Background: Aplasia Cutis Congenita (ACC) causes local skin absence, usually on the scalp, with a potential underlying skin layer involvement. It carries severe risks, including hemorrhage and a 20-55% mortality rate. Despite various classification systems, a validated treatment algorithm is lacking. This study validates a novel classification-guided treatment approach for ACC that has been used over the past two decades.

Methods: A retrospective analysis of ACC cases at Soroka University Medical Center (2000-2024) was conducted. Patients were classified based on defect size, tissue involvement, and vascular exposure. Characteristics, treatment, outcomes, and mortality rates before and after implementing our classification-guided approach were investigated.

Results: 77 ACC cases were evaluated over 24 years. The scalp was affected in 96.1% of cases, with defect size ranging between 0.25 and 150 cm2. Surgical intervention was performed in 18.2% of cases. Limb anomalies were present in 20.8% of cases, with prevalence increasing with an increase of defect severity (P = 0.001). Adam-Oliver syndrome was diagnosed in 10.4% of patients. The mortality rate decreased from 37.5% (3/8) pre-2008 to 0% (0/22) post-2008 in the high-risk group (P = 0.017). All deaths occurred in Type III patients due to massive hemorrhage following delayed surgical intervention. Integra® dermal regeneration template was used successfully as an effective single-stage treatment for some extensive defects.

Conclusions: Our classification-guided treatment approach has improved ACC outcomes in high-risk cases. Immediate surgical intervention for severe cases has significantly reduced mortality.