A rare case of hypereosinophilic syndrome presenting

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this clinical and histological masquerade when assessing tumors and inflammatory conditions of the eyelid. This lesion is reported infrequently among oculoplastic pathology. Though it closely mimics malignant lesions of eyelid, it cannot be proved unless there is a high index of suspicion especially in trichoepithelioma.

3. Jakobiec FA. Tumours of the lids. In: Anderson RL, editor. Symposium on diseases and surgery of the lids, lacrimal apparatus and orbit. Transactions of the New Orleans Acad. Ophthalmol. Mosby Co., St. Louis; 1982. pp 264‑307.

Conclusion

5. Hidayat AA, Font RL. Trichilemmoma of eyelid and eyebrow. A clinicopathologic study of 31 cases. Arch Ophthalmol 1980;98:844‑7.

This article aims at recreating an awareness among oculoplastic surgeons and ocular pathologists about the masquerades of malignant lesions and thus avoids extensive lid reconstruction. Declaration of patient consent The authors 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 the 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 The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.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.

References 1. Apple DJ, Naumann GOH. Okul/ire Adnexe: Lider, Tr/inenapparat und Orbita. In: Naumann GOH, editor. Pathologie des Auges. Berlin: Springer Verlag; 1980; pp. 815‑33. 2. Aurora AL, Blodi FC. Lesions of the eyelids: A clinicopathological study. Surv Ophthal‑ tool 1970;15:94‑104.

A rare case of hypereosinophilic syndrome presenting with unilateral proptosis and torticollis Jayanta K Das, Krati Gupta1, Saurabh Deshmukh1, Richa Shrivastava Access this article online Quick Response Code:

Website: www.ijo.in DOI: 10.4103/ijo.IJO_316_18 PMID: *****

Departments of Oculoplasty, 1Ophthalmology Sri Sankaradeva Nethralaya, Guwahati, Assam, India Correspondence to: Dr. Jayanta Kumar Das, Department of Oculoplasty, Sri Sankaradeva Nethralaya, 96, Basistha Road, Guwahati - 781 028, Assam, India. E-mail: [email protected] Manuscript received: 25.03.18; Revision accepted: 01.06.18

4. Vanessa Ngan, Sta Writer. Hair follicle tumours. https://www. dermnetnz.org/topics/hair follicle tumours/2013/31/08/2017. [Last accessed on 2017 Aug 13].

6. Salhi A, Bornholdt D, Oeffner F, Malik S, Heid E, Happle R, et al. Multiple familial trichoepithelioma caused by mutations in the cylindromatosis tumor suppressor gene. Cancer Res 2004;64:5113‑7. 7. Font RL. Eyelids and lacrimal drainage system. In: Ophthalmic Pathology. An Atlas and Textbook. 3rd ed. Philadelphia: WB Saunders; 1996. pp 2229‑32. 8. Córdoba A, Guerrero D, Larrinaga B, Iglesias ME, Arrechea MA, Yanguas JI. Bcl‑2 and CD10 expression in the differential diagnosis of trichoblastoma, basal cell carcinoma, and basal cell carcinoma with follicular differentiation. Int J Dermatol 2009;48:713‑7. 9. Schirren CG, Rütten A, Kaudewitz P, Diaz C, McClain S, Burgdorf WH. Trichoblastoma and basal cell carcinoma are neoplasms with follicular differentiation sharing the same profile of cytokeratin intermediate filaments. Am J Dermatopathol 1997;19:341‑50. 10. Kurzen H, Esposito L, Langbein L, Hartschuh W. Cytokeratins as markers of follicular differentiation: An immunohistochemical study of trichoblastoma and basal cell carcinoma. Am J Dermatopathol 2001;23:501‑9. 11. Yeh I, McCalmont TH, LeBoit PE. Differential expression of PHLDA1 (TDAG51) in basal cell carcinoma and trichoepithelioma. Br J Dermatol 2012;167:1106‑10. 12. Schulz T, Proske S, Hartschuh W, Kurzen H, Paul E, Wünsch PH. High‑grade trichoblastic carcinoma arising in trichoblastoma: A rare adnexal neoplasm often showing metastatic spread. Am J Dermatopathol 2005;27:9‑16.

