Abstract
The persistent vitellointestinal duct (PVID) is the rarest of all the vitelline duct anomalies. The enteric end of the vitelline duct mostly connects to the terminal ileum. The appendico-umbilical fistula (appendiceal-umbilical fistula) in which the proximal end of the vitelline duct connects to the appendix is a rare clinical entity. This systematic literature review was carried out to review the demographics, clinical characteristics and operative procedures executed for the appendico-umbilical fistula. Literature/cases with incomplete desired details for the above-mentioned anomaly were excluded. The guidelines of the preferred reporting items for systematic reviews and meta-analyses are followed for presenting this manuscript. An online search was made for the retrieval of the published manuscripts. The search was performed for the previous 100 years from 1921 to December 2021. A total of n = 21 (n = 12 female and n = 9 males) cases of the appendico-umbilical fistula were included in the systematic review. All the cases, except one, were infants and n = 10 of the cases were neonates/newborn babies. Their ages ranged from 1 day to 5 years. Three-fourths (n = 16) of the children presented with the chief complaint of discharge of mucus/stool through the umbilicus. An appendicectomy with resection of the PVID/fistulous duct/prolapsed mucosa was executed as the standard operative procedure for the entire case. A peri-umbilical incision was preferred and frequently employed for surgical therapy. This review revealed only one (4.76%) post-operative death that was unrelated to the surgical procedure. PVID connected to the appendix, presented clinically as appendico-umbilical fistula is a rare entity. The standard surgical procedure performed for the appendico-umbilical fistula was an appendicectomy with resection of the PVID/fistulous duct/prolapsed mucosa.