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288
Letters to the Editor
12 Shields JA, Shields CL, Eagle RC, Jr., Diniz W. Intravascular papillary endothelial hyperplasia with presumed bilateral orbital varices. Arch Ophthalmol 1999; 117 (9): 1247–9. 13 Schulze S, Hoerle S, Koop G, Barth PJ, Huegens-Penzel M, Strempel I. Intravascular papillary endothelial hyperplasia – a rare finding in the orbital region. Ophthalmologica 2008; 222 (3): 213–5. 14 Zakka FR, Jakobiec FA, Thakker MM. Eyelid varix with phlebolith formation, thrombus recanalization, and early intravascular papillary endothelial hyperplasia. Ophthal Plast Reconstruct Surg 2011; 27 (1): e8–11.
Perineural squamous cell carcinoma infiltration of infraorbital nerve treated with endoscopic nerve resection up to foramen rotundum Perineural infiltration by squamous cell carcinomas (SCC) is not uncommon and occurs in 2.5–14% of cases, more commonly in the head and neck region.1 However, few reports documented isolated infraorbital nerve (ION) involvement.2–4 The spread occurs directly through the perineural and endoneural spaces but not through the perineural lymphatics as was believed earlier.5 Although extension along the ION can produce distinctive radiological signs, the diagnosis often remains elusive since clinical symptoms appear late.1–4 In the absence of intracranial extension, a complete excision with adjuvant radiotherapy is usually curative.2–4 A 66-year-old patient presented with numbness of right cheek and upper lip of 2 months duration. He was diagnosed with cutaneous SCC over right malar region, 6 months earlier, which was completely excised with frozen section control. Examination revealed hypoesthesia of the malar region and upper lip. Magnetic resonance imaging (MRI) with contrast showed demonstrated thickening and enhancement of the right ION up to orbital apex without any apparent residual disease in the vicinity of the nerve anteriorly (Fig. 1a,b). An endoscopic pre-lacrimal approach medial maxillectomy along with a middle meatal antrostomy was performed to gain a clear access to the entire length of infraorbital canal (Fig. 1c,d). The bony canal was carefully removed to expose the ION in its entirety before performing a complete length excision (Fig. 1e,f). Subsequently, the complete pterygopalatine fossa course of the nerve backwards was completely exposed up to the foramen rotundum (Fig. 2a), and excised (Fig. 2b) and sent for frozen section, where the posterior Competing/conflicts of interest: Peter J Wormald receives royalties from Medtronic for design of instruments and is a consultant for Nielmed, both not related to this study. All other authors have no conflicts of interest. Funding sources: No stated funding sources.
end was free of the tumour. Postoperatively, the patient received adjuvant radiotherapy. At 6 months follow-up, there was no evidence of any recurrence. The gross pathological examination revealed the first nerve specimen to measure 16 × 5 mm and the second was 26 × 3 mm. Microscopic examination revealed a moderately differentiated keratinizing SCC infiltrating perineural connective tissue and focally involving the nerve fibres (intraneural invasion) (Fig. 2c,d). Focal perineural lymphohistiocytic inflammation with occasional histiocytes and multinucleate giant cells related to keratin were noted (Fig. 2c). Immunocytochemistry staining for cytokeratin 5 revealed perineural keratin labelled squamous cells (Fig. 2e). There was no perineural or intraneural SCC or inflammatory reaction at the distal foramen rotundum end of the nerve (Fig. 2f). Neurotropism is the tendency of carcinomas to grow along the nerve trunks producing neoplastic foci some distance from the primary lesion.3 Isolated infraorbital invasion has been reported with basal cell carcinomas, SCC and malignant melanoma.4 Following invasion, the ION concentrically expands within its bony canal causing nerve degeneration as a result of direct pressure and interference with blood flow. Subsequently, erosion of infraorbital canal can occur and a tissue mass can be detected in the roof of the maxillary antrum.2–4 Of patients with perineural invasion, 60–70% may be asymptomatic initially, although