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 Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 7  |  Issue : 3  |  Page : 117-121

Retrospective analysis of clinical profile of pediatric proptosis in a tertiary care hospital of Eastern India


1 Department of Ophthalmology, North Bengal Medical College, Siliguri, West Bengal, India
2 Department of Ophthalmology, Purulia Government Medical College, Purulia, West Bengal, India
3 Department of Ophthalmology, Calcutta National Medical College, Kolkata, West Bengal, India

Date of Submission23-Sep-2018
Date of Acceptance18-Feb-2019
Date of Web Publication11-Dec-2019

Correspondence Address:
Nabanita Barua
21, Rabindranath Tagore Road, P. O. Bediapara, P.S. Dumdum, Kolkata - - 700 077, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_73_18

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  Abstract 


Background: Pediatric proptosis can often present with puzzling clinical features and needs a systematic approach to avoid diagnostic dilemma. Aims and Objectives: To study the clinical and etiological profile, management, and outcome of pediatric proptosis. Materials and Methods: This retrospective noncomparative study involved the analysis of record files of 41 cases of childhood proptosis, who presented in the eye outpatient department and oculoplastic clinic between January 2013 and December 2016. Results: There were a total of 41 children, age varied from 6 months to 15 years. The maximum number of cases 25 (61%) were in the age group of 0–5 years. Male:female ratio was 1.77:1. Unilateral cases were found to be 31 (76%) and bilateral cases be 10 (24%). Neoplastic lesions constituted 22 (54%) of which benign lesions were 3 (14%) and malignant were 19 (86%). Among malignant, primary were 11 (58%) and secondary were 8 (42%). Inflammatory/infective lesions were 8 (20%). Conservative/medical management was given to 11 cases (27%). Surgery with pre- or post-operative chemo- or radio-therapy was the treatment of choice. Conclusion: Neoplastic lesions were found to the most common cause of proptosis in this study. Apart from detail ocular and systemic examination, final etiological diagnosis is possible in certain cases only after investigations such as peripheral smear, bone marrow examination, histopathological examinations, ultrasonography B-scan, computed tomography scan, and magnetic resonance imaging. Timely diagnosis and management can save the vision and many a times lives.

Keywords: Neoplasia, neuroimaging, pediatric proptosis


How to cite this article:
Chakraborti C, Barua N, Chisthi RK, Manna S. Retrospective analysis of clinical profile of pediatric proptosis in a tertiary care hospital of Eastern India. J Clin Ophthalmol Res 2019;7:117-21

How to cite this URL:
Chakraborti C, Barua N, Chisthi RK, Manna S. Retrospective analysis of clinical profile of pediatric proptosis in a tertiary care hospital of Eastern India. J Clin Ophthalmol Res [serial online] 2019 [cited 2020 Apr 1];7:117-21. Available from: http://www.jcor.in/text.asp?2019/7/3/117/272714



Proptosis is defined as an abnormal protrusion of the eyeball which may be axial or nonaxial. The causes of pediatric proptosis are relatively distinct from those in adults and require urgent interference as they are commonly caused by infection or malignant lesions and can lead to early loss of vision.[1] Intraconal lesions such as orbital cellulitis, retinoblastoma (RB), glioma, neuroblastoma, and arteriovenous (AV) malformations produce axial proptosis in children. Extraconal lesions, e.g., dermoid cyst, parasitic cyst, rhabdomyosarcoma, lymphangioma, varices, neurofibroma, chloroma, and fibrous dysplasia can cause nonaxial proptosis in children.[2],[3]

Various studies have reported different frequencies of etiology of childhood proptosis.[4],[5],[6],[7],[8] In this present study, we have looked for causes of childhood proptosis with regard to their clinical and management profile.


