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Updated: Aug 13, 2026

Removal of an Intravesical Gellhorn Pessary and Staged Repair of a Residual Large Vesico-Vaginal Fistula
Published on: April 14, 2026
A Systematic Literature Review of Demographics, Clinical Presentation and Surgical Procedures Performed for the
1Department of Pediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals, Bhopal, Madhya Pradesh, India.
Insights
Persistent vitellointestinal duct (PVID) anomalies are rare. Appendico-umbilical fistula, a PVID variant, presents with umbilical discharge. Surgical resection of the fistula is the standard treatment, with good outcomes.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Genetics
Background:
- Persistent vitellointestinal duct (PVID) anomalies are rare congenital malformations.
- Appendico-umbilical fistula is an exceptionally rare variant where the vitelline duct connects the appendix to the umbilicus.
Purpose of the Study:
- To systematically review the demographics, clinical characteristics, and surgical management of appendico-umbilical fistula.
- To consolidate existing literature on this rare congenital anomaly.
Main Methods:
- A systematic literature review was conducted for appendico-umbilical fistula cases from 1921 to 2021.
- Data extraction followed PRISMA guidelines, including demographics, clinical presentation, and operative procedures.
- Cases with incomplete data were excluded, resulting in 21 included cases.
Main Results:
- Twenty-one cases (12 female, 9 male) were reviewed, primarily infants (10 neonates).
- The main clinical presentation was umbilical discharge of mucus/stool (16 cases).
- Appendectomy with resection of the PVID/fistulous duct was the standard surgical approach, often via a peri-umbilical incision.
Conclusions:
- Appendico-umbilical fistula is a rare PVID manifestation requiring surgical intervention.
- Standard treatment involves appendectomy and fistula resection, demonstrating a low mortality rate unrelated to surgery.
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.
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