Kimura disease of naso-orbito-ethmoid region and review of surgical approaches to naso-orbito-ethmoid region

  1. Milind Sagar 1,
  2. Prem Sagar 1,
  3. Rajeev Kumar 1 and
  4. Kavneet Kaur 2
  1. 1 Otolaryngology & Head Neck Surgery, All India Institute of Medical Sciences, New Delhi, Delhi, India
  2. 2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
  1. Correspondence to Dr Prem Sagar; sagardrprem@gmail.com

Publication history

Accepted:26 Jul 2023
First published:04 Aug 2023
Online issue publication:04 Aug 2023

Case reports

Case reports are not necessarily evidence-based in the same way that the other content on BMJ Best Practice is. They should not be relied on to guide clinical practice. Please check the date of publication.

Summary

A man in 30s had complaints of glabellar and upper nasal swelling for 8 years. It was insidious in onset and gradually progressive causing epiphora and restriction of nasal visual field. Fine-needle aspiration cytology and biopsy revealed features which were suggestive of Kimura’s disease (KD). CT scans showed a well-defined subcutaneous swelling in the naso-orbito-ethmoid (NOE) region. KD presents as lymphoglandular swelling; however, NOE region is an uncommon site of occurrence. A thyroid-shaped tumour was excised by H-shaped incision approach to the NOE region.

Background

Kimura disease (KD) is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and/or salivary gland involvement. This condition has a predilection for men of Asian descent and may clinically simulate a neoplasm. KD is sometimes confused with angiolymphoid hyperplasia with eosinophilia, which occurs in the superficial skin of the head and neck region.1–3 Emphatically, the presentation is classic and the site of occurrence, the naso-orbito-ethmoid (NOE) region, is rare. Additionally, we have reviewed the surgical approaches to the NOE region, which will provide a ready reckoner to the practicing surgeons.

Case presentation

A man in his 30s noticed right parotid swelling 10 years ago for which no intervention was done. Then medial canthal swellings appeared 8 years ago following which he was diagnosed as a case of KD on fine-needle aspiration cytology (FNAC) from the swellings. Same year, he underwent a left submandibular gland excision biopsy. Histopathology was suggestive of KD.

Five years ago, he underwent excision of left medial canthal swelling for complaints of epiphora but the medial canthal swellings have progressively increased to the present size which are causing restriction of nasal visual field as well as he has aesthetic concerns due to the swelling. He was started on oral steroids for a week which were stopped in view of systemic side effects.

His recent eosinophil counts (6.04×10−3/μL) and total IgE level (>5000 KUA/L) were raised. The clinical picture as well as blood parameters, in consonance, confirming the diagnosis of KD.

The decision of surgical excision of the tumour located in the NOE region was made after studying the radiology (figure 1). A subcutaneous swelling with a well-defined tissue plane surrounding the swelling substantiated the surgical excision.

Figure 1

Axial cuts of CT of paranasal sinuses showing the tumour involving subcutaneous tissue of NOE region. NOE, naso-orbito-ethmoid.

Various skin incisions have been described to approach NOE region primarily to reduce fractures. They include glabellar approach, bicoronal approach, butterfly, vertical, Gullwing and H-shaped incision.4 Owing to the size and site of the tumour, H-shaped incision was deemed suitable for the excision (figure 2).

Figure 2

H-shaped incision to excise the tumour.

The tumour was exposed and dissected in a subcutaneous plane (figure 3). A thyroid-shaped tumour was removed and sent for histopathology which confirmed the diagnosis (figure 4).

Figure 3

Skin flaps raised to expose the entire tumour.

Figure 4

Tumour specimen.

Outcomes and follow-up

Two weeks after surgical excision, he was started on oral prednisolone 40 mg once a day. The swelling has reduced significantly 2 weeks after starting the treatment (figures 5 and 6). He is satisfied with the cosmetic outcomes. The nasal visual field has improved. However, he is concerned about the side effects of systemic steroids which have not yet appeared. The plan is to continue the steroids for 6–8 weeks in tapering dose.

