A prospective, multicentre study of malignant and premalignant lesions at the base of periocular cutaneous horns.

PURPOSE: The primary objective of the study was to determine the incidence of the various pathological conditions present at the base of the periocular cutaneous horns. The secondary objective was to study the presentation of these cutaneous horns with a view to finding any clinical indicators for premalignant, malignant and benign lesions at the base. METHODS: Prospective multicentre study of patients presenting with cutaneous horns. Informed consent followed by excision biopsy of the base lesion was performed in all the cases included for the study. The biopsy specimens were examined histologically and results analysed. RESULTS: Twenty-six patients presented with cutaneous horns in the periocular region over a period of 2 years, of these two patients presented with more than one cutaneous horn. 23 patients underwent excision biopsy. There were a total of 25 specimens. Of the base lesions 2/25 (8%) were malignant, 7/25 (28%) were pre-malignant and the remaining 16/25 (64%) were benign. Analysis of the findings on clinical presentation revealed no clinical indicators to correlate with the final histology. CONCLUSION: The incidence of malignant and pre-malignant lesions present at the base of periocular cutaneous horns was 36% with 8% of them being malignant. It is important to perform an excision biopsy with histological diagnosis of the base of the cutaneous horn as there are no definite clinical features that point towards a potential for malignancy.

Ophthalmic manifestations of atypical IgD multiple myeloma.

A previously healthy 32-year-old Caucasian female presented with sudden-onset horizontal diplopia following a paroxysm of coughing. She had recently sustained a pubic ramus fracture during an innocuous fall and had also noted a firm lump developing at the right side of her forehead. On examination, she had a right fronto-temporal mass. Visual acuities were 6/6 bilaterally. There was reduced abduction of the right eye, bilateral white, granular corneal opacities and evidence of bilateral optic disc swelling. Haematological investigations revealed normocytic anaemia, hypercalcaemia and raised erythrocyte sedimentation rate (ESR). CT showed lytic foci throughout the skull, ribs, scapulae, spine, pelvis and upper femora. Serum protein electrophoresis revealed immunoglobulin D (IgD)-kappa paraproteinaemia; urine electrophoresis showed free light chain kappa and bone marrow biopsy demonstrated 87% plasma cells. A diagnosis of IgD multiple myeloma was made, with subsequent chemotherapeutic treatment and eventual autologous stem cell transplant resulting in resolution of neuro-ophthalmic manifestations and prolonged disease remission.

Management of Marcus Gunn jaw winking synkinesis.

PURPOSE: To report the outcomes of a management protocol for Marcus Gunn jaw winking synkinesis. METHODS: The records of 31 patients (16 female, 15 male) presenting to a tertiary referral center with Marcus Gunn jaw winking synkinesis between 1993 and 2003 were retrospectively analyzed. Generally, patients with mild wink and a small degree of ptosis underwent unilateral upper eyelid retractor surgery. Patients with a moderate or marked wink and ptosis underwent bilateral levator weakening procedures and brow suspension. Patients were assessed and treated for amblyopia and vertical strabismus before ptosis surgery. RESULTS: Mean patient age was 11.23 years (median, 8 years; range, 10 weeks to 31 years). Ipsilateral hypotropia was noted in 8 patients (26%), with a median visual acuity in the ptotic eye of 20/30 (range, 20/20 to 20/40), compared with 20/20 in those without hypotropia. Patients who underwent unilateral surgery had good correction of ptosis, with equal palpebral apertures and symmetrical contour but had a detectable wink on formal testing. Patients who underwent bilateral surgery had equal palpebral apertures and symmetrical contour, with wink elimination. Mean follow-up was 31 months (all patients) and mean postoperative follow-up was 22 months. CONCLUSIONS: Patients with Marcus Gunn jaw winking synkinesis can present at a wide age range. There may be an associated vertical muscle imbalance, which should be managed before ptosis surgery to avoid ptosis undercorrection and allow amblyopia management. Nearly all patients use the synkinesis to reduce the underlying true ptosis to a less noticeable "habitual" ptosis or to maintain binocularity. Surgical approach will differ, depending on whether the synkinesis, ptosis, or both are the main concern. Satisfactory results with a low complication rate can be achieved with the use of this management protocol.

The management of perineural spread of squamous cell carcinoma to the ocular adnexae.

PURPOSE: To analyze a series of patients with periorbital perineural spread of squamous cell carcinoma and propose treatment guidelines. METHODS: Retrospective, noncomparative, interventional case series of 17 patients with clinical, radiologic, or histologic evidence of distant perineural spread. Treatment, recurrence, progression, and mortality rates were recorded. RESULTS: Numbness and pain were the most common symptoms, whereas ophthalmoplegia, ptosis, and facial palsy were the most frequent signs. All cases received wide-field radiotherapy to at least 50 Gy. Chemotherapy and surgery (biopsy, debulking, exenteration) were used in selected cases. Disease progression occurred in 6 patients, 4 of whom died. Median disease-free survival in the remainder was 37.5 months. CONCLUSIONS: The diagnosis of perineural spread is largely clinical and may be confirmed with imaging findings in the majority of cases. The role of biopsy is usually complementary but may be essential in some cases. In patients with evidence of distant perineural spread, radiotherapy volumes inclusive of potential antegrade and retrograde spread are recommended. Three-dimensional conformal planning or intensity-modulated radiation therapy assists in minimizing damage to adjacent structures. Synchronous chemotherapy should be considered to potentiate the effectiveness of radiation. The place of surgery in the treatment of perineural spread is palliative.