Hypereosinophilic syndrome (HES) is a spectrum of myeloproliferative disorder, which is characterized by persistent and marked blood eosinophilia and damage to multiple organs due to eosinophilic infiltration. Idiopathic HES is identified after ruling out all other causes of eosinophilia. Poor prognosis is usually associated with cardiac involvement and malignant transformation of blood cells. We report a rare case of HES in an 8‑year‑old boy who presented with unilateral proptosis and torticollis. The patient responded well to corticosteroid therapy with reduction of proptosis and torticollis and normalization of serum eosinophil count. Key words: Eosinophilia, hypereosinophilic syndrome, torticollis This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite this article as: Das JK, Gupta K, Deshmukh S, Shrivastava R. A rare case of hypereosinophilic syndrome presenting with unilateral proptosis and torticollis. Indian J Ophthalmol 2018;66:1508-11.

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Case Report

Hypereosinophilia has been defined as eosinophil count ≥1,500 eosinophils/µL. Hardy and Anderson in 1968 first described hypereosinophilic syndrome (HES) as sustained overproduction of eosinophils.[1] The term idiopathic HES as defined by Chusid et al. was characterized by three criteria: (1) eosinophil count greater than 1,500 cells/μL persisting longer than 6 months, (2) single or multiple organ system dysfunction attributable to cytotoxic injury by eosinophils, and (3) without an identifiable etiology to explain the eosinophilia.[2,3] The mortality in untreated patients can be as high as 75%.[4] Inspite of significant amount progress in the understanding of the pathogenesis of HES, our knowledge is insufficient in formulating a new comprehensive definition of HES based on the etiology.[5] Only a handful of reports can be retrieved from the literature that describe ocular involvement in HES. We present a unique case of HES, wherein an 8‑year‑old boy presented with unilateral proptosis with torticollis and eosinophilia. The child responded well to steroid therapy.

Case Report An 8‑year‑old boy was referred to us with protrusion of his left eye (LE) for 1 month and neck stiffness for 7 days by the consulting pediatrician. Protrusion of LE was insidious in onset, slowly progressive, and was associated with mild pain. There was no history of diplopia, postural variation, and visual disturbance associated with protrusion of the eye. Neck stiffness was associated with mild pain. His medical and family history was unremarkable. On examination [Fig. 1], child was of average built, well oriented to surroundings and afebrile. Visual acuity in both eyes was 6/6, N6. Head tilt to right was present. Child was orthophoric. An axial proptosis of LE was noted and was recorded to be 24 mm with the Hertel’s exophthalmometer. There was fullness of periorbital area with mild tenderness. On palpation of left orbit, orbital margins were intact, though insinuation with fingers was not possible. Proptosis was noncompressible and nonreducible. Bruits, thrills, and pulsations were absent. Slit lamp examination and fundus examination were within normal limit. Pupils were round, regular, and reacting to light. Intraocular pressure (Goldman’s applanation tonometry) was 19 mmHg in the right eye and 22 mmHg in the left eye. On systemic examination, vitals were stable. Mild pallor was present. Peripheral blood showed eosinophils were 62% with absolute eosinophil count to be 4,082 cells/μL [Fig. 2]. ESR was raised to 102 mm. There was no increase in the number of blasts or atypical cells in peripheral blood smear. All other laboratory work was within normal limits. Stool examination showed cysts of Giardia lamblia. No space occupying lesion in orbit was visible in USG B‑scan of the orbit. MRI of orbits [Fig. 3a and b] showed thickening of orbital septum and lacrimal gland enlargement with fuzzy outline in left orbit. An MRI scan of cervical spine [Fig. 3c] showed loss of cervical lordosis. His chest x‑ray [Fig. 3d] revealed bilateral paracardiac lower zone opacities. Ultrasonography of the abdomen showed mild hepatomegaly. The high eosinophil count could not be explained by the Giardia infection therefore, a diagnosis of HES was made and the same was conveyed to the consulting pediatrician. The child was started on corticosteroids in tapering doses (with a starting dose of 1 mg/

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kg), antiallergic, and antiprotozoal medications. He responded well to the therapy. After 2 weeks of treatment, there was marked reduction in proptosis and neck stiffness [Fig. 4]. At 6 months follow‑up there was complete resolution of torticollis with reduction in proptosis and normal eosinophils counts. The patient is on 6 monthly follow‑up with the ophthalmologist and the pediatrician and undergoes routine blood examination at each visit. This case is a rare one because, to our knowledge, unilateral proptosis with torticollis in a patient of HES has not been reported in the literature.

