Congenital fistulisation of Meckelís diverticulum in omphalocele sac: case report
Mohammed Tazi Charki1,&, Hicham Abdellaoui1, Saad Andaloussi1, Mohammed Amine Oukhouya1, Abdelhalim Mahmoudi1, Aziz El madi1, Khalid Khattala1, Youssef Bouabdallah1
1Department of Pediatric Surgery, University Hospital of Hassan II,
University of Sidi Mohamed Ben Abdellah, Fez, Morocco
Mohammed Tazi Charki, Department of Pediatric Surgery, University Hospital of
Hassan II, University of Sidi Mohamed Ben Abdellah, Fez, Morocco
Fistulisation of Meckel's diverticulum in the top of an omphalocele sac is very rare. To our Knowledge, three cases were reported in the literature. We presente in this report a new case of this uncommon presentation.
Minor degrees of omphalocele have been known to be associated with
vitellointestinal duct (VID) anomalies. The most frequent association
is Meckel's diverticulum (MD) . But the fistulisation
of the diverticulum in the top of the omphalocele sac is very rare.
To our Knowledge, three cases were reported in the literature [2-4].
Herein, we report a new case of this uncommon presentation.
Patient and observation
A one-day old, full term male, was referred with a swelling at umbilical
region. Prenatal ultrasonography was not performed. Birth weight
was 3500g. In physical examination he had normal morphological
features. There was
an omphalocele making 6cm with a collar of 4cm, covered with a
thin, semi-transparent membrane with fistula draining meconium
1). The umbilical cord above the omphalocele sac measured approximately
2cm. The small bowel loops were visible through the thin membrane.
Rest of the neonatal examination was unremarkable. Echocardiography
After optimization of the general condition, surgery was performed.
After excision of the membrane, exploration found a fistulisation
of a Meckel's diverticulum (MD) adhered to the omphalocele sac.
The diverticulum size
was 2.5cm, located at the 4th intestinal loop before Bauhin
Valve (Figure 2, Figure
3). There were no meconium in peritoneal cavity. A resection of the
loop including the MD was performed with end-to-end anastomosis.
The post operative was simple; breastfeeding was started on the
after surgery and meconium was emitted by the anus. The newborn
was discharged without complication. Histopathology of the excised
specimen revealed wall
of small intestine without ectopic mucosa.
Omphalocele is a midline abdominal wall defect in which a thin membrane
surrounds the protruding organs that can include small intestine,
liver, bladder, spleen, stomach, uterus and ovaries .
It occurs in 1 in 4,000 to 6,000 live births  and
can be associated to others abnormalities in 50 to 75 % of cases,
including heart defects, cleft lip or palate, intestinal, vesical, genital,
malformations; and chromosomal anomaly [7, 8].
Small omphalocele size is associated with fewer cardiac anomalies
but with an increased prevalence of gastrointestinal anomalies
such as ileal or colonic
atresia and VID abnormalities . In fact, Minor degrees
of omphalocele have been known to be associated with VID abnormalities,
the most frequent is MD . In review of 49 omphalocele,
MD was found in 8 cases (16%), the incidence was higher in small
omphalocele (28%) than large one (4%) . Prenatal
discovery of an omphalocele associated with an inner umbilical
cord MD was described
in only one case . In our case, we described a fistulisation
of MD in the top of omphalocele sac. To our Knowledge, only 3 similar
cases were reported in the literature [2-4]. In all
cases, omphalocele was minor and resection of the loop including
MD with end-to-end
anastomosis was performed. Other cases of congenital intestinal
fistulisation in minor exomphalos were reported. In the series
of Ng J et
5 cases on 2006, there were two cases of a patent VID with a fistula,
one case of ileal prolapse and in two cases there was a fistulous
communication of the ileum directly with the exomphalos Sac .
In these two cases, the authors suggest a prolapsed of ileum through
a patent VID which subsequently underwent spontaneous regression.
In summary, if fistula is noted in an omphalocele sac, a co-existing patent VID should be considered. The treatment involves a resection of small bowel including the VID anomalies with end-to-end anastomosis.
The authors declare no competing interests.
All the authors have contributed to this article in ways that comply to ICMJE authorship criteria. All the authors have read and approved the final version of the manuscript.
Figure 1: omphalocele with fistula draining a greenish meconium (white arrow)
Figure 2: peroperative picture showing fistulisation of a Meckelís diverticulum adhered to the omphalocele sac. (white arrow)
Figure 3: peroperative picture showing Meckelís diverticulum located at the 4th intestinal loop before Bauhin valve (white arrow)
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