  Materials and Methods Top


A retrospective analysis of the case records of pediatric proptosis was carried out at a tertiary care hospital in Eastern India over a period of 4 years from January 2013 to December 2016. The case records of proptosis patients in the age group of 0–15 years were obtained from the outpatient department and oculoplastic clinic of the hospital. Cases with insufficient records were excluded from the study. Written consents for publication of the patient's photos were obtained from the parents. History included duration, mode of onset, laterality, progression, and associated systemic illness. Any significant perinatal history, surgery, and family history were noted. Ocular examination included visual acuity, eyelids, and anterior- and posterior-segment evaluation. Exophthalmometry (with plastic ruler), extraocular muscle movements, retropulsion, palpation of orbital margins and eyelids for thrills and auscultation for bruits, slit-lamp biomicroscopy, examination under anesthesia, indirect ophthalmoscopy, intraocular pressure (IOP) measurement, and forced-duction test were done as and when required.

Investigations included routine hemogram with peripheral blood smear (PBS) examination, culture sensitivity, ultrasonography (USG) B-scan, computed tomography (CT)/magnetic resonance imaging (MRI), X-ray of orbital and paranasal sinuses, biopsy with histopathology, and bone marrow examinations. In various cases, patients were referred to different departments such as otorhinolaryngology, pediatrics, hematology, neurology, endocrinology, oncology, and radiotherapy for evaluation and treated accordingly. Hospital ethical committee approval was taken.


  Results Top


Cases were divided into five groups: congenital (dermoid cyst, craniosynostosis), inflammatory diseases (cellulitis), vascular lesions (AV malformations, hemangiomas), cystic lesion (cysticercosis), and neoplasia (benign and malignant).

[Table 1] shows the incidence of patients being highest in the age group of 0–5 years (n = 25) rest two groups have similar incidence. Male: female ratio was 1.77:1. Thirty-one cases (76%) were unilateral, and 10 cases (24%) were bilateral [Table 2]. Out of 41 cases, 30 presented with axial proptosis. Nineteen cases presented with acute proptosis. Acute cases were those of orbital cellulitis, rhabdomyosarcoma, RB, lymphangioma, and cysticercosis.
Table 1: Age group and sex distribution

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Table 2: Laterality

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[Table 3] depicts the causes of proptosis in all patients. The highest number of patients (n = 22) suffered from neoplastic lesions, followed by inflammatory disease (n = 8). Other diagnosis included cystic lesion (n = 3), vascular disease (n = 6), and congenital disorder (n = 2).
Table 3: Causes of proptosis in 41 patients

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Out of six RB cases, two were bilateral and three of them presented with fungating mass [Figure 1]. RB was associated with leukocoria (2), strabismus (1), uveitis (1), pseudohypopyon (1), proptosis (4), cellulitis (2), red eye (2), and fungating mass (2). On systemic examination, neurofibromatosis patients were found to have café-au-lait spots and pulsatile eye mass. On ocular examination, various features were decreased vision, strabismus, ophthalmoplegia, dystopia of the globe, high IOP, inflammation of the eyelids or conjunctiva, vitreous hemorrhage or inflammation, and afferent pupillary defect. In RB, calcification and orbital involvement were present in two cases [Figure 2]. One case had metastasis in brain leading to death of the child. Among leukemic patients (n = 5), three cases were acute lymphocytic lymphoma (ALL) and two were acute myeloid leukemia (AML). All ALL cases were bilateral. One of the AML cases presented with unilateral proptosis. One case of ALL presented with unilateral ciliary congestion and hyphema with raised IOP in that eye. One of the AML case presented with unilateral proptosis in which orbital encroachment was from granulocytic sarcoma of the maxillary sinus. In this case, blood picture was negative initially, and incisional biopsy and bone marrow aspiration clinched the diagnosis. Chemotherapy saved the child, but delay in diagnosis led to loss of vision in that eye.
Figure 1: Clinical picture of bilateral retinoblastoma. Left eye showing fungating mass

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Figure 2: Contrast-enhanced computed tomography scan showing bilateral calcification in retinoblastoma, proptosis in the left eye and right eye phthisis bulbi

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Optic nerve glioma presented with unilateral down and out proptosis, complete loss of vision, optic atrophy, and an intraconal mass in contrast-enhanced CT (CECT) with mild bony erosion. The tumor was removed through a lateral orbitotomy, but the eye became phthisical. Fibrous dysplasia of greater wing of sphenoid presented with mild axial proptosis; however, as the visual acuity was normal, he was asked for regular follow-up.

