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| CASE REPORT |
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| Year : 2017 | Volume
: 6
| Issue : 1 | Page : 40-42 |
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Umbilical mass resembling ram's horn: An unusual presentation
Niraj Kumar Dipak, Sidarth Parab, Vaidehi Dande, Sudha Rao
Department of Neonatology, B J Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Acharya Donde Marg, Mumbai, Maharashtra, India
| Date of Web Publication | 8-Feb-2017 |
Correspondence Address:
Dr. Niraj Kumar Dipak
Department of Neonatology, B J Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Acharya Donde Marg, Parel, Mumbai - 400 012, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2249-4847.199755
Vitello-intestinal duct anomalies are secondary to the persistence of embryonic vitelline duct.Remnant of connection of mid-gut with yolk sac results in a number of congenital anomalies. We present a case of patent vitello-intestinal duct in which prolapse of bowel through a patent omphalomesentric fistula occurred and presented like an umbilical mass. Keywords: Enteroumbilical fistula, prolapse of ileum, surgical emergency, vitellointestinal duct
How to cite this article:
Dipak NK, Parab S, Dande V, Rao S. Umbilical mass resembling ram's horn: An unusual presentation. J Clin Neonatol 2017;6:40-2 |
| Introduction | |  |
Vitellointestinal duct (VID) or omphalomesenteric duct anomalies are secondary to the persistence of the embryonic vitelline duct which connects the yolk sac to the gut in the developing embryo and provides nutrition until the placenta is established; the duct constricts and separates from the intestine between the 5th and 9th weeks of gestation.[1],[2] Persistence of embryonic VID leads to various congenital anomalies such as patent vitelline duct covered by skin, Meckel's diverticulum with fibrous cord, enteric cyst with fibrous cord, fibrous cord, enteric fistula, and hemorrhagic umbilical mass.[2],[3],[4] The VID anomalies occur in approximately 2% of newborns, and 6% of these, the duct remains patent.[2] Patent omphalomesenteric duct is an enteroumbilical fistula, manifesting with the passage of meconium or fecal matter through the umbilicus. The condition may begin at birth or occur within 1–2 weeks. The most significant complication of this lesion is prolapse of the small intestine through the umbilicus, with a significant increase in mortality.[4] We report a case of patent VID with prolapse of small intestine through umbilicus. This is a very rare occurrence and only a few cases have been reported.[5],[6],[7] Once this lesion is diagnosed, it should be corrected by surgical excision of the umbilicus and the duct.
| Case Report | | |
An 11-day-old male newborn was brought to the pediatric emergency unit with complaints of fever, sudden onset of profuse bleeding from the umbilicus, and swelling over nape of the neck. On admission, remnants of umbilical cord were removed and local pressure was applied at the bleeding site so as to stop oozing of blood. Baby had cellulitis over nape of the neck, for which incision and drainage was done on the day of admission [Figure 1].
On day 12th, fecal matter was seen coming out of the umbilicus. Following this, a red-colored mass resembling Ram's horn was seen protruding from the umbilicus [Figure 2]. The mass was irreducible and bled on touch. The general condition deteriorated and baby went into hypotensive shock. After fluid resuscitation, laparotomy was performed under general anesthesia. A small, transverse incision of 5 cm was made just above the umbilicus. The outer surface of the emerging loop was free of any adhesion to the anterior abdominal wall. It was seen that a single loop of small intestine was prolapsing through the umbilicus giving rise to Ram's horn-like mass to the exterior.
The herniated loop through umbilicus was reduced into the peritoneal cavity cautiously, leading the diminution and disappearance in size of the outer mass. After complete reduction, a defect was found in the small intestine at the point of adherence with the abdominal wall, suggesting its patency with external environment through the umbilicus.
A patent VID with prolapse of ileum was diagnosed; on reduction, approximately 6 cm of the protruded mass was found to be gangrenous, for which primary resection anastomosis was done [Figure 3]. Appearance of umbilicus after repair is seen in [Figure 4]. Histopathological examination of the excised duct revealed the ileal mucosa with gangrenous changes.
