External dacryocystorhinostomy in Wegener’s granulomatosis

Abstract

Wegener’s granulomatosis (WG) is a small vessel vasculitis, typically located in the upper and lower respiratory tract and the kidneys. The characteristic pathological findings consist of necrotizing granulomatous inflammation, vasculitis and glomerulonephritis. Lacrimal obstruction is reported in 7% of patients with WG (Bullen et al. 1983). Standard treatment for severe nasolacrimal obstruction is a dacryocystorhinostomy (DCR), but after a case report of postoperative wound necrosis and nasocutaneous fistula in both of two patients with WG (Jordan et al. 1987), DCR was considered controversial in this patient category. However, favourable results have since been reported in several studies (Hardwig et al. (1992), Kwan & Rose (2000), Cannady et al. 2009). We used our electronic medical record system to review all 336 cases of DCR performed from 1 October 1998 to 1 July 2011 at the Lacrimal Clinic. Eight cases with concurrent WG were identified (Table 1). One patient underwent bilateral DCR. Lacrimal symptoms emerged 2–6 years (mean, 5 years) after diagnosis of WG. One patient (case 8) had been treated elsewhere with silicone stenting of the lacrimal system. In each case, DCR was planned in cooperation with rheumatologists and rhinologists at Aarhus University Hospital. Patient demographics and morbidity prior to DCR are shown in Table 2. All patients had been in remission and on stable anti-inflammatory medication for at least ½ year preoperatively. The state of remission was based on the rheumatologist′s general clinical evaluation. All DCR procedures were performed by the same surgeon (KN), using a standardized technique: a curved paranasal incision was followed by release of the inferior half of the medial canthal tendon and dissection of the lacrimal fossa. A rhinostomy of at least 10 × 10 mm and a partial anterior ethmoidectomy were performed. Mucosal flaps were formed. The orbicularis/subcutis and the skin were sutured separately. A bicanalicular silicone tube (Crawford, FCI, S1-1270) was inserted and removed after 4 weeks. Intraoperatively, 1 g of Ampicillin was given intravenously. Probing at the time of surgery revealed a complete stenosis of the common canaliculusin all cases. Dacryocystorhinostomy surgery was uncomplicated, but two cases with extensive intranasal lesions required assistance from a rhinologist: in case 7, the thick bony structures required removal from both the nasal and the external side. In case 8, extensive synechiae in the upper part of the nose were released. Case 7 developed a painful ipsilateral orbital granuloma 3 months after surgery (Fig. 1A). The incidence was treated successfully with high-dose immunosuppressive medication. Revision of the latest CT scan, performed 3 years prior to surgery, showed an intact medial orbital wall. A new CT scan at the time of the orbital granuloma revealed a destruction of the upper medial orbital wall and contiguous ethmoidal inflammation. A similar case has previously been described Kwan & Rose (1998). Orbital granuloma occurs in 13% of patients with WG and is most often the result of sinus disease, bone erosion and subsequent contagious spread (Bullen et al. 1983). There were no other postoperative complications and no reoperations. (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 following left DCR in Wegener′s granulomatosis. Open nasal fistula with large amounts of fluorescein. Note slightly inflamed nasal mucosa and synechiae between medial turbinate and lateral nasal wall in the lower part of the figure. One patient died 3 years after surgery of unrelated causes. This patient had successful DCR with resolution of lacrimal symptoms and no postoperative complications. Six patients, representing seven surgical procedures, were available for follow-up from 2 months to 11.2 years (mean, 5.4 years) after surgery. At follow-up, all patients reported resolution of lacrimal symptoms after DCR. Endoscopic rhinoscopy revealed patent fistulae in 6/7 cases (Fig. 1B). In case 1, the fistula was closed, and probing revealed a complete stenosis at the level of the common canaliculus. Probing and/or syringing were not performed in patients with patent fistulae. From this small and rare material with varying follow-up times, the following conclusions are drawn: DCR in WG is an efficient and safe procedure, when performed in a quiescent WG state. We recommend a multidisciplinary approach, a recent scan and a preoperative endoscopic rhinoscopy. Patients with extensive intranasal lesions may be in a higher risk for postoperative extension of granulomatous disease from the surgical site to adjacent regions. Edith Nielsen, MD, medical director of the Department of Neuroradiology, Aarhus University Hospital, performed the review of the CT and MRI scans.