Translate this page into:
What is behind umbilical appendix in neonates that we need to know?
-
Received: ,
Accepted: ,
How to cite this article: Ajaj OA, Mousa MI. What is behind umbilical appendix in neonates that we need to know? Adesh Univ J Med Sci Res. doi: 10.25259/AUJMSR_96_2025
Abstract
Anomalies of the vermiform appendix involving the umbilicus are exceptionally uncommon. The few cases described in the literature reveal a broad clinical spectrum, highlight diagnostic challenges, and provide important insights into embryological mechanisms. We report a 7-day-old female presented with a flashy tubular structure from the umbilicus, there was a dark-red structure about 3.5 cm in length and having a diameter of about 3 mm. Exploration of the umbilicus with incision extended to the right transverse of the abdomen revealed an external tubular structure communicating with the cecum as the vermiform appendix. Umbilical appendix, though rare, presents important diagnostic and surgical challenges. They may mimic common umbilical conditions. Careful inspection of the umbilicus in neonates and precise use of terminology can further improve diagnosis, prevent iatrogenic injury, and guide appropriate management.
Keywords
Anomalies
Appendix
Umbilical
INTRODUCTION
The most prevalent congenital umbilical anomalies described in the literature are umbilical granuloma, allantois (patent urachal remnant), and patent omphalomesenteric duct. However, herniation of abdominal viscera through the umbilicus is rare and unusual, including appendix, liver, gallbladder, midgut with and without atresias.[1,2] Umbilical appendix is a congenital protrusion of the appendix through the umbilicus. In this article, we report a very rare case of a newborn with an umbilical appendix. We searched the common literature for published case reports concerning umbilical appendices and found a total of 13 cases.[3]
CASE REPORT
A full-term female neonate was presented at 7 days of age with abnormal umbilical protrusion, which appeared 1 day after separation of the umbilical stump. She was fed normally, no vomiting, and passed stool normally. There was no redness or feculent discharge per umbilicus. On examination, it was seen that, projecting a flashy tubular structure from the umbilicus, there was a dark-red structure about 3.5 cm in length and having a diameter of about 3 mm [Figure 1]. After pro-operative preparation, biochemical investigations are not remarkable, abdominal plain X-ray is normal, and ultrasound shows bowel connected to the umbilicus. Exploration of the umbilicus with incision extended to the right transverse of abdomen revealed an external tubular structure communicating with the cecum as vermiform appendix [Figure 2]. The appendix was removed, and the newborn made good post-operative follow-up [Figure 3]. Histopathological examination confirmed appendicular tissue [Figure 4].
DISCUSSION
Anomalies of the vermiform appendix involving the umbilicus are exceptionally uncommon. The few cases described in the literature reveal a broad clinical spectrum, highlight diagnostic challenges, and provide important insights into embryological mechanisms.[3]
Clinical implications and terminology
Although the true incidence of appendicoumbilical anomalies is extremely low—estimated at 0.006% in large series of appendix specimens, the clinical impact is considerable.[4]Misdiagnosis as a granuloma or patent omphalomesentric remnant may delay definitive care, while inadvertent injury during umbilical catheterization or cord clamping can create preventable fistulas.[4,5] As the umbilicus is increasingly used for laparoscopic access, awareness of these anomalies is also relevant in minimally invasive surgery.[6] Recent reports recommend refining terminology: “Umbilical appendix” should describe congenital persistence of the appendix at the umbilicus, whereas “appendicoumbilical fistula” is more appropriate for acquired or iatrogenic tracts.[5,7]
Clinical presentation and diagnostic challenges
Infants most often present with umbilical abnormalities such as persistent granulomas, reddish, moist tissue, or discharge. These findings are easily attributed to more common conditions such as remnants of the omphalomesenteric duct (POMD) or urachal anomalies.[4,5,8] In several reports, repeated treatment with silver nitrate for presumed granuloma failed, and further exploration revealed an appendix extending to the umbilicus.[5,9] Feculent discharge tends to suggest an enteric connection, whereas serous drainage can mimic a benign granuloma. In neonatal intensive care settings, attempts at umbilical catheterization have inadvertently cannulated or even transected an umbilical appendix, with the anomaly recognized only when meconial material was noted at the cord site.[6,7,10] These experiences emphasize the importance of maintaining suspicion when umbilical conditions do not resolve with standard therapy.
