Keywords
esophageal atresia - long gap - magnamosis
Introduction
Esophageal atresia (EA) Gross type A (long gap without tracheoesophageal fistula)
is a rare and surgical challenging variation in EA that constitutes ∼7%[1] of the children born with EA. A primary esophagoesophagostomy will almost always
be impossible, and several surgical techniques to establish the continuity of the
gut have been developed with small or large intestinal interposition, gastric tube
or pull-up, and active elongation of the pouches as the most common.[2] All procedures carry an inherent high risk of postoperative complications and long-term
functional problems. The incidence of subclinical musculoskeletal deformities after
EA repair with muscle-sparing thoracotomy has recently been reported as high as 25%.[3] We present a case with successful delayed esophagoesophagostomy obtained by magnetic
compression of a long-gap EA type A without thoracotomy.
Case Report
The boy was delivered in gestational week 32 + 5 by emergency cesarean section due
to leaking amniotic fluid. Birth weight was 1920 g, and Apgar score at 1, 5, and 10
minutes was 8, 5, and 10, respectively. Prenatal ultrasound screening had given the
suspicion of EA due to absence of a gastric bubble and polyhydramnios. The diagnosis
was confirmed at birth by failure to place a gastric tube and plain abdominal X-ray
showed no visible gas in the stomach or intestine compatible with type A EA based
on Gross classification.[4] No other congenital malformation was found at general physical examination, echocardiography,
or ultrasound of the cerebrum.
One-day old a Ch 14 gastrostomy tube (Flocare, Nutricia, Denmark) was placed by laparotomy
and the distance between the upper and lower pouch was measured by the Hegar method
and was found to be equivalent to the height of 3.5 thoracic vertebrae ([Fig. 1]). Postoperative permanent suction of the upper pouch was instituted and spontaneous
growth of the lower and upper pouch was awaited. Full enteral nutrition via the gastrostomy
was launched as boluses. The parents gently pushed on the esophageal tube daily to
stimulate elongation and growth of the upper esophageal pouch.
Fig. 1 Anteroposterior X-ray demonstrating measurement of the esophageal gap on day 1 after
birth.
At the age of 54 days, bodyweight was 3,100 g and the distance between the upper and
lower pouch was measured to be less than 5 mm. After several information sessions
with the parents, it was decided in agreement to try to approximate the two pouches
by magnet force as previously reported. The parents were informed about the possibilities
for a surgical approximation with the pros and cons including the risk of stenosis
in relation to the magnetic method.[5]
[6] Two identical cylindrical magnets with a diameter of 5 mm and a strength of 12,000
G (Hindsbo Magneter ApS, Roskilde, Denmark) were mounted and secured in the tip of
two separate gastric tubes CH 18 (Biofarma Logistik A/S, Glostrup, Denmark). The magnets
were specially designed for the purpose with a power similar to other reports. The
magnets were sterilized prior to use. Ethical approval was not necessary according
to Danish law. At the age of 62 days, each magnet was placed intraluminal at the tip
of each pouch under fluoroscopic guidance ([Fig. 2]), the lower tube via the gastrostomy tube and the upper peroral. Contact and alignment
between the magnets was achieved after 15 minutes. Enteral nutrition was continued
via the lower pouch tube where a side hole was created corresponding to the part located
in the stomach. A temporary increase in leucocyte count in peripheral blood to a maximum
of 19.8 109/L (reference values 6.0–13.3 × 109/L) and C-reactive protein to 106 mg/L (reference value < 10 mg/L) was observed and
normalized within 3 days. No antibiotics were administered. On postoperative day 5,
the tubes including the magnets were removed and replaced with a nasogastric tube
without any problems. Enteral nutrition was changed to the nasogastric tube and the
gastrostomy tube was removed. Peroral nutrition was tolerated on day 10. On day 16,
the boy underwent esophagoscopy due to dysphagia. A stenosis was found, which was
dilated (5 mm balloon). Early and frequent dilations at any symptoms on stenosis were
decided to prevent fibrous stenosis that might become refractory to dilatation with
the need of stenting or surgery. Thus, during the first 3 months, 12 endoscopic dilations
where performed. During the next 9 months, additional five endoscopic dilations were
performed. The child is now 15 months old and we have not seen him for dilation for
3 months. Otherwise normal psychomotor development and normal height and weight gain
according to standard curve for Danish children were observed.
Fig. 2 (A) A 5 mm cylindrical magnet (12,000 G) was placed in the upper pouch and lower pouch
under fluoroscopic guidance at the age of 62 days. (B) Immediate contact and alignment between the magnets was observed.
Discussion
This is the seventh report case of using magnamosis in the repair of a patient with
type A EA without any other surgical interventions than the placement of a gastrostomy
tube. In the previous cases, the magnets and tube system from Cook Medical have been
used.[7] The equipment used in the current case is noncommercial. The principles of the two
systems are almost equal with some minor difference. Our system did not include the
ability for suction of saliva from the upper pouch and the shape of the magnets used
in the previous cases was bullet-shaped opposite cylindrical shaped magnets in the
present case. The strength of the magnets was equal around 12,000 G.
In the present case, an elongation of the pouches occurred within 2 months to obtain
a distance of 5 mm, which we considered sufficient for applying the method. To promote
growth, it is our practice that parents are instructed to gently push the tube in
the upper pouch daily, which in theory may facilitate growth. There is no evidence
of how short the distance between the pouches must be, but magnamosis has been suggested
in gaps up to 3 cm.[6] The presented equipment in its present form is not considered suitable for such
large gaps due to the lack of control over the magnetic contraction and thereby the
risk of perforation in one or both pouch.
Magnetic contact occurred almost immediately and the anastomosis was achieved within
5th postoperative day in the present cases, which is comparable to the previously
reported cases where the average was 4.2 days (range 3–6 days).[6]
One of the advantages for nonsurgical magnamosis is the avoidance of the extensive
mobilization and dissection of the pouches with the risk of tracheal injury, devascularization,
denervation of the esophagus, and the long-term consequences of thoracotomy.[5] A disadvantage with the method might be high rate of early anastomotic stenosis,
as seen in the present and previously published cases, which requires early and frequent
dilation. Some strictures are persistent and require stenting or surgical reconstruction.[7] Another disadvantage is the waiting period of 2 to 3 months for the natural growth
of the esophageal pouches[8] that may require long hospital stay and the constant risk of aspiration pneumonia.
These factors must be balanced against the disadvantages of esophageal replacement
that seems to have more long-term complications compared with delayed primary anastomosis
when considering the method.[9]
[10] In the presented case, a thoracoscopic repair with its advantages in terms of avoiding
musculoskeletal morbidity could have been another solution due to the short distance
of 5 mm after the waiting period. This method might have reduced the number of dilatations.
Almost only positive results have been reported regarding the use of magnamosis and
one could suspect some publication bias and thus lack of reporting postoperative complications
to the method.
For the magnamosis principle, further design refinements are necessary especially
to reduce the rate of postanastomotic stenosis. The shape and size compared with the
esophageal diameter and strength of magnets may be important. The method could be
a promising modality in selected cases with a long-gap EA and were a significant spontaneous
growth and elongation of the pouches occurs within a few weeks postnatal. Further
investigation and refinement of the method is required before the method can be recommended
in general.
