Successful orthognathic surgery in a patient with severe congenital neutropenia

Severe congenital neutropenia (SCN) is a rare heterogeneous genetic disorder characterized by marked chronic neutropenia leading to recurrent bacterial infections. Perioperative management is vital for surgical treatment in these patients because of the high risk of postoperative infection. Herein, we report a case of a woman with SCN who underwent orthognathic surgery for mandibular protrusion. The treatment was carried out in collaboration with the related specialist medical department because of the possibility of severe postoperative infection. Although several cases of relatively minor interventions in patients with SCN have been reported in the literature, to the best of our knowledge, this is the first reported case of orthognathic surgery. Multidisciplinary collaborations with related specialists are imperative to prevent postoperative infections in high-risk patients.

Automated summary

Severe congenital neutropenia (SCN) is a rare genetic disorder characterized by chronic neutropenia and recurrent bacterial infections. We report the first case of a patient with SCN undergoing orthognathic surgery, under the collaboration of relevant specialist departments to prevent postoperative infection. Multidisciplinary collaborations are imperative in high-risk patients to prevent postoperative infections.