We report a rare case of low-grade systemic B-cell non-. Hodgkin's lymphoma (NHL) causing central retinal artery and vein occlusion, which was the only ...
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stasis during delivery may cause marked chemosis and prolapse of the conjunctiva, causing eversion of the eyelids.8 Once everted orbicularis spasm may act as sphincter, that leads to a vicious cycle of conjunctival strangulation and edema, secondary to venous stasis. 9 The chemosed conjunctiva protects the cornea from exposure and hence, corneal complications are rare.
References
Congenital eyelid eversion can be treated conservatively. The goal of management is to prevent dessication of the exposed conjunctiva and allow spontaneous inversion of the lid. Surgical treatment options include temporary tarsorrhaphy, subconjunctival injection of hyaluronic acid, fornix sutures and full thickness skin graft to the upper lid. Our case was managed by topical lubrication, antibiotics and patching of the eyelid. This case illustrates the need for early successful repositioning of the eyelid, to break a cycle leading to increasing lid edema, conjunctival chemosis and eversion. Congenital eyelid eversion is very rare and may present “once in a life time” to the ophthalmologist. However, it is important to note that the condition is very amenable to conservative treatment, if started early.
Combined central retinal artery and vein occlusion secondary to systemic non-Hodgkin’s lymphoma Dhananjay Shukla, MS; Amit Arora, MS;
Khazaei M Hadi, MS; Mahesh Kumar, DNB;
Satish Baddela, MS; Ramasamy Kim, DNB
We report a rare case of low-grade systemic B-cell nonHodgkin’s lymphoma (NHL) causing central retinal artery and vein occlusion, which was the only manifestation of disease recurrence. A young man with resolved systemic NHL underwent fluorescein angiography, magnetic resonance imaging and computed tomography to investigate a severe unilateral visual loss. A combined vascular occlusion was observed in the right eye. Neuroimaging detected optic nerve infiltration; but no systemic/ central nervous system involvement was observed. The patient was treated with highdoses of corticosteroids and optic nerve irradiation. The optic neuropathy and vascular occlusion were resistant to treatment. The subsequent neovascular glaucoma was treated by Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India Correspondence to Dhananjay Shukla, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, 1 Anna Nagar, Madurai 625 020, Tamil Nadu, India. E-mail: Manuscript received: 18.4.05; Revision accepted: 28.11.05
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Adams AL. A case of double congenital ectropion. Med Forthnight 1896;9:137-8.
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Watts MT, Dapling RB. Congenital eversion of the upper eyelid: A case report. Ophthal Plast Reconstr Surg 1995;11:293-5.
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Dawodu OA. Total eversion of the upper eyelids in a newborn. Niger Postgrad Med J 2001;8:145-7.
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Al Hussain HA, Al-Rajhi AA, Al-Qahtani S, Meyer D. Congenital upper eyelid eversion complicated by corneal perforation. Br J Ophthalmol 2005;89:771.
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Conlon MR, Sutula FC. Congenital eyelid anomalies. In Albert DM, Jakobiac FA (editors): Principles and Practice of Ophthalmology, vol 4, 2nd ed. WB Saunders Co: Philadelphia, PA; p. 3336.
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Rabb EL, Saphir RL. Congenital eyelid eversion with orbicularis spasm. J Pediatr Ophthal Strabismus 1985;22:125-8.
panretinal photocoagulation, which relieved the pain, but vision was not recovered. No further recurrence was observed over the following year.
Key words: Central retinal artery, central retinal vein, combined occlusion, non-hodgkin’s lymphoma. Indian J Ophthalmol 2006;54:204-6
In a post-treatment non-Hodgkin’s lymphoma (NHL), ocular lymphoma may be the first sign of a systemic relapse.1 We report, probably the first case of a benign systemic NHL-in remission, causing central retinal artery and vein occlusion, without any other associated central nervous system (CNS) involvement.
