Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features

Kimura disease is a rare chronic inflammatory disorder mostly reported in males in second to third decades from south-east Asia. Head and neck is the most commonly involved region. The usual presentation is painless facial swelling with salivary gland involvement and regional lymphadenopathy. The diagnosis can be suspected on imaging but needs to be proven on histopathology. We describe three histopathology proven cases of Kimura disease and emphasize the specific clinic-radiological findings which can help in early identification of the condition and its differentiation from other diseases with similar presentation. Gray scale Ultrasound, contrast-enhanced ultrasound, and contrast-enhanced Magnetic Resonance Imaging (CE-MRI) were done in all patients and Computed Tomography (CT) was done in one. The salient imaging features were ill-defined solid mass in the subcutaneous location of the facial region which was heteroechoic on Gray scale Ultrasound, heterogeneously hyperintense on T2W MRI and without much diffusion restriction. There was involvement of salivary glands with ipsilateral cervical adenopathy. The lesion showed avid contrast enhancement on contrast-enhanced ultrasound and CE-MRI. We also present a systematic literature review of clinico-radiological features in Kimura disease in the head and neck. The articles with histopathologically proven cases and satisfactory description of imaging findings were included and we have compared the findings in our cases with published literature. In addition, we have included examples of radiological mimics of this disease. Certain imaging findings are highly suggestive and can aid in early diagnosis of Kimura disease which helps in early initiation of treatment with better patient prognosis.

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Introduction/Background

Kimura disease is a chronic inflammatory disease of unknown etiology, seen predominantly in males in the second to third decades of life, mainly in Southeast Asian countries. Initially described in 1948 by Kimura et al,1 it presents with soft tissue swelling most commonly involving the head and neck region.2 Imaging can raise the suspicion of this entity but definitive diagnosis is made by histopathological analysis. We present 3 cases of Kimura disease, 2 in adolescent boys and one in a young

Case 1

A 13 years old boy presented with a complaint of painless, noncolored swelling on the right side of his face, for the past 6 years (Fig 1a). The lesion was gradually increasing in size with no history of fever, trauma, or bleeding. On physical examination, a firm noncompressible swelling measuring 18 × 14 cm in size was present, involving almost the entire right side of the face. No facial deviation or drooling of saliva was seen. There was no change in size of the swelling with change in

Systematic Review and Discussion

We reviewed all the research articles as well as case series / reports on imaging in Kimura disease as per methodology delineated in Table 1, adhering to PRISMA guidelines.3 Literature search was performed from June to July 2020 and studies and reports from 1989 to 2020 were reviewed and compiled. Review of literature was performed on PubMed (MEDLINE) and EMBASE and the bibliographies of each study were also reviewed manually. Table 2 delineates the references eventually included for systematic

Conclusion

Kimura disease is a rare chronic inflammatory disorder presenting as facial soft tissue swelling, mostly encountered in South-East Asia. Although the diagnosis is established on the basis of histopathology, imaging investigations and lab findings can help in early identification and differentiation from other disorders affecting head and neck in the typical age group. Features like ill-defined diffuse involvement of subcutaneous tissues and salivary glands, presence of lymphadenopathy with

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