Orbitofacial neurofibromatosis: clinical characteristics and treatment outcome.

PURPOSE: To report clinical observations and surgical management in a large series of patients with orbitofacial neurofibromatosis type 1 (OFNF). PATIENTS AND METHODS: Patients were identified and medical records reviewed for demographic data, ophthalmologic examinations, surgical interventions, and procedure outcome to create a retrospective, non-comparative case series of patients with OFNF seen at one medical centre over a 23-year period. RESULTS: Sixty patients with OFNF (31 females and 29 males; mean age, 14 years) were followed for an average of 5.7 years. Presenting signs and symptoms included eyelid swelling in all patients, ptosis in 56 (93.3%), proptosis in 34 (56.6%), dystopia or strabismus in 30 (50%), and decreased visual acuity in 50 (83.3%). Surgical intervention included ptosis repair in 54 (90%; mean 1.6 surgical procedures), facial and orbital tumour debulking in 54 (90%; mean 2.3 surgeries), and canthoplasty in 28 (46.6%) patients. Eleven patients required enucleation or exenteration of a blind eye. CONCLUSION: Patients with OFNF often require multiple procedures to preserve vision, prevent additional disfigurement, and achieve cosmetic rehabilitation. Patients need regular ophthalmological monitoring given the potential for progressive visual and cosmetic consequences.

Periocular basal cell carcinoma: 5-year outcome following Slow Mohs surgery with formalin-fixed paraffin-embedded sections and delayed closure.

AIM: The aim of the study was to determine the 5-year outcome of periocular basal cell carcinoma (BCC) managed by Mohs surgery using formalin-fixed, paraffin-embedded sections (Slow Mohs). METHODS: This was a prospective, non-comparative, interventional case series of all patients with periocular BCC treated by Slow Mohs in Newcastle upon Tyne, UK, between 1985 and 1999. Data collected included demographic information, indication for Slow Mohs, tumour site, histology, recurrence rate after 5 years and cosmetic outcome. RESULTS: Of 287 BCCs in 278 patients, 5-year follow-up data were available for 173 (60.2%). Recurrence following Slow Mohs occurred in one patient: 0.34% of total and 0.58% of those with 5-year follow-up. The main indication for Slow Mohs was most frequently due to the tumour site. Cosmetic outcome was deemed excellent in 56%, good in 18%, adequate in 8%, unknown in 14% and revision advised in only 4%. CONCLUSION: The low 5-year recurrence rate (0.58%) reported in this prospective series confirms the importance of margin-controlled removal of recurrent, poorly defined or critically sited BCCs, and illustrates that Slow Mohs is equivalent to standard Mohs. While delayed closure does not appear to compromise cosmetic outcome, this technique offers a histologically superior and cheaper alternative to frozen-section Mohs surgery.

Inpatient preseptal cellulitis: experience from a tertiary eye care centre.

OBJECTIVE: To describe causes of preseptal cellulitis (PSC) and outcome of treatment in patients admitted to a tertiary eye-care centre. METHODS: A 15-year (January 1991 to December 2005) review of inpatients with clinical signs and symptoms or radiological evidence suggestive of PSC was conducted. Patients with infection anterior to the orbital septum which is characterised by acute onset of eyelid oedema, tenderness, erythema, warmth and chemosis were included in the study. RESULTS: Among the 104 patients (male:female 64:40) fulfilling the diagnostic criteria for PSC, acute dacryocystitis (ADC) was the most common predisposing cause in 32.6% patients, followed by sinusitis/upper-respiratory infection (URI) in 28.8% and trauma/recent surgery in 27.8% patients. Fifty-per cent required surgical intervention including dacryocystorhinostomy/probing/stenting in 74% and abscess/chalazian drainage in 28.8%. In 38.5% of the patients who had surgical intervention, microbiological investigations were carried out, cultures were positive in 90%. Most common micro-organisms recovered included Staphylococcus and Streptococcus species followed by Haemophilus influenzae and Klebsiella pneumonia. Blood cultures were positive in two of the 34 patients in whom blood was drawn. Most patients responded to systemic antibiotics with resolution of PSC. Seven patients developed late complications which included subacute lid abscesses, eyelid necrosis and cicatricial ectropion. CONCLUSIONS: Sinusitis/URI, ADC and recent history of trauma/surgery were the most common cause of PSC in admitted patients. Although most patients responded to systemic antibiotics, surgical intervention was necessary in some patients to prevent associated complications.

Optic nerve avulsion from door-handle trauma in children.

AIM: To report severe visual loss caused by optic nerve avulsion (ONA) in children with door-handle trauma. METHODS: Clinical records at a tertiary eye care hospital, of 14 children who sustained severe visual loss as a result of door-handle injuries, were reviewed. The data were analysed for location, presenting symptoms and signs, diagnostic studies, intervention, and the cause of visual loss. RESULTS: There were 11 males and three females with an average age of 8 years and an average height of 125 cm. The place of trauma was home in 11 and school in three children. Presenting visual acuity (VA) was light perception (LP) in five patients and no light perception (NLP) in nine. All the 14 children had evidence of ONA and four patients had ruptured eye globes that required initial repair. The diagnosis of ONA was made clinically or by imaging studies and confirmed histopathologically in eyes that were enucleated. Average follow up was 28.8 months (range 4 months to 8 years). Final VA was LP in one patient and NLP in 13 patients, eight eyes required enucleation for painful blind eye or to achieve optimal cosmesis. CONCLUSION: ONA was the common cause of visual loss in children who sustained ocular trauma caused by door-handles.