Grand Rounds at the New England Eye Center

Woman presents with lid swelling and proptosis

The left eye had progressive proptosis and lid edema associated with foul-smelling nasal discharge.

A 34-year-old woman was transferred to the University of New Mexico emergency department with a 3-day history of progressive left eye swelling. She complained of mild blurry vision, diplopia, discomfort with eye movements and difficulty opening the left eye. She also noted foul-smelling left-sided nasal discharge, subjective fevers and nausea for the past 3 days. She reported having a history of left-sided epistaxis requiring nasal cautery about 2 or 3 years before presentation. She also had a 1-year history of increased tearing in the left eye. She had no other significant medical or surgical history. Her family history was noncontributory. She used over-the-counter nasal spray. She denied tobacco and recreational drug use and reported occasional alcohol use.

Examination

Upon examination, best corrected visual acuity was 20/20 in both eyes. Both pupils were minimally reactive without afferent pupillary defect. IOP was within normal limits in both eyes. Extraocular movements were limited to 80% in lateral gazes and 20% in up- and downgazes in the left eye. Anterior segment exam of the left eye revealed 1+ edema and erythema of the left upper lid with significant proptosis (Figure 1). The left conjunctiva had moderate diffuse injection, moderate follicles and mild yellow mucoid discharge. There was moderate chemosis of the conjunctiva medially and laterally in the left eye. The rest of the anterior segment and dilated fundus exams were within normal limits in both eyes.

Figure 1. External photograph of patient at presentation.

Source: Deborah Witkin, MD, and Amar Joshi, MD

What is your diagnosis?

See answer on next page.

Progressive eye swelling

Based on the presentation, the differential diagnosis included orbital cellulitis, fungal infection, nonspecific orbital inflammation, sarcoidosis, granulomatosis with polyangiitis, thyroid eye disease, IgG4 disease, orbital lymphoma, rhabdomyosarcoma, metastasis or other mass lesion. Less likely diagnoses included cavernous sinus thrombosis, retrobulbar hemorrhage, arteriovenous malformation or orbital varix. The patient underwent CT of the sinuses and MRI of the brain.

Diagnosis

CT of the sinuses with contrast showed a large, heterogeneously enhancing mass centered in the left nasal cavity and ethmoid sinus. There was destruction of the bony nasal septum and lamina papyracea (Figure 2). The MRI contrast showed enhancement of the mass and marked left orbital proptosis secondary to orbital tumor invasion and left frontal mucocele. Otolaryngology performed flexible endoscopy, which showed a large, firm, rubbery, irregular mass with overlying necrotic material on the left extending into the middle meatus. The patient was brought to the operating room the following day with otolaryngology for debridement of the left sinonasal mass with frozen sections, left functional endoscopic sinus surgery and drainage of left orbital subperiosteal abscess. Frozen sections showed a polypoid lesion without signs of malignancy, so the mass was grossly debrided intraoperatively. Pathology slides showed a Schneiderian papilloma with exophytic and oncocytic features that was negative for dysplasia or carcinoma.

Figure 2. Axial and coronal views from CT maxillofacial with contrast.

Management

The patient was treated with intravenous ceftriaxone, metronidazole, vancomycin and dexamethasone for 3 days. Extraocular movements, proptosis and lid edema significantly improved over the next few days. She was discharged home on postoperative day 4 on oral amoxicillin-clavulanate and prednisone taper. She was seen in the otolaryngology clinic 2 weeks after discharge, and rigid endoscopy showed a clear nasal passage. She underwent repeat surgery 3 months after initial presentation for complete excision of the tumor.

Discussion

Schneiderian papillomas, also known as sinonasal papillomas, are papillomas arising from the pseudostratified ciliated Schneiderian epithelium, which lines the sinonasal tract. These papillomas are typically subdivided into three types: inverted, oncocytic and exophytic. Schneiderian papillomas typically present with nasal obstruction, epistaxis and/or nasal discharge. The exophytic type may have an association with the human papilloma virus, but this link has not been well-established. The inverted type is the most common, while oncocytic is the rarest. Exophytic papillomas tend to arise in younger patients (ages 40 to 50 years) vs. the other two types (ages 60 to 70 years).

All three types of Schneiderian papillomas have a risk for malignant transformation, with highest risk in the inverted type and lowest risk in the oncocytic type. A recent meta-analysis found a range of rates of malignant transformation to be 5% to 27% with an average rate of 10%. Complete excision is the recommended treatment due to malignant potential of these lesions as well as a high recurrence rate of 20% to 30% with incomplete excision. While radical debridement via open approach was historically recommended, there has been an increasing trend toward endoscopic excision of Schneiderian papillomas. There have also been some recent case reports that show that radiotherapy may be a viable treatment modality for unresectable or incompletely excised tumors.

Our patient presented with a large tumor that was obstructing the left nasal passage. Acuity of her ocular symptoms was most likely due to a secondary infection in the setting of an obstructive lesion. During surgical resection, no tumor material was noted inside the orbit, but a subperiosteal abscess was found. She will continue to be monitored closely for recurrence of the papilloma.

