J Ped Surg Case Reports 2 (2014) 12e14

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Journal of Pediatric Surgery CASE REPORTS

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Pyloric atresia in a healthy newborn e Two stage procedure

Sonal Nagra a, b, Jitoko K. Cama c, *
a
General Surgical Department, Dunedin Hospital, Southern District Health Board, Private Mail Bag 1921, Dunedin 9054, New Zealand
b
Apartment 4/44A Filleul Street, Dunedin