|Year : 2008 | Volume
| Issue : 2 | Page : 157-159
Solitary ectopic lacrimal gland tissue of the orbit presenting with unilateral proptosis in a 5-year-old child
Nurullah Yuceer1, Erdener Ozer2
1 Department of Neurosurgery, School of Medicine, University of Dokuz Eylul, Izmir, Turkey
2 Department of Pathology, School of Medicine, University of Dokuz Eylul, Izmir, Turkey
Department of Neurosurgery, School of Medicine, University of Dokuz Eylul, Balcova, 35340 Izmir
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Ectopic lacrimal gland tissue (ELGT) of the orbit is seen rarely. A 5-year-old child with an ELGT of the right orbit causing to unilateral proptosis is presented. Computerized tomography and magnetic resonance imaging studies showed an irregular, soft-tissue in the right orbit, without bony erosion. The lesion was excised subtotally by right frontal transcranial approach.
Keywords: Chilhood, magnetic resonance imaging, neoplasm, orbit, proptosis
|How to cite this article:|
Yuceer N, Ozer E. Solitary ectopic lacrimal gland tissue of the orbit presenting with unilateral proptosis in a 5-year-old child. J Pediatr Neurosci 2008;3:157-9
| Introduction|| |
Unilateral proptosis in childhood is usually an ominous symptom suggesting intraorbital malignancy, such as non-Hodgkin's lymphoma, rhabdomyosarcoma, metastatic neuroblastoma, and Ewing's sarcoma. ,, Differentiating between orbital benign lesions and malignant tumors is imperative. Ectopic lacrimal gland tissue (ELGT) of the orbit as a cause of unilateral proptosis is relatively uncommon in childhood. , We describe a pediatric case with ELGT of the orbit causing to unilateral proptosis.
| Case Report|| |
A 5-year-old girl was admitted to our clinic with 3 months history of proptosis in the right eye. Physical examination revealed proptosis in the right eye. The patient's ophthalmological examinations consisted of testing visual acuity, fundoscopy, and Goldman perimetry for visual field defects revealed decrease of visual acuity in the right eye (R, 2/10; L, 4/10). Mobility of the right eyeball was slightly restricted in lateral direction. Computerized tomography (CT) demonstrated an irregular, soft-tissue in the neighborhood of the optic nerve in the right orbit without bony erosion. The most likely diagnosis from the CT was intraorbital neoplasm [Figure 1]. T1-weighted and T2-weighted magnetic resonance imaging (MRI) studies of the orbit revealed a well-encapsulated globular intraorbital lesion [Figure 2]. T1-weighted MR images showed a globular lesion that consisted of hyperintense and hypointense areas around the optic nerve in posterior of globe [Figure 2A],[Figure 2B]. T2-weighted MR images demonstrated a hyperintense lesion [Figure 2C],[Figure 2D]. Contrast-enhanced T1-weighted MR images revealed a hyperintense lesion [Figure 2E],[Figure 2F]. The most likely diagnosis from the MRI was intraorbital cavernous hemangioma. Right frontal craniotomy and orbital unroofing were used to remove the lesion. After the orbital unroofing was complete, it was approached to the lesion by retracting the superior rectus and levator muscles laterally. The lesion was appeared as a nodular, pinkish, fleshy mass, and the lesion was excised. Then, the mass was excised by retracting the lateral rectus and levator muscles laterally. The lesion was adherent to neighborhood tissues. For this reason, it could be subtotally removed. Pathological examination disclosed a nest of normal gland tissue surrounded by a cystic lesion lined two layers of lacrimal duct epithelium cells [Figure 3A],[Figure 3B]. Right lateral rectus muscle paresis developed in early postoperative stage. Both proptosis and lateral rectus muscle paresis improved partially during a 12-month period of follow-up, and there was absent recurrence of the mass.
| Discussion|| |
The lacrimal gland is composed of the main lacrimal gland located in the orbital lacrimal fossa. Ectopic lacrimal gland comprises lacrimal gland tissue outside of the orbital lacrimal fossa.  ELGT may present as an isolated solitary mass,  as in our case, or as part of a complex choristoma, where lacrimal gland tissue is seen in combination with a variety of mesodermal tissues.  ELGT of the orbit as a solitary mass or as a complex choristoma is relatively uncommon. ,,,,, The most common extraconal tumors in childhood are dermoid cyst and capillary hemangioma. The most common intraconal tumors in childhood are optic nerve glioma, hemorrhagic lymphangioma, and rhabdomyosarcoma. , The most common malignant tumors involving the orbit in children are rhabdomyosarcoma and metastatic tumors. Benign orbital lesions in children include congenital vascular or dermoid tumors. 
