PDF Otolaryngology Case Reports

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Otolaryngology Case Reports 24 (2022) 100440

Available online 10 May 2022

Bilateral necrotizing otitis externa in a patient with hemophagocytic lymphohistiocytosis

Ratna Dwi Restuti

^a^,^*

, Ayu Astria Sriyana

^a

, Ludi Dhyani Rahmartani

^b

, Harim Priyono

^a

, Rangga Rayendra Saleh

^a

, Reyhan Eddy Yunus

^c

, Lisnawati

^d

aEar Nose Throat Head and Neck Surgery Department Dr. Cipto Mangunkusumo General Hospital-Faculty of Medicine University of Indonesia, Indonesia

bPediatric Department Dr. Cipto Mangunkusumo General Hospital-Faculty of Medicine University of Indonesia, Indonesia

cRadiology Department Dr. Cipto Mangunkusumo General Hospital-Faculty of Medicine University of Indonesia, Indonesia

dPathology Anatomy Department Dr. Cipto Mangunkusumo General Hospital-Faculty of Medicine University of Indonesia, Indonesia

A R T I C L E I N F O Keywords:

Necrotizing otitis externa Primary immunodeficiency Hemophagocytic lymphohistiocytosis Children

A B S T R A C T

Necrotizing otitis externa (NOE), an infection involving the temporal bone and adjacent structures, poses a high risk of life-threatening complications. The disease occurs mostly in immunocompromised patients. In this paper, we reported a case of a 5-year-old patient presenting with bilateral NOE and hemophagocytic lymphohistiocytosis (HLH).

1. Introduction

Necrotizing otitis externa (NOE), largely caused by Pseudomonas aeruginosa infection, is a progressive and invasive infection of the external auditory canal and the deep periauricular tissues potentially leading to osteomyelitis and other life-threatening complications [1].

This condition often accompanies patients with comorbidities such as diabetes mellitus and immune dysregulation [2], including hemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening disorder associated with an uncontrolled hyperinflammatory response. HLH, which predominantly occurs in children, may occur either as a primary genetic disorder or as a secondary case triggered by infection, malignant diseases, or autoimmune disorders [3]. In this paper, we reported a case of HLH in a pediatric patient presenting with bilateral NOE.

2. Case report

A 5-year-old girl was consulted by her pediatrician with bilateral ear discharge and left-sided drooping of her face since four months ago. On physical examination, both ears showed purulent discharge with necrotic tissues overlying the exposed bone. The tympanic membrane was necrotic, and the malleus was exposed (Fig. 1). In addition, facial nerve examination revealed House-Brackmann grade V paralysis of the left side of the face. Blood tests showed pancytopenia, hypocellular bone

marrow, and marked hemophagocytosis. High-resolution computed to- mography (HRCT) scan of the mastoids showed opacification of both mastoids with the destruction of the air cells, the tegmen tympani, and the sigmoid plate (Fig. 2). The patient was then diagnosed with bilateral NOE, left-facial paralysis, and HLH. The diagnosis of HLH was based on the HLH-2004 criteria which include prolonged fever, pancytopenia, hepatosplenomegaly, hypertriglyceridemia, high ferritin serum (14,363 ng/mL), and bone marrow hemophagocytosis.

The patient received intravenous ceftazidime and metronidazole for four weeks and underwent canal wall mastoidectomy and facial nerve decompression. During the procedure, granulation and necrotic tissues, as well as bone sequestrum were found in both mastoids, while the left sigmoid sinus was covered with granulation tissues. The tympanic segment of the left facial nerve was edematous, pale, and was also covered with granulation tissues. The necrotic tissues were sent for bacterial culture and histopathological examination, which revealed necrotic tissues and necrotic bone trabecula with neutrophil and chronic inflammatory cells (Fig. 3). Pseudomonas aeruginosa was found from bacterial culture and was sensitive to ceftazidime. The patient respon- ded well to the treatments, and the inflammation and infection subsided.

The patient was then discharged on oral antibiotics. Six weeks after- ward, the patient had achieved dry ears and had continued with her HLH chemotherapy protocol. Physical examination at three months follow-up revealed dry ears without any signs of discharge or necrotic tissue.

* Corresponding author. ENT Head and Neck Surgery Department Dr. Cipto Mangunkusumo General Hospital - Faculty of Medicine University of Indonesia, Jakarta, Indonesia.

E-mail address: [email protected] (R.D. Restuti).

