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Umbilical Fistula

An umbilical fistula is a term that describes a connection between organs and the external environment through the umbilicus, but vascular fistulas may also arise at this anatomical site. Various forms of intestinal and urinary fistulas are described in the literature, and extensive imaging studies are required to confirm the exact type.


Presentation

Earlier reports considered umbilical fistulas to be rare and were primarily diagnosed as complications of abdominal surgery [1], but numerous congenital or secondary conditions have subsequently shown to be involved in their pathogenesis. The clinical presentation of an umbilical fistula significantly depends on its type [1] [2] [3] [4] [5]:

  • Persistent urachus and urachal sinus - Urachus, also termed the median umbilical ligament, is a remnant structure of the primitive bladder that should normally obliterate during fetal life [5] [6]. In rare cases, it's incomplete transformation can lead to several anatomical disorders, and a persistent urachus, as well as urachal sinus, can result in a connection of the bowels (or the urinary system) and the external environment through the umbilicus [3] [5]. In such instances, umbilical discharge (fecal, urinary, or purulent in the setting of abscess formation) is the most important and often the only symptom [3] [5] [6]. An abdominal mass and erythema around the umbilicus can be seen in some patients.
  • Arteriovenous fistulas - Vascular malformations of the umbilical arteries and veins (sometimes in association with a persistent urachus) can be asymptomatic and pose no risk for infants and neonates, in whom this diagnosis is most frequently made [2] [5]. In more severe blood shunting, however, congestive heart failure, life-threatening hemorrhage (most frequently after trauma) or thrombus formation can occur, in which case immediate therapeutic measures are mandatory [2] [7]. Having in mind the vascular origin of the fistula, a pulsatile mass may be a valid clinical sign encountered during a physical examination.
  • Omphalomesenteric fistula - The omphalomesenteric duct connects the terminal ileum and the umbilicus during fetal life and its regression is observed during the early months of gestation. In rare cases, its persistence can lead to an open connection between the bowels and the skin (similarly to urachal abnormalities) [3] [8], with a similar clinical presentation consisting of umbilical discharge.
Neglect
  • First, strangulation in a neglected Richter’s hernia that causes fistula is typically an urgent, rapidly developing, and critical condition with high mortality. In 1986,J.M.[bmcsurg.biomedcentral.com]

Workup

Umbilical discharge or presence of an abdominal mass mandates a thorough physical examination and the use of various imaging studies to confirm the etiology. A detailed inspection and palpation of the abdomen may provide important clues, but ultrasonography is considered to be the first diagnostic method used in such circumstances, especially when urachal diseases and vascular fistulas are present [2] [5] [7]. If the diagnosis remains inconclusive, computed tomography (CT), magnetic resonance imaging (MRI), voiding cystourethrography (when urinary discharge from the umbilicus is reported) and a fistulogram are recommended, with CT carrying the highest rate of detection [5]. Although rare, diagnostic modalities may fail to confirm the underlying cause, which is why some patients receive a diagnosis after surgery [5]. Apart from imaging studies, the value of laboratory workup should be pointed out, especially in patients who suffer from hemorrhage in arteriovenous fistulas. For this reason, a complete blood count (CBC), a full coagulation panel and renal function tests should be obtained.

Granulomatous Tissue
  • Pathological finding of the surgically removed fistula demonstrated granulomatous tissues containing silk ligature. Acquired umbilical fistula is a rare complication of inguinal herniorrhaphy.[ncbi.nlm.nih.gov]

Treatment

  • Treatment Treatment of uvelular fistula treatment Department of Anesthesiology Anesthesiology Medicine Pediatric Surgery Pediatric Pediatric Surgery Treatment: Drug treatment Surgery treatment Rehabilitation treatment Treatment cycle: 1 month Cure rate[healthfrom.com]
  • In view of the complete closure of the fistulas with medical treatment, we recommended conservative medical management, at least initially, in such patients.[ncbi.nlm.nih.gov]
  • Complete excision is the correct treatment.[ncbi.nlm.nih.gov]
  • […] usually seen in paediatric patients as persistent discharge from umbilicus 1 ; however cases have also been reported in adults 2 .It can also present as small bowel obstruction due to prolapse of ileum through the fistula, requiring early diagnosis and treatment[pakjr.com]
  • We performed surgical treatment after drainage for spontaneous umbilical fistula in Crohn's disease.[jstage.jst.go.jp]

