Image-guided lacrimal drainage surgery in congenital arhinia-microphthalmia syndrome

The aim of this study was to illustrate the surgical techniques and utility of stereotactic or image-guided navigation in the management of lacrimal drainage obstruction in congenital arhinia-microphtalmia syndrome and review the relevant literature. Image-guided combined external and endoscopic dacryocystorhinostomy was performed in a female, aged 16 years with congenital partial arhinia and ipsilateral microphthalmus. The lacrimal sac was bypassed to the contra lateral nasal cavity through a septal window. The surgical procedure was performed using the intra-operative optical image-guided Nav 1 Pico^TM ENT navigation system with real-time intra-operative instrument geometry. Different phases of the surgical technique, adjunctive endoscopic procedures, intra-operative anatomical guidance, and utility at crucial phases of surgery were noted. A review of the literature was performed pertinent to arhinia and navigation guided lacrimal surgeries. Lacrimal bypass into the contra lateral nasal cavity even through a malformed septum is possible in partial arhinia syndromes. Detailed preoperative evaluation including 3D imaging studies, navigation guided planning of risk structures with intra-operative distance control and construction of meticulous surgical roadmaps were found to be essential factors in successful outcomes. At six months follow up after surgery, there was a complete and contiguous healed mucosal anastomosis with lacrimal system patent on irrigation and resolution of epiphora. Combined external and endoscopic approach is useful in partial arhinia syndromes. Image guidance is a very useful adjunctive tool that facilitates safe and precise surgery in the management of such complex lacrimal surgeries.