(4) Reconstructive Technique for Congenital Hemiarhinia in a Low-Income Country: A Case Report and Review of the Literature

Abstract: Hemiarhinia, unilateral absence of an external nose and internal nasal cavity, is a rare congenital malformation with under 100 known cases, presenting functional and reconstructive challenges. Likely due to multifactorial etiology during early development, it commonly occurs sporadically with inconsistent genetic correlations. Most incidences have associated anomalies, such as proboscis lateralis, coloboma, and facial clefts. Reconstruction is not standardized due to its rarity, but often utilizes cartilage grafts for the nasal framework and pedicled or free flaps for skin coverage. Optimal approach and timing of reconstruction is debated, with degree of deformity and extranasal abnormalities important considerations. We highlight a reconstructive technique for hemiarhinia performed in a low-income country, as well as literature review pertaining to this rare disease process. A 7-year-old otherwise healthy male was found to have right hemiarhinia, medial canthal dystopia, colobomas, and intermittent epiphora on examination. Imaging showed right hypertrophied medial orbital wall and alveolar cleft. An inferiorly pedicled skin flap of the right cheek and hemi-nose including the nasal tip was created to form new nasal lining, and colobomas of the right eyelids were excised. Osteotomies of hypertrophied medial orbital wall were then performed, with harvested bone placed in the alveolar cleft. Bony tunnels were created through abnormal nasal bones to the right medial orbit for medial canthopexy. A burr was used to create a new right nasal passage. Adherent nasal septum to the bony sidewall was resected to expose underlying nasal mucosa, which was elevated on a superior pedicle, along with turbinectomy, to create a 1 cm opening in the nasal cavity. The elevated skin flap was tubed distally and inset to create a neo-nasal passage. The nasal mucosal flap was interpolated with the dorsal and nasal sidewall components of the tunneled flap to create the deeper portions of the nasal passage into the greater nasal cavity. Costal cartilage was used to create a dorsal graft and columellar strut in a tongue-in-groove fashion. Auricular chondral bowl cartilage was used to create the right lower lateral and alar supporting cartilage. A paramedian forehead flap was inset on the neo-nose with pedicle division at 11 weeks postoperatively. The patient healed well with no complications at 2 months follow-up. This case presents modifications of hemiarhinia reconstruction techniques possible in a low-income country, with improvements in function and cosmesis. Reconstruction was able to be limited to 2 stage by creating neo-nasal lining with existing cheek and nasal skin without need for tubing or debulking the forehead flap. While this patient underwent reconstruction at age 7 due to resource limitations, there remains no standardized timeline or technique for repair in the literature, with individualization of management critical for successful outcomes.References1. Abulezz T. Congenital Heminasal Aplasia: Clinical Picture, Radiological Findings, and Follow-up After Early Surgical Intervention. J Craniofac Surg. 2019;30(3):e199-e202. doi:10.1097/SCS.0000000000005091. 2. Meel R, Samdani A, Agrawal S, Das D. Opening a dacryocystorhinostomy into the opposite nasal cavity in a case of hemiarhinia. BMJ Case Rep. 2022;15(1):e245424. doi:10.1136/bcr-2021-245424. 3. Ramamoorthy D, Damke S. Hemiarhinia: a rare presentation of the unilateral absence of nasal structure. Pan Afr Med J. 2022;42:166. doi:10.11604/pamj.2022.42.166.35225

Figure 1: A). Preoperative 3D reconstruction of the skull and facial bones demonstrating hemiarhinia B) Preoperative photograph.

Figure 2: A) Skin flap to form the neo-nasal lining. B) Creation of dorsal graft and columellar strut. C) Forehead flap inset. D) Postoperative result after division of forehead flap pedicle.