<%server.execute "isdev.asp"%> A case of intraorbital intraconal lymphangioma with post-traumatic apoplexy Rao G P, Reddy M S, Mohammed I, Reddy K A, Reddy E S - J Pediatr Neurosci
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Year : 2018  |  Volume : 13  |  Issue : 2  |  Page : 208-210

A case of intraorbital intraconal lymphangioma with post-traumatic apoplexy

1 Department of Neurosurgery, Gandhi Medical College and Hospital, Padmarao Nagar, Secunderabad, Telangana, India
2 Department of Ophthalmology, Pushpagiri Vitreo Retinal Institute and Hospital, Secunderabad, Telangana, India

Date of Web Publication5-Jul-2018

Correspondence Address:
M Siddartha Reddy
Department of Neurosurgery, 4th Floor, Gandhi Medical College and Hospital, Padmarao Nagar, Secunderabad, Telangana 500025
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JPN.JPN_39_18

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Orbital lymphangioma is an infrequent benign cystic lesion manifesting in childhood and presenting with slowly progressive proptosis, and restriction of eye movements. Here we report a rare case of 8 year old male patient presenting with unilateral painful proptosis and subconjunctival hemorrhage with decrease of vision and restriction of eye movements. CT scan and MRI were done which revealed an intraorbital and intraconal cystic space occupying lesion. Fronto-orbito zygomatic craniotomy was done for orbital decompression and subtotal excision of tumor and blood evacuation. Histopathological findings were suggestive of lymphangioma.

Keywords: Intraconal, intraorbital, lymphangioma, orbital decompression, proptosis

How to cite this article:
Rao G P, Reddy M S, Mohammed I, Reddy K A, Reddy E S. A case of intraorbital intraconal lymphangioma with post-traumatic apoplexy. J Pediatr Neurosci 2018;13:208-10

How to cite this URL:
Rao G P, Reddy M S, Mohammed I, Reddy K A, Reddy E S. A case of intraorbital intraconal lymphangioma with post-traumatic apoplexy. J Pediatr Neurosci [serial online] 2018 [cited 2023 Feb 5];13:208-10. Available from: https://www.pediatricneurosciences.com/text.asp?2018/13/2/208/235955

   Introduction Top

Orbital lymphangioma is a rare, benign, slow-growing, cystic, vascular malformation constituting up to 0.3%–1.5% of diagnosed orbital tumors. It occurs in children and teenagers, but more frequently during the first decade of life. Spontaneous hemorrhages can occur in 55% of the cases. These cases may often present with acute proptosis and loss of vision. Different modes of treatment for orbital lymphangioma are conservative management, partial resection of the cystic lesion, and local radiotherapy. Orbital decompression can also be performed occasionally to restore vision. Recurrences are quite common as complete surgical resection is very difficult.

   Case Report Top

An 8-year-old male patient was brought to outpatient department with complaints of bulging of left eye for 10 days. The patient was apparently asymptomatic before 10 days, following which he started developing bulging of the left eye, gradually progressive, associated with continuous pain and watery discharge from the left eye and continuous frontal headaches. The child’s father reported a history of trauma 12 days back. The child denies the history of fever, a decrease of vision, or vomiting. The child did not have a history of surgery or upper respiratory tract infection.

On examination, there was a unilateral proptosis of the left eye with subconjunctival hemorrhage and restriction of eye movements. There was a loss of vision in the affected eye (counting fingers up to 3 m). Computed tomography (CT) scan revealed multiple, hypodense, nonenhancing, intraconal lobulated lesions. Magnetic resonance imaging (MRI) revealed a heterogeneous cystic multiseptated soft-tissue lesion measuring 2.4×2.5×3cm with intraconal and extraconal component noted in the left orbit [Figure 1]. The cyst contained fluid of varying intensity suggestive of left orbital lymphangioma with internal hemorrhage. Left orbitozygomatic craniotomy was performed, and the cystic space–occupying lesion was excised and hematoma was evacuated. The specimen was sent to histopathology examination, which suggested features of lymphangioma [Figure 2]. The postoperative course was uneventful with good aesthetic result, complete reduction of proptosis and pain, and improvement of vision [Figure 3]. The patient was followed up at the end of 1 and 3 months and had a good recovery with no recurrence.
Figure 1: Magnetic resonance imaging (MRI) revealed a heterogeneous cystic multiseptated soft-tissue lesion measuring 2.4 × 2.5 × 3 cm with intraconal and extraconal component noted in the left orbit

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Figure 2: Histopathological picture showing lymphatic channels

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Figure 3: Preoperative and postoperative proptosis

