Non-Sacrococcygeal Pilonidal Sinus: A Systematic Review of a Growing Rare Disease

Abstract Introduction Clinical courses and management options for non-sacrococcygeal pilonidal sinus diseases have never been agreed on by the experts. The aim of this review is to collect, analyze, present, and discuss the management of pilonidal sinuses occurring in the body other than the sacrococcygeal regions. Methods PubMed/MEDLINE, Web of Science, Cochrane Library, and EMBASE and Google Scholar were explored for the related keywords. The inclusion criteria included those articles that mentioned at least the presentation and management of non-sacrococcygeal pilonidal sinuses. The collected data were re-analyzed (meta-data) and presented in the form of tables for a few variables and others were presented without analysis (review data). Results In 56 studies, 447 cases of non-sacrococcygeal pilonidal sinuses were found. About 367 (82%) were male. The mean age of presentation was 24.66 ranging from 8 to 70 years. The most prevalent site was the umbilicus (390 cases; 87.2%) followed by the mammary region (17 cases; 3.8%). The most prevalent clinical presentation of umbilical pilonidal sinus was swelling, discharge, and pain (83%). Only discharge was found in 20% and in 1.5% of the cases associated with bleeding. The most common management modality (187, 47.9%) was hair removal with regular dressing. Conclusion Non-sacrococcygeal pilonidal sinus is a rare variant of the disease. Among them, umbilical pilonidal sinus is the most prevalent one. Swelling, discomfort, and discharge are the usual presenting symptoms. Non-sacrococcygeal PNDs are managed by excision and primary suturing apart from umbilical PND which is usually treated by conservative measures.