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Disorders of the Umbilicus   355

          umbilical  hernia  may  continue  to  stretch  and  result  in  a  proboscoid
          umbilical  hernia.  In  Africa,  most  parents  are  very  accepting  of  its
          appearance, in contrast to parents from developed countries. Once the
          umbilical  defect  has  spontaneously  closed,  the  nipple-like  umbilical
          skin may continue to flatten, even during adolescence.
          Aetiology
          An  umbilical  hernia  results  when  the  umbilical  ring  fails  to  close.
          Umbilical hernias are more frequent in premature, low birth weight,
          and black infants. They also occur more often in children with ventricu-
          loperitoneal shunts, ascites, obesity, and certain syndromes, including
          Beckwith-Wiedemann, Trisomy 21, and Marfan’s syndromes.
          Demographics
          Umbilical hernias are common in Africa. In one study from Nigeria,
          umbilical hernias were found in 91% of under-6-year-olds; 64% of 6- to
                                           15
          9-year-olds, and 46% of 10- to 15-year-olds.  Meier found umbilical
          hernias with a fascial defect >1 cm in 23% of Nigerian children younger
                        16
          than  18  years  old.   Surprisingly,  when  6-  to  9-year-old  Nigerian
          children of high socioeconomic class were evaluated for an umbilical
                                                     17
          hernia, only 1.3% of 7,968 children had an umbilical hernia.  It is pos-
          sible that nutrition may be a factor. Jelliffe found a higher incidence of   Figure 57.6: Giant ulcerated umbilical hernia.
          umbilical hernias in malnourished versus well-nourished adults (27%
          versus 14%). 15                                        umbilicus, and pain. On examination, a child with an umbilical hernia
          Complications                                          usually presents with a protrusion of the umbilicus with contents that
                                                                 are  easily  reducible. After  reduction,  the  size  of  the  fascial  ring  can
          Complications,  including  incarceration,  strangulation,  and  rupture  of
                                                                 be palpated; it can range from a few millimeters to more than 4 cm in
          umbilical hernias, may occur. In developed countries, the incidence of
                                                                 diameter. No other investigations are required for diagnosis.
          incarceration or strangulation is rare—one paper reported an incidence
                                  18
          of  1  in  1,500  umbilical  hernias.   Rupture  of  umbilical  hernias  with   Umbilical  hernia  repair  is  one  of  the  most  frequent  procedures
                                                                                                            1
                                                                 performed by paediatric surgeons in developed countries.  In Africa,
          evisceration is even more rare, but has been reported in infants younger
          than 6 months of age. 19,20                            however,  umbilical  hernia  repairs  are  more  infrequent  because  the
                                                                 hernias  are  usually  repaired  only  if  symptomatic  or  complicated.
            Even  though  the  incidence  of  incarceration  and  strangulation  in
                                                                 Generally accepted indications for management in Africa compared to
          children  with  umbilical  hernias  in  Africa  is  not  known,  it  appears
                                                                 the West are highlighted next.
          to  be  higher  than  in  the West  (although  this  may  in  part  reflect  the
                                                                   Due to the high rate of spontaneous closure and the fact that the
          significantly higher prevalence of umbilical hernias in black children).
                                                                 appearance of a proboscoid umbilical hernia is well tolerated by parents
          For  instance,  at A.  Le  Dantec  Hospital  in  Senegal,  over  a  five-year
                                                                 in Africa, conservative management of asymptomatic, easily reducible
          period,  41  children  had  emergency  operations  for  incarcerated  or
                                21
          strangulated umbilical hernias.  At Jos University Teaching Hospital in   umbilical hernias is recommended. In the West, conservative treatment
                                                                 is  generally  recommended  for  small  asymptomatic  umbilical  hernias
          Nigeria, over an eight-year period, 23 children underwent surgery for
          acute or recurrent incarceration. 16,19,22–24          (<2 cm fascial defect) in children younger than 4–5 years of age.
                                                                   In  Africa,  surgical  repair  is  reserved  for  symptomatic  umbilical
            In contrast, Okada et al. reviewed the literature from 1957 to 1999
                                                            25
          and found a total of only 38 cases reported in children worldwide.    hernias.  Rarely,  parents  may  request  to  have  a  proboscoid  hernia
                                                                 repaired.  (For  further  discussion,  see  the  “Ethical  Issues”  section  in
          In King’s College Hospital in London, only 3 incarcerated umbilical
                                                                 this chapter.) In the West, surgical repair is generally recommended for
          hernias  were  treated  in  children  over  a  20-year  period  (and  all  3  of
                                  26
          these occurred in black children).  The fact that most umbilical hernias   hernias with large fascial defects (>1.5 cm), hernias that have failed to
                                                                 spontaneously close by 4 to 5 years of age, and umbilical hernias with
          in the West are repaired by 4–5 years of age does not account for the
                                                                 significant proboscoid components.
          apparent difference in the frequency of incarceration between the West
                                                                   A classic Mayo “vest-over-pants” procedure or simple approximation
          and Africa.  In  both  Senegal  and  Nigeria,  most  of  the  incarcerations
                                                                 with long-lasting absorbable suture are both acceptable for conventional
          reported occurred in patients younger than 5 years of age; in Senegal,
                                                                 umbilical hernia repairs. For complicated umbilical hernia repairs, the
          the average age at incarceration was 14 months (range, 8 months–10
                                                                 use of mesh may be considered in the closure of a very large uninfected
          years); in Nigeria, the median was 4 years (range, 3 weeks–12 years).
                                                                 umbilical hernia to prevent excess tension on the fascia. The use of mesh
          Most  incarcerated  hernias  do  not  have  an  inciting  factor;  however,
                                                                 also prevents the development of an abdominal compartment syndrome,
          bezoars,  digested  vegetable  matter,  parasitic  worms,  or  ascites  have
          been implicated. 21,22,25                              which could result with significant fascial tension. Surgical complications
                                                                 are  rare  after  umbilical  hernia  repairs.  The  outcome  is  excellent  and
            Umbilical hernias can incarcerate regardless of the size of the fascial
                                                                 the  mortality  approaches  zero  for  elective  repairs.  Rare  postoperative
          defect (Figure 57.6). In one report, a majority (52%) of the patients
                                                                 eviscerations can be prevented by meticulous surgical technique.
          with  incarcerated  hernias  had  medium-sized  (0.5–1.5  cm)  fascial
          defects, whereas 24% occurred in small defects (<0.5 cm), and 24%   Other Umbilical Problems
                              25
          in  large  defects  (>1.5  cm).   Of  the  incarcerated  hernias  in  which  a   Absent umbilicus
          measurement was documented in one study in Nigeria, all had defects   Malposition  or  absence  of  the  umbilicus  is  encountered  frequently
          greater than 1.5 cm in diameter (not all, however, were measured). 22
                                                                 in patients with bladder exstrophy. When the umbilicus is absent, an
          Management                                             omphaloplasty may be performed, as many ethnic groups are culturally
          Factors that lead parents to seek medical care for their child in Africa   sensitive to the absence of the navel. Research has been performed to
          include the age of the child, size of the defect, height of protruding   help the reconstructive surgeon locate the umbilicus in an aesthetically
                                                                 pleasing location. 27
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