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   Discussion Top

Orbital lymphangiomas are rare benign vascular tumors and constitute up to 0.3%–1.5% of histopathologically diagnosed orbital tumors.[1] These tumors are usually congenital and slow growing, and may not become apparent for months or years.[2],[3],[4],[5] Lymphangiomas are common during the first decade of life.[6] Spontaneous hemorrhage occurs in 55% of the cases, resulting in sudden increase in size of tumor, loss of vision, proptosis, and compressive optic neuropathy.[2],[3],[7] Lymphangiomas are more common in males.[8] Histologically, lymphatic malformations consist of nonencapsulated channels lined by a single layer of endothelium. The International Orbital Society classified the orbital vascular malformations into three groups based on their clinical and hemodynamic relationships: (1) no-flow malformations, (2) venous-flow malformations, and (3) arterial-flow malformations.[9] The lymphangioma or lymphatic malformation falls into the no-flow malformation category because it is hemodynamically isolated.

Most of the lesions are asymptomatic until hemorrhage or upper respiratory tract infection occurs, resulting in proptosis, decreased vision, decreased motility, and pain. CT and MRI show a cluster of nonenhancing “grape-like” cystic lesions with internal septations.[10] Old bleeds within the lesion appear hyperintense on T1 imaging, whereas recent ones appear hypointense.[11],[12] Lymphangiomas can be classified radiographically as macrocystic (cystic structures >1cm in size), microcystic (cystic structures <1cm in size), or combined.[13],[14]

Treatment options for lymphangioma include conservative therapy, partial surgical excision of the cystic lesion, intralesional injection of the sclerosing agent, and local radiotherapy; in case of intralesional hemorrhage, orbital decompression can be performed.[2],[3],[7],[15] Recurrences are quite common as complete surgical excision is very difficult.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Sekhar LN, Tariq F. Orbital lymphangiomas: surgical treatment and clinical outcome. World Neurosurg 2013;13:710-11.  Back to cited text no. 1
Kalisa P, Zieleghem BV, Roux P, Meire F. Orbital lymphangioma: clinical features and management. Bull So Belge Ophtalmol 2001;13:59-70.  Back to cited text no. 2
Malhotra AD, Parikh M, Garibaldi DC, Merbs SL, Miller NR, Murphy K. Resection of an orbital lymphangioma with the aid of an intralesional liquid polymer. AJNR Am J Neuroradiol 2005;13:2630-4.  Back to cited text no. 3
Rootman J, Hay E, Graeb D, Miller R. Orbital-adnexal lymphangiomas. A spectrum of hemodynamically isolated vascular hamartomas. Ophthalmology 1986;13:1558-70.  Back to cited text no. 4
Goto H, Usui M, Okada S. Histopathological study of orbital lymphangioma in an infant. Jpn J Ophthalmol 2004;13:594-7.  Back to cited text no. 5
Simas N, Farias JP. Orbital lymphangiomas: surgical treatment and clinical outcomes. World Neurosurg 2014;13: 842.e5-10.  Back to cited text no. 6
Oyama T, Eguchi K, Cho H, Abe H. [A variety of orbital lymphangioma treatments: one case treated with orbital decompression therapy and the other case with intralesional injection of OK-432 therapy]. Nippon Ganka Gakkai Zasshi 2009;13:732-40.  Back to cited text no. 7
Tunç M, Sadri E, Char DH. Orbital lymphangioma: an analysis of 26 patients. Br J Ophthalmol 1999;13:76-80.  Back to cited text no. 8
Harris GJ. Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Orbital society. Am J Ophthalmol 1999;13:453-5.  Back to cited text no. 9
Khan SN, Sepahdari AR. Orbital masses: CT and MRI of common vascular lesions, benign tumors, and malignancies. Saudi j Ophthalmol 2012;13:373-83.  Back to cited text no. 10
Bilaniuk LT. Vascular lesions of the orbit in children. Neuroimaging Clin N Am 2005;13:107-20.  Back to cited text no. 11
Pahwa S, Sharma S, Das CJ, Dhamija E, Agrawal S. Intraorbital cystic lesions: an imaging spectrum. Curr Probl Diagn Radiol 2015;13:437-48.  Back to cited text no. 12
Hill RH III, Shiels WE II, Foster JA, Czyz CN, Stacey A, Everman KR, et al. Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg 2012;13:119-25. Available from: https://journals.lww.com/op-rs/Fulltext/2012/03000/Percutaneous_Drainage_and_Ablation_as_First_Line.6.aspx.  Back to cited text no. 13
Gooding C, Meyer D. Intralesional bleomycin: a potential treatment for refractory orbital lymphangiomas. Ophthal Plast Reconstr Surg 2014;13:e65-7.  Back to cited text no. 14
Gündüz K, Demirel S, Yagmurlu B, Erden E. Correlation of surgical outcome with neuroimaging findings in periocular lymphangiomas. Ophthalmology 2006;13:1231.e1-8.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3]

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