All cases of orbital cellulitis presented with fever, discharge, and proptosis; out of the seven cases, two were due to periocular and facial skin furunculosis, two due to sinusitis, two due to lid injury, and one due to tooth abscess. The cases of pseudotumor presented with features of orbital cellulitis and were diagnosed on the basis of clinical signs, CT scan, and response to steroid therapy.

A child with myocysticercosis presented with features of orbital cellulitis and was being treated initially in that line, but USG-B scan of orbit revealed cystic mass lesion [Figure 3]. It was treated with oral albendazole and steroid.
Figure 3: A case of myocysticercosis. Ultrasonography B scan of orbit revealed cystic mass lesion in orbit with a scolex. A-scan ultrasonography showing high amplitude spikes corresponding to the cyst wall. It was associated with thickening of the muscle. A small nonenhancing well-defined, hypodense lesion in medial rectus muscle was identified in computed tomography scan of left orbit

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Patient with Apert's syndrome had bilateral secondary optic atrophy in both eyes along with exposure keratopathy in one eye. In contrast to Cruzon's where proptosis is due to shallow orbital floor, lateral orbital margin is markedly protruded in Apert's syndrome, resulting in protrusion of eyeball. CECT brain showed ventriculomegaly with increased intracranial pressure [Figure 4]. Ventriculoperitoneal shunt was planned for the patient by neurosurgeon.
Figure 4: Proptosis in Apert's syndrome. Contrast-enhanced computed tomography showing ventriculomegaly as a sign of increased intracranial pressure

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Vascular lesions such as AV malformation and lymphangioma were treated conservatively with intralesional triamcinolone, leading to reduction of proptosis.

We gave more importance on clinical diagnosis, and the investigations were tailored accordingly. Few investigations such as immunohistochemistry could have been done in some cases as those facilities are not available in our center. The investigation which finally clinched the diagnosis was recorded as key investigation. In 24%, neuroimaging (CT scan/MRI) was the key investigation for diagnosis followed by clinical diagnosis as shown in [Table 4]. Next important investigation was hematological examination (29%); in 20%, histopathologic diagnosis was the most important investigation. Orbital USG-B scan aided to the diagnosis in few cases. In orbital cellulitis, CT revealed increased soft-tissue intensity with cavernous sinus involvement in two cases.
Table 4: Modalities of investigations

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As in our study, most patients had neoplastic lesions (54%), surgical removal with treatment along with pre- or post-operative radio- or chemo-therapy was mainstay of therapy. In rest of the cases, inflammatory conditions were managed by medical therapy; few congenital disorders required observation only [Table 5]. Cases of pseudotumor were treated successfully with high dose of oral steroid. In one out of the two cases, recurrence was found after 2 years.
Table 5: Modality of treatment

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Unilateral loss of vision was seen in 20 cases and bilateral loss in two cases which included patients of RB and ALL. Three children of RB and two cases of ALL died on follow-up.


  Discussion Top


Proptosis in pediatric age group is different than adults. Grave's/dysthyroid orbitopathy is the leading cause of proptosis in adults, whereas inflammatory and neoplastic lesions are the leading cause for proptosis in children.[9] Henderson's review of 764 tumors shows female: male ratio is 24:26.[10] In our study, this ratio was 43:57. A ratio of 40:60 was found in the study by Loganathan and Radhakrishnan.[2] In this study, majority of cases (n = 31) had unilateral presentation (76%). Inflammatory orbital cellulitis was found to be the most common cause of acute proptosis followed by rhabdomyosarcoma, RB, and cysticercosis. Similar results have been reported by Brazzo et al. and Dallow et al.[11],[12]

