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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.
Wound Infection
  • In a retrospective descriptive study, Brandi CD et al reported that mesh repair is not correlated with the incidence of wound infection and leads to low recurrence of ECF; these findings further contribute to the controversy regarding the application[ncbi.nlm.nih.gov]
Cardiomegaly
  • Upon performing fetal echocardiography assessment, mild cardiomegaly and dilatation of the inferior vena cava were detected. Chromosomal testing revealed a normal karyotype.[degruyter.com]
Burning Sensation
  • She reported abdominal pain similar to a burning sensation that accompanied the discharge of faecal matter. These symptoms and signs waxed and waned but lasted for 5years.[ncbi.nlm.nih.gov]
Microscopic Hematuria
  • Detection of microscopic hematuria with proper evaluation and treatment options is understudied in females.[urologyannals.com]
Vaginal Discharge
  • She was admitted to the referring hospital with complaints of on-and-off watery vaginal discharge and mild vaginal bleeding.[urologyannals.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

  • 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]
  • Spontaneous external biliary fistulae are now extremely rare events because of the relative safety of early treatment of biliary tract disease.[birpublications.org]
  • […] 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]
  • In the rare case where a segment of colon is strangulated with resulting gangrene and there is perforation and egress of stool, previously reported and recommended methods of treatment are not applicable.[jamanetwork.com]

Prognosis

  • Timely surgical intervention is crucial for reducing mortality and improving prognosis.[ncbi.nlm.nih.gov]
  • (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]
  • 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]

Etiology

  • 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]
  • Neurol India 2018;66:1831-3 Sir, A ventriculoperitoneal (VP) shunt is a commonly performed surgical procedure for hydrocephalus of different etiologies.[neurologyindia.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]
  • Etiology of neonatal gastric perforation: a review of 20 years' experience. Pediatr Surg Int, 28: 9-14, 2012. 吉田英生, 大植孝治, 大野康治, 寺倉宏嗣, 尾藤祐子, 増本幸二, 韮澤融司.[m.chiba-u.ac.jp]

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 embryological aspect of this anomalous communication is described, along with all the 7 cases previously reported with their pathophysiological causes. Conclusions: The CSF fistula through the umbilicus may be due to varying pathological causes.[karger.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]

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]
  • 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]
  • The shunt catheter could neither be seen nor palpated per abdomen Click here to view As far as management of such cases is considered, a persistent omphalomesentric tract, when identified, should be operated upon and closed to prevent late complications[neurologyindia.com]

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