- being placed because the lacrimal sacs were gery at Moorfields Eye Hospital between Janu- found to be markedly inflamed with adherent Correspondence to: Mr G E Rose, Lacrimal ary 1982 to July 1999. Most patients had been surrounding tissues, evidence of peridacryo- Clinic, Moorfields Eye treated for sinus and nasal involvement before cystitis. The nasal mucosa was also inflamed Hospital, City Road, London their lacrimal surgery. The modes of presenta- and, in view of the operative findings, a course EC1V 2PD tion, duration of disease, preoperative medical of high dose oral prednisolone (reducing over 3 Accepted for publication therapy, and the results and complications of weeks) was given. He remained asymptomatic 25 October 1999 the surgery were recorded. at 2 years after surgery. 330 Kwan, Rose

Table 1 Patients’ characteristics, medications and follow up periods. Br J Ophthalmol: first published as 10.1136/bjo.84.3.329 on 1 March 2000. Downloaded from Duration of Perioperative Patient Age at Wegener’s increase in Follow up Symptoms No surgery (years) Preoperative immunosuppression Operation (side) immunosuppression Complications (months) resolved

1 60 5 Prednisolone 7 mg once daily DCR (L) No Nil 6 Yes 2 60 22 Prednisolone 15 mg once daily DCR (R) No Nil 60 Yes Cyclophosphamide 50 mg every 3days 3 56 4 Prednisolone 5 mg once daily DCR and tube (L) Yes Nil 24 Yes Prednisolone 5 mg once daily DCR and tube (R) Yes Nil Yes 4 55 30 Nil DCR and tube (R) No Early failure with 9No membrane formation Nil DCR and tube (L) No Punctal granuloma Ye s formation at 1 month 5 44 5 Prednisolone 10mg once daily DCR and tube (L) No Extension of nasal 48 Yes Azathioprine 150 mg once daily granulomatosis to orbit Prednisolone 7.5 mg once daily DCR and tube (R) Yes Nil Yes Cyclophosphamide 100 mg once daily 6 49 5 Prednisolone 10 mg once daily DCR and tube (R) Yes Nil 20 Yes 7 57 5 Prednisolone 7.5 mg once daily DCR and tube (L) No Nil 6 Yes Methotrexate 15 mg per week 8 49 10 Prednisolone 7.5 mg once daily DCR and tube (R) Yes Nil 3 Yes Azathioprine 150 mg once daily Prednisolone 7.5 mg once daily DCR and tube (L) Yes Nil Yes Azathioprine 150 mg once daily 9 63 9 (recently Nil DCR and tube (L) and No Large reformed 12 Yes conﬁrmed) excision of intranasal intranasal cyst excised cyst with canalicular ablation 10 34 8 Prednisolone 20 mg once daily DCR (L) and removal No Nil 96 Yes Azathioprine 100 mg once daily of lid granuloma 11 48 5 (recently Prednisolone 20 mg once daily DCR and tube (L) Yes Nil 3 Yes conﬁrmed)

PATIENT 4 patient declined placement of a right canalicu- A 55 year old woman with a 30 year history of lar bypass tube. Wegener’s granulomatosis presented with a 2 year history of bilateral epiphora. The disease had been quiescent for over 10 years on no PATIENT 5 treatment, although her nasal structure was Left DCR was performed on a 44 year old man grossly disorganised (Fig 1). who had Wegener’s disease for 5 years, control- Dacryocystography showed bilateral small led on oral prednisolone and azathioprine; mucocoeles. A right DCR with intubation was biopsy from the lacrimal sac showed that it was performed, together with division of multiple clear of disease. One year after the operation, intranasal adhesions, but the symptoms re- he presented with non-axial left proptosis and

curred due to gross intranasal scarring over the computed tomograph scan revealed a pre- http://bjo.bmj.com/ common canaliculus. Four months later a left sumed intraorbital extension of nasal granulo- DCR with intubation was performed and, at matous disease through the rhinostomy.2 This the same time, right canalicular trephination was successfully treated with high dose pred- and intubation was performed. No immuno- nisolone and cyclophosphamide and, 6 months suppressants were given. A pyogenic granu- later, a successful right DCR was performed loma was excised from the left inferior with prophylactic high dose systemic pred- punctum at 1 month after surgery and the left nisolone and cyclophosphamide cover. Both on October 1, 2021 by guest. Protected copyright. side remains symptom free. Right epiphora sides remained symptom free at 48 months persisted at 9 months postoperatively, but the after surgery.

