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Q: are there any known complications later in life of omphalocele? ( Answered,   0 Comments )
Question  
Subject: are there any known complications later in life of omphalocele?
Category: Health > Conditions and Diseases
Asked by: jodygold-ga
List Price: $50.00
Posted: 15 Sep 2005 09:52 PDT
Expires: 15 Oct 2005 09:52 PDT
Question ID: 568384
I had omphalocele when I was born. Surgery was successful and besides
an ugly belly button, I have had no known subsequent problems with it.
Now I am 31.

Two weeks ago, I underwent emergency abdominal surgery due to a
"twisted colon", specifically at the cecum. I was told that part of my
colon was "floppy". They removed one foot of the intestine, including
appendix and cecum. Could this be related at all to the omphalocele I
had at birth? Are there any known incidences?

Request for Question Clarification by pafalafa-ga on 16 Sep 2005 07:29 PDT
jodygold-ga

The answer to your question is almost certainly "Yes...it COULD be
related".  Whether it IS, in fact, related, is a diagnosis only your
doctor could make.

A high proportion of infants born with an omphalocele also have
abnormalities in other parts of their body.  These abnormalities may
or may not be recognized at the time the omphalocele is treated.

I did not see much in the way of medical literature that links the
presence of an omphalocele specifically to colon disorders.  Nor is
there much in terms of long-term (adult) follow-up of patients.

Here's a link, though, to one article that mentions omphalocele and
colon disorder (but not twisted colon) together:


http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=21213118


Note in the above that the colon disorder was evident in the newborn
infant, as would be expected with most serious colon disorders. 
Although I'm not a medical professional (so take all this with a grain
of salt) it seems to me that a mild defect in the colon may not
present any problems until later in life.

There's a pretty good overview of omphaloceles here:


http://www.childrenshospital.org/az/Site1384/mainpageS1384P0.html


that discusses how often other sorts of defects or complications are
present (the percentage is pretty high!).  If you haven't seen this
sort of information already, it's certainly worth a careful read.

Bottom line, of course, is that only your doctor can offer
professional guidance on your question.

However, please do let me know what sort of additional information you
would like here to make for a complete answer to your question.

All the best, and stay healthy!

pafalafa-ga
Answer  
Subject: Re: are there any known complications later in life of omphalocele?
Answered By: crabcakes-ga on 16 Sep 2005 18:43 PDT
 
Hello  Jodygold,

   In the beginning... during the first two mnths of fetal
development, something called malrotation occurs in an estimated 1 in
5,000* live births.

Malrotation occurs when the part of the colon, as a normal part of
fetal development, ( between the three and 8 weeks of gestation)passes
into the umbilical cord, but returns misplaced and askew (Around the
10th and 12th week of gestation). This occurs because of lack of space
in the fetal abdominal area. As the fetus grows, the colon returns to
the abdominal cavity of the fetus. Normally, this portion of the
intestine rotates into place. When the intestine malrotates, or turns
the wrong way, the intestine becomes suspended by tissue (mesentery)
http://www.emedicine.com/ped/topic1205.htm

*?The incidence of omphalocele reported in the literature varies
considerably, ranging from 0.8 to 3.9 cases per 10,000 births (2).?.
http://radiology.rsnajnls.org/cgi/content/full/232/1/191

Colonic volvulus, better known as twisted colon, may indeed be
connected to a congenital omphalocele.  Adhesions, or scar tissue,
over or undergrowth of intestinal mesentery may precipitate twisted
colon. However, one can have twisted colon without having had an
omphalocele. Some studies show that a poor western diet, low in fiber
may  be an underlying cause of twisted colon.


?Background: Malrotation of the intestine results when the normal
embryologic sequence of bowel development and fixation is interrupted.
The malrotated bowel is prone to torsion, resulting in midgut
volvulus.

