A Systematic Literature Review of Demographics, Clinical Presentation and Surgical Procedures Performed for the Appendico-Umbilical Fistula for the PasQAt 100 Years

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.

Patent vitellointestinal duct treated during infancy: Case series with literature review

“…A recent systematic literature review of the past 50 years (1971-2021), comprised of n=280 infants, treated for the PVID, found/revealed that 72% of cases were diagnosed and treated during the neonatal period. Further review revealed that 90% of the infants were treated for PVID during the first 3 months of their life [6]. The literature review further supports that three-fourths of PVID cases were boys [6,10].…”

“…Further review revealed that 90% of the infants were treated for PVID during the first 3 months of their life [6]. The literature review further supports that three-fourths of PVID cases were boys [6,10].…”

“…The persistent or patent VID (PVID) is the rarest (5%) of all the VID anomalies and is the result of the complete failure of obliteration of the VID [1,4,5]. In the literature, the above-mentioned congenital anomaly is also described by various names, namely, patent omphalomesenteric/ omphaloenteric duct, patent VID, PVID, omphalo-ileal fistula, umbilical-enteric fistula, and umbilicointestinal fistula [5,6]. Approximately three-fourths of the cases are clinically present during the neonatal period [6].…”

“…In the literature, the above-mentioned congenital anomaly is also described by various names, namely, patent omphalomesenteric/ omphaloenteric duct, patent VID, PVID, omphalo-ileal fistula, umbilical-enteric fistula, and umbilicointestinal fistula [5,6]. Approximately three-fourths of the cases are clinically present during the neonatal period [6]. PVID is also detected after infancy and in older children, but it is not frequent and is extremely rare in adults/older people [5,7,8].…”

“…The clinical presentation varies from mucus/flatus/fecal discharge through the umbilicus to the acute intestinal obstruction [9]. The standard surgical procedure executed for PVID is the resection of the patent duct along with resection of an adjacent small segment of the ileum or wedge resection of the ileum, and ileo-ileal anastomosis or ileal repair [6,9].…”