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Show Journal of Neuro- Ophthalmology 20( 3): 207- 212, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia The Spectrum of Presentation of Silent Sinus Syndrome Meng Kong Wan, MBBS, Ian C. Francis, FRACS, FRACO, FASOPRS, Peter R. Carter, FRACS, Robert Griffits, FRACO, Miriam L. van Rooijen, FRACR, and Minas T. Coroneo, FRACS, FRACO, MD, MS, MSc The general clinical, ophthalmologic, and radiologic features of three patients with silent sinus syndrome are presented. All three patients were treated surgically. The cases of these patients illustrate the spectrum of presentation of silent sinus syndrome, including enophthalmos, hypophthalmos, transient vertical diplopia, lid retraction, lagophthalmos, and blurred vision. All patients had sinus disease, and all patients improved after surgery using functional endoscopic sinus surgery techniques. The protean manifestations of silent sinus syndrome can be identified, thereby allowing appropriate management. Key Words: Diplopia- Lid retraction- Silent sinus syndrome. Silent sinus syndrome ( SSS) was first described in 1994 by Soparkar et al. ( 1). Orbital signs ( enophthalmos and hypophthalmos) are secondary to asymptomatic ip-silateral maxillary sinus disease, and there is usually no history of trauma. Patients may also present to clinicians with subtle eye movement symptoms and signs, including upper lid retraction ( 2,3) and transient vertical diplopia ( 4). The peak age of presentation is typically the fourth decade, and most patients are asymptomatic at that time. Computed tomography scans show ipsilateral max-illary sinus atrophy and focal orbital floor thinning and resorption ( 1). The orbital floor and globe usually prolapse inferiorly into the maxillary sinus. This paper describes the presentation of SSS in three patients. CASE REPORTS Case 1: Classical Presentation A 38- year- old woman noticed her left eye appeared lower than her right eye 3 years before our initial assess- Manuscript received May 2, 2000; accepted June 14, 2000. From the Ocular Plastics Unit ( MKW, ICF, MTC), the Department of Otorhinolaryngology ( PRC), the Department of Radiology ( M van R), Prince of Wales Hospital, Randwick, Sydney, Australia; and the Department of Ophthalmology ( RG), John Hunter Hospital, Newcastle, Australia. Address correspondence and reprint requests to Ian C. Francis, FRACS, FRACO, FASOPRS, Suite 1, Malvern Court, 16- 18 Malvern Avenue, Chatswood, New South Wales 2067, Australia. ment. She had a long history of sinus symptomatology. Examination findings showed left hypophthalmos and enophthalmos, with visual acuity of 6/ 4 OD and 6/ 6 OS. Findings from the neuro- ophthalmic examination were otherwise normal. There was 2 mm of left hypophthalmos ( Fig. 1). With Luedde proptometry ( 5), readings were 16 mm OD and 11 mm OS, which suggested 5 mm of left enophthalmos. Computed tomography showed the left maxillary sinus to be contracted and completely opacified. The sinus roof was concave, and the left globe had prolapsed inferiorly ( Fig. 2). Her nasal septum appeared to be deviated to the left, which possibly exacerbated her defective maxillary drainage. The maxillary sinus was successfully drained by functional endoscopic sinus surgery ( FESS). The orbital floor was not repaired at that stage. Case 2: Presentation With Transient Vertical Diplopia In 1996, a 44- year- old man noticed while shaving that his right eye was lower than the left eye. For the previous 12 months, he had had intermittent vertical diplopia on upgaze. On examination, visual acuity was 6/ 6 bilaterally, and he had 5 mm of right hypophthalmos. Findings from neuro- ophthalmic examination, and in particular from ocular rotations, were otherwise normal. Luedde proptometry ( 5) demonstrated 4 mm of right enophthalmos; the right eye had a reading of 14 mm, and the left eye had a reading of 18 mm. The right superior sulcus was deeper than the left superior sulcus ( Fig. 3). Computed tomography scanning 4 months previously showed an opacified right maxillary sinus with an intact roof, but a repeated scan at presentation showed a contracted right maxillary sinus with a collapsed orbital floor that resulted in inferior displacement of the right globe. The patient underwent right maxillary sinus FESS without orbital floor repair. Postoperatively, his hypophthalmos, enophthalmos, and diplopia resolved. Examination 3 years later showed right hypophthalmos of 3 mm ( an improvement of 2 mm). Right enophthalmos improved by 3 mm ( the right proptometry reading was 17 mm, and the left reading was unchanged) ( Fig. 4). 