A Systematic Literature Review of Demographics, Clinical Presentation and Surgical Procedures Performed for the

Rajendra Kumar Ghritlaharey1

  • 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.

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