Contents lists available at ScienceDirect

Otolaryngology Case Reports

journal homepage: www.elsevier.com/locate/xocr

https://doi.org/10.1016/j.xocr.2022.100440

Received 21 March 2022; Received in revised form 14 April 2022; Accepted 4 May 2022

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Otolaryngology Case Reports 24 (2022) 100440

2 3. Discussion

The prompt diagnosis and treatment of NOE, which includes surgical debridement and the use of antimicrobial agents, are essential to prevent life-threatening complications. In this patient, the diagnosis of NOE was made clinically based on the presence of otalgia, otorrhea, necrotic tissues of the ears, non-responsiveness to antibiotics, and facial nerve paralysis. The presence of facial nerve paralysis indicates an advanced stage of the disease and has been linked to poorer outcomes [4]. These clinical manifestations, coupled with an HRCT scan of the mastoids to evaluate the extension of the disease, enabled us to decide which surgical approach was best for the patient [5]. In addition, the histological findings which revealed necrotic tissues and bone sequestrum with inflammatory infiltrates further supported our diagnosis. Hence, we decided to perform canal wall mastoidectomy and facial nerve decompression to remove the source of infection and the necrotic tissues [5].

In our patient, HLH was identified as a risk factor for the development and progression of NOE. HLH is a spectrum of hyperinflammatory disorders characterized by the excessive activation of natural killer cells, cytotoxic T cells, and macrophages. It is known that HLH may occur primarily due to genetic mutations, or secondarily due to infections caused by viral (e.g., cytomegalovirus, Epstein-Barr virus, parvovirus, varicella-zoster virus, measles virus, herpes simplex virus, human herpesvirus 8, H1N1 influenza virus, and Human Immunodeficiency Virus), bacterial (P. aeruginosa or Streptococcus pneumoniae), fungal, or parasitic etiologies [3]. The excessive immune activation in HLH results in fever, hepatosplenomegaly, and pancytopenia, which was also seen in our patient. These results in immune dysfunction which predisposes the patients to infection, including NOE. Therefore, the prompt identifica- tion and treatment of HLH, alongside NOE, are imperative to improve the overall prognosis of the patients.

4. Conclusion

NOE should be promptly diagnosed and treated due to its potentially life-threatening complications. Furthermore, other comorbidities which may contribute to the development and the progression of NOE, including HLH, should also be identified. Surgery is currently the mainstay treatment of NOE in patients with an advanced disease who are not responsive to medical therapy.

Funding

No sources of funding to be declared.

Ethics and consent statement

Individual patient informed consent was obtained for this case report.

Credit author statement

Ratna Dwi Restuti: Conceptualization, Supervision, Writing-original draft. Ayu Astria Sriyana: Resources, Writing-review and editing,

Fig. 1. Right (R) and left (L) ear examination before the surgery revealed necrotic tissues in the tympanic membrane (green) and the ossicles (purple) of both ear canals (blue arrow). In the left (L) ear, the bony part of the ear canal was exposed (orange arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2. High-resolution computed tomographic scan of the mastoids showed destructions of the air cells (red arrow), the sigmoid plate (blue arrow), and the tegmen tympani (orange arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.) R.D. Restuti et al.

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Otolaryngology Case Reports 24 (2022) 100440

3 Visualization. Ludi Dhyani Rahmartani: Resources, writing-review and editing. Harim Priyono: Writing-review and editing.^:Rangga Rayendra

Saleh^:Writing-review and editing. Reyhan Eddy Yunus: Resources. Lis- nawati: Resources.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

The authors declare that they have no competing interests.

References

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[2] Boyce TG, Balakrishnan K. Otitis externa and necrotizing otitis externa. In:

Principles and practice of pediatric infectious diseases. Elsevier; 2018. p. 223–5.

[3] George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. Hematol Res Rev 2014;5:69–86. https://doi.org/10.2147/JBM.

S46255. Published 2014 Jun 12.

[4] Soudry E, Hamzany Y, Preis M, Joshua B, Hadar T, Nageris BI. Malignant external otitis: analysis of severe cases. Otolaryngol Neck Surg 2011;144(5):758–62.

[5] Peled C, Parra A, El-Saied S, Kraus M, Kaplan DM. Surgery for necrotizing otitis externa—indications and surgical findings. Eur Arch Oto-Rhino-Laryngology. 2020:

1–8. Published online.

Fig. 3. Histopathological examination of the intraoperative frozen sections taken from the mastoid showed necrotic tissues and necrotic bone sequestrum with neutrophil and chronic inflammatory infiltrates.

R.D. Restuti et al.