207 208 M. K. WAN ET AL. lit •• 2 /> ie: is FIG. 1. Patient 1. Note this patient's left hypophthalmos and enophthalmos. FIG. 2. This computed tomography scan of patient 1 demonstrates the typical appearance of a patient with silent sinus syndrome. The left sinus roof is concave, resulting in orbital expansion. The left globe prolapses interiorly into a contracted, obstructed, and opaque maxillary sinus. FIG. 3. Patient 2 at presentation demonstrated right hypophthalmos and enophthalmos with a deep right superior sulcus. Case 3: Presentation With Lid Retraction and Blurred Vision A 41- year- old man presented with bilateral blurred vision that had occurred for several months. Two months earlier, a left superior orbital abscess had been treated successfully with antibiotics and surgical drainage. Similar treatment for a right orbital abscess did not resolve the discharge. There was evidence of marked bilateral enophthalmos ( Fig. 5) and upper lid retraction on downgaze that was more obvious on the left side ( Fig. 6). Abscess sinus tracks were present that drained into each upper lid ( Fig. 7). There was 2.5 mm of left lagophthalmos. Both corneas showed enough extensive punctate staining with fluorescein to have reduced his visual acuity. Acuity OD was recorded as 6/ 12, and it corrected with pinhole to 6/ 6; acuity OS was 6/ 18, and it corrected with pinhole to 6/ 9. While he always had rather deep- set eyes, as seen in a wedding photograph from 20 years previously ( Fig. 8), Luedde proptometry ( 5) showed measurements of 8 mm OD and 10 mm OS that confirmed his current marked bilateral enophthalmos. Findings from the neuro-ophthalmic examination were otherwise normal. Pus cultured from the right sinus had numerous poly- J Neuro- Ophthalmol. Vol. 20, No. 3, 2000 THE SPECTRUM OF PRESENTATION OF SILENT SINUS SYNDROME 209 FIG. 4. Patient 2. Three years after functional endoscopic sinus surgery alone, the patient's right hypophthalmos and enophthal-mos have almost resolved. rnorphs, but no organisms were grown, probably because he had been treated with numerous courses of antibiotics before referral. Computed tomography scanning showed pansinusitis of all except the sphenoid sinuses ( Fig. 9), a 9- mm soft tissue mass in the preseptal space of his right upper lid ( Fig. 10), early cortical erosion of the right superior orbital margin, and bilateral enophthalmos ( Fig. 10), but no inflammatory change in the orbital fat. The provisional diagnosis was bilateral SSS with bilateral fronto- orbito- cutaneous fistulae. He underwent external bilateral fronto- ethmoidectomy surgery and bilateral FESS to the maxillary sinuses, along with excision of the fistulae. On the left side, a full- thickness skin graft was needed in the upper lid because of the inflammatory contraction within the eyelid substance that was related l: o the chronic fistula. The patient did well postoperatively, but a further abscess in the right upper lid developed that settled with antibiotics. The left upper lid underwent further retraction and may need revision surgery. DISCUSSION Silent sinus syndrome typically presents with hypophthalmos and enophthalmos during the fourth decade. Patients are generally asymptomatic, but may have had previous sinus disease. Patients with SSS may have upper lid retraction ( 2,3), lagophthalmos ( 2), and episodes of transient vertical diplopia ( 4). While these symptoms may suggest a significant neuro- ophthalmic problem, the presence of enophthalmos and hypophthalmos help to pinpoint the diagnosis of SSS. Patients are often not aware of their globe malposition because of its insidious chronicity, and the fact that it may occasionally be bilateral. Table 1 discusses the differential diagnosis of enophthalmos. In 2 recent publications, 67 causes of upper lid retraction were described, including SSS ( 6,7). Recently in this journal, a patient was described to have presented with transient vertical diplopia ( 4). In this patient, 8 mm of hypophthalmos was believed to have prevented him from maintaining orthophoria. Our first patient initially presented with unilateral hypophthalmos and enophthalmos. We await her surgical result in terms of improvement of the orbital position after FESS; our second patient's orbital anatomy improved significantly after FESS alone. Patient 2 improved to near- normal in terms of globe position after FESS alone, without orbital floor reconstruction. We believe that this may be the first report of this FIG. 5. Patient 3. Note the marked bilateral enophthalmos. J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 210 M. K. WAN ETAL. occurrence. The fact that it occurs may reflect the tendency of the orbital contents to resume normal configuration after an external deforming force has been removed. This patient also had transient diplopia on upward gaze over 12 months, and his symptoms resolved after surgery. The patient described by Borruat et al. ( 4) also improved after sinus surgery, but orbital floor reconstruction was necessitated. Patient 3, who presented with bilateral fronto- orbital-cutaneous fistulae, probably demonstrated early manifestations of bilateral SSS. We were surprised that there was no contraction of his maxillary sinuses, considering his marked enophthalmos. Contraction of his maxillary sinuses may have occurred if he had presented at a later date. His lid retraction on downgaze may have been a result of a combination of enophthalmos and upper lid scarring. The patient had reduced vision caused by exposure keratopathy because of the inability to achieve adequate ocular surface coverage with blinking. We believe this is the first report of a patient with probable early SSS who presented with blurred vision. Pathophysiology The pathophysiology of SSS is related to chronic hypoventilation of obstructed maxillary sinuses. This cre- FIG. 6. Patient 3. On downgaze, this patients developed marked upper lid retraction on the