Pathophysiology: Development of the human gut takes place during the
first months of fetal life. In the normal embryo, physiologic
herniation of the gut through the umbilicus at 6 weeks is accompanied
by a 270° counterclockwise rotation of the developing intestine around
the superior mesenteric artery (SMA). By 10-12 weeks, the intestine
returns to the abdomen and assumes its normal adult anatomic position.
Normal small bowel mesentery has a broad attachment stretching
diagonally from the duodenojejunal junction (DJJ) (in the left upper
quadrant) to the cecum (in the right lower quadrant). The point of
attachment at the DJJ is referred to as the ligament of Treitz.? If
you sign up for a free membership, you can continue on with the
article, which includes some illustrations and x-rays.
http://www.emedicine.com/radio/byname/midgut-volvulus.htm


?Etiology ? Fetal abdominal wall defects form as a result of
disturbances in organogenesis during the embryonic period. (See
"Ultrasound diagnosis ofetal abdominal wall defects", section on
Embryology). There are three theories on the etiology of omphalocele
formation: persistence of the primitive body stalk, failure of the
bowel to return to the abdomen, and failure of complete lateral-body
fold migration and body wall closure [4]. The latter theory helps to
explain why some omphalocele sacs contain liver.

Most omphaloceles occur sporadically, but are associated with a high
risk of concomitant chromosomal abnormality (40 to 60 percent) if an
intracorporeal liver is present with exclusively small bowel in the
omphalocele sac [4-6]. Karyotypic abnormalities include trisomies 13,
15, 16, and 18 and Beckwith-Wiedemann syndrome [1]. Fetuses with an
omphalocele containing liver typically, but not invariably, have a
normal karyotype [6,7].?
http://patients.uptodate.com/topic.asp?file=pregcomp/18882&title=Amniocentesis


?Aetiology of the condition remains speculative. While chronic
constipation is blamed for the Western type of sigmoid volvulus, a
high fibre diet has been deemed a major factor in the development of
sigmoid volvulus in the African population.?
?Underlying condition causes of Colonic volvulus: The list of possible
underlying conditions (see also Misdiagnosis of underlying causes of
Colonic volvulus) mentioned in various sources as possible causes of
Colonic volvulus includes:
·	Chronic constipation (type of Constipation) 
·	Abdominal adhesions (type of Adhesions) 
·	Congenital bowel defects ?
http://www.wrongdiagnosis.com/c/colonic_volvulus/causes.htm

?Volvulus is a condition of the large bowel in which the bowel twists
on its pedicle (mesentery) causing a blockage of the blood vessels and
thus may lead to necrosis of the bowel, necessitating surgery to
remove the damaged portion.?
http://encyclopedia.laborlawtalk.com/volvulus

?The small bowel and colon are held in position by tissue known as the
mesentery. The ascending colon and descending colon are fixed in place
in the abdominal cavity. The cecum, transverse colon, and sigmoid
colon are suspended from the back of the abdominal wall by the
mesentery.?


?Volvulus refers to the twisting of a portion of the intestine around
itself or a stalk of mesentery tissue to cause an obstruction.
Volvulus occurs most frequently in the colon, although the stomach and
small bowel can also twist. The part of the digestive system above the
volvulus continues to function and may swell as it fills with digested
food, fluid, and gas. A condition called strangulation develops if the
mesentery of the bowel is twisted so tightly that blood flow is cut
off and the tissue dies. This condition is called gangrene. Volvulus
is a surgical emergency because gangrene can develop quickly, cause a
hole in the wall of the bowel (perforation), and become
life-threatening.?
http://digestive.niddk.nih.gov/ddiseases/pubs/anatomiccolon/#Fistulas

?In volvulus a loop of bowel twists on itself, usually in association
with fibrous peritoneal adhesions which, in turn, are usually the
result of previous abdominal surgery."
 Page 18
http://www.fleshandbones.com/readingroom/pdf/285.pdf


?Background: Volvulus is defined as a complete twisting of a loop of
intestine around its mesenteric attachment site. Such twisting can
occur at various sites of the GI tract, including the stomach, small
intestine, cecum, transverse colon, and sigmoid colon. Midgut volvulus
refers to twisting of the entire midgut about the axis of the superior
mesenteric artery. This article mainly concerns midgut volvulus
because it is the most common and is a very serious type of volvulus
in infants and children.

