Atretic cephaloceles: a comprehensive analysis of historical cohort

This study aims to analyze the historical cohort of patients with atretic cephaloceles (ACs) comprehensively, since the literature regarding ACs is limited by small case series or case reports and to report a new venous anomaly concomitant with a parietal AC in our exemplary case. After a PubMed/Medline search with atretic cephalocele in April 2016, we retrieved 42 articles, 41 of which were relevant with the topic. Full texts of accessible articles and abstracts of inaccessible articles were read comprehensively. References of retrieved articles were manually searched. Additional 29 articles reporting the cases of atretic, rudimentary, occult, or sequestered cephaloceles were accessed, since these terms have been used interchangeably in the literature. Median age and mean age of the patients were 1 and 6.69 years, respectively. Female/male ratio was 1.03:1. Atretic cephaloceles were located in different regions, in decreasing order of frequency: parietal (55 %; n = 105), occipital (37 %; n = 71), parieto-occipital (n = 8), frontal (n = 4), asterion (n = 1), and sincipital (n = 1). Concomitant central nervous system (CNS) anomalies were present in 73.3 % of patients with parietal atretic cephaloceles and in 32.4 % of patients with occipital atretic cephaloceles (p < 0.0001). We realized that all patients with worse outcomes had concomitant CNS anomalies (100 %), whereas only 48 % of those with good clinical outcomes had concomitant anomalies (p = 0.0248). Atretic cephaloceles present as nodular extrusions on the parietal and occipital regions. Concomitant CNS anomalies, which impair the clinical outcomes, are more common in patients with parietal ACs. Observation-only approach is enough in patients with asymptomatic ACs. However, life-long follow-ups are required, because of recurrence after the surgery.