they may complain of hypoesthesia, parasthesia and pain.1,2 Patients with past history of SCC, especially over the malar region, can present several months to years later with numb cheeks, and this could be an early sign of perineural ION invasion and is an indication for an MRI study.3,4 The advantages of MRI in this setting include greater multiplanar capacity, greater soft tissue contrast with better nerve study and the absence of artefacts from bone or dental fillings. The radiological signs can be distinctive and include concentric enlargement of the nerve and diffuse enhancement with contrast, and in advance cases soft tissue mass in the roof of maxillary antrum. In patients with spread beyond the infraorbital canal, MRI accurately delineates the extent of involvement in pterygopalatine fossa, meninges and cranial nerves.2–4 Histopathological evaluation usually reveals classical features of an SCC; however, the cleavage plane between the nerve sheath and nerve trunk is commonly utilized by the tumour cells for their spread since this plane offers less resistance.4 A high proportion of fusiform cells in SCC has been described as a risk factor for perineural invasion.1 In the treatment of perineural ION invasions, the surgeon should accurately estimate the extent of disease spread before surgical planning. Perineural SCC can occasionally be accompanied by regional lymph node metastasis and rarely distant metastasis.1–4 Fortunately, the tumour spread along regional nerves is a slow process. In the absence of intracranial extension, the treatment recommended includes a complete excision with frozen section clearance, followed by adjuvant radiotherapy or a concurrent chemoradiotherapy.1–4 It is difficult to completely excise the lesion in cases of intracranial extension, and the prognosis is poor.1–4
© 2014 Royal Australian and New Zealand College of Ophthalmologists
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Letters to the Editor
289 Figure 1. Magnetic resonance imaging (coronal plane, T1 weighted, fat suppression with contrast) shows a thickened and enhancing right infraorbital nerve. Compare the thickness as well as enhancement with the opposite normal site (a). MRI (sagittal plane, T1 weighted with contrast) showing the enhancement of the nerve (arrow) up to the orbital apex (b). Intraoperative endoscopic photographs showing the exposed medial maxillary wall (star) (c), exposed lacrimal system (arrow) (d), complete exposure of the infraorbital nerve (arrow) prior to excision (e) and empty infraorbital canal (arrow) following a complete nerve excision (f).
(a)
(b)
(c)
(d)
(e)
(f)
Figure 2. Intraoperative endoscopic photograph showing a complete exposure of the nerve (arrow) in the pterygopalatine fossa (a). Endoscopic view of the nerve being cut at foramen rotundum (arrow) (b). Microscopic photograph showing areas of perineural infiltration by squamous cell carcinoma. Note few keratin pearls and areas of focal inflammation (H&E ×100) (c). Microphotograph at high magnification showing the carcinomatous infiltration beneath the nerve sheath (H&E ×400) (d). Immunohistochemistry by cytokeratin showing perineural keratin labelled squamous cells (CK5 ×400) (e). Microphotograph at the foramen rotundum end showing a normal myelinated nerve and absence of any tumour (H&E ×40) (f). In conclusion, although rare, perineural invasion should be kept in the differential diagnosis of numb cheek in patients with history of SCC.3 Early diagnosis with complete surgical resection and adjuvant radiotherapy is curative in most cases.
Prasad Eye Institute) in literature search and its provisions thereof. Mohammad J Ali MD FRCS,1 Jae Murphy MBBS,2 Craig L James MD3 and Peter J Wormald MD FRACS2 1
ACKNOWLEDGEMENT The authors acknowledge the help of Ms Lyn Martin (The Queen Elizabeth Hospital) and Ms Sabera Banu (L. V. © 2014 Royal Australian and New Zealand College of Ophthalmologists
Dacryology Service, L.V. Prasad Eye Institute, Hyderabad, India; and 2Department of Surgery-Otolaryngology, Head and Neck Surgery, University of Adelaide, and 3Adelaide Pathology Partners, Adelaide, South Australia, Australia Received 1 August 2014; accepted 6 August 2014.