In most studies, orbital cellulitis is the most common cause in children;[2],[4],[5] however, in our study, neoplasm (54%) was found to be the most common orbital lesion. Similar results were found by Masud et al.[13] The causes of less number of orbital cellulitis are manifold. One reason we presume better management of orbital cellulitis with newer generation antibiotics has brought down the rate of conversion of preseptal to orbital cellulitis. The second reason can be as it is a tertiary care center; a few cases may have been admitted in pediatric and otorhinolaryngology department which were not included in the study. Similar results were seen by Loganathan and Radhakrishnan and Dallow et al.[2],[12] Bajaj et al. and Jha and Lamba reported malignancy as the most common cause of childhood proptosis.[6],[7] None of the three bilateral RB cases had positive familial history.

Proptosis as a primary presentation is more common in ALL than AML.[14] Out of two AML cases, one had proptosis as primary presentation in which the peripheral blood smear was negative initially. Incisional biopsy of the orbital mass followed by bone marrow biopsy revealed the diagnosis.

Previous studies have shown decreased incidence of proptosis due to primary intraocular malignancy like RB. Due to availability of modern equipment, early diagnosis and treatment of such deadly tumors are possible.[2] This was, however, not true in our study. We have found three cases of advanced RB out of the total six cases. This difference may be due to lack of awareness among the low socioeconomic strata to avail the health care facilities.

Secondary neoplastic cases were lymphoma and leukemia. In previous study, leukemia and neuroblastoma were the main causes.[12] In a number of malignant lesions can present as masquerading syndrome, e.g., RB or rhabdomyoma presenting as orbital cellulitis.[15] Rizvi et al. in a retrospective study of 25 proptosis cases found pediatric orbital tumor in 7, i.e., 46.66%.[16]

The causes of inflammations were found to be orbital cellulitis and pseudotumor. It shows similar results of previous studies by Dallow et al. of 257 cases of childhood proptosis.[12]

Noninvasive modalities such as neuroimaging (CT/MRI) were the primary modality in diagnosing and staging of the disease. Similar results were also reported by Bakhshi et al.[17] MRI is challenging in pediatric cases. The availability, low cost, and relatively faster speed of CT scanning make the CT scan a valuable diagnostic tool.[10] Our study shows that CT/MRI provided accurate diagnosis in 24% of the cases and CT-guided fine-needle aspiration cytology aided in diagnosis in 14% (n = 5) of cases. This finding is supported by previous studies and considered the best investigation for proptosis in a child.[8]

In cases of bilateral proptosis in children, initial investigations should include PBS examination for diagnosing hematological malignancies such as AML or ALL rather than going for much costlier neuroimaging modalities. Murthy et al. recommended that PBS should be done in all cases of sudden onset proptosis or an orbital mass in children along with an orbital biopsy.[18]

In our study, surgery was the main modality of treatment in neoplastic lesions and some infective conditions such as orbital abscess. Inflammatory conditions which constituted bulk of cases were managed by medical therapy, and few congenital disorders required observation only.

Small sample size, only one tertiary care hospital data, and nonavailability of few investigative modalities are the main limitations of our study. Nevertheless, the study may be valuable to general ophthalmologist, pediatrician, otolaryngologist, and radiologist regarding diagnosis and management of pediatric proptosis.


  Conclusion Top


The current study shows that neoplastic lesions are the major cause of pediatric proptosis followed by inflammatory conditions. Among the neoplasms, malignant lesions ere more common, of these majority were metastasis from leukemia. Each case of pediatric proptosis needs to be analyzed individually with special attention to ocular as well as systemic disabilities. Thorough knowledge of orbital and periorbital anatomy and appropriate investigations are required for a correct diagnosis and a timely referral to save the vision and lives of these patients.