Co-incidentally, pus culture from neck cellulitis site grew methicillin-sensitive Staphylococcus aureus for which antibiotics were given according to sensitivity report. Postoperatively, infant recovered well and discharged subsequently.
| Discussion | | |
The baby in the present case came with hemorrhagic discharge from umbilical site. The differential diagnosis of umbilical drainage includes omphalitis, omphalomesenteric duct remnant, or an umbilical granuloma and some rare conditions such as vascular lesions of umbilical cord (hemangioma, varix, true knot).[7],[8] Medical causes of umbilical bleeding in neonatal period include thrombocytopenia, Vitamin K deficiency, and coagulopathy, especially factor XIII deficiency.[7],[8] The quantity and character of the drainage may indicate the origin of the lesion.[9],[10],[11] Omphalitis is characterized by purulent discharge from the umbilical cord stump and bleeding, resulting from delayed obliteration of the umbilical vessels.[2],[10] Clear or yellowish drainage signifies urachal anomaly, whereas an omphalomesenteric duct remnant manifests as feculent drainage.[2],[10]
VID anomalies are secondary to the persistence of the embryonic vitelline duct. It represents the remnant of connection of midgut with the yolk sac and is noted between 5 and 9 weeks of fetal life and disappears by 9th week, just before the midgut returns to the abdomen.[5] Persistence of some portion of the vitelline duct, of which connection is usually to the ileum and rarely to the appendix or colon, results in a number of congenital anomalies, of which Meckel's diverticulum is the most common.[2] In other cases, part of the vitelline duct within the abdominal wall persists, forming an open omphalomesenteric fistula, an enterocyst, or a fibrous band connecting the small bowel to the umbilicus.[2] These anomalies occur in approximately 2% of the population and may remain silent throughout life or may present incidentally sometimes with an intra-abdominal complication.[5],[10] Intestinal obstruction may be caused by a Meckel's diverticulum attached to the umbilicus by a fibrous cord or by a fibrous cord between the ileum and the umbilicus. Herniation or prolapse of the bowel through a patent omphalomesenteric fistula at the anterior abdominal wall as a discolored, mucosa-covered mass (with a characteristic “Ram's Horn” appearance) like in the present case report is a rare occurrence in the neonatal period.[7],[8],[10]
It is difficult to predict beforehand which one of the patent VID cases will develop this complication. It is difficult similarly to explain the mechanism of ileal intussusceptions into the patent VID, but two reasons have been hypothesized; wide mouth of patent VID and shorter distance between VID and ileocecal valve in infants leading to higher intraluminal pressure.[11],[12]
The diagnosis of a patent vitelline duct anomaly is based on the history of type of discharge from the umbilicus and physical examination.
Newborns with obstructive symptoms should be resuscitated as rapidly as possible to obviate the need for ischemic bowel resection. Those with significant hemorrhage should be transfused.[13] The principle of surgical management is a reduction of the intussuscepted gut along with complete excision of the vitelline duct and restoring the ileal continuity as well as umbilical reconstruction. Three surgical approaches are described, i.e., infraumbilical, supraumbilical, or through the umbilicus.[2],[12],[13] The incision chosen varies with the symptoms and the age of the patient. Newborns with feculent umbilical drainage or prolapse of the omphalomesenteric duct remnant can be explored by a small infraumbilical incision. Postoperative complications are generally the same as that of other operations such as bleeding, infection, intra-abdominal abscess formation, wound dehiscence, incisional hernia, and postoperative adhesive intestinal obstruction.[11],[12],[13]
The cellulitis of neck in the case was a coexisting infective pathology.
| Conclusion | | |
A patent vitelline duct with umbilical fecal drainage indicates omphalomesenteric fistula and should be kept under monitoring as the real threat existed of herniation of bowel through it and repaired electively as well. Patent VID with ileal prolapse is a surgical emergency as any delay can lead to catastrophic consequences.
Acknowledgments
We thank MD Dr. Amdekar and MS Dr. Aswini, BJ Wadia Hospital for Children, Mumbai, for permitting them to publish the manuscript.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 9. | St-Vil D, Brandt ML, Panic S, Bensoussan AL, Blanchard H. Meckel's diverticulum in children: A 20-year review. J Pediatr Surg 1991;26:1289-92. |
| 10. | Fraser N, Davies BW, Cusack J. Neonatal omphalitis: A review of its serious complications. Acta Paediatr 2006;95:519-22. |
| 11. | Zea M, Chana R, Anees A, Khan S. Inverted ileal prolapse through patent vitellointestinal duct: A case report. Internet J Pediatr Neonatol 2008;10:2. |
| 12. | Mohite PN, Bhatnagar AM, Hathila VP, Mistry JH. Patent vitellointestinal duct with prolapse of inverted loop of small intestine: A case report. J Med Case Rep 2007;1:49. |
| 13. | Snyder CL. Current management of umbilical abnormalities and related anomalies. Semin Pediatr Surg 2007;16:41-9. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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