Orbital Pseudotumor as a Rare Extrahepatic Manifestation of Hepatitis C Infection

Abstract

Hepatitis C is frequently accompanied by immune-related extrahepatic manifestations affecting the skin, kidneys, central and peripheral nervous system and exocrine glands. We present the case of a 40-year-old man with left-sided ptosis, exophthalmos and headache. MRI demonstrated left-sided orbital pseudotumor with lacrimal and retro-orbital contrast enhancement extending to the cavernous sinus and the vestibulocochlear nerve. Immunological tests of serum and cerebrospinal fluid identified hepatitis C virus (HCV) as a potential causative agent but did not indicate any additional infectious, malignant or immunological disorder. Hepatological evaluation revealed no signs of advanced liver disease. After initial spontaneous improvement, the patient subsequently developed vestibulocochlear failure with gait disorder, tinnitus and transient left-parietal sensory loss. Lacrimal biopsy demonstrated lymphocytic infiltrate, prompting steroid treatment. After initial improvement, steroids could not be tapered below 40 mg daily for several months due to recurrent symptoms. Twelve months after the initial presentation, the patient’s chronic HCV infection was successfully treated with sofosbuvir, simeprevir and ribavirin and he remains now free of symptoms without steroids. In patients with chronic hepatitis C, lymphocytic infiltrate of the salivary and lacrimal glands is a frequent phenomenon. However, the extent of the lymphocytic infiltrate beyond the lacrimal gland to the tip of the orbit, cavernous sinus and vestibulocochlear nerve as in our patient is highly unusual. For all symptomatic extrahepatic manifestations of hepatitis C infection, treatment of HCV as the underlying immune stimulus is recommended, and it helped to control the symptoms in our patient. In addition, long-term follow-up for recurrent lymphocyte infiltrate and development of lymphoma is warranted.

Clinical characteristics, histopathology, and treatment outcomes in adult and pediatric patients with nonspecific orbital inflammation

“…Misselwitz et al. described dacryoadenitis with symptomatic retro-orbital and vestibulocochlear involvement as an extrahepatic manifestation of hepatitis C 25 . A case of rheumatoid arthritis was reported who developed orbital signs and symptoms due to producing autoantibodies targeting orbital tissues 26 .…”

Orbital mucosa-associated lymphoid tissue (MALT) lymphoma as the initial presentation in patients with hepatitis C virus infection

“…A pathogenic link between HCV and lymphoma, mostly MALT, has been widely suggested, and there have been several studies reporting an increased HCV prevalence in patients with systemic NHL (5,6) . However, few studies have explored the association between HCV and ocular adnexal lymphomas (e.g., conjunctiva, lacrimal gland, and orbital soft tissue) (3)(4)(5)7,8) .…”

Orbital pseudotumor as a result of chronic sinusitis in an HIV‐positive patient

“…Similarly, Brunelle et al noted a case of orbital inflammatory pseudotumor due to sarcoidosis . Orbital inflammatory pseudotumor has also been noted as an extra‐abdominal manifestation of hepatitis C infection and as a result of rheumatoid arthritis . Interestingly, Li et al noted an ~30% concomitance between orbital pseudotumor and sinusitis in their group of 46 patients with IgG4+ dacryoadenitis subtype orbital inflammatory pseudotumor.…”