A number of rotational abnormalities have been described correlating
with abnormal embryologic development of the midgut. Mall first
described intestinal rotation during the embryologic period in 1898.
However, Waugh in 1911 first described the clinical presentation of
intestinal malrotation. Ladd, reporting in 1936, wrote the classic
paper on surgical treatment of malrotation.?
?Malrotation leaves the cecum high in the mid abdomen with its
peritoneal attachments, called the Ladd bands. These bands stretch
from the cecum to the right lateral abdominal wall, causing
compression of the duodenum and mechanical obstruction. Abnormal
fixation of the ligament of Treitz causes narrowing and kinking of the
duodenum near its junction with the jejunum. Along with it, the
mesentery remains bunched up in the epigastrium and does not fan out.?

?Omphalocele -Abdominal musculature fails to form; infant born with
herniated abdominal contents into ventral membranous sac?
http://pathologyoutlines.com/colon.html#omphalocele


?In summary, the human embryo initially is disc-shaped and composed of
2 cell layers. It acquires a third cell layer as it grows above the
umbilical ring and becomes cylindrical by elongation and inward
folding. The body folds (cephalic, caudal, lateral) meet in the center
of the embryo where the amnion invests the yolk sac. Defective
development at this critical location results in a spectrum of
abdominal wall defects. By the sixth week, rapid growth of the midgut
causes a physiologic hernia of the intestine through the umbilical
ring. The intestine returns to the abdominal cavity during the tenth
week, and rotation and fixation of the midgut occur. This process does
not occur in babies with gastroschisis or omphalocele, resulting in an
increased risk of midgut volvulus.?

?·  Paradoxically, babies with small (unimpressive) omphaloceles are
most likely to have associated abnormalities, including intestinal
problems (Meckel diverticulum, atresia), genetic syndromes
(Beckwith-Wiedemann), and congenital heart disease.
·  Babies with giant omphaloceles usually have small, bell-shaped,
thoracic cavities and minimal pulmonary reserve; reduction and repair
of the omphalocele frequently precipitates respiratory failure, which
may be chronic and require a tracheotomy and long-term ventilator
support. The authors recently cared for a baby with giant omphalocele
and diaphragmatic hernia. Both conditions are associated with
pulmonary hypoplasia, and when they are combined, the severity of the
pulmonary deficit precludes survival, even with Extracorporeal
Membrane Oxygenation (ECMO) support, as provided for our patient.:
http://www.emedicine.com/ped/topic1642.htm

?According to the Online Mendelian Inheritance in Man database (17),
omphalocele may be a part of 52 syndromes. Therefore, a wide range of
differential diagnoses should be considered for fetuses in whom
omphalocele is observed. Detailed repeat targeted US examinations of
all fetal organs are mandatory in these cases because the detection of
associated structural anomalies may help direct the observer in
diagnosing a specific syndrome. Because of the reported high incidence
of chromosomal aberrations in fetuses with omphalocele, in this study
we offered chromosomal analysis to all patients with this abnormality.
Despite the fact that there were no cases of an abnormal karyotype in
the fetuses with isolated omphalocele in our series, we still believe
that karyotyping should be offered to all patients in whom this
anomaly is detected because our series was too small for us to draw a
definitive conclusion regarding the possibility of an abnormal
karyotype in such fetuses.

Of special interest are the nine fetuses in whom the omphalocele
disappeared according to follow-up US findings. All of these fetuses
had a normal karyotype, and the initial US scan revealed only a few
herniated intestinal loops in the umbilical cord. In all except one
(fetus 22 in Table 1) of these cases, the omphalocele was a solitary
finding.?
http://radiology.rsnajnls.org/cgi/content/full/232/1/191



?Colonic volvulus accounts for 1-7% of cases of large bowel
obstruction in the United States and Western Europe. It is, however, a
much commoner condition in parts of Africa, South Asia and South
America. Volvulus is thought to be an idiopathic condition, probably
with an anatomical basis, particularly in cecal volvulus.?
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=2663257&dopt=Citation

?I was able to find two recent reports which assess the long term
morbidity and quality of life in survivors of gastroschisis. In the
first study 83/94 patients with omphalocele or gastroschisis survived
initial treatment and 61 had long term follow-up. If congenital
abnormalities were not present survival was favorable. Nineteen
patients required 31 reoperations, most for abdominal wall hernias and
the sequelae of intestinal atresia. Current quality of life (10-20
years after birth) was described as good in 80% of patients. In the
second study 23/35 patients with gastroschisis (age range 12-23 years
old) were questioned about their general health, growth, abdominal
symptoms, cosmetic concerns, education, employment and fertility.

