Panophthalmitis associated with scleral necrosis in dengue hemorrhagic fever.

Dengue is a mosquito-borne flavivirus disease affecting humans. The Aedes aegypti mosquito spreads it. Ophthalmic manifestations of dengue range from subconjunctival hemorrhage to optic neuropathy. Panophthalmitis in dengue fever is a rare finding. We report a case of a 22-year-old male having dengue fever, who presented with pain, redness, swelling and loss of vision in his right eye. He was diagnosed as panophthalmitis with subretinal hemorrhage and required right eye evisceration.

Dengue fever, a mosquito-borne disease commonly found in the tropics, is the most prevalent form of Flavivirus infection in humans. It is transmitted to humans by the bite of infected female Aedes aegypti mosquito, and is characterized by an acute onset of fever associated with symptoms of malaise, sore throat, rhinitis, cough, headache, muscle ache, retro-orbital pain, joint pain, abdominal discomfort, rash, and bleeding diathesis. It is a self-limiting illness.[1]

Ophthalmic complications in dengue fever are uncommon. The most common ophthalmic symptom is blurred vision, secondary to macular involvement by edema, ischemia or hemorrhage. Other reported ocular findings are subconjunctival hemorrhage, central scotoma, retinal hemorrhage, optic neuropathy, retinal cotton wool spots, retinal vasculitis, exudative retinal detachment and anterior uveitis.[2] Proptosis in patients of dengue fever is very uncommon and has been reported secondary to panophthalmitis or retrobulbar hemorrhage. The precise pathophysiologic mechanism of ocular involvement is not very well understood and an immune mediated process is the most hypothesized possibility.[3]

Case Report

A 22-year-old man presented to our hospital with one day history of pain, watering, redness, swelling and loss of vision in the right eye. He gave history of fever associated with bodyache, malaise and severe joint pains since past 4 days and was diagnosed as having dengue fever based on the symptoms and dengue serology reports, which was positive for dengue non-structural protein 1 (NS 1) antigen. There was no history of skin rash or bleeding from any site or any blood or platelet transfusion. Blood investigations showed haemoglobin level of 16.3 g/dl, total leucocyte count of 32400 cells/mm2, thrombocytopenia (platelet count: 58000/cu mm), along with mildly elevated liver enzymes (SGOT: 74.21 U/L, SGPT: 54.81 IU/Alkaline phosphatase: 157 U/L). Blood and urine cultures showed no growth.

On examination, there was no perception of light in the right eye. The upper and lower eyelids were edematous with periocular ecchymosis and severe proptosis [Fig. 1a]. The conjunctiva showed subconjunctival hemorrhage and chemosis, cornea was hazy and anterior chamber and retinal details could not be visualized. Extraocular movements were limited in all gazes. Visual acuity in the left eye was 6/6 and anterior and posterior segment findings were within normal limits in left eye. Ultrasound B scan of the right eye showed mounds of subretinal hypereflective echoes suggestive of sub retinal haemorrhage, along with diffuse chorioretinal thickening and vitreous echoes [Fig. 1b]. CT scan showed fuzzy ocular coats, a hyperdense intraocular opacity with vertical fluid level suggestive of intraocular hemorrhage or possibly exudates.

Figure 1

Right eye tense proptosis, eyelid edema and ecchymoses, subconjunctival hemorrhage in a patient with dengue fever (a). Ultrasound B scan of the right eye showing mounds of subretinal hemorrhage and hype reflective echoes in vitreous cavity suggestive of exudates and (b). Histopathology of the scleral tissue showing non specific inflammatory infiltration and areas of scleral necrosis (c). Postoperative appearance of the patient after a secondary orbital implantation and customized prosthesis fitting. (d)

A clinical diagnosis of right eye panophthalmitis with intraocular hemorrhage was made. Patient was started on conservative management with intravenous fluids and antibiotics with close monitoring of platelet counts. The platelet count increased to 251000/mm2 after 2 days, with no improvement in ocular findings. Thereafter, right eye evisceration was done, and intra-operatively, vitreous hemorrhage was noted along with purulent discharge in the vitreous cavity. The Tenon capsule and sclera showed necrosis and extensive areas of scleral melt with fragmentation of the sclera. The eviscerated ocular specimen along with scleral biopsy was sent for histopathology and microbiological examination.

