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CHAPTER 64

                             Vitelline Duct Anomalies



                                                      Bankole S. Rouma
                                                        Kokila Lakhoo






                             Introduction                                          Demographics
          Vitelline  duct  or  omphalomesenteric  duct  anomalies  are  secondary   The  most  frequent  malformation  is  Meckel’s  diverticulum,  with  an
          to  the  persistence  of  the  embryonic  vitelline  duct,  which  normally   incidence of 2–3% of the population, but it is one of the most unlikely
          obliterates by weeks 5–9 of intrauterine life. These anomalies occur in   to cause symptoms. About 4% of children with a Meckel’s diverticulum
          approximately 2% of the population and may remain silent throughout   develop symptoms, and more than 60% of those who develop symp-
                                                                                            2–5
          life, or may present incidentally sometimes with an intraabdominal com-  toms are younger than 2 years of age.  The male-to-female complica-
          plication. Although Meckel’s diverticulum is the most common vitelline   tion rate ratio is about 3:1. 3
          duct anomaly (Figure 64.1(G)), a patent vitelline duct (Figure 64.1(A))   Embryology
          is the most common symptomatic presentation in developing countries. 1
                                                                 During week 3 of gestation, the midgut is open into the yolk sac, which
                                                                 does not grow as rapidly as the rest of the embryo. Subsequently, by
                                                                 week 5, the connection with the yolk sac becomes narrowed and is
                                                                 then termed a yolk stalk, vitelline duct, or omphalomesenteric duct.
                                                                 Normally,  the  vitelline  duct  disappears  by  gestational  week  9,  just
                                                                 before the midgut returns to the abdomen. Persistence of some portion
                                                                 of the vitelline duct results in a number of congenital anomalies, of
                                                                 which Meckel’s diverticulum is the most common. This anomaly is
                                                                 variable in length and location, but most often it is observed as a 1–5
                                                                 cm intestinal diverticulum projecting from the antimesenteric wall of
                                                                 the ileum within 100 cm of the caecum. It possesses all three layers
                                                                 of the intestinal wall and has its own blood supply. The connection in
                                                                 a patent vitelline duct is usually to the ileum, but less commonly may
                                                                 be to the appendix or colon.  In other cases, part of the vitelline duct
                                                                                      1
                                                                 within  the  abdominal  wall  persists,  forming  an  open  omphalomes-
                                                                 enteric fistula, an enterocyst, or a fibrous band connecting the small
                                                                 bowel to the umbilicus. 2–7
                                                                                  Pathophysiology
                                                                 Vitelline  duct  malformations  comprise  a  wide  spectrum  of  anatomic

                                                                 structures, depending on the degree of involution of the vitelline duct.
                                                                 The  most  common  anomaly  is  Meckel’s  diverticulum,  described  as
                                                                 being 60 cm from the ileocaecal valve, 2 cm in diameter, 3 cm in length,
                                                                 and not attached to the abdominal wall. Most complications of these
                                                                 abnormalities are related to ectopic tissue (gastric, pancreatic, colonic,
                                                                 endometriosis, or hepatobiliary). 7
                                                                   Ectopic  gastric  tissue  usually  causes  bleeding  from  ulceration  of
                                                                 the adjacent ileal mucosa. The ileal mucosa is not equipped to buffer
                                                                 the acid produced by the ectopic gastric mucosa and thus is prone to

                                                                 ulceration. The site of the ulceration is most often at the junction of the

                                                                 normal ileal mucosa and the ectopic gastric mucosa. Some studies have
                                                                 shown a very low colonisation rate with Helicobacter pylori in children
                                                                 with ulcerative bleeding of Meckel’s diverticulum. 3
                                                                   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.  This  may  lead  to  a  volvulus  around
                                                                 the fibrous cord. A persistent vitelline artery, an end artery from the
                                                                 superior mesenteric artery, may cause obstruction and volvulus. Bowel
          Figure 64.1: Remnants of the omphalomesenteric duct: (A) patent vitelline duct; (B)   obstruction  can  also  occur  by  intussusception  with  the  diverticulum

          patent vitelline duct covered by skin; (C) Meckel’s diverticulum with fibrous cord;   as  a  lead  point  or  by  herniation  or  prolapse  of  the  bowel  through  a
          (D) cyst with fibrous cord; (E) cyst; (F) fibrous cord; (G) Meckel’s diverticulum.  patent  omphalomesenteric  fistula  (with  a  characteristic  “ram’s  horn”
                                                                 appearance).  Obstruction may be caused by phytobezoar. 6,7
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