Figure 1 Patient with extensive loss of nasal structure due to long standing Wegener’s granuloma (case 4). The collapse of her nasal bridge is associated with loss of the upper portion of the anterior nasal air space, that which normally lies alongside the lacrimal sac fossa. Lacrimal drainage surgery in Wegener’s granulomatosis 331

PATIENT 9 rhinostomy and failure; surgery on the contral-

A 63 year old woman with a 9 year history of ateral side, without adjuvant systemic immu- Br J Ophthalmol: first published as 10.1136/bjo.84.3.329 on 1 March 2000. Downloaded from epiphora, chronic conjunctivitis, and a severe nosuppression, was curative. Despite pred- left toxic keratopathy with stromal vascularisa- nisolone and azathioprine cover, there was an tion, who had undergone multiple endonasal intraorbital progression of the nasal granuloma DCRs, was referred to our unit. An open revi- through the rhinostomy in one patient (patient sional DCR with intubation was performed 5), this orbital disease settling with increased without perioperative systemic steroid. At the immunosuppression and later surgery to the time of the operation a large cystic mass was other eye was uncomplicated after giving found in the upper half of the nasal space and prophylactic high dose systemic steroids.2 the septum; this mass being encysted secre- Wegener’s disease was not recognised in tions from the frontonasal sinus duct. Excision patient 9 and multiple previous attempts of biopsy of this mass showed features consistent endoscopic laser DCR (before referral) were with Wegener’s granulomatosis and helped to without immunosuppression and associated confirm the clinical diagnosis; serum cANCA with extensive nasal scarring and formation of was also raised. Owing to reformation of the a massive intranasal cyst—these changes un- cyst, her symptoms recurred and revisional doubtedly compromising the definitive lac- dacryocystectomy, with placement of a Jones rimal surgery. Endoscopic DCR may be canalicular bypass tube was planned. During inappropriate in the presence of major nasal operation the intranasal cyst was found to be inflammatory disease, as illustrated in this case, draining from the frontal sinus into the and we consider that the large rhinostomy canaliculi and the cyst was, therefore, excised achievable with external surgery may help in and a frontal sinus drainage tube placed. The the long term success of the operation. There canalicular system was ablated and there has have been similar recommendations for pa- been a complete resolution of the toxic tients with sarcoidosis who are undergoing lac- keratopathy. With good control of her symp- rimal surgery.8 In contrast with a previous toms, the patient has declined placement of a report,3 wound necrosis and nasocutaneous canalicular bypass tube. fistula were not seen in our series and there was only one early failure because of canalicular Discussion occlusion developing in an extensively scarred Nasolacrimal duct obstruction in Wegener’s nasal space. granulomatosis probably occurs as a direct We recommend open DCR as a safe and extension of nasopharyngeal disease and typi- eVective treatment for nasolacrimal obstruc- cally is a late manifestation. Postoperative tion in patients with controlled Wegener’s wound necrosis and the formation of nasocuta- granulomatosis. An increase in systemic immu- neous fistula have been reported after DCR in nosuppression should be considered for a few 3 these patients and dacryocystectomy has been weeks after surgery in unstable disease, or suggested as a treatment for nasolacrimal duct when active disease is seen during surgery, and 4 obstruction in this disease. Others have also the patient monitored closely for recurrent or reported more encouraging results with spreading postoperative disease. http://bjo.bmj.com/ DCR.56 or have suggested the use of endoscopic DCR in Wegener’s disease.7 In the present series, all the patients 1 Bullen CL, Liesegang TJ, McDonald TJ, et al. Ocular complications of Wegener’s granulomatosis. Ophthalmology presented in the third to sixth decades, and 1983;90:279–90. either had a short (4–5 years) or long (8–30 2 Kwan ASL, Rose GE. Orbital Wegener’s granuloma result- ing from direct extension of nasal disease through a surgi- years) history of symptoms compatible with cal rhinostomy. Br J Ophthalmol 1998;82:198. Wegener’s granuloma. Four patients (patients 3 Jordan DR, Miller D, Anderson RL. Wound necrosis following dacryocystorhinostomy in patients with Wegen- on October 1, 2021 by guest. Protected copyright. 1, 2, 7, and 10) underwent uneventful and suc- er’s granulomatosis. Ophthalmic Surg 1987;18:800–3. cessful DCR on their normal maintenance 4 Holds JB, Anderson RL, Wolin MJ. Dacryocystectomy for the treatment of dacryocystitis in patients with Wegener’s dose of immunosuppressants and three (pa- granulomatosis. Ophthalmic Surg 1989;20:443–4. tients 6, 8, and 11) achieved symptomatic relief 5 Glatt HJ, Putterman AM. Dacryocystorhinostomy in Wege- ner’s granulomatosis. Ophthalmic Plast Reconstr Surg 1990; with perioperative immunosuppression. Pa- 6:207–10. tient 3 achieved a good result with a short 6 Hargwig PW, Bartley GB, Garrity JA. Surgical management of nasolacrimal duct obstruction in patients with Wegener’s course of high dose prednisolone following dis- granulomatosis. Ophthalmology 1992;99:133–9. covery of inflamed tissue at the operative site. 7 Wong RJ, Gliklich RE, Rubin PA, et al. Bilateral nasolacrimal duct obstruction managed with endoscopic In patient 4, the granulomatous disease had techniques. Arch Otolaryngol Head Neck Surg 1998;124: been quiescent for over 10 years, but the 703–6. extensive scarring in the nose was associated 8 Chapman KL, Bartley GB, Garrity JA, et al. Lacrimal bypass surgery in patients with sarcoidosis. Am J Ophthal- with dense membrane formation over the mol 1999;127:443–6.