Embryological and pathophysiological considerations
Several mechanisms have been proposed to explain these anomalies. During normal development, the midgut herniates into the umbilical cord at 6 weeks and returns to the abdominal cavity by 10–12 weeks, with the appendix forming from the cecal diverticulum.[7,11] Failure of retraction, particularly in the setting of a mobile cecum, may leave the appendix attached at the umbilicus.[7] Persistence of the vitelline duct with abnormal connection to the appendix rather than the ileum has also been reported.[3] Others suggest that elongated vascular connections, such as an appendicular artery extending to the umbilicus, may tether the appendix.[9]In some infants, what appears to be a congenital anomaly may actually represent iatrogenic injury: unrecognized appendiceal tissue clamped during cord ligation can later form a fistula.[5]
Congenital versus acquired lesions
Most neonatal cases reflect congenital persistence of embryological attachments.[5,11] In contrast, acquired appendicoumbilical fistulas are more often encountered in older children and usually follow appendicitis, adhesions, trauma, or prior operations.[9,10] Differentiating the two is important, as acquired lesions may present with recurrent infection or drainage rather than neonatal umbilical abnormalities.
Management and surgical considerations
Surgical exploration with appendectomy has consistently been curative.[5,8] In cases of umbilical cord hernia, dense adhesions between the appendix and sac wall made reduction difficult, requiring careful dissection.[11] In catheter-related cases, the key to safe management was recognition of abnormal discharge at the time of insertion; reinserting injured tissue into the abdomen risked sepsis.[6]For these reasons, neonatologists and surgeons must remain alert to unusual umbilical findings and manage them with early operative evaluation. Abdominal ultrasound is the 1st imaging that may be helpful in the management of umbilical anomalies. Computed tomography scan can also be applicable for diagnosis but it is of high risk of radiation.[12]
CONCLUSION
Umbilical appendix, though rare, presents important diagnostic and surgical challenges. They may mimic common umbilical conditions, fail to respond to conservative measures, or complicate routine neonatal procedures. Awareness of their embryological basis, combined with timely surgical exploration and appendectomy, results in excellent outcomes. Careful inspection of the umbilicus in neonates and precise use of terminology can further improve diagnosis, prevent iatrogenic injury, and guide appropriate management. Abdominal ultrasound was part of the evaluation in a case of umbilical anomalies.
Ethical approval:
Institutional Review Board approval is not required.
Declaration of patient consent:
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Conflict of interest:
There are no conflict of interest.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation:
The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
Financial support and sponsorship: Nil.
References
- Clinical presentation of rare appendiculo-omphalic anomalies. J Pediatr Surg. 1995;30:1702-3.
- [CrossRef] [PubMed] [Google Scholar]
- A case of persistent vitelline duct attached to the vermiform appendix. J Br Surg. 1921;9:304-5.
- [CrossRef] [Google Scholar]
- A study of 50,000 specimens of the human vermiform appendix. Am J Surg. 1974;127:704-7.
- [CrossRef] [PubMed] [Google Scholar]
- Herniation of the appendix through the umbilical ring following umbilical artery catheterization. J Pediatr Surg. 1980;15:672-3.
- [CrossRef] [PubMed] [Google Scholar]
- A neonate with an intact congenital umbilical appendix: An alternative theory on the etiology of the appendico-umbilical fistula. Pediatr Surg Int. 2006;22:689-93.
- [CrossRef] [PubMed] [Google Scholar]
- Vitelline duct anomalies. Experience with 217 childhood cases. Arch Surg. 1987;122:542-7.
- [CrossRef] [PubMed] [Google Scholar]
- Surgical complications of congenital anomalies of the umbilical region. Surg Gynecol Obstet. 1950;91:71-80.
- [Google Scholar]
- Umbilical cord hernia containing vermiform appendix adherent to the sac. J Korean Surg Soc. 2011;80:161-3.
- [CrossRef] [Google Scholar]
- Congenital appendicoumbilical fistula. Pediatr Neonatol. 2019;60:684-5.
- [CrossRef] [PubMed] [Google Scholar]