A review of solitary fibrous tumours of the orbit and ocular adnexa.

Solitary fibrous tumour (SFT) is an uncommon spindle cell tumour of mesenchymal origin characterised by NAB2-STAT6 gene fusion. Although it was first described in the pleura, it can occur in connective tissue in any part of the body, but rarely presents in the orbit and ocular adnexa. SFT, which is part of the same disease spectrum as other fibroblastic tumours such as giant cell angiofibroma, haemangiopericytoma and fibrous histiocytoma, usually presents as a painless, slow-growing mass in any age group and generally follows a benign course, with a good prognosis after complete excision. However, malignant forms rarely occur. Even for benign tumours a more aggressive clinical behaviour is possible, with relentless infiltrative local growth, frequent recurrence following surgery, and malignant transformation with the potential for metastatic spread. Careful long-term follow-up is essential. The published literature on SFTs of the orbit and ocular adnexa is reviewed, and the aetiology, clinical presentation, epidemiology, radiological features, histopathology, immunohistochemistry, risk stratification, clinical management, and prognosis are discussed, reflecting on our own experience.

Mascara-induced nasolacrimal duct obstruction.

A 35-year-old woman underwent left external dacryocystorhinostomy (DCR) following a recent bout of left acute dacryocystitis. She had a right DCR 14 years earlier. Her relatively young age of presentation prompted suspicion of secondary nasolacrimal duct obstruction and, although the left lacrimal sac appeared macroscopically normal peroperatively, a lacrimal sac biopsy was taken. Histopathology revealed florid chronic inflammation, with abundant granular brown pigment and polarisable crystals suggestive of an exogenous material in the lacrimal sac mucosa compatible with mascara. After initial improvement, her epiphora has recurred 1-year postoperatively, but her ocular discharge has resolved. Mascara-induced conjunctival pigmentation is well established. However, there are very few published reports of nasolacrimal duct obstruction due to mascara. Since cosmetic application of mascara and kohl eyeliner is widespread, patients and practitioners should be aware of their potential to migrate into the lacrimal apparatus and cause chronic inflammation with secondary nasolacrimal duct obstruction.

Delayed migration of soft tissue fillers in the periocular area masquerading as eyelid and orbital pathology.

Soft tissue fillers used for facial rejuvenation can cause complications. We present two cases of late migration of injected fillers mimicking other pathology in the periocular area. Case 1 is a 52-year-old woman referred with chronic bilateral upper lid swelling, mimicking blepharochalasis syndrome, 51/2 years after undergoing injection of hyaluronic acid filler in both brows. Extensive blood investigations were normal. Bilateral, sequential upper lid biopsy revealed migrated hyaluronic acid filler, which was successfully treated with hyaluronidase. Case 2 is a 62-year-old woman who presented with a right lower lid mass 8 years after undergoing injection of polyalkylimide gel into both cheeks. CT scanning confirmed an intermediate density soft tissue mass overlying the inferior orbital rim. Histology from surgical excision reported chronic granulomatous inflammation due to migrated polyalkylimide gel. An awareness of late migration of fillers causing eyelid swelling and masses in the periocular area will prevent unnecessary investigations and facilitate prompt management.

Secondary Resistance to Vismodegib After Initial Successful Treatment of Extensive Recurrent Periocular Basal Cell Carcinoma with Orbital Invasion.

Vismodegib is proven to be effective in the treatment of locally advanced and metastatic basal cell carcinoma, but evidence of resistance is beginning to emerge. A case of advanced recurrent periocular basal cell carcinoma which responded dramatically to vismodegib after 3 months but recurred after 9 months due to drug resistance, eventually requiring orbital exenteration, is presented. The mechanism of vismodegib resistance is discussed.

Periocular necrotising fasciitis: a multicentre case series.