Case Report A 28-year-old man presented with abdominal pain to his physician. There was no history of weight loss or fever. Ultrasonography of abdomen showed hepatomegaly and abdominal lymphadenopathy. Systemic examination showed palpable axillary lymph nodes. Fine-needle aspiration cytology from the left axillary lymph nodes showed a nodal marginal zone B-cell NHL. Bone marrow biopsy was normal. Six cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone were given. The patient responded well, with complete remission of the lymphoma, radiologically and ultrasonographically. Four months later, his right eye suffered a sudden drop in
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visual acuity to light perception; the left eye was 20/20. An afferent pupillary defect was noted in the right eye. The anterior segment and angles were unremarkable on slit-lamp biomicroscopy and gonioscopy in either eye. Intra-ocular pressure was normal in both the eyes. Fundus examination of the right eye showed a grossly swollen optic disc, pale macular edema with a cherry-red spot, venous tortuosity and extensive retinal hemorrhages [Figure 1a]. Fluorescein angiography showed no retinal vascular perfusion [Figure 1b]. Left fundus was normal. Magnetic resonance imaging (MRI) and computerized tomography (CT) showed thickening of the right optic nerve and enhancement of the optic-nerve-sheath complex in a post-contrast study [Figure 2]. CNS was not involved. Chest X-ray, abdominal ultrasound and CT failed to detect any systemic recurrence. Intravenous methyl prednisolone (15 mg/kg/day) was given for three days, followed by oral prednisolone, 1 mg/kg. The patient was referred to an oncologist for radiotherapy of the optic nerve. Two months later, he returned with pain in right eye. The vision remained light perception. Anterior segment examination showed a steamy cornea, a persistent afferent pupillary defect and rubeosis of the iris and angles. Intraocular pressure was 40 mmHg by applanation tonometry. Fundus
showed reduced severity of hemorrhages and disc edema. Panretinal photocoagulation was done to treat the neovascular glaucoma. The rubeosis and pain regressed; a stable ocular and systemic status was observed for the next 12 months.
Discussion NHLs are a heterogeneous group of malignancies of the lymphoid system. The WHO classification of hematopoietic and lymphoid tumors classifies lymphomas into B-cell and T-cell neoplasms; the former account for more than 85% of all lymphomas. 2 Intraocular lymphomas are usually B-cell neoplasms.1 While retinal hemorrhages and cotton-wool spots may occur due to anemia or thrombocytopenia, direct retinal involvement is extremely rare in systemic NHL.3 There are three reports of combined vascular occlusion, secondary to NHL, in the literature.4-6 These occlusions were primarily due to lymphomatous infiltration of the optic nerve. Septic emboli and paraneoplastic hypercoagulability were the additional causes. We also suspected an infiltrative optic neuropathy, with subsequent infiltration/compression of the vascular wall, in our case. Similar to the report of Saatci et al.,4 our case also experienced a vascular occlusion during disease remission. However, there were some distinctive features: In spite of a well-differentiated systemic B-cell lymphoma, our patient suffered a severe combined vascular occlusion, with neovascular glaucoma. We were unable to find such an association in the literature; the above-mentioned combined occlusions occurred due to large/mixed B-cell or T-cell lymphoma. Further, in the previous reports of optic nerve infiltration and combined vascular occlusion in the background of regressing NHL, CNS was involved in the relapse.4,5 A systemic/CNS relapse of lymphoma did not occur in our patient. Optic neuropathy may occur as the sole manifestation of NHL recurrence.7
Figure 1a: Fundus of the right eye shows swollen optic disc, dilated and tortuous veins, pale retina, a cherry-red spot at the fovea and extensive retinal hemorrhages
We observed combined vascular occlusion as the only manifestation of recurrent systemic NHL. Visual prognosis was poor in spite of aggressive treatment with corticosteroids and irradiation. Even a benign systemic NHL can be deceptively destructive to the eyes; it is important to explain the guarded visual prognosis to the patient, even after achieving successful remission of the tumor.
Figure 1b: Fluorescein angiogram fails to show any dye in the retinal vessels, 10 minutes after injection
Figure 2: Post-contrast computed tomogram (axial view) shows tramline enhancement of the right optic-nerve-sheath complex
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References 1.
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Combined occlusion of central retinal artery and vein occlusion in a child with systemic non-Hodgkin’s lymphoma. Int Ophthalmol 1999;22:125-7.
Harbour JW, Char DH. Intraocular lymphoid tumors. In: Guyer DR, Yannuzzi LA, Chang S, Shields JA, Green WR, editors. Retina-Vitreous-Macula. W.B. Saunders Co: Philadelphia; 1994. p. 1204-16.
