A 32-year-old man presented with watering and an inability to close his left eye for 5 years. On local examination, there were periorbital hypopigmented skin changes, loss of forehead creases and sensations, and nodular swellings felt in the lateral two-thirds of the left side of the forehead Fig. 1a. Similar swellings suggestive of thickened nerves were felt in the medial aspect of the left upper and lower eyelids. The orbicularis muscle function was affected due to paresis, and severe lagophthalmos was noted for which a permanent tarsorrhaphy was done elsewhere Fig. 1b. The upper eyelid had entropion, and the lower had grade III ectropion (visible palpebral conjunctiva) with maximum severity corresponding to the thickened nerves, which led to the eyelid notch. Vision was 6/6 OD and 6/18 OS. There was a reduction in corneal sensations and opacity in the left eye. The rest of the eye and systemic examination was normal. The lower half of the face was normal.Figure 1: (a) Shows a left-sided nodular forehead swelling with upper eyelid entropion and lower eyelid ectropion with sagged eyelid and notch along the thickened nerves and a lateral tarsorrhaphy. Periorbital hypopigmentation can also be seen. (b) Shows a severe grade of lagophthalmos. (c) Intra-operative pictures of the upper and lower (inset) eyelids show thickened nerve branches reaching till the eyelid margin. (d) Microphotograph shows granulomas along the nerve (×400, H and E stain). (e) The late post-operative picture shows correction of eyelid margin rotation and notch with a persistent sagged eyelid. (f) Post-operative picture shows correction of sagged lower eyelid using auricular skin-cartilage graftWhat is the possible diagnosis? Neurofibromatosis type I Hansen’s disease Amyloidosis Bell’s palsy. Answer: B. Hansen’s disease. On taking specific history, he informed that he was treated for borderline tuberculoid leprosy with multidrug anti-leprosy therapy for 14 months in 2019. On palpation, the swollen areas had well-delineated, serpiginous, nodular, thickened nerves in the distribution of the supraorbital nerve on the left side of the forehead. A similar nodular and taut nerve bundle was felt in the upper eyelid, medially, reaching to the eyelid margin, which led to mechanical entropion. The lower eyelid ectropion could also be explained by the thickened and taut branches of the infraorbital nerve pulling the eyelid outwards. The pull from the taut and thickened nerves of the upper and lower eyelid aggravated the lagophthalmos and led to the notch. Patients with neurofibromatosis type I have associated typical S-shaped mechanical ptosis with diffuse thickening of the eyelid structures (“bag of worms” feel) with associated temple involvement, dysplastic/absent greater wing of sphenoid/roof, secondary glaucoma, and other features such as café-au-lait spots and freckling in the armpits and groin, neurofibroma, Lisch nodules, etc. The presentation of eyelid amyloidosis is usually as mechanical ptosis with diffuse swelling and occasional bleed; paresis is uncommon. Bell’s palsy is sudden facial nerve paralysis and usually a temporary phenomenon with no thickening of nerves. None of these features was seen in the present case. The patient underwent excision of thickened nerves and release of the mechanical effect on the eyelid margins of both eyelids Fig. 1c. The histopathology revealed mild chronic granulomatous inflammation with few fragmented acid-fast bacilli Fig. 1d. Post-operatively, the eyelid margin and contour correction were seen with a reduction in lagophthalmos but persistent sagging of the lower eyelid Fig. 1e. Six months later, he underwent a skin-cartilage graft using the sandwich technique, which corrected the sagged lower eyelid Fig. 1f. What is true about Hansen’s disease? It is caused by Mycobacterium leprae It primarily affects skin, peripheral nerves, and eyes It is a curable disease Acid-fast bacilli fragments might be seen on histopathology even after remission of the disease All of the above. Answer: E. Discussion Hansen’s disease is a potentially blinding disease with ocular involvement reported in two-thirds of cases.1,2 The ocular manifestations are common in the lepromatous type of leprosy than in the tuberculoid type; the anterior segment is involved in the former, and the peripheral nerves are involved in the latter, as was seen in the present case. The periorbital involvement includes skin changes, madarosis, loss of sensations, ptosis, lagophthalmos due to orbicularis oculi weakness, ectropion, and other conditions. The involvement of the branches of the supraorbital nerve and the infraorbital nerve, which were thickened, beaded, and taut, aggravated the pull on the eyelid, making it more difficult for the patient to close his eye in the presence of peripheral facial paresis. It is usual to have lagophthalmos due to facial nerve branches’ involvement but unusual to have an add-on mechanical pull on the eyelid margin by the grossly thickened nerves, aggravating the lagophthalmos and its related complications. The patient underwent a two-stage surgical procedure and is currently on topical lubricants, night taping, and regular follow-up. Declaration of patient consent The author certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in this journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.
Pushker et al. (Thu,) studied this question.