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290
Letters to the Editor
REFERENCES 1. McNab AA, Francis IC, Benger R. Perineural spread of cutaneous squamous cell carcinoma via the orbit. Clinical features and outcomes in 21 cases. Ophthalmology 1997; 104: 1457–62. 2. Jafek BW, Ward PH. Infraorbital perineural invasion by tumor. Radiology 1971; 101: 617–8. 3. Campbell WW Jr. The numb cheek syndrome: a sign of infraorbital neuropathy. Neurology 1986; 36: 421– 3. 4. Terashi H, Kurata S, Tadokoro T et al. Perineural and neural involvement in skin cancers. Dermatol Surg 1997; 23: 259–64. 5. Larson DL, Rodin AE, Roberts DK, O’Steen WK, Rapperport AS, Lewis SR. Perineural lymphatics: myth or fact. Am J Surg 1966; 112: 488–92.
The Royal Australian and New Zealand College of Ophthalmologists’ ophthalmic support to developing countries We read the recent letter, ‘Ophthalmic support in developing world countries’1 with interest and applaud the authors for highlighting important issues pertaining to volunteer activities. We agree that such activities play a vital part in enabling developing countries to work towards eliminating avoidable blindness, and should be in alignment with the World Health Organisation Global Action Plan 2014–2019.2 International development is embedded in the current Royal Australian and New Zealand College of Ophthalmologists (RANZCO) strategic plan as an organizational response to this need.3 RANZCO is leveraging its core competencies in ophthalmic education and professional standards to contribute to international development, focusing on the Asia-Pacific region.4 The approach is organizational, but also recognizes the ongoing place for individual activities. A critical component, uncommon in the international development sector, is the fact that Fellows (members of RANZCO) give their time and expertise on a pro bono basis. RANZCO is compliant with best development practice through its accreditation with the peak body for Australian not-for-profit aid and development organizations, the Australian Council for International Development (ACFID).5 The ACFID Code of Conduct seeks to ensure good development outcomes through good governance,
Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.
transparency and accountability. RANZCO is an active member of the peak international eye health advocacy body, the International Association for the Prevention of Blindness.6 RANZCO works in partnership with local stakeholders in the Asia Pacific, and as part of consortia such as Vision2020Australia Global Consortium and the Commonwealth Eye Health Consortium, to advocate for, and build capacity in, ophthalmic education and professional standards for eye care. It is important to highlight that this typically involves working with national ophthalmological societies, to apply and implement best practice principles to ophthalmic education. The peer-reviewed and validated resources of the International Council of Ophthalmology are utilized as the foundation for educational programmes. This not only promotes and demonstrates standard setting to benchmarked international standards, but also provides an in-built mechanism for ongoing development and improvement. RANZCO has a track record, which continues, of working in partnership with ophthalmic organizations in the Pacific, Cambodia, Vietnam and Indonesia. Call for expressions of interest are regularly made for Fellows who wish to participate. The final matching of Fellows for these development projects is based on relevant experience, expertise, subspecialty interest and availability. RANZCO does not limit its engagement just to ophthalmic curriculum and teaching development. Leadership development is an important part of long-term capacity building. RANZCO has established a Regional Leadership Development Program for key regional ophthalmic personnel. The programme involves several leadership capacity building and mentoring activities and includes sponsored attendance at the RANZCO Annual Scientific Congress. Additionally, regional ophthalmologists and residents are offered international scholarships to the annual RANZCO Congress. These are popular and sought after. Alongside the organizational response, RANZCO recognizes and respects that individual volunteer activities are ongoing. Good practice guidelines have been developed, and are easily accessible on the RANZCO website to assist these activities. Those considering, or engaged in, volunteering can use them as a developmentally sound framework for their activities. The guidelines cover, among other things, • • • • •
Assessing one’s individual contribution to international ophthalmology activities Clinical teaching of local eye care practitioners Teaching ophthalmology in developing countries Donated items of equipment or consumables Use of recycled spectacles.