Prognosis

  • (Outcomes/Resolutions) The prognosis of Arteriovenous Malformation of Umbilical Cord is poor in a majority of cases. There is an increased incidence of fetal mortality with this condition.[dovemed.com]
  • Prognosis linked with malignization of the urachus is often poor. This is usually due to a delayed diagnosis and early peritoneal metastatization, which can lead to pseudomyxoma peritonei (PMP).[omicsonline.org]
  • Timely surgical intervention is crucial for reducing mortality and improving prognosis.[bmcsurg.biomedcentral.com]
  • Prognosis The location and severity of the fistula play a major role in determining treatment. A fistula is a sign of serious inflammatory bowel disease (IBD), and without proper care, can lead to serious complications.[verywell.com]
  • Prognosis ECF is a common condition in most general surgical wards.[emedicine.medscape.com]

Etiology

  • Etiology Urogenital fistula etiology Etiology (30%): The etiology of urachus fistula is not yet fully understood, although some people think that with the urinary tract obstruction, and in fact in the uveal fistula neonatal, only 14% found evidence of[healthfrom.com]
  • Umbilical discharge or presence of an abdominal mass mandates a thorough physical examination and the use of various imaging studies to confirm the etiology.[symptoma.com]
  • A neonate with an intact congenital umbilical appendix: an alternative theory on the etiology of the appendico umbilical fistula. Pediatr Surg Int 2006; 22:689–693. 2. Nagar H. Umbilical granuloma: a new approach to an old problem.[journals.lww.com]
  • (Etiology) The exact cause of Arteriovenous Malformation of Umbilical Cord is unknown Research is currently being performed to identify the relevant causative factors It is not caused by what the expectant mother does or does not do, either prior to or[dovemed.com]
  • Infectious inflammation of navel skin (omphalitis) in adults has also bacterial or fungal etiology. And discharge from navel during pregnancy can occur just because of omphalitis.[iliveok.com]

Epidemiology

  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.es]
  • Epidemiology of the Malformations The incidence rate of malformations is 4-6% and consists of 2-3% of all newborns and another 2-3% of children up to the age of 5.[lecturio.com]
Sex distribution
Age distribution

Pathophysiology

  • Spontaneous umbilical fistula pathophysiology and pathways are reviewed.[ncbi.nlm.nih.gov]
  • Spontaneous umbilical fistula pathophysiology and pathways are reviewed. Key words Spontaneous umbilical fistula This is a preview of subscription content, log in to check access. Preview Unable to display preview. Download preview PDF.[link.springer.com]
  • The pathophysiologic mechanism is unclear but several explanations are proposed. Umbilicus location in the median raphe naturally renders it a place of lesser resistance.[bioline.org.br]
  • The pathophysiology of Richter’s hernia is not well understood. To date, a small abdominal wall defect with hard peripheral tissue was regarded as a key feature of the evolution and progression of this type of hernia.[bmcsurg.biomedcentral.com]

Prevention

  • Knowledge of the variations in anatomical structure of the urachus and umbilical arteries at birth will help to prevent this complication.[ncbi.nlm.nih.gov]
  • , severe posterior urethral valve are not both There is uvelular fistula, so the disease can not be prevented, so early detection, early diagnosis, early treatment of indirect prevention of this disease is of great significance.[healthfrom.com]
  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.es]
  • Careful inspection and palpation of the umbilical cord prior to these procedures may prevent a fistula being created.[ncbi.nlm.nih.gov]

References

Article

  1. Pracyk JB, Pollard SG, Calne RY. The development of spontaneous colo-umbilical fistula. Postgrad Med J. 1993;69(815):750-751.
  2. Gozar H, Gozar L, Badiu CC, et al. Interact Cardiovasc Thorac Surg. 2014;18(5):688-689,
  3. Alessandrini P, Derlon S. Congenital umbilical fistulas. A report of 12 cases. [Article in French] Pediatrie. 1992;47(1):67-71.
  4. Pust A, Ovenbeck R, Erbersdobler A, Dieckmann KP. Laparoscopic management of patent urachus in an adult man. Urol Int. 2007;79(2):184-6.
  5. Choi YJ, Kim JM, Ahn SY, Oh J-T, Han SW, Lee JS. Urachal Anomalies in Children: A Single Center Experience. Yonsei Med J. 2006;47(6):782-786.
  6. El Ammari JE, Ahallal Y, El Yazami Adli O, El Fassi MJ, Farih MH. Urachal Sinus Presenting with Abscess Formation. ISRN Urol. 2011;2011:820924.
  7. Shibata M, Kanehiro H, Shinkawa T, et al. A neonate with umbilical arteriovenous malformation showing hemorrhagic shock from massive umbilical hemorrhage. Am J Perinatol. 2009;26(8):583-586.
  8. Hunald FA, Rajaonarivony MF, Rakotovao M, Ravololoniaina T, Rakoto-Ratsimba H, Andriamanarivo ML. Unusual discovery of omphalomesenteric fistula. A case report. [Article in French]. Arch Pediatr. 2011;18(4):420-422.

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Last updated: 2018-06-22 07:28