 22 patients were in good health and overall growth was normal. Eight
patients underwent further surgery related to gastroschisis, including
two for adhesive small bowel obstruction (scar tissue forming in the
abdomen after surgery-this can cause intestinal blockage) and three
for scar complications. In 13 patients the absence of an umbilicus
(belly button) caused emotional distress during childhood. These
reports suggest that once corrective surgery is performed most
gastroschisis survivors can expect normal growth and good health,
however, a significant percentage of patients will require
reoperation.?
http://www.medhelp.org/forums/gastro/archive/221.html

?Twisted colon: Twisted colon is listed as a type of (or associated
with) the following medical conditions in our database: Digestive
Diseases Twisted colon (medical condition): Twisting of the colon.?
http://www.wrongdiagnosis.com/medical/twisted_colon.htm

?Omphalomesenteric remnants: Persistence of all or portions of the
omphalomesenteric duct can result in fistulas, sinus tracts, cysts,
congenital bands, and mucosal remnants. Patients with mucosal remnants
can present with an umbilical polyp or within an umbilical cyst.?
http://www.emedicine.com/ped/topic2948.htm


?Many babies have correctable lesions and simply require routine
pediatric care. For others, the abdominal wall defect is part of a
larger constellation of unresolved problems, and further care by
specialists is necessary. All of these children, however, require
general management by pediatricians who have knowledge of their
particular anomalies and their past surgical histories. For example,
physicians should know if an associated malrotation was corrected (to
prevent midgut volvulus) and whether an abnormally located appendix
was removed (to prevent occurrence of atypical appendicitis).?
http://www.emedicine.com/ped/topic1642.htm

?Volvulus has multifactoral causes. It occurs worldwide, and is a
common cause of large bowel obstruction. Current knowledge suggests
that primary prevention is not possible, so better management is
needed if the number of deaths are to be reduced. Most cases of
sigmoid volvulus can be nonoperatively decompressed. However,
operation is required to remove gangrenous bowel and stop recurrence.
Treatment needs to be individualized as appropriate for medically
compromised patients. Sound clinical judgment still has an important
role in the diagnosis and management of this disorder.?
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8719999&dopt=Citation

An interesting German study:
?From 1970 to 1998, 35 children with omphalocele (OC) and 31 with
gastroschisis (GS) were treated at the Department of Paediatric
Surgery at Lubeck Medical University. Forty of 43 survivors were
examined in 1990, the data of 30 patients were renewed in 1999 and 12
new cases added.?
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11077588&dopt=Abstract


Floppy Colon/Cecum
?"Floppy cecum" is a general term that refers to a cecum that is on a
redundant or long mesentery and therefore can move in the abdominal
cavity. Sometimes, the cecum is at an unusual location, whereas at
other times it is located in its regular location. The condition is
relatively common. Anecdotally, as many as 10% of people having a
barium enema may have a floppy cecum. There are no reported
consequences of having a floppy cecum.
This material is provided for general informational purposes only and
should not be considered a formal medical evaluation. If you have
specific questions, please contact your primary physician.?
http://www.medhelp.org/forums/gastro/archive/278.html


?There may be a relationship between redundant colon and rectal
prolapse, in which a portion of the rectum protrudes through the anus.
Although the protruding tissue can be easily mistaken for a prolapsed
hemorrhoid when it is palpated, a physician's examination will confirm
the correct diagnosis. Many patients with rectal prolapse also have
redundant colons. A common cause for both conditions could well be
chronic constipation. Other possibilities for rectal prolapse include
a weakening of the support tissues that hold the rectum in place,
which allows it to slide down and out through the anus. Whether such a
defect in the anatomy of the pelvic floor is at all related to
redundant colon is conjectural.?
http://www.intelihealth.com/IH/ihtIH/WSIHW000/8270/8438/371716.html



I hope this answer has helped you understand the possible link, or
not, of omphalocele and twisted colon. If any part of my answer is
unclear, please request an Answer Clarification, before rating. This
will allow me to assist  you further, on this question.

Sincerely, Crabcakes

Search Terms
=============
Colonic volvulus 
Omphalocele sequelae
Twisted colon + omphalocele
incidence + Colonic volvulus + after omphalocele
intestinal mesentery
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