Taken together, all of these initiatives build upon the remarkable track record of the many individual Fellows activities in the past. Collectively, they represent a responsible, coherent and considered response to eliminating avoidable blindness and the Global Action Plan. This response is only possible through continued
© 2014 Royal Australian and New Zealand College of Ophthalmologists
Copyright of Clinical & Experimental Ophthalmology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
288
Letters to the Editor
12 Shields JA, Shields CL, Eagle RC, Jr., Diniz W. Intravascular papillary endothelial hyperplasia with presumed bilateral orbital varices. Arch Ophthalmol 1999; 117 (9): 1247–9. 13 Schulze S, Hoerle S, Koop G, Barth PJ, Huegens-Penzel M, Strempel I. Intravascular papillary endothelial hyperplasia – a rare finding in the orbital region. Ophthalmologica 2008; 222 (3): 213–5. 14 Zakka FR, Jakobiec FA, Thakker MM. Eyelid varix with phlebolith formation, thrombus recanalization, and early intravascular papillary endothelial hyperplasia. Ophthal Plast Reconstruct Surg 2011; 27 (1): e8–11.
Perineural squamous cell carcinoma infiltration of infraorbital nerve treated with endoscopic nerve resection up to foramen rotundum Perineural infiltration by squamous cell carcinomas (SCC) is not uncommon and occurs in 2.5–14% of cases, more commonly in the head and neck region.1 However, few reports documented isolated infraorbital nerve (ION) involvement.2–4 The spread occurs directly through the perineural and endoneural spaces but not through the perineural lymphatics as was believed earlier.5 Although extension along the ION can produce distinctive radiological signs, the diagnosis often remains elusive since clinical symptoms appear late.1–4 In the absence of intracranial extension, a complete excision with adjuvant radiotherapy is usually curative.2–4 A 66-year-old patient presented with numbness of right cheek and upper lip of 2 months duration. He was diagnosed with cutaneous SCC over right malar region, 6 months earlier, which was completely excised with frozen section control. Examination revealed hypoesthesia of the malar region and upper lip. Magnetic resonance imaging (MRI) with contrast showed demonstrated thickening and enhancement of the right ION up to orbital apex without any apparent residual disease in the vicinity of the nerve anteriorly (Fig. 1a,b). An endoscopic pre-lacrimal approach medial maxillectomy along with a middle meatal antrostomy was performed to gain a clear access to the entire length of infraorbital canal (Fig. 1c,d). The bony canal was carefully removed to expose the ION in its entirety before performing a complete length excision (Fig. 1e,f). Subsequently, the complete pterygopalatine fossa course of the nerve backwards was completely exposed up to the foramen rotundum (Fig. 2a), and excised (Fig. 2b) and sent for frozen section, where the posterior Competing/conflicts of interest: Peter J Wormald receives royalties from Medtronic for design of instruments and is a consultant for Nielmed, both not related to this study. All other authors have no conflicts of interest. Funding sources: No stated funding sources.
end was free of the tumour. Postoperatively, the patient received adjuvant radiotherapy. At 6 months follow-up, there was no evidence of any recurrence. The gross pathological examination revealed the first nerve specimen to measure 16 × 5 mm and the second was 26 × 3 mm. Microscopic examination revealed a moderately differentiated keratinizing SCC infiltrating perineural connective tissue and focally involving the nerve fibres (intraneural invasion) (Fig. 2c,d). Focal perineural lymphohistiocytic inflammation with occasional histiocytes and multinucleate giant cells related to keratin were noted (Fig. 2c). Immunocytochemistry staining for cytokeratin 5 revealed perineural keratin labelled squamous cells (Fig. 2e). There was no perineural or intraneural SCC or inflammatory reaction at the distal foramen rotundum end of the nerve (Fig. 2f). Neurotropism is the tendency of carcinomas to grow along the nerve trunks producing neoplastic foci some distance from the primary lesion.3 Isolated infraorbital invasion has been reported with basal cell carcinomas, SCC and malignant melanoma.4 Following invasion, the ION concentrically expands within its bony canal causing nerve degeneration as a result of direct pressure and interference