Wegener’s granulomatosis (WG) is asmall vessel vasculitis, typically locatedin the upper and lower respiratory tractand the kidneys. The characteristicpathological findings consist of necro-tizing granulomatous inflammation,vasculitis and glomerulonephritis. Lac-rimal obstruction is reported in 7% ofpatients with WG (Bullen et al. 1983).

Standard treatment for severe naso-lacrimal obstruction is a dacryocysto-rhinostomy (DCR), but after a casereport of postoperative wound necro-sis and nasocutaneous fistula in bothof two patients with WG (Jordanet al. 1987), DCR was considered con-troversial in this patient category.

However, favourable results havesince been reported in several studies(Hardwig et al. (1992), Kwan & Rose(2000), Cannady et al. 2009).

We used our electronic medicalrecord system to review all 336 casesof DCR performed from 1 October1998 to 1 July 2011 at the LacrimalClinic. Eight cases with concurrentWG were identified (Table 1). Onepatient underwent bilateral DCR.Lacrimal symptoms emerged 2–6years (mean, 5 years) after diagnosisof WG. One patient (case 8) had beentreated elsewhere with silicone stentingof the lacrimal system. In each case,DCR was planned in cooperation with

rheumatologists and rhinologists atAarhus University Hospital. Patientdemographics and morbidity prior toDCR are shown in Table 2. Allpatients had been in remission and onstable anti-inflammatory medicationfor at least ½ year preoperatively.The state of remission was based onthe rheumatologist¢s general clinicalevaluation.

All DCR procedures were per-formed by the same surgeon (KN),using a standardized technique: acurved paranasal incision was fol-lowed by release of the inferior half ofthe medial canthal tendon and dissectionof the lacrimal fossa. A rhinostomy of

Table 1. Preoperative symptoms and outcome of dacryocystorhinostomy (DCR) in 7 patients with Wegener¢s granulomatosis (WG).

Case Age at surgery Eye Duration in years Symptoms Follow-up in years Patency of fistula

1 34 R 3 Chronic dacryocystitis. Acute dacryocystitis 11.2 No

2 63 L 5 Chronic dacryocystitis. Acute dacryocystitis 8.5 Yes

3 68 L 3 Epiphora. Chronic dacryocystitis – –

4 71 R 7 Chronic dacryocystitis 7.6 Yes

5 59 R 7 Epiphora. Chronic dacryocystitis 4.9 Yes

6 60 L 7 Epiphora 4.6 Yes

7 39 L 15 Chronic dacryocystitis 0.9 Yes

8 34 L 10 Epiphora. Chronic dacryocystitis 0.2 Yes

Case 5 and 6 represent bilateral operations in one patient. Patient 3 died 3 years after surgery. Duration is the period from diagnosis of WG till

surgery. Chronic dacryocystitis indicates the presence of a pyocele, which usually could be emptied by digital pressure. Acute dacryocystitis indi-

cates a clinical situation having required percutaneous drainage. The patency of the surgical fistula was evaluated by an endoscopic rhinoscopy

preceded by installation of fluorescein eye drops.

Diagnosis and Therapy in Ophthalmology

External dacryocystorhinostomyin Wegener’s granulomatosis

Esben Næser,1 Per Friis,2 Ib Tønder Hansen3 and Kristian Næser1

1Department of Ophthalmology, Aarhus University Hospital, Denmark2Department of Ear, Nose and Throat Diseases, Aarhus University Hospital,Denmark3Department of Rheumatology, Aarhus University Hospital, Denmark

Acta Ophthalmol.ª 2012 The Authors

Acta Ophthalmologica ª 2012 Acta Ophthalmologica Scandinavica Foundation

doi: 10.1111/j.1755-3768.2012.02530.x

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at least 10 · 10 mm and a partialanterior ethmoidectomy were per-formed. Mucosal flaps were formed.The orbicularis ⁄ subcutis and the skinwere sutured separately. A bicanalicu-lar silicone tube (Crawford, FCI, S1-1270) was inserted and removed after4 weeks. Intraoperatively, 1 g ofAmpicillin was given intravenously.Probing at the time of surgeryrevealed a complete stenosis of thecommon canaliculusin all cases.

Dacryocystorhinostomy surgery wasuncomplicated, but two cases withextensive intranasal lesions requiredassistance from a rhinologist: in case7, the thick bony structures requiredremoval from both the nasal and theexternal side. In case 8, extensivesynechiae in the upper part of thenose were released.

Case 7 developed a painful ipsilat-eral orbital granuloma 3 months aftersurgery (Fig. 1A). The incidence wastreated successfully with high-doseimmunosuppressive medication. Revi-sion of the latest CT scan, performed3 years prior to surgery, showed anintact medial orbital wall. A new CTscan at the time of the orbital granu-loma revealed a destruction of theupper medial orbital wall and contigu-ous ethmoidal inflammation. A similar

case has previously been describedKwan & Rose (1998). Orbital granu-loma occurs in 13% of patients withWG and is most often the result ofsinus disease, bone erosion and subse-quent contagious spread (Bullen et al.1983). There were no other postopera-tive complications and no reopera-tions.