INTRODUCTION: Necrotising fasciitis (NF) is a severe infection of deep subcutaneous soft tissues with high morbidity and mortality. Periocular necrotising fasciitis (PONF) is a very rare condition with many unanswered questions about the presentation and management. We present a retrospective case series of patients with PONF from three centres in Australia and two in the UK to investigate the clinical and microbiological characteristics and outcomes and report on patients treated with antibiotics alone. RESULTS: Twenty-nine patients (20 men; 69%) with PONF were identified and followed up for between 2 months and 10 years (median 57, mean 52.6 months) between 1990 and 2013. Conditions associated with chronic immunocompromise were present in 16/29 (55%). Twenty-one (75%) recalled minor periocular trauma or an infected lesion, two having been assaulted by the same assailant. Systemic shock occurred in 6/29 (21%) patients and 1 died. Group A, ß-haemolytic Streptococcus was the most common bacterium identified (25/29, 86%). Intravenous antibiotics were used in all patients, and up to five tissue debridements were required to control the disease in 23/29 (74%); reconstructive surgery was required in 12/29 (41%) patients. One patient died from the disease and visual loss occurred in four eyes of four patients (14%). CONCLUSIONS: PONF has a better prognosis than disease elsewhere in the body, but is still associated with significant risk of visual loss and a small risk of death. Intravenous antibiotic treatment with cautious observation may be reasonable in selected patients with a low threshold for debridement.

Radiological Comparison of the Lacrimal Sac Fossa Anatomy Between Black Africans and Caucasians.

PURPOSE: To compare, in black Africans and Caucasians, the radiological anatomy of the intranasal structures and lacrimal sac fossa as relevant to dacryocystorhinostomy. METHODS: 0.75 mm section cranio-orbital computed tomography scans from 72 patients (42 black Africans and 30 Caucasian) were included in this retrospective observational case series. Only one orbit from each scan was utilized. The main outcome measures were: the thickness and proportions of the lacrimal bone and frontal process of the maxilla evaluated at 3 axial planes (upper, middle, lower) in the lacrimal sac fossa; 2 measurements of maxillary thickness were obtained at each plane-namely, the "midpoint thickness" and the "maximum thickness." The anterior extent of the nasal mucosa was also evaluated. RESULTS: The frontal process of the maxilla was thickest inferiorly (p < 0.001) and the maximum maxillary thickness was significantly thicker in black Africans as compared with Caucasians (p < 0.001) at all planes. At midfossa level, the proportion of maxillary bone forming the lacrimal fossa wall was significantly greater in black Africans (p < 0.01). In contrast, the length of nasal mucosa available for creation of an anastomosis, as estimated from the greatest mucosal height, was significantly greater in Caucasians (p < 0.01). CONCLUSIONS: Black Africans have a lacrimal sac fossa bounded by thicker maxillary bone, this bone constituting a higher proportion of the fossa wall at its midpoint, and also have significantly less nasal mucosa available for soft-tissue anastomosis during lacrimal drainage surgery. The anterior lacrimal crest, comprising the frontal process of the maxilla, was thickest at the lowest plane in both black Africans and Caucasians.

Atypical Presentation of Low-Grade Small Lymphocytic Lymphoma of the Lacrimal Sac.

Lymphoma of the lacrimal sac is uncommon and usually presents as a lacrimal sac mass, against a background of known systemic lymphoma. This study presents the case of a 70-year-old man with small lymphocytic lymphoma of the lacrimal sac and widespread systemic involvement presenting as common canalicular obstruction without a palpable mass or systemic symptoms.

Reconstruction of complex orbital exenteration defects: a single center's experience with a five-year follow-up.

BACKGROUND: Orbital exenteration presents a challenge to the reconstructive surgeon. Defects may be treated with split skin grafts, local advancement flaps, or free flap coverage. There are few published series showing the long-term outcomes of reconstruction. Our results, with at least a 5-year follow-up, are presented. METHODS: A retrospective review of 15 immediate reconstructions after orbital exenterations for malignancies in 12 patients at a tertiary referral center over a 5-year period, was done. All flaps were followed up for at least 5 years (mean, 75 months; range, 3-118 months). RESULTS: Malignancies included squamous cell carcinoma, basal cell carcinoma, meningioma, sebaceous gland carcinoma, and rhabdomyosarcoma. Eight cervicofacial rotation-advancement flaps and 4 anterolateral thigh, 1 rectus abdominis, and 1 radial forearm free tissue transfers were used. Aggressive postoperative radiotherapy (9/15) was well tolerated by both regional and free flaps. Both cervicofacial flaps in previously irradiated patients had wound dehiscence or fistula formation. Six (50%) patients died during follow-up, 4 of whom (33%) died of tumor recurrence. CONCLUSIONS: Flap reconstruction after complex orbital exenteration is associated with low morbidity. Cervicofacial rotation-advancement flaps offer reliable, single-stage, aesthetically pleasing reconstructions. They should be avoided in the previously irradiated. Free tissue transfer is indicated for volume replacement, after previous radiotherapy, after tumor recurrence and previous use of locoregional flaps. Reconstruction of complex orbital exenteration defects for malignancies should be undertaken in centers with experience in the management of these procedures.

