Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
Users Online: 663
 
About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Advertise Login 
     


 
 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 40-42

Umbilical mass resembling ram's horn: An unusual presentation


Department of Neonatology, B J Wadia Hospital for Children, Nowrosjee Wadia Maternity Hospital, Acharya Donde Marg, Mumbai, Maharashtra, India

Date of Web Publication8-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
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4847.199755

Rights and Permissions
  Abstract 

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

How to cite this URL:
Dipak NK, Parab S, Dande V, Rao S. Umbilical mass resembling ram's horn: An unusual presentation. J Clin Neonatol [serial online] 2017 [cited 2020 Apr 6];6:40-2. Available from: http://www.jcnonweb.com/text.asp?2017/6/1/40/199755


  Introduction Top


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 Top


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].
Figure 1: Cellulitis at nape of the neck

Click here to view


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.
Figure 2: Umbilical mass resembling Ram's horn

Click here to view


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.
Figure 3: Perioperative intestinal mass

Click here to view
Figure 4: Postrepair appearance

Click here to view


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 Top


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 Top


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 Top

1.
Jauniaux E, De Munter C, Vanesse M, Wilkin P, Hustin J. Embryonic remnants of the umbilical cord: Morphologic and clinical aspects. Hum Pathol 1989;20:458-62.  Back to cited text no. 1
    
2.
Vane DW, West KW, Grosfeld JL. Vitelline duct anomalies. Experience with 217 childhood cases. Arch Surg 1987;122:542-7.  Back to cited text no. 2
    
3.
Mariño LP, Fraga JI, Rubio S, Segarra J, Gaetano M, Ossés JA. Patent omphalomesenteric duct. Arch Argent Pediatr 2009;107:57-9.  Back to cited text no. 3
    
4.
Moore TC. Omphalomesenteric duct malformations. Semin Pediatr Surg 1996;5:116-23.  Back to cited text no. 4
    
5.
Elebute EA, Ransome-Kuti O. Patent vitello-intestinal duct with ileal prolapse. Arch Surg 1965;91:456-60.  Back to cited text no. 5
    
6.
Gvalani AK, Acharya GV, Rao RV, Dawookkar VV. Ileal prolapse through a patent vitellointestinal. Indian Pract1985;38:629-32.  Back to cited text no. 6
    
7.
Keshava Murthy M, Naveen S, Hanumanthaiah AE, Sunil B. Patent urachus in a neonate presenting with severe umbilical bleeding: A case report. JIMSA 2014;27:27-8.  Back to cited text no. 7
    
8.
Piparsaliya S, Joshi M, Rajput N, Zade P. Patent vitellointestinal duct: A close differential diagnosis of umbilical granuloma. A case report and review of literature. Surg Sci 2011;2:134-6.  Back to cited text no. 8
    
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.  Back to cited text no. 9
    
10.
Fraser N, Davies BW, Cusack J. Neonatal omphalitis: A review of its serious complications. Acta Paediatr 2006;95:519-22.  Back to cited text no. 10
    
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.  Back to cited text no. 11
    
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.  Back to cited text no. 12
    
13.
Snyder CL. Current management of umbilical abnormalities and related anomalies. Semin Pediatr Surg 2007;16:41-9.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
 
 
  Search
 
Similar in PUBMED
  Search Pubmed for
  Search in Google Scholar for
Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed1637    
    Printed11    
    Emailed0    
    PDF Downloaded105    
    Comments [Add]    

Recommend this journal