Department of ENT, JIPMER, Pondicherry, India.

Allergic fungal sinusitis is a hyperintense response to fungi in the paranasal sinuses [1]. Paediatric allergic fungal sinusitis, even in extensive cases, can present with few symptoms. Other associated systemic diseases such as ciliary dyskinesias and cystic fibrosis must be ruled out in these cases. Special care is needed for children to treat the disease early, limit disfiguring sequelae, and avoid intracranial and intra-orbital complications. Timely treatment, with an emphasis on follow-up, is critical in cases of allergic fungal sinusitis.

A 13-year-old male adolescent with no known medical co-morbidities presented with a chronic, progressive bilateral nasal obstruction for ten months, along with gross facial deformity and abaxial proptosis for six months. His visual acuity was within normal limits at six feet. Abaxial proptosis, which is the protrusion of the eyeball from the orbit, with increased interpupillary distance (hypertelorism) was noted on examination [Fig.1a,c]. On external examination of the nose, the nasal bridge was flattened and widened, a characteristic finding in patients with chronic rhinosinusitis with nasal polyps. The child had a hypo-nasal voice and was mouth-breathing most of the time due to the extensive blockage in his nasal cavities. Anterior rhinoscopy revealed that pale polypoidal tissue, which appeared as a mass of grape-like clusters, completely filled both nasal cavities. It did not bleed on touch and was insensitive, consistent with the diagnosis of nasal polyps. His visual acuity was 6/12 in both eyes, and extraocular movements were complete and symmetrical. Blood evaluation showed peripheral eosinophilia, with an absolute eosinophil count of 3570 cells/µL, indicative of an allergic reaction.

Allergic fungal rhinosinusitis (AFRS) is a non-invasive fungal sinusitis that is characterized by alterations in the innate immunity, type 2 inflammation with alteration in sino nasal adaptive immune response, and direct involvement of fungal elements in the pathogenesis [2]. Although AFRS is more commonly seen among adults, it can also occur in children, but there is a scarcity of literature on its clinical course in this age group [3]. Studies suggest that the clinical presentation of AFRS in children is subtle, with a slow onset of symptoms, and often involves facial dysmorphism [4,5]. Facial deformities, proptosis, intra-orbital and intracranial extension, and an increased risk of recurrence are more commonly seen in children than in adults [6]. The pliable nature of the pediatric skeleton and the expansile force of allergic fungal sinusitis are believed to contribute to the higher incidence of facial deformities in children.

Allergic fungal rhinosinusitis (AFRS) is a rare condition in children, which can lead to significant facial deformities, proptosis, intra-orbital and intracranial extension, and increased chances of recurrence. AFRS is managed with a combination of medical and surgical treatment, with pre-operative knowledge of the disease extent helping in precise intra-operative management. Tapering courses of peri-operative steroids play a pivotal role in pediatric AFRS. With appropriate management and follow-up, the chances of recurrence can be reduced, and the child can have a complete resolution of symptoms and remodelling of facial bones. Furthermore, educating parents and caregivers on the importance of regular follow-up visits and adherence to medical therapy can help prevent recurrences and improve outcomes for pediatric patients with AFRS.

Contributors: RR: manuscript writing, patient management; LKP: manuscript editing, patient management; SP: critical inputs into the manuscript. RR will act as a study guarantor. All authors approved the final version of this manuscript and are responsible for all aspects of this study.

Funding: None; Competing interests: None stated.