left. FIG. 7. Patient 3. Abscess sinus tracks ( arrows) draining into each upper lid. ates a negative pressure within the sinus ( 8,9) that leads to osteolysis of its walls. Eventually, the negative pressure gradient across the weakened orbital floor ( into the maxillary sinus) causes it to collapse inferiorly, manifesting as enophthalmos and hypophthalmos ( 10,11). We suspect that the enophthalmos of patient 3 was a result of fat atrophy caused by negative pressure from obstructed maxillary sinuses. All of the cases described by Soparkar ( 1) had thinning or complete resorption of the maxillary roof, followed by the medial, posterior, and anterior walls. The lateral wall is seldom resorbed. Chronic sinusitis does not usually cause roof thinning because it induces wall thickening or maintains thickness. Occasionally, if chronic sinusitis is complicated by osteomyelitis, resorption occurs ( 12,13). Rubin and Rumelt ( 2) suggested two theories to explain upper lid retraction in the primary position. The first theory suggests that when the anterior orbital floor is disrupted, the globe, including the Lockwood ligament, prolapses inferiorly. At the same time, the Whitnall ligament and the levator palpebrae superioris remain in their normal position. Therefore, the intact levator causes upper lid retraction relative to the prolapsed globe. J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 THE SPECTRUM OF PRESENTATION OF SILENT SINUS SYNDROME 211 FIG. 8. Photograph of patient 3 taken 20 years before development of bilateral SSS. The second theory suggests that inferior prolapse of the globe may increase innervation of the superior division of the oculomotor nerve. The inferior rectus is stretched when the floor prolapses, increasing its force of contraction. To maintain binocular fusion, the superior rectus needs to balance the inferior rectus by increasing its contraction. Because the superior division of the oculomotor nerve supplies the superior rectus and levator muscles, upper lid retraction occurs. This theory cannot explain lid retraction in downgaze because innervation to the complex is reduced. Treatment Treatment is directed toward restoration of maxillary sinus drainage. Functional endoscopic sinus surgery is the preferred procedure. The Caldwell- Luc procedure may be complicated by adhesions that reduce mucus clearance with resultant obstruction of maxillary sinus drainage ( 14). If hypophthalmos is severe, the orbital floor may be reconstructed during the same operation ( 15,16). Our preferred implant is titanium mesh fixed with Leibinger screws. Porous polyethylene, iliac crest bone graft, cranial vault bone graft, bone from the anterior wall of the maxillary sinus, autologous nasal septal FIG. 9. Computed tomography scan demonstrating pansinusitis of all except the sphenoidal sinuses. cartilage, and banked irradiated costochondral cartilage have been used ( 16,17). Mild cases of hypophthalmos and enophthalmos may be correctable with FESS alone. Drainage abolishes the negative pressure gradient, which allows the globe to elevate spontaneously. In patient two, there was satisfactory resolution of hypophthalmos and enophthalmos from FESS alone, and we remain optimistic regarding the treatment outcome for patients 1 and 3. In addition, the diplopia of patient 2 resolved postoperatively. Our practice is to restore maxillary sinus drainage by FESS and to observe the ocular signs. If there is insufficient FIG. 10. Patient 3. Note the right preseptal soft tissue mass ( arrow) in the right upper lid, as well as bilateral enophthalmos. J Neuro- Ophtkalmol, Vol. 20, No. 3, 2000 212 M. K. WAN ET AL. TABLE 1. Differential diagnosis of enophthalmos* Unilateral Blowout fracture Scirrhous breast carcinoma Orbital inflammatory disease ( pseudotumor) Silent sinus syndrome Bilateral Involutional Malnutrition Silent sinus syndrome Pulsating enophthalmos Neurofibromatosis type 1 Encephalocele Pseudo- enophthalmos Horner syndrome Ipsilateral microphthalmos Contralateral high myopia Persistent hyperplastic primary vitreous * Taylor D. Pediatric ophthalmology. In: Pediatric Ophthalmology. Melbourne: Blackwell Scientific Publications, 1990: 235. resolution of ocular signs, the orbital floor may be reconstructed later. Silent sinus syndrome is an uncommon condition that presents with orbital and apparent neuro- ophthalmologic signs. The cause is ipsilateral maxillary sinus blockage. Key points for diagnosis are asymptomatic presentation with classical signs of hypophthalmos and enophthalmos, confirmed by computed tomography scanning, demonstrating an ipsilateral contracted maxillary sinus. The orbital floor may be depressed or disrupted. Patients may present early with transient vertical diplopia, upper lid retraction, lagophthalmos, or blurred vision. REFERENCES 1. Soparkar CNS, Patrinely JR, Cauycong MJ, et al. The silent sinus syndrome. A cause of spontaneous enophthalmos. Ophthalmology 1994; 101: 772- 8. 2. Rubin PAD, Rumelt S. Functional indications for enophthalmos repair. Ophthalmic Plast Reconstr Surg 1999; 15: 284- 92. 3. Garber PF, Abramson AL, Stallman PT, et al. Globe ptosis secondary to maxillary sinus mucocele. Ophthalmic Plast Reconstr Surg 1995; 11: 254- 60. 4. 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