A 34-year-old woman was transferred to the University of New Mexico emergency department with a 3-day history of progressive left eye swelling. She complained of mild blurry vision, diplopia, discomfort with eye movements and difficulty opening the left eye. She also noted foul-smelling left-sided nasal discharge, subjective fevers and nausea for the past 3 days. She reported having a history of left-sided epistaxis requiring nasal cautery about 2 or 3 years before presentation. She also had a 1-year history of increased tearing in the left eye. She had no other significant medical or surgical history. Her family history was noncontributory. She used over-the-counter nasal spray. She denied tobacco and recreational drug use and reported occasional alcohol use.

Examination

Upon examination, best corrected visual acuity was 20/20 in both eyes. Both pupils were minimally reactive without afferent pupillary defect. IOP was within normal limits in both eyes. Extraocular movements were limited to 80% in lateral gazes and 20% in up- and downgazes in the left eye. Anterior segment exam of the left eye revealed 1+ edema and erythema of the left upper lid with significant proptosis (Figure 1). The left conjunctiva had moderate diffuse injection, moderate follicles and mild yellow mucoid discharge. There was moderate chemosis of the conjunctiva medially and laterally in the left eye. The rest of the anterior segment and dilated fundus exams were within normal limits in both eyes.

Figure 1. External photograph of patient at presentation.

Source: Deborah Witkin, MD, and Amar Joshi, MD

What is your diagnosis?

See answer on next page.

PAGE BREAK

Progressive eye swelling

Based on the presentation, the differential diagnosis included orbital cellulitis, fungal infection, nonspecific orbital inflammation, sarcoidosis, granulomatosis with polyangiitis, thyroid eye disease, IgG4 disease, orbital lymphoma, rhabdomyosarcoma, metastasis or other mass lesion. Less likely diagnoses included cavernous sinus thrombosis, retrobulbar hemorrhage, arteriovenous malformation or orbital varix. The patient underwent CT of the sinuses and MRI of the brain.

Diagnosis

CT of the sinuses with contrast showed a large, heterogeneously enhancing mass centered in the left nasal cavity and ethmoid sinus. There was destruction of the bony nasal septum and lamina papyracea (Figure 2). The MRI contrast showed enhancement of the mass and marked left orbital proptosis secondary to orbital tumor invasion and left frontal mucocele. Otolaryngology performed flexible endoscopy, which showed a large, firm, rubbery, irregular mass with overlying necrotic material on the left extending into the middle meatus. The patient was brought to the operating room the following day with otolaryngology for debridement of the left sinonasal mass with frozen sections, left functional endoscopic sinus surgery and drainage of left orbital subperiosteal abscess. Frozen sections showed a polypoid lesion without signs of malignancy, so the mass was grossly debrided intraoperatively. Pathology slides showed a Schneiderian papilloma with exophytic and oncocytic features that was negative for dysplasia or carcinoma.

Figure 2. Axial and coronal views from CT maxillofacial with contrast.

Management

The patient was treated with intravenous ceftriaxone, metronidazole, vancomycin and dexamethasone for 3 days. Extraocular movements, proptosis and lid edema significantly improved over the next few days. She was discharged home on postoperative day 4 on oral amoxicillin-clavulanate and prednisone taper. She was seen in the otolaryngology clinic 2 weeks after discharge, and rigid endoscopy showed a clear nasal passage. She underwent repeat surgery 3 months after initial presentation for complete excision of the tumor.

Discussion

Schneiderian papillomas, also known as sinonasal papillomas, are papillomas arising from the pseudostratified ciliated Schneiderian epithelium, which lines the sinonasal tract. These papillomas are typically subdivided into three types: inverted, oncocytic and exophytic. Schneiderian papillomas typically present with nasal obstruction, epistaxis and/or nasal discharge. The exophytic type may have an association with the human papilloma virus, but this link has not been well-established. The inverted type is the most common, while oncocytic is the rarest. Exophytic papillomas tend to arise in younger patients (ages 40 to 50 years) vs. the other two types (ages 60 to 70 years).

All three types of Schneiderian papillomas have a risk for malignant transformation, with highest risk in the inverted type and lowest risk in the oncocytic type. A recent meta-analysis found a range of rates of malignant transformation to be 5% to 27% with an average rate of 10%. Complete excision is the recommended treatment due to malignant potential of these lesions as well as a high recurrence rate of 20% to 30% with incomplete excision. While radical debridement via open approach was historically recommended, there has been an increasing trend toward endoscopic excision of Schneiderian papillomas. There have also been some recent case reports that show that radiotherapy may be a viable treatment modality for unresectable or incompletely excised tumors.

Our patient presented with a large tumor that was obstructing the left nasal passage. Acuity of her ocular symptoms was most likely due to a secondary infection in the setting of an obstructive lesion. During surgical resection, no tumor material was noted inside the orbit, but a subperiosteal abscess was found. She will continue to be monitored closely for recurrence of the papilloma.