Management of periocular basal cell carcinoma by Mohs micrographic surgery.

BACKGROUND/AIMS: To determine the success of Mohs micrographic surgery (MMS) for periocular basal cell carcinoma (BCC) at a tertiary referral centre in the UK. DESIGN: A retrospective interventional case series covering 5 years of practice. METHODS: Review of medical records of 104 consecutive patients who underwent MMS for confirmed periocular BCC. The main outcome measure was biopsy-proven recurrence of BCC at the same anatomical location after MMS. Secondary outcome measures included tumour site, histological subtype and length of follow-up. RESULTS: 104 patients underwent MMS for periocular BCC from January 2003 to July 2008. 63 (62%) of the surgeries were for primary BCC and 25 procedures (25%) were for recurrent or residual BCC. 64% of tumours were nodular BCC. The mean follow-up was 28 months (range 1-85 months). Six recurrences were identified in total. The recurrence rate following MMS for primary BCC was 1.6% (1 patient) compared to 20% (5 patients) in the patient group treated for residual or recurrent tumours. The mean time to recurrence was 39 months (range 16-71 months). CONCLUSIONS: MMS for primary BCC has a very high success rate but the recurrence rate increases significantly in cases of recurrent or residual tumours.

A benign or malignant eyelid lump--can you tell? An unusual collision tumour highlighting the difficulty differentiating a hidrocystoma from a basal cell carcinoma.

Hidrocystoma and basal cell carcinoma (BCC) are common eyelid lesions. The former is benign while the latter is malignant and can cause significant destruction of the adnexal structures, orbital invasion and visual loss. The authors describe a case of a 76-year-old female with a slow growing right upper lid lesion which was diagnosed as a hidrocystoma but excision biopsy revealed a collision tumour comprised of a BCC abutting a hidrocystoma in the same lesion. Cystic BCC can masquerade as hidrocystoma but there are no previous case reports of BCC coexisting with hidrocystoma in the same lesion. This case highlights the fact that apparently benign lesions, such as hidrocystomas, may actually be malignant or coexist with malignant pathology. Clinicians should have a low threshold for appropriate excision biopsy and histological examination of such lesions, especially if there are sinister features, such as lash loss, induration, pigmentation or a pearly appearance.

Discrepancy between fluorescence in situ hybridization and multiplex ligation-dependent probe amplification in orbital recurrence of uveal melanoma 26 years after enucleation.

Cytogenetic analysis has transformed the management of uveal melanoma in recent years and allows categorization of such tumors into low-grade tumors with a favorable prognosis and high-grade tumors that metastasize with a fatal outcome. The authors report the case of a 73-year-old man who presented with recurrent melanoma in his left socket, 26 years after enucleation for uveal melanoma. Chromosomal analysis by multiplex ligation-dependent probe amplification revealed partial loss of chromosome 3 and gains in chromosomes 6 and 8, which were missed with fluorescence in situ hybridization. The patient developed multiple liver metastases 14 months after orbital exenteration and died 8 months later. To the best of authors' knowledge, this is the first report of late recurrence of uveal melanoma after enucleation, in which multiplex ligation-dependent probe amplification chromosomal analysis has been used. The case also highlights the limitations of fluorescence in situ hybridization and the benefits of multiplex ligation-dependent probe amplification, which is more reliable at predicting survival.

Dacryocystorhinostomy precipitating keratoconjunctivitis sicca in aplasia of lacrimal and major salivary glands (ALSG).

We report a 16-month-old girl referred for bilateral epiphora and sticky eyes since birth. Examination revealed a refluxible left lacrimal sac mucocele, agenesis of the left lower punctum, and agenesis of both puncta on the right side. Complete bony obstruction was noted on probing of the left nasolacrimal duct. At 4 years of age, she underwent left external dacryocystorhinostomy (DCR) with silicone intubation because of chronic dacryocystitis. Her epiphora and stickiness improved significantly in the first postoperative year, but she subsequently developed dryness of the left eye, dry mouth, and dental caries. CT and MRI scans revealed the absence of the lacrimal and salivary glands. The clinical signs and symptoms improved with plugging the left upper punctum and topical lubricants. Aplasia of the lacrimal and salivary glands may present with symptoms of congenital lacrimal obstruction, and failure to make an early diagnosis will result in inappropriate lacrimal surgery and dry eye.