Unusual masquerading infraorbital mass – First case report of human ocular Dirofilariasis found in an Ukrainian patient in Switzerland

doi:10.21203/rs.3.rs-2884085/v1

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Unusual masquerading infraorbital mass – First case report of human ocular Dirofilariasis found in an Ukrainian patient in Switzerland

https://doi.org/10.21203/rs.3.rs-2884085/v1

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A 78-year-old Ukrainian woman immigrated to Switzerland presented with a rapid growing subcutaneous infraorbital mass. Surgical excision of the mass revealed a well-circumscribed, encapsulated tumor, adherent to the skin. The excision showed a soft tissue inflammation with parts of helminthozoonosis diagnosed as Dirofilaria repens.

The number of cases of human Dirofilariasis reported in the last 50 years has gradually increased. Dirofilaria repens is now endemic in many countries and is currently considered to be one of the fast spreading zoonoses in Central, Eastern and Northern Europe. The first empirical evidence of Swiss spreading of Dirofilaria infections was in a dog from southern Switzerland in 1998. Ours is the first case of human orbital Dirofilariasis reported in Switzerland. Our purpose is to inform the ophthalmologist to consider orbital dirofilariasis in the differential diagnosis of inflammatory masses of the orbit and to warn about the spread of this infection in Switzerland.

infraorbital cyst

subcutaneous dirofilariasis

dirofilaria repens

orbital tumor

A wide variety of processes can produce space-occupying lesions in and around the orbit. These include benign neoplasms, malignant neoplasms, vascular lesions, inflammatory disease, congenital lesions, and infection, among other causes. Imaging can be used to precisely localize a lesion, to help establish a diagnosis or generate a differential diagnosis that guides management, to follow a known lesion for progression, or some combination of these¹.

In this report we describe a case of a rapid growing subcutaneous infraorbital mass - masquerading as orbital tumor - secondary to a Dirofilariasis infection.

This case report was prepared in accordance with the principles of the Declaration of Helsinki. As this article pertains only to a case report, specific ethics approval is not mandated. Written informed consent was taken from the patient to publish the photographs before inclusion in the article.

A 78-year-old Ukrainian woman, who had moved to Switzerland about 6 months ago complained of a three weeks history of sudden relatively painless swelling in the lower right orbit (Fig. 1). At the time of presentation in our clinic she had already been treated with corticosteroids and antibiotics, with no improvement. Since the patient had been recently diagnosed with breast cancer, she was referred to us with the suspect of orbital tumor or metastasis. She had no dogs of her own but lived in a family with dogs in Switzerland. She reported no recent foreign travel and could not recall any mosquito bite. Vision was 20/20 in both the eyes and intraocular pressure and fundus were normal. There was no evidence of conjunctival infection and hemorrhage. A round palpable hard and not very mobile subcutaneous mass was in the area of the lower lid/ anterior orbit. CT scan showed a round well-defined mass in the lower left orbit (Fig. 2). An excision in local anesthesia was performed. The histological section showed a florid nodular soft tissue inflammation measuring 1.4x1x1 cm with abundant neutrophils and eosinophils. Centrally, there were parts of a helminthozoonosis. The pathogens had an average cross-section of 450 µm with an approximately 7 µm thick cuticle with typical longitudinal grooves (Fig. 3A, B). Within the worm, parts of the intestine and a paired uterus were visible. Based on this typical morphology, it was confirmed that the parasite was a female form of Dirofilaria repens. The postoperative course went well and she had no complaints after the surgical excision of the mass (Fig. 4).

Dirofilaria repens is a nematode affecting domestic and wild canids, transmitted by several species of mosquitoes. It usually causes a non-pathogenic subcutaneous infection in dogs and is the principal agent of human dirofilariosis in Europe. In the last decades, Dirofilaria repens has increased in prevalence in areas where it has already been reported and its distribution range has expanded into new areas of Europe, representing a paradigmatic example of an emergent pathogen².

In the ophthalmic literature, the number of cases of human dirofilariasis reported in the last 50 years has gradually increased. Global warming in particular, has created conditions favouring the development of infective larvae in mosquitoes, and facilitated the recent spread of Dirofilaria spp. to Central Europe. Dirofilaria repens is now endemic in many countries in the region (Poland, Ukraine, Germany, Austria, Hungary, Netherlands), and is currently considered to be one of the fast spreading zoonoses in Central, Eastern and Northern Europe³^,⁴.

The first empirical evidence of Swiss spreading of Dirofilaria infections was in a dog from southern Switzerland in 1998. A few years later, another two positive dogs were found in Canton Ticino in 2001⁵׳⁶.

Dirofilaria is a common parasite of dogs, who constitute the main source of infection. Humans are accidental hosts and many infected subjects are asymptomatic. Transmission occurs through the bite of zooanthropophilic types of Aedes, Culex, or Anopheles mosquitoes carrying infective larvae acquired from the microfilariae-rich blood of animal hosts parasitized with either deep-seated or subcutaneous worms of the Dirofilaria species. In humans, the nematode causes a subcutaneous or superficially located inflammatory reaction that traps it within a nodule, where it may survive for many years⁷. Such lesions are always associated with moderate to severe inflammation. However, it may also present as a noninflammatory lid tumor.

Dirofilaria is well known to affect the eye and the adnexa⁸. The infection may be periocular, subconjunctival, or intraocular. The first case of ocular dirofilariasis was reported in 1885 by Addario⁹, an Italian ophthalmologist. He found a Dirofilaria worm in the conjunctiva of a Sicilian woman and called it Dirofilaria conjunctiva. The largest series of cases–six with periocular involvement by Dirofilaria was described by Font in 1980¹⁰. More recently reports included one involving the lateral rectus muscle of a 20-year-old man¹¹, conjunctival tissue of a 27-year-old female¹² and a 35-year-old male¹³ and a superficial orbital dirofilariasis in a 24-year-old female¹⁴. It was recently reported a case of ocular dirofilariosis in a 76- year-old patient in the course of cataract surgery in Bulgaria caused by gravid female nematode¹⁵ and a case of a 12-year-old patient with a rapid growing deep orbital mass and imaging findings suggestive of Rhabdomyosarcoma that was found to be Dirofilariasis after mass resection¹⁶.

In the literature there are up-to-date 38 reported cases of ocular/orbital/eyelid dirofilariosis. The majority of articles were case reports of up to 3 patients, with the exception of Dzamic et al.¹⁷ who presented 19 cases of human dirofilariasis in Serbia, both ocular and subcutaneous and Kalogeropoulos et al.¹⁸ who presented 8 cases of ocular dirofilariasis. In published reports of ocular dirofilariasis, most of the cases were located under the conjunctiva (> 60% of all cases) followed by orbital/eyelid dirofilariasis (approximately 25%).

In general, the diagnosis of human dirofilariasis is based on histologic examination. Useful characteristics for differentiating between the different Dirofilaria species, are the size and the features of the body wall, i.e., thickness of the cuticle and its structure, ridges, lateral chords, and number and type of muscle cells¹⁹.

Therapy with systemic antibiotics has proved useless and surgical removal of the worm is the only known treatment. Usually, the clinical symptoms disappear after the parasite is removed and no adjunct therapy is necessary²⁰.

This is the first report of human infraorbital Dirofilariasis masquerading as orbital tumor reported in Switzerland.

Climate change affecting mosquito vectors and the facilitation of pet travel seem to have contributed to this expansion; however, the major factor is likely the rate of undiagnosed dogs perpetuating the life-cycle of Dirofilaria repens. Many infected dogs remain undetected due to the subclinical nature of the disease, the lack of rapid and reliable diagnostic tools and the poor knowledge and still low awareness of Dirofilaria repens in non-endemic areas.

Because the clinical onset of orbital Dirofilariasis is usually marked by common symptoms of orbital inflammation such as redness and swelling, ophthalmologists are advised to consider this parasite in the differential diagnosis of inflammation of the orbital tissues. The differential diagnosis in these cases incudes dermoid cysts, sarcoidosis, idiopathic pseudotumor, benign tumor and malignant tumor including metastatic disease.

The purpose of this report is to inform Swiss ophthalmologist to consider orbital Dirofilariasis in the differential diagnosis of inflammatory masses of the orbit and to warn about the spread of this infection in Switzerland.

In our view, it is critical to register all new cases in order to assess the epidemiological status and raise awareness of this rare infection.

Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials

discussed in this article.

No conflicting relationship exists for any author.

No competing interests among authors.

Précis:

Dirofilariosis presents as a rapid growing infraorbital mass in a 78-year-old Ukrainian woman immigrated to Switzerland

Statement of Ethics

Ethical approval is not required for this study in accordance with local or national guidelines.

Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

Declaration of conflicting interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

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You are reading this latest preprint version

Unusual masquerading infraorbital mass – First case report of human ocular Dirofilariasis found in an Ukrainian patient in Switzerland

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