Preoperative diagnosis of ELGT is very difficult. Alyahya et al ,  reported that only two lesions from 61 tissue with ELGT was preoperatively diagnosed. CT scan of the intraorbital ELGT shows an irregular, soft-tissue mass of increased density that demonstrates variable degrees of enhancement. ,, Solaroglu et al ,  reported a case of intraorbital ELGT that destroyed the lateral wall of the orbit on CT scan. In the presented case, MRI studies of the orbit revealed a well-encapsulated globular intraorbital lesion. The lesion had a hyperintense appearance on MR images. After the contrast administration, the mass was appeared as hyperintense. Radionuclide studies may be useful for the differential diagnosis between intraorbital malignancy and ELGT. Technetium-99 m-pertechnetate SPECT demonstrates marked uptake in ELGT. 
Selecting the most suitable surgical treatment for orbital tumors requires a thorough knowledge of the localization and size of the lesion, its apparent site of origin, and its probable histologic status. These features are gleaned from clinical signs, radiologic findings of especially CT scans, and MR images.  Lesions of the apex are the best approached transcranially or laterally. In the presented patient, lateral orbital approach or transcranial approach may be choiced. The lesion was located both in the apex of orbita and in medial, lateral, superior, and inferior of the optic nevre. We preferred the transcranial approach in result of the preoperative clinic and radiologic evaluation. The lesion was subtotally excised. Postoperatively, the patient had paresis of right lateral rectus muscle. However, both proptosis and lateral rectus muscle paresis improved partially during a 12-month period of follow-up.
In conclusion, intraorbital ELGT as a cause of unilateral proptosis in childhood is a rare lesion. Preoperative diagnosis of the intraorbital ELGT is difficult. However, especially preoperative MR images can be helped for the diagnosis.
| References|| |
|1.||Shields J, Shields C, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997-1008. |
|2.||Ohtsuka K, Hashimoto M, Suzuki Y. A Review of 244 orbital tumors in Japanese patients during a 21-year period: Origins and locations. Jpn J Ophthalmol 2005;49:49-55. [PUBMED] [FULLTEXT]|
|3.||Sindhu K, Downie J, Ghabrial R, Martin F. Aetiology of childhood proptosis. J Paediatr Child Health 1998;34:374-6. [PUBMED] |
|4.||Sakurai H, Mitsuhashi N, Hayakawa K, Nozaki M, Kurosaki H, Kishi S, et al . Ectopic lacrimal gland of the orbit. J Nucl Med 1997;38:1498-500. [PUBMED] [FULLTEXT]|
|5.||Alyahya GA, Bangsgaard R, Prause JU, Heegaard S. Occurrence of lacrimal gland tissue outside the lacrimal fossa: Comparison of clinical and histopathological findings. Acta Ophthalmol Scand 2005;83:100-3. [PUBMED] [FULLTEXT]|
|6.||Appel N, Som PM. Ectopic orbital lacrimal gland. J Comput Assist Tomogr 1982;6:1010-2. [PUBMED] |
|7.||Pfaffenbach DD, Green WR. Ectopic lacrimal gland. Int Ophthalmol Clin 1971;11:149-59. [PUBMED] |
|8.||Jacobs L, Sirkin S, Kinkel W. Ectopic lacrimal gland in the orbit identified by computerized axial transverse tomography. Ann Ophthalmol 1977;9:591-3. [PUBMED] |
|9.||Solaroglu ?, Okutan O, Kaptanoglu E, Beskonakl? E. Unilateral proptosis arising from an ectopic lacrimal gland of the orbit. J Neurosurg (Pediatrics 3) 2005;103:289-91. |
|10.||Kennerdell JS, Maron JC, Malton ML. Surgical approaches to orbital tumors. Clin Plast Surg 1988;15:273-82. |
[Figure 1], [Figure 2], [Figure 2A], [Figure 2B], [Figure 2C], [Figure 2D], [Figure 2E], [Figure 2F], [Figure 3A], [Figure 3B]