One patient died 3 years after sur-gery of unrelated causes. This patienthad successful DCR with resolution oflacrimal symptoms and no postopera-tive complications. Six patients, repre-senting seven surgical procedures, wereavailable for follow-up from 2 monthsto 11.2 years (mean, 5.4 years) aftersurgery. At follow-up, all patientsreported resolution of lacrimal symp-toms after DCR. Endoscopic rhinos-copy revealed patent fistulae in 6 ⁄ 7cases (Fig. 1B). In case 1, the fistulawas closed, and probing revealed acomplete stenosis at the level of thecommon canaliculus. Probing and ⁄orsyringing were not performed inpatients with patent fistulae.

From this small and rare materialwith varying follow-up times, the fol-lowing conclusions are drawn: DCRin WG is an efficient and safe proce-dure, when performed in a quiescentWG state. We recommend a multidis-

ciplinary approach, a recent scan anda preoperative endoscopic rhinoscopy.Patients with extensive intranasallesions may be in a higher risk forpostoperative extension of granuloma-tous disease from the surgical site toadjacent regions.

Acknowledgements

Edith Nielsen, MD, medical directorof the Department of Neuroradiology,Aarhus University Hospital, per-formed the review of the CT andMRI scans.

ReferencesBullen CL, Liesegang TJ, McDonald TJ &

DeRemee RA (1983): Ocular complications

of Wegener’s granulomatosis. Ophthalmol-

ogy 90: 279–290.

Cannady SB, Batra PS, Koening C, Lorenz

RR, Citardi MJ, Langford C & Hoffman

GS (2009): Sinonasal Wegener granuloma-

tosis: a single-institution experience with

120 cases. Laryngoscope 119: 757–761.

Hardwig PW, Bartley GB & Garrity JA (1992):

Surgical management of nasolacrimal duct

obstruction in patients with Wegener’s gran-

ulomatosis. Ophthalmology 99: 133–139.

Jordan DR, Miller D & Anderson RL (1987):

Wound necrosis following dacryocystorhi-

nostomy in patients with Wegener’s granu-

lomatosis. Ophthalmic Surg 18: 800–803.

Table 2. Morbidity and systemic treatment prior to dacryocystorhinostomy (DCR).

Case Age Gender Cumulative organ Sinonasal surgery CT ⁄MR scan Treatment

1 31 Male Upper respiratory

tract, lungs

Lysis of nasal

synechiae, R

Sinusitis Prednisolone, 30 mg

Cyclophosphamide

pulse, 1 g ⁄month

2 58 Male Upper respiratory tract,

lungs, kidneys, CNS

Nasal mucosal

biopsy, R + L

Sinusitis,

neo-ossification,

bone destruction

Sulfatrim 400 ⁄ 800 mg

3 65 Male Upper respiratory tract,

lungs, CNS

Turbinectomy, L;

Luc Caldwell, L;

Ethmoidectomy, L

Sinusitis, bone

destruction

Prednisolone, 15 mg

Cyclophosphamide, 100 mg

4 65 Male Upper respiratory tract Turbinectomy, R;

Exploration max.

sinus, R

Sinusitis,

neo-ossification,

bone destruction

Prednisolone, 5 mg

Methotrexate, 10 mg ⁄week

5 52 Male Upper respiratory tract,

lungs, CNS, skin

None Sinusitis Prednisolone, 5 mg

Methotrexate, 7.5 mg ⁄week6 52 Male Upper respiratory tract,

lungs, CNS, skin

None Sinusitis Prednisolone, 5 mg

Methotrexate, 7.5 mg ⁄week7 25 Female Upper respiratory tract Nasal septum plasty;

Ext. nasal plasty x 2;

Ethmoidectomy, R

Sinusitis,

neo-ossification,

bone destruction

Prednisolone,

10 mg Sendoxan,

50 mg Rituximab, 1 g · 2

8 24 Male Upper respiratory tract,

lungs, kidneys, skin

None Sinusitis,

neo-ossification

Prednisolone 5 mg

Rituximab, 1 g · 2

Age at diagnosis of Wegener’s granulomatosis (WG), gender, organs cumulatively involved during the course of WG, prior sinonasal surgery,

last image analysis and current treatment at the time of surgery. The treatment refers to daily doses, unless otherwise stated. Treatment with

Rituximab was given as 1 g, 2 weeks apart and every 6 months. R, right nasal cavity; L, left nasal cavity.

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Kwan AS & Rose GE (1998): Orbital Wegen-

er’s granuloma resulting from direct exten-

sion of nasal disease through a surgical

rhinostomy. Br J Ophthalmol 82: 198. 6:4:

929–934.

Kwan AS & Rose GE (2000): Lacrimal

drainage surgery in Wegener’s granuloma-

tosis. Br J Ophthalmol 84: 329–331.

Received on December 15th, 2011.

Accepted on July 3rd, 2012.

Correspondence:

Kristian Næser, MD

Department of Ophthalmology

Randers Regional Hospital

Skovlyvej 1

8930 Randers Øst

Denmark

Tel: (+45)78422048

Fax (+45)78424336

Email: krisnaes@rm.dk

(A)

(B)

Fig. 1. (A: upper figure) Case 7. Left orbital granuloma, which seems to be an extension from

an opacified ethmoid through a defect in the upper medial orbital wall. The rhinostomy is seen

inferiorly as a separate osseous defect. The globe is displaced laterally by an ill-defined mass of

tissue located medially and superiorly in the orbit. (B: lower figure) Endoscopic rhinoscopy fol-

lowing left DCR in Wegener¢s granulomatosis. Open nasal fistula with large amounts of fluores-

cein. Note slightly inflamed nasal mucosa and synechiae between medial turbinate and lateral

nasal wall in the lower part of the figure.

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