Discussion Almost any organ or tissue may be affected in HES but mostly clinical presentation has been found to be due to skin, heart, lung, and CNS involvement.[6] In the present case, lung, liver, cervical spine, and lacrimal gland along with periorbital tissue were involved. The clinical presentation of patients with HES is diverse and depends on the severity of the disease and pattern of end‑organ involvement.[7] Ocular manifestations in HES remains poorly described, thus highlighting its rarity. In one such report of a patient suffering from HES, transient visual obscuration preceded the systemic manifestations of the disease such as the renal infiltration.[8] There are only few reports of ocular involvement in HES. These reports mostly show retinochoroidal vasculature involvement due to increased blood coagulability. [1] Involvement of periorbital tissue with eosinophilic infiltration has not been reported so far. In the present case to identify possible cause for eosinophilia elaborate history taking, examination, laboratory work‑up, and investigations were done. Only possible cause for eosinophilia in this case may be Giardiasis. However, Giardiasis is rarely associated with eosinophilia and extremely rarely associated with such a high eosinophil count. Corticosteroids constitute the 1st line of treatment. Oral prednisolone acetate in 1 mg/kg dosing in present case showed rapid improvement in proptosis and torticollis. Second line drugs include hydroxyureas, cyclosporin, and interferon‑alpha. Newer modalities of therapy include the use of imatinib mesylate, monoclonal anti‑IL5 antibody, and stem cell transplantation after nonmyeloablative conditioning.[7‑10] In the present case, there was multisystem involvement in the form of orbital, musculoskeletal, pulmonary, and hepatic dysfunctions. We could not fulfill criteria of persistent elevation of eosinophil count for 6 months as it was not practically feasible to observe the patient for 6 months without proper management.

Conclusion HES is a multisystem dysfunction secondary to eosinophilic infiltration. Rare presenting features of torticollis and proptosis and rewarding response to treatment in freeing the child of symptoms and a possible cure is unique in the present case. Declaration of patient consent The authors 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


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Figure 1: Clinical photograph of the patient showing proptosis of the left eye with right sided head tilt

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Figure 2: Blood smear showing eosinophilia

b Figure 4: Post treatment clinical photograph showing resolution of proptosis and head tilt

References 1. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994;83:2759‑79.

c

d

2. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: Analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975;54:1‑27.

Figure 3: (a and b) MRI of the orbits showing thickening of orbital septum and lacrimal gland enlargement with fuzzy outline in left orbit. (c) MRI of the cervical spine showing loss of cervical lordosis with normal appearing vertebral bodies. (d) Chest X-ray PA view showing bilateral paracardiac lower zone opacities

3. Seifert M, Gerth J, Gajda M, Pester F, Pfeifer R, Wolf G. Eosinophilia‑a challenging differential diagnosis. Med Klin (Munich) 2008;103:591‑7.

clinical information to be reported in the 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.

5. Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol 2010;126:45‑9.

Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

4. Boxer LA. Hypereosinophilic syndrome. In Behrman RE, Kliegman RM, Jenson HB, eds. Nelson textbook of Pediatrics. 17th ed. Philadelphia: Saunders; 2004. pp 710.

6. Balaskas K, Wyss P, Guex-Crosier Y. Ocular involvement in idiopathic hypereosinophilic syndrome: a rare finding. Klin Monbl Augenheilkd 2012;229:437-8. 7. Chaine G, Davies J, Kohner EM, Hawarth S, Spry CJ. Ophthalmologic abnormalities in the hypereosinophilic syndrome. Ophthalmology 1982;89:1348‑56. 8. Farcet JP, Binaghi M, Kuentz M, Merlier JF, Mayaud C, Nebout T,


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et al. A hypereosinophilic syndrome with retinal arteritis and tuberculosis. Arch Intern Med 1982;142:625‑7. 9. Alfaham MA, Ferguson SD, Sihra B, Davies A. The idiopathic