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 Top

1.
Ganessan K, Bakhshi S. Proptosis in children: Approach. Indian J Med Paediatr Oncol 2003;24:33-4.  Back to cited text no. 1
    
2.
Loganathan M, Radhakrishnan M. An etiological analysis of childhood proptosis. J Evol Med Dent Sci 2014;3:6158-61.  Back to cited text no. 2
    
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Rootman J, editor. Diseases of the Orbit: A Multidisciplinary Approach. 2nd ed. Philadelphia: Lippincott Williams and Wilkins; 2003.  Back to cited text no. 3
    
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Satpute K, Chingsuingamba Y. A retrospective analysis of presentation and management outcome of proptosis in a tertiary care centre of North-East, India-a case series. J Dent Med Sci 2013;5:30-2.  Back to cited text no. 4
    
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Sindhu K, Downie J, Ghabrial R, Martin F. Aetiology of childhood proptosis. J Paediatr Child Health 1998;34:374-6.  Back to cited text no. 5
    
6.
Bajaj MS, Pushker N, Chaturvedi A, Betharia SM, Kashyap S, Balasubramanya R, et al. Orbital space-occupying lesions in Indian children. J Pediatr Ophthalmol Strabismus 2007;44:106-11.  Back to cited text no. 6
    
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Jha BK, Lamba PA. Proptosis as a manifestation of acute myeloid leukaemia. Br J Ophthalmol 1971;55:844-7.  Back to cited text no. 7
    
8.
Ghosh S, Shinde SC, Kumaran GS, Sapre RS, Dhond SR, Badrinath Y, et al. Haematologic and immunophenotypic profile of acute myeloid leukemia: An experience of Tata memorial hospital. Indian J Cancer 2003;40:71-6.  Back to cited text no. 8
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Char DH. Tumors of the Eye and Ocular Adnexia. Hamilton: BC. Decker, Inc.; 2001.  Back to cited text no. 9
    
10.
Garrity AG, Henderson WJ. Vascular hamartomas hyperplasia neoplasm. In: Garrity AG, Henderson WJ, Cameron DJ editors. Henderson's Orbital Tumours. 4th ed. USA: Lippincott Williams & Wilkins; 2007. p. 210-5.  Back to cited text no. 10
    
11.
Macdonald D Jr, Schneider K, Della Rocca R, Brazzo B. Child-hood disorders of the orbit and ocular adnexa. In: Nesi F, LismanR, Levine M, eds. Smith's ophthalmic plastic and reconstructivesurgery. 2nd ed. St Louis: Mosby, 1998:835-52.  Back to cited text no. 11
    
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Dallow RL, Pratt SG, Green JP. Approach to orbital disorders and frequency of disease occurrence. In Albert & Jakobiec's Principles and Practice of Ophthalmology. Volume 4, 2nd ed. WB Saunders; 2000. p. 3056-67.  Back to cited text no. 12
    
13.
Masud MZ, Babar TF, Iqbal A, Khan MT, Zaffar UI, Khan MD, et al. Proptosis: Etiology and demographic patterns. J Coll Physicians Surg Pak 2006;16:38-41.  Back to cited text no. 13
    
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Charif Chefchaouni M, Belmekki M, Hajji Z, Tahiri H, Amrani R, El Bakkali M, et al. Ophthalmic manifestations of acute leukemia. J Fr Ophthalmol 2002;25:62-6.  Back to cited text no. 14
    
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Gutierrez JC, Fischer AC, Sola JE, Perez EA, Koniaris LG. Markedly improving survival of neuroblastoma: A 30-year analysis of 1,646 patients. Pediatr Surg Int 2007;23:637-46.  Back to cited text no. 15
    
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Rizvi SA, Gupta Y, Gupta M. Surgical treatment and histopathological analysis of proptosis. Nepal J Ophthalmol 2010;2:31-4.  Back to cited text no. 16
    
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Bakhshi S, Singh P, Chawla N. Malignant childhood proptosis: Study of 104 cases. J Pediatr Hematol Oncol 2008;30:73-6.  Back to cited text no. 17
    
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Murthy R, Vemuganti GK, Honavar SG, Naik M, Reddy V. Extramedullary leukemia in children presenting with proptosis. J Hematol Oncol 2009;2:4.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]



 

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