Key-words:
Intraconal - lymphangioma - orbital
Introduction
Orbital lymphangioma is a rare vascular malformation. It is a benign but severe anomaly
because of its infiltrative, diffuse, and hemorrhagic nature, and its high morbidity
rate. Surgical resection is a real challenge on account of the intricate architecture
of the lesion. It constitutes 0.3% to 4% of all orbital tumors.[[1]]
Case Report
A 68-year-old male patient presented with a history of decreased vision in the left
eye and diplopia. The symptoms started following a trauma to the left eye 6 months
back. He is a known case of coronary artery disease on antiplatelet medications. Examination
showed restricted movements of the left eye, proptosis, and decreased vision of 6/60
on Snellen chart. Contrast magnetic resonance imaging (MRI) scan showed a well-defined
2.2 cm × 1.8 cm × 1.8 cm fairly rounded, slightly lobulated intraconal tumor in the
retrobulbar region inferior to optic nerve. The optic nerve is displaced superiorly.
Moderate heterogeneous contrast enhancement of the tumor was noted [[Figure 1]]. A provisional diagnosis of optic nerve sheath meningioma was made.
Figure 1: Axial (a), sagittal (b), and coronal (c) contrast magnetic resonance imaging scan
showing an intraorbital intraconal tumor in the retrobulbar region. (MRI- Magnetic
resonance imaging)
Antiplatelet medication clopidogrel was stopped before surgery. Patient underwent
the left fronto-temporo-orbito-zygomatic (FTOZ) craniotomy using three piece technique
[[Figure 2]]. Zygomatic arch was cut at two ends and separated along with its attached masseter
muscle. Fronto-temporal craniotomy was done and the orbital wall was removed separately.
Intraconal tumor was accessed through the window between levator muscle and lateral
rectus muscle [[Figure 3]]. The tumor was grayish red in color, firm in consistency, moderately vascular with
lobulated surface. Few foci of hemorrhages were also seen. Near total excision was
done [[Figure 4]]. Small parts of the tumor adhering to the optic nerve and surrounding structures
were left behind. FTOZ bones fixed back [[Figure 5]]. Patient's vision improved in the postoperative period. Diplopia and proptosis
were subsided. On the postoperative day 3, patient developed desaturation of oxygen
with myocardial infarction. Patient was kept on ventilator, and injection heparin
infusion was started. Following this, patient developed extradural and intraorbital
bleeding with subgaleal and periorbital swelling. Aspiration of the blood under the
scalp flap was done. After stabilization of the patient, vision was assessed and found
to be deteriorated to become perception of light only.
Figure 2: Exposed orbital contents, frontal and temporal dura following left fronto-temporo-orbito-zygomatic
craniotomy
Figure 3: Intraconal tumor was exposed through the window between levator muscle and lateral
rectus muscle
Figure 4: Computed tomography scan showing near total excision of left orbital intraconal tumor
(CT - Computed tomography)
Figure 5: Left fronto-temporo-orbito-zygomatic bones fixed with mini plates and screws
Histopathology showed a fibrocollagenous tissue composed of congested blood vessels
and dilated lymphatic channels with lymphoid cell aggregates. Focal areas showed lymphoid
follicles. Lymphatic channels of varying sizes were noted [[Figure 6]]a and [[Figure 6]]b. Immunohistochemistry showed epithelial membrane antigen [[Figure 7]] and vementin negative. CD34 showed strong positivity in the vascular and lymphatic
channels [[Figure 8]]. These features were suggestive of lymphangioma.
Figure 6: Hematoxylin and Eosin stain showing dilated lymphatic channels of varying sizes (a)
and dense lymphoid aggregates forming lymphoid follicles (b). (H and E - Hematoxylin
and Eosin)
Figure 7: Immunohistochemistry showing epithelial membrane antigen negative cells (EMA - Epithelial
membrane antigen)
Figure 8: Immunohistochemistry showing CD 34 positive cells in lymphatic channels
Discussion
A study evaluated retrospective data of 26 orbital lymphangioma patients managed over
16 years. At presentation, proptosis was present in 85%, ptosis in 73%, and restrictive
eye movements in 46% of patients. Most patients developed symptoms in the first (77%)
or second decades (15%) of life. Two lymphangiomas were diagnosed during adult life,
at 48 and 64-year-old, respectively. The accuracy of the initial radiology interpretations
was 77%. Only two patients had an isolated intraconal lesion. Twenty-four cases required
one or more surgeries. The mean follow-up was 9.2 years (range 1–14 years). Recurrence
was noted in 58% of patients.[[2]]
Park et al., reviewed the experience of 19 patients with orbital tumors. The surgical
approaches used were transcranial in 17 patients. Tumors located in the intraconal
or perioptic space were surgically excised using a frontoorbital approach (8 cases),
whereas pterional (3 cases), orbital (2 cases), and combined approaches (6 cases)
were used for tumors in other sites. Total resection of tumors was achieved in 12
of 19 patients. In 4 patients with glioma and lymphoma, only diagnostic biopsy was
done. Three patients experienced visual deterioration postoperatively. Surgical treatment
could be the mainstay of therapy for the majority of symptomatic orbital tumors. Many
orbital tumors can be treated safely via a transcranial approach. Frontoorbital approach
allows the surgeon to reach both the intraorbital and intracranial structures.[[3]]
Orbital lymphangioma may be difficult to diagnose in young patients who present with
sudden proptosis due to hemorrhage into a previously unrecognized lesion. MRI should
be ideally suited for evaluating lymphangiomas due to its unique ability to characterize
hemorrhage because of the paramagnetic qualities of hemoglobin. It differentiates
hemorrhagic cysts, and has unique ability to detect tumor feeding vessels by the flow
void phenomenon.[[4]]
Orbital lymphangioma is a disease of children and constitutes 0.3%–4% of all orbital
tumors. Adult orbital lymphangioma and isolated intraconal lymphangioma are rare.
Authors reported a very rare case of isolated intraconal orbital lymphangioma which
has occurred in an adult patient of 68 years old.
Conclusion
Although rare, intraconal orbital lymphangioma should be kept in the differential
diagnosis of orbital tumors presenting in adult patients following a trauma. It can
radiologically mimic other intraorbital tumors. Intraconal orbital lymphangioma is
a surgical challenge and FTOZ craniotomy provides direct access to the intraconal
orbital compartment. FTOZ is an ideal technique for the neurosurgeon for accessing
the intraconal compartment due to the familiarity with the technique.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient has given his consent for his 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.