A challenging case of a large intraorbital foreign body perforating the nasal septum in a child Tanie Natung, Wakaru Shullai, Donbok Lynser1, Taraprasad Tripathy1 A 5 year old boy with a history of fall from a height of about 4 feet, presented after one week with swelling, watering and discharge of the right eye. He had severe conjunctival chemosis with superior displacement of the globe. Computed Tomography (CT) showed a linear low attenuation tract in the right orbit extending from the inferolateral wall up to the left uncinate process of the ethmoid bone with increasing Hounsfield unit after 10 days. The parents did not agree for early exploration. After 10 days an exploration was done and a large linear and irregular wooden foreign body (FB) measuring 4.5 x 1.5 cm2 was removed from the right orbit and a smaller one from the nasal cavity. Four weeks post surgery, his vision was 6/9 in the right eye with the eyeball in the normal position. This case was challenging because of the late presentation, parents not agreeing for early exploration, difficulty in diagnosing by CT and a large and very deep penetrating FB. Key words: Foreign body, intra-orbital, nasal septum, wooden

Orbital wooden foreign bodies (FBs) penetrating into the orbital and facial areas are common during falls, with children and young adults being more susceptible to these injuries.[1] Wooden FBs being organic and porous act as a good medium for bacterial growth and if not removed promptly may lead to infections and severe complications such as orbital cellulitis,

Access this article online Quick Response Code:

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Department of Ophthalmology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, 1Department of Radiology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India Correspondence to: Dr. Wakaru Shullai, Department of Ophthalmology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), P.O. Mawdiangdiang, Shillong ‑ 793 018, Meghalaya, India. E‑mail: [email protected] Manuscript received: 07.03.18; Revision accepted: 12.06.18

hypereosinophilic syndrome. Arch Dis Child 1987;62:601‑13. 10. Bain BJ. Eosinophilic leukemias and the idiopathic hypereosinophilic syndrome. Br J Haematol 1996;95:2‑9.

orbital abscess, fistulas, optic neuropathy, and so on. [2-4] Moreover, radiological imaging of wooden FBs is complex due to their varied appearances and presents a unique diagnostic challenge.[5] We report here a challenging case of a large intraorbital wooden FB in the right orbit of a child following a fall, perforating the nasal septum and extending up to the left uncinate process of the ethmoid bone.

Case Report A 5 year old presented to the casualty with swelling, watering and discharge of the right eye (RE) for 4 days. The parents gave a history of fall from a height of about 4 feet, one week before the presentation. On examination, there was severe periorbital oedema. Examination of the eye could be done only with the help of lid retractors. The vision in the RE was perception of light with accurate projection of rays. However, there was severe conjunctival chemosis and the eyeball was not visible and appeared to be displaced superiorly [Fig. 1]. Extra ocular movements could not be assessed. On palpation, a hard structure was felt below the displaced globe. The consensual pupillary light reaction was present in the left eye. A superficial, small linear lacerated wound was noted in the lateral aspect of the right inferior eyelid. Ocular ultrasound (USG) showed displaced but intact globe; no FB was seen. Axial CT soft tissue window showed a linear low attenuation track with attenuation values of −300 Hounsfield unit (HU) approximately in the right orbit extending from inferolateral wall through the medial orbital wall, bony nasal septum and medial turbinate reaching up to the left uncinate process of the ethmoid bone, causing superior displacement of the right globe [Fig. 2a]. Coronal section showed the globe to be intact and displaced superiorly due to mass effect [Fig. 2c]. Extraocular muscles were found to be intact. Axial CT bone window showed fracture of the medial wall of the right orbit and nasal septum with hyperattenuating contents in the right and left maxillary sinus and nasal cavity. The parents refused exploration and took discharge against medical advice. However, they returned back after 10 days and a repeat CT scan showed the presence of the linear attenuation track but the attenuation values increased to approximately −30 HU [Fig. 2b]. With full written consent of the parents, an exploration was done under general anaesthesia. A linear and irregular wooden FB measuring 4.5 × 1.5 cm2 [Fig. 3] was

This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite this article as: Natung T, Shullai W, Lynser D, Tripathy T. A challenging case of a large intraorbital foreign body perforating the nasal septum in a child. Indian J Ophthalmol 2018;66:1511-3.