The microbiological examination revealed no growth on culture mediums. Histopathological examination of the scleral tissue showed severe nonspecific inflammatory infiltration with replacement of the scleral lamellae with areas of necrosis and inflammatory infiltration [Fig. 1c].

On subsequent follow-up visits, socket inflammation healed. Secondary acrylic spherical orbital implant was placed into the retro scleral space. Intraoperatively, shrinkage of the scleral shell was noted. Scleral shell was opened, 4 radial anterior and posterior sclerotomies were made, optic nerve head disinserted and the implant was placed in the retro scleral space. A customized ocular prosthesis was fitted subsequently for cosmetic rehabilitation [Fig. 1d].

Discussion

A variety of ocular complications have been reported in dengue fever and are mostly attributed to the thrombocytopenia and the associated bleeding diathesis. Most dengue fever related ocular involvement are limited to posterior segment and manifest in the form of retinal vasculitis, macular edema or optic neuropathy. Proptosis and panophthalmitis associated with dengue fever has been rarely reported.

Siva Saranappa et al. has reported a case of proptosis secondary to panophthalmitis in a 6-year-old child diagnosed as dengue fever.[3] It presented as angle closure glaucoma which progressed to exudates in vitreous cavity and imaging showed inflammatory thickening of retinochoroidal and orbital tissues. The authors predicted the panophthalmitis to be part of the inflammatory or immune response to the dengue virus infection. Hussain et al. report 2 cases of unilateral proptosis which were secondary to anterior orbital and retrobulbar hemorrhage respectively.[4] A case of globe rupture in a patient of dengue fever has also been reported by Nagaraj et al. wherein the proptosis was secondary to the retrobulbar haemorrhage.[5] They concluded that vitreous, suprachoroidal and retrobulbar haemorrhage could have occurred spontaneously due to thrombocytopenia and/or due to trauma from vigorous rubbing of the eye.

The pathogenesis of ocular manifestations of dengue is not understood completely. An immune-mediated inflammatory process, an infective etiology due to direct viral invasion or the coagulopthay associated with thrombocytopenia could be the possible causes of the various ocular manifestations.[6789] The possible causes of hemorrhage could be reduced platelet count, i.e., thrombocytopenia with coagulation defects, capillary fragility, consumptive coagulopathy, and platelet dysfunction. Kamal R et al. have reported a culture positive case of panophthalmitis caused by Bacillus Cereus in a patient with serology positive dengue hemorrhagic fever.[10] They postulated that disintegration of the endothelial cells caused by antibodies against NS 1 antigen facilitated the direct entry of the bacteria into the uveal and retinal circulation causing septic focus and secondary endophthalmitis. The onset of symptoms in our patients at the lowest level of thrombocytopenia correlates with the previous reports. However, scleral melt associated with dengue fever has not been reported in previous cases. This is possibly the result of scleral ischemia secondary to the immune mediated vasculitis or due to thrombocytopenia and coagulopathy associated with dengue hemorrhagic fever. Steroids are the mainstay of treatment in patients who have ocular manifestations and have poor vision due to dengue-related ocular complications. Proptosis due to to hemorrahage or endopthalmits in dengue fever patients have a poor prognosis and vision and globe salvage is rarely possible.

In conclusion, a case of culture negative panophthalmitis with scleral necrosis associated with dengue fever is reported by the authors. This ocular manifestation of dengue hemorrhagic fever is rare and carries poor prognosis for eye salvage. With increasing epidemicity and cocirculation of multiple dengue serotypes, an increase in the occurrence of dengue-related ophthalmic morbidity can be expected. An awareness of this and other ocular complications and prompt referral for ophthalmologic assessment is of utmost importance for initiating early treatment to salvage globe and vision.

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.