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Coffey J, Hodgson DC, Gospodarowicz MK. Therapy of nonHodgkin’s lymphoma. Eur J Nucl Med Mol Imaging 2003;30:S28 36.
Guyer DR, Green WR, Schachat AP, Bastacky S, Miller NR. Bilateral ischemic optic neuropathy and retinal vascular occlusions associated with lymphoma and sepsis. Clinicopathologic correlation. Ophthalmology 1990;97:882-8.
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Dhaliwal RS, Schachat AP. Leukemias and lymphomas. In: Ryan SJ, editor. Retina. Mosby: St Louis; 2001. p. 842-58.
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Primary orbital neuroblastoma in a neonate Hasan Mirzai, MD; Esin F Baser, MD; Nermin Tansug, MD; Nalan Nese, MD; Aydin Isisag, MD Neuroblastoma is an undifferentiated malignant tumor of the primitive neuroblasts. Orbital neuroblastoma is typically a metastatic tumor. We describe a two-days-old girl, who presented with a large tumor in her left orbit. Magnetic resonance imaging revealed that the tumor originated from the retrobulbar area, extending into the upper and lateral orbit. She was operated on the fifth day of life. A histopathologic diagnosis of neuroblastoma was made. Medical evaluation including chest roentgenogram, ultrasonography of the abdomen, whole body computerized tomogram and bone scintigraphy showed no evidence of systemic involvement or metastasis. Neuroblastoma should be considered in the differential diagnosis of neonatal orbital tumors.
Key words: Neonatal tumors, neuroblastoma orbital tumors, primary neuroblastoma. Indian J Ophthalmol 2006;54:206-8
Tumors diagnosed during the first month of life are rare, constituting only 0.5-2% of all childhood neoplasms. 1 Neuroblastoma is among the most common solid tumors of the neonatal tumors.1-6 The most frequent sites of primary neonatal neuroblastoma are thoracal, cervical, abdominal and pelvic locations.1,2,7,8 Only 8% of neuroblastomas first present with an orbital lesion.9 Primary orbital neuroblastoma has been reported before, in adults.10-11 Departments of Neurosurgery (HM), Ophthalmology (EFB), Pediatrics (NT), and Pathology (NN, AI), Celal Bayar University School of Medicine, Manisa, Turkey Correspondence to Hasan Mirzai, 2040 Sok. Pamukkale 4/60 D:67, Mavisehir Izmir, 35540 Turkey. E-mail: Manuscript received: 28.4.05; Revision accepted: 28.11.05
In this article, we describe a neonate with primary orbital neuroblastoma. To the best of our knowledge, primary orbital neuroblastoma in a neonate has not been reported in the English literature before.
Case Report A two-days-old girl was admitted to our university hospital with a giant mass in her left eye. The mass was red and firm in appearance and protruded underneath her left upper eyelid, impeding any view of her left globe [Figure 1]. She had no ecchymosis of the periorbital area. She was otherwise physically normal and showed no signs of any congenital malformations. The patient was the product of an uncomplicated pregnancy and was born at full term by normal delivery. Magnetic resonance imaging showed a large (4×2.5 cm) posterolateral solid orbital tumor. The tumor originated from the retrobulbar area, extended into the upper and lateral orbit and then protruded externally, significantly displacing the globe medially and inferiorly. The left globe was distorted due to the neighboring mass, but was otherwise normal. There was no evidence of a mass in the sinuses, nasal cavities or intracranial cavities [Figure 2]. Because of the serious concern about amblyopia, she was operated on the fifth day of life, by a team of a neurosurgeon and an ophthalmologist. The operation was as follows: Under general anesthesia, the lids were retracted and a lateral canthotomy was performed. With the aid of an operating microscope, first, the top portion protruding between the globe and the upper eyelid was debulked and sent for frozen section. On frozen section examination of the specimen, a malignant small-cell tumor was seen. Dissection was carried down deep into to the retrobulbar area, between the globe and the lateral orbital wall, taking special care to preserve the muscles and the globe. None of these structures were infiltrated by the tumor. The tumor was excised totally. Examination of the left globe after tumor excision revealed no pathologies. Microscopic examination revealed a tumor consisting of round cells with high mitotic activity, in a neurofibrillary and hemorrhagic background. The chromatin of the cells was moderately condensed. Not infrequently, the tumor cells were
