Urachal Remnants

Pediatric Surgery, AlSadik Hospital, Qatif, Saudi Arabia


16.1 Introduction

  • The urachus is a fibrous remnant of the allantois.

  • The allantois is a canal that drains the urinary bladder of the fetus and runs within the umbilical cord.

  • The urachus is a band of fibrous tissue extending from the dome of the bladder to the umbilical cord.

  • By 32 weeks, the urachus is obliterated and becomes a vestigial structure known as the median umbilical ligament (not to be confused with the medial umbilical ligament, which is a separate structure that lies laterally to the median umbilical ligament).

  • The urachus remnant lies in the space of Retzius, between the transversalis fascia anteriorly and the peritoneum posteriorly.

  • In 0.1–2.0 % of the population, the urachus remains patent and may result in urine leaking from the umbilicus or infected urachal cysts.

  • Persistence of urachal remnant can give rise to various clinical problems:

    • Urachal cyst

    • Urachal fistula

    • Urachal diverticulum (Vesicourachal diverticulum)

    • Urachal sinus

  • Because urachal remnants are uncommon and manifest with nonspecific abdominal or urinary signs and symptoms, definitive presurgical diagnosis is not easily made.

  • The urachus is also known to undergo malignant transformation and adenocarcinoma is the commonest.

  • A rare urachal tumor has been reported as a manifestation of IgG4-related disease.

16.2 Embryology

  • Embryologically, the amniotic cavity bulges over the embryo and attaches to the yolk sac and the connecting stalk to form the umbilical cord.

  • Further caudal folding of the embryo incorporates the proximal yolk sac into the hindgut and the allantois which is a diverticulum of the yolk sac into the urogenital sinus.

  • The yolk and body stalks fuse to become the umbilical cord.

  • In the third week of gestation, the allantois, which grows into the body stalk is formed as a diverticulum from the yolk sac.

  • The allantois appears on about day 16 as a tiny, finger-like outpouching from the caudal wall of the yolk sac.

  • The bladder develops from the ventral portion of the expanded terminal part of the hindgut, the cloaca, which is contiguous with the allantois ventrally.

  • The cranioventral end of the bladder opens into the allantois at the level of the umbilicus; thus, the bladder initially extends all the way to the umbilicus.

  • By the fourth or fifth month of gestation, the bladder descends into the pelvis and its apical portion progressively narrows to a small, epithelialized fibromuscular strand, the urachus (Figs. 16.1, 16.2, and 16.3).


    Figs. 16.1, 16.2, and 16.3
    Diagrammatic representations of the embryo at 3, 4 and 5 weeks of intra-uterine life. Note the developing allantosis and omphalomesenteric duct

  • In late embryonic and fetal life and early postnatal life, the urachal portion, which is still microscopic, fails to grow; thus, its lumen remains narrow and is usually obliterated by fibrous proliferation.

  • In one-third of adults, it may be visible at microscopic examination as a structure communicating with the lumen of the bladder; however, in terms of function it can be considered closed by the latter half of fetal life.

  • The urachus varies from 3 to 10 cm in length and from 8 to 10 mm in diameter.

  • It is a three-layered tubular structure, the innermost layer being lined with transitional epithelium in 70 % of cases and with columnar epithelium in 30 %.

  • The structure is surrounded by connective tissue and an outermost muscular layer in continuity with the detrusor muscle.

  • Occasionally, the urachus may merge with one or both of the obliterated umbilical arteries, and there may be a slight deviation to the right or left of the midline.

  • As the distal hindgut and the urogenital sinus separate, the developing bladder remains connected to the allantois through a connection called the urachus.

  • Persistence of this communication leads to urachal remnants.

  • The urachal remnant anomalies include:

    • Patent urachus

    • Urachal sinus

    • Urachal cyst

    • Urachal diverticulum

  • Subsequently, the yolk and body stalks fuse to become the umbilical cord.

  • Development of the abdominal wall narrows the umbilical ring, which should close before birth.

  • Persistence of the umbilical ring results in an umbilical hernia.

16.3 Classification

  • There are four types of congenital urachal remnant anomalies.

  • They are:

    • Patent urachuss(50 %)

    • Urachal cyst (30 %)

    • Urachal-umbilical sinus (15 %)

    • Vesicourachal diverticulum (5 %)

  • Urachal cyst:

    • A urachal cyst is a cyst which occurs in a persistent portion of the urachus between the umbilicus and the urinary bladder.

    • It presents as an extraperitoneal mass in the umbilical region.

    • Urachal cysts are usually silent clinically until infection, calculi or adenocarcinoma develop

    • It is characterized by:

      • Abdominal pain, and fever if infected

      • It may rupture, leading to peritonitis

      • It may drain through the umbilicus.

  • Patent urachus:

    • This is the commonest congenital malformation of the urachus.

    • In patent urachus, the whole urachus fails to obliterate and there is a patent communication between the urinary bladder and the umbilicus.

  • Umbilical sinus:

    • In umbilical sinus, the distal part of the urachus remains open to the umbilicus.

    • The usual presentation is persistent umbilical discharge.

  • Vesico-urachal diverticulum:

    • In this, there is a wide patent urachal opening to the urinary bladder

  • Inflammation and malignancy are the commonest complications of urachal remnants.

  • Inflammation occurs more frequently in children and young adults.

Jul 10, 2017 | Posted by in UROLOGY | Comments Off on Urachal Remnants

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