Clinical clues to the early diagnosis and management of ocular surface neoplasia following keratoplasty in xeroderma pigmentosa

The purpose of this report is to highlight the early clinical signs and management of ocular surface neoplasia following penetrating keratoplasty in xeroderma pigmentosa. A 14-year-old girl reported increased conjunctival hyperemia, tearing, photophobia in the right eye at 6 months after penetrating keratoplasty that worsened rapidly over the subsequent 2 weeks. Slit lamp examination showed increased vascularity, epithelial haze and raised whitish lesions at the graft–host junction. Alcohol keratoepithelectomy was performed for confirming the diagnosis. Histology of the sample revealed severe dysplasia. The lesion resolved after surface plaque brachytherapy. The graft clarity was restored and maintained until the last follow-up of 15 months following brachytherapy. Exacerbated symptoms and clinical signs of increased vascularisation and epithelial lesions should arouse the suspicion of ocular surface neoplasia in grafted eyes. Early diagnosis and management can salvage the graft clarity.

Cockayne syndrome, xeroderma pigmentosa: a rare case report

Cockayne syndrome is a rare autosomal recessive disorder characterized by premature ageing (progeria), facial anomalies, cachectic dwarfism, mental retardation, cutaneous photosensitivity, and retinopathy, loss of adipose tissue and muscle, and neurological abnormality which are associated with the changes in the brain parenchyma. The findings of computed tomography scan and especially magnetic resonance imaging of the brain support the clinical diagnosis of CS. There is no permanent cure of this condition and death usually occurs in the second or third decade due to functional disability and multiple infections.

Clinico-Demographic Profile and Management Outcomes of Patients With Ocular Surface Squamous Neoplasia in a Tertiary Eye Care Hospital in Nepal

Background: To evaluate the Clinico-demographic profile and management outcomes of patients with Ocular Surface Squamous Neoplasia (OSSN) in a tertiary eye care hospital in NepalMethods: All the patients with biopsy proven OSSN presenting to Oculoplasty and Ocular Oncology department of Tilganga Institute of Ophthalmology, Nepal over 1 year were retrospectively studied for clinical and demographic profiles, medical and surgical management and the outcomes of the management. Results: A total of 39 eyes of 37 patients were included in this study. The average age of patients was 44.56 years (range- 15 years to 84 years) consisting of 21(56.8%) male and 16 (43.2%) female. The ration of participating male and female patients (M:F) was 1.31:1. 35 cases (94.6%) were unilateral whereas 2 cases with Xeroderma pigmentosa had bilateral involvement. Nasal bulbar conjunctiva in (43.58%) was the most common. 6 (16.2%), 19 (51.4%) and 12 (32.4%) patients received primary medical management alone, surgical management alone and both surgical and adjuvant medical managements respectively.Histologically, the most common finding was CIN I with 10 (31.25%) patients. Surgical margins were free in 15 eyes (46.87%), whereas 17 eyes (53.12%) had one or more margins involved and hence, they received adjuvant topical chemotherapy. Moreover, 5 (15.38%) out of 39 eyes had recurrence.Conclusion: OSSN is usually present in middle age group though it may occur early and become rapidly progressive as in the cases of Xeroderma pigmentosa. Recurrence after surgery can occur despite optimal management.

Single Stage Nasal Reconstruction in a Near Total Nasal Defect

Reconstruction of nasal defect is difficult and challenging. A full-thickness defect of nose requires reconstruction of thin inner lining, middle skeletal (bony/cartilaginous) support, and outer skin layer cover. Large full-thickness defects of nose require complex multistage reconstruction to achieve good functional and aesthetic result. We present here a case of 12-year-old boy, a known case of xeroderma pigmentosa, who underwent wide local excision for squamous cell carcinoma of the nose, leaving a near total defect of the external nose. The reconstruction was done with a suprafascial, thin radial artery forearm free-flap for the external cover as well as the inner lining along with the septal cartilage graft for skeletal support in a single stage.