Median Upper Lip Cleft: Phenotypic Features and Reconstructive Considerations

BACKGROUND: Median (Tessier 0) cleft lip includes phenotypes ranging from preserved midline dysraphia to hypoplastic midline deficiency associated with primary palate agenesis. Despite these differences, management is often based on principles used for conventional cleft lip repair. The longitudinal effects of reconstruction in hypoplastic variants remain poorly defined. METHODS: This retrospective descriptive case series included 13 patients with median upper lip cleft treated at a single craniofacial center. Patients were grouped into dysraphic and hypoplastic phenotypes. Clinical presentation, prior treatment, operative approach, and longitudinal changes in nasal morphology were evaluated using serial photographs over 2 to 5 years (mean: 3.6 y). RESULTS: Four patients demonstrated a dysraphic phenotype with preserved midline tissue and underwent early repair without observed progressive nasal narrowing during follow-up. Nine patients demonstrated a hypoplastic phenotype with varying degrees of midline deficiency. Within this subgroup, all patients referred after prior early direct closure performed elsewhere (4/4) developed progressive nasal narrowing during follow-up. Five hypoplastic patients underwent delayed reconstruction; 3 maintained stable nasal morphology, and 2 demonstrated progressive narrowing during follow-up. CONCLUSIONS: Hypoplastic median cleft demonstrated morphologic features consistent with midline developmental deficiency rather than simple tissue separation. Progressive nasal narrowing was observed during follow-up in all 4 hypoplastic patients referred after prior early direct closure performed elsewhere; 2 of 5 hypoplastic patients undergoing delayed reconstruction also demonstrated progressive narrowing. Because of the small sample size, referral bias, heterogeneous treatment history, incomplete operative data, and the lack of standardized morphometric and airway assessment, these observations are descriptive and do not establish treatment efficacy or causal associations.