with blood flow. Subsequently, erosion of infraorbital canal can occur and a tissue mass can be detected in the roof of the maxillary antrum.2–4 Of patients with perineural invasion, 60–70% may be asymptomatic initially, although they may complain of hypoesthesia, parasthesia and pain.1,2 Patients with past history of SCC, especially over the malar region, can present several months to years later with numb cheeks, and this could be an early sign of perineural ION invasion and is an indication for an MRI study.3,4 The advantages of MRI in this setting include greater multiplanar capacity, greater soft tissue contrast with better nerve study and the absence of artefacts from bone or dental fillings. The radiological signs can be distinctive and include concentric enlargement of the nerve and diffuse enhancement with contrast, and in advance cases soft tissue mass in the roof of maxillary antrum. In patients with spread beyond the infraorbital canal, MRI accurately delineates the extent of involvement in pterygopalatine fossa, meninges and cranial nerves.2–4 Histopathological evaluation usually reveals classical features of an SCC; however, the cleavage plane between the nerve sheath and nerve trunk is commonly utilized by the tumour cells for their spread since this plane offers less resistance.4 A high proportion of fusiform cells in SCC has been described as a risk factor for perineural invasion.1 In the treatment of perineural ION invasions, the surgeon should accurately estimate the extent of disease spread before surgical planning. Perineural SCC can occasionally be accompanied by regional lymph node metastasis and rarely distant metastasis.1–4 Fortunately, the tumour spread along regional nerves is a slow process. In the absence of intracranial extension, the treatment recommended includes a complete excision with frozen section clearance, followed by adjuvant radiotherapy or a concurrent chemoradiotherapy.1–4 It is difficult to completely excise the lesion in cases of intracranial extension, and the prognosis is poor.1–4
© 2014 Royal Australian and New Zealand College of Ophthalmologists
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Letters to the Editor
289 Figure 1. Magnetic resonance imaging (coronal plane, T1 weighted, fat suppression with contrast) shows a thickened and enhancing right infraorbital nerve. Compare the thickness as well as enhancement with the opposite normal site (a). MRI (sagittal plane, T1 weighted with contrast) showing the enhancement of the nerve (arrow) up to the orbital apex (b). Intraoperative endoscopic photographs showing the exposed medial maxillary wall (star) (c), exposed lacrimal system (arrow) (d), complete exposure of the infraorbital nerve (arrow) prior to excision (e) and empty infraorbital canal (arrow) following a complete nerve excision (f).
(a)
(b)
(c)
(d)
(e)
(f)
Figure 2. Intraoperative endoscopic photograph showing a complete exposure of the nerve (arrow) in the pterygopalatine fossa (a). Endoscopic view of the nerve being cut at foramen rotundum (arrow) (b). Microscopic photograph showing areas of perineural infiltration by squamous cell carcinoma. Note few keratin pearls and areas of focal inflammation (H&E ×100) (c). Microphotograph at high magnification showing the carcinomatous infiltration beneath the nerve sheath (H&E ×400) (d). Immunohistochemistry by cytokeratin showing perineural keratin labelled squamous cells (CK5 ×400) (e). Microphotograph at the foramen rotundum end showing a normal myelinated nerve and absence of any tumour (H&E ×40) (f). In conclusion, although rare, perineural invasion should be kept in the differential diagnosis of numb cheek in patients with history of SCC.3 Early diagnosis with complete surgical resection and adjuvant radiotherapy is curative in most cases.
Prasad Eye Institute) in literature search and its provisions thereof. Mohammad J Ali MD FRCS,1 Jae Murphy MBBS,2 Craig L James MD3 and Peter J Wormald MD FRACS2 1
ACKNOWLEDGEMENT The authors acknowledge the help of Ms Lyn Martin (The Queen Elizabeth Hospital) and Ms Sabera Banu (L. V. © 2014 Royal Australian and New Zealand College of Ophthalmologists
Dacryology Service, L.V. Prasad Eye Institute, Hyderabad, India; and 2Department of Surgery-Otolaryngology, Head and Neck Surgery, University of Adelaide, and 3Adelaide Pathology Partners, Adelaide, South Australia, Australia Received 1 August 2014; accepted 6 August 2014.
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290
Letters to the Editor
REFERENCES 1. McNab AA, Francis IC, Benger R. Perineural spread of cutaneous squamous cell carcinoma via the orbit. Clinical features and outcomes in 21 cases. Ophthalmology 1997; 104: 1457–62. 2. Jafek BW, Ward PH. Infraorbital perineural invasion by tumor. Radiology 1971; 101: 617–8. 3. Campbell WW Jr. The numb cheek syndrome: a sign of infraorbital neuropathy. Neurology 1986; 36: 421– 3. 4. Terashi H, Kurata S, Tadokoro T et al. Perineural and neural involvement in skin cancers. Dermatol Surg 1997; 23: 259–64. 5. Larson DL, Rodin AE, Roberts DK, O’Steen WK, Rapperport AS, Lewis SR. Perineural lymphatics: myth or fact. Am J Surg 1966; 112: 488–92.
The Royal Australian and New Zealand College of Ophthalmologists’ ophthalmic support to developing countries We read the recent letter, ‘Ophthalmic support in developing world countries’1 with interest and applaud the authors for highlighting important issues pertaining to volunteer activities. We agree that such activities play a vital part in enabling developing countries to work towards eliminating avoidable blindness, and should be in alignment with the World Health Organisation Global Action Plan 2014–2019.2 International development is embedded in the current Royal Australian and New Zealand College of Ophthalmologists (RANZCO) strategic plan as an organizational response to this need.3 RANZCO is leveraging its core competencies in ophthalmic education and professional standards to contribute to international development, focusing on the Asia-Pacific region.4 The approach is organizational, but also recognizes the ongoing place for individual activities. A critical component, uncommon in the international development sector, is the fact that Fellows (members of RANZCO) give their time and expertise on a pro bono basis. RANZCO is compliant with best development practice through its accreditation with the peak body for Australian not-for-profit aid and development organizations, the Australian Council for International Development (ACFID).5 The ACFID Code of Conduct seeks to ensure good development outcomes through good governance,
Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.
transparency and accountability. RANZCO is an active member of the peak international eye health advocacy body, the International Association for the Prevention of Blindness.6 RANZCO works in partnership with local stakeholders in the Asia Pacific, and as part of consortia such as Vision2020Australia Global Consortium and the Commonwealth Eye Health Consortium, to advocate for, and build capacity in, ophthalmic education and professional standards for eye care. It is important to highlight that this typically involves working with national ophthalmological societies, to apply and implement best practice principles to ophthalmic education. The peer-reviewed and validated resources of the International Council of Ophthalmology are utilized as the foundation for educational programmes. This not only promotes and demonstrates standard setting to benchmarked international standards, but also provides an in-built mechanism for ongoing development and improvement. RANZCO has a track record, which continues, of working in partnership with ophthalmic organizations in the Pacific, Cambodia, Vietnam and Indonesia. Call for expressions of interest are regularly made for Fellows who wish to participate. The final matching of Fellows for these development projects is based on relevant experience, expertise, subspecialty interest and availability. RANZCO does not limit its engagement just to ophthalmic curriculum and teaching development. Leadership development is an important part of long-term capacity building. RANZCO has established a Regional Leadership Development Program for key regional ophthalmic personnel. The programme involves several leadership capacity building and mentoring activities and includes sponsored attendance at the RANZCO Annual Scientific Congress. Additionally, regional ophthalmologists and residents are offered international scholarships to the annual RANZCO Congress. These are popular and sought after. Alongside the organizational response, RANZCO recognizes and respects that individual volunteer activities are ongoing. Good practice guidelines have been developed, and are easily accessible on the RANZCO website to assist these activities. Those considering, or engaged in, volunteering can use them as a developmentally sound framework for their activities. The guidelines cover, among other things, • • • • •
Assessing one’s individual contribution to international ophthalmology activities Clinical teaching of local eye care practitioners Teaching ophthalmology in developing countries Donated items of equipment or consumables Use of recycled spectacles.
Taken together, all of these initiatives build upon the remarkable track record of the many individual Fellows activities in the past. Collectively, they represent a responsible, coherent and considered response to eliminating avoidable blindness and the Global Action Plan. This response is only possible through continued
© 2014 Royal Australian and New Zealand College of Ophthalmologists
Copyright of Clinical & Experimental Ophthalmology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
