Pediatric temporal bone langerhans cell histiocytosis: report of 2 cases with review of literature

Abstract

Introduction:Langerhans cell histiocytosis is a rare multiorgan disorder, commonly seen in the pediatric age group. Commonest presentation of the disease involving the temporal bone is ear discharge. This entity is often underdiagnosed and the patient is treated with multiple courses of antibiotics which leads to delay in diagnosis and initiating apropriate treatment. Case report:We present case reports of 2 children who presented to us with this rare disease. The line of management followed is discussed briefly. Our aim is to increase awareness of this disease amongst otolaryngologists and pediatricians so that they can identify such cases in a timely manner. Conclusion:Langerhans cell histiocytosis can affect any age group but is commonly seen in children. It can involve any organ system but the temporal bone is involved in most cases. The differential diagnosis of this disease should be kept in mind for any patient presenting with chronic ear discharge that is not responding to the appropriate line of management.

Recurrent Otorrhea in A Child: Early Sign of Temporal Bone Langerhans Cell Histiocytosis

“…The characteristic feature in CT scan includes diffusely destructive lytic bony lesions at the ear canal, mastoid process, posterior and lateral semicircular canal, but rarely involve ossicular chain, facial canal, and petrous apex. 3,[5][6][7]10 A typical "punched-out lesion" at the ear canal and squamous part of the temporal bone is characteristic as per this case. 5 Zheng et al hypothesized that the ossicular chain is resistant to granulation tissue of LCH and ossicles destruction likely contributed by existing chronic otitis media.…”

“…LCH is more commonly seen in boys than females in a ratio of 2:1. 6,7 There is no specific clinical manifestation that is pathognomonic for temporal bone LCH, it depends on the site and extension of the disease. Our case presented with recurrent refractory otorrhea and aural polyp, which correlate with findings of Fernandez et al and Skoulakis et al However, recent literature reported most common manifestations are temporal bone lesion or mastoid swelling (74.1%), periauricular swelling (44.4%), otorrhea (33.3%), otalgia (22.2%) and conductive hearing loss.…”

“…Our case presented with recurrent refractory otorrhea and aural polyp, which correlate with findings of Fernandez et al and Skoulakis et al However, recent literature reported most common manifestations are temporal bone lesion or mastoid swelling (74.1%), periauricular swelling (44.4%), otorrhea (33.3%), otalgia (22.2%) and conductive hearing loss. 2,[5][6][7][8] Other symptoms such as ear canal polyp, sensorineural hearing loss, vertigo, and facial nerve palsy are rare. 7,9 Saliba et al postulated that the bony labyrinth is rarely susceptible to erosion in LCH and Postovsky et al stated that inert growth of LCH tends to not infiltrate neural tissue.…”

“…The treatment options for LCH include surgery, chemotherapy, radiotherapy, local steroids injection, or a combination of them, depending on the extension of LCH. 6,7 Histiocyte Society established that combination treatment with vinblastine and prednisolone are effective with minimal side effects; and are the standard therapy for multisystem LCH. 2,3 This is supported by recent literature where chemotherapy is preferred especially for multisystem LCH.…”

“…3,4 LCH of the head and neck accounts to 50-80% of all cases, with temporal bone involvement around 15-60%. 5,6 Temporal bone LCH has a similar presentation to other common otology diseases such as mastoiditis, recurrent otitis media, or otitis externa. The presence of proliferating Langerhans dendritic cells, Birbeck granules, and positive immunohistochemical staining for CD1a and S100 are pivotal to diagnose LCH.…”