Olaryngologic manifestations in children with chronic neutropenia

Chronic neutropenia of childhood (CNC) is a rare disorder in which the absolute neutrophil count is below 1500/μL over an extended period of time. The objective of this study is to describe the otolaryngolgic manifestations associated with CNC to facilitate diagnosis and treatment of this condition.

We performed a retrospective chart review of patients with the diagnosis of CNC between 1970 and 2005 at a tertiary pediatric hematology center. After Institutional Review Board approval, 43 patients were evaluated. The average age at hematologic diagnosis was 49 months (range: 1 month - 15 years with 35% <1 year, 44% 1-10 years, 21% >10 years). A total of 2049 encounters were analyzed from the hospital charts.

Twenty four subjects (56%) presented with recurrent otitis media (ROM), sinusitis or pharyngo-tonsillitis, while 11% presented with oral mucosal lesions. After diagnosis, otolaryngologic problems persisted, including ROM (81%), viral upper respiratory tract infection (67%), oral ulcers or gingivitis (53%), tonsillitis (39%) and sinusitis (37%) and were more common than other systemic infections. Myringotomy tube placement, endoscopic sinus debridement, adeno-tonsillectomy or tracheostomy were required in 42% of patients. After G-CSF (granulocyte colony-stimulating factor) became available in the early 1990s, the infection rate markedly decreased. Five deaths occurred (12% mortality) including one due to sepsis from otolaryngologic infection.

The majority of children with CNC had otolaryngologic problems at presentation and these continued after diagnosis. While managing common otolaryngologic infections in children, a high index of suspicion for chronic neutropenia is necessary. An otolaryngologist is frequently one of the first physicians to encounter children with this condition. Awareness of CNC and its management will enhance earlier diagnosis and more effective treatment for these children.