Figure 5

Surgical site at 2 weeks follow-up.

Figure 6

Surgical site 2 weeks after starting oral steroids.

Discussion

KD is a rare chronic inflammatory disorder affecting salivary glands, lymph nodes and subcutaneous tissue of the head and neck region. It is seen predominantly in young-aged to middle-aged Asian men. The patient presents with indolent painless facial swelling involving salivary glands, lymph nodes and subcutaneous tissue along with raised serum eosinophils.2 3

Above-mentioned patient, therefore, is a classic case of KD because he is a middle-aged man with history of submandibular, parotid and subcutaneous medial cantal swelling with serum eosinophilia (6.04×10−3/μL) and raised serum IgE (>5000 KUA/L).

His clinical features, male gender, serum eosinophilia and raised serum IgE differentiated the KD from angio lymphoid hyperplasia with eosinophilia (ALHE).3 Serum eosinophilia and raised serum IgE are invariably seen in patients of KD.1

FNAC is not specific but can help in diagnosis in pertinent clinical setting. Biopsy is required to confirm the diagnosis and differentiate from various benign and malignant lesions particularly from ALHE.5

Three main histological features of KD includes: cellular (inflammatory infiltrate including increased eosinophils and follicular hyperplasia), fibrocollagenous and vascular (arborizing vascular proliferation of the postcapillary venule). The histology of this patient in low power magnification showed many secondary follicles with germinal centres and expanded interfollicular areas and the interfollicular areas revealed vascular proliferation and numerous eosinophils consistent with the diagnosis of KD (figure 7A,B).1

Figure 7

(A) Low power magnification showed many secondary follicles with germinal centres and expanded interfollicular areas (HE ×40); (B) interfollicular areas revealed vascular proliferation and numerous eosinophils.

Radiology showed well-defined subcutaneous density in NOE region with ill-defined margins around the nasal bones (figure 1).

Even though, he underwent multiple surgeries for excision of left submandibular gland and medial canthal swellings, they recurred; which is in consonance with high recurrence following surgical excision (30.5%).3

However, surgical excision forms the mainstay of treatment with lowest recurrence rate (30.5%) when compared with corticosteroids (45%) and radiotherapy (60%).3 He was keen on getting the tumour removed, for better cosmesis and reluctant to take oral steroids apropos side effects. Therefore, tumour excision was planned.

The NOE region can be approached by various incisions. Balraman et al have explained such approaches to NOE region in detail in their textbook.4 The incisions include glabellar approach, bicoronal approach, butterfly, vertical, Gullwing and H-shaped incisions (figure 8).4 Since the tumour was large measuring roughly 7–8 cm in greatest dimension and involved frontal, nasal and both medial canthi, H-shaped incision was marked and tumour was dissected in toto in subcutaneous plane (figures 2–4).

Figure 8

Approaches to NOE region. NOE, naso-orbito-ethmoid.

Therefore, in cases where KD involves NOE region it can be removed by either of the soft tissue approaches described above which can be tailored according to the site and size of tumour whereas oral steroids can be used as an effective adjuvant modality.

Summarising, the presentation is classic along with the rare site of occurrence, the NOE region. Additionally, we have reviewed the surgical approaches to the NOE region, which will provide a ready reckoner to the practising surgeons.

Learning points

  • Kimura disease is a rare chronic inflammatory disease affecting salivary glands, nodes and subcutaneous tissue of head and neck region.

  • It is more commonly seen in young-aged to middle-aged Asian men.

  • Surgery is the mainstay of treatment for optimum aesthetic and symptomatic results with least rate of recurrence.

  • Naso-orbito-ethmoid lesions can be approached via various incisions tailored according to the site and size of the tumour.

  • Other adjuvant modalities of treatment include steroids and radiotherapy.

Ethics statements

Patient consent for publication

Footnotes

  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: MS, PS and KK. The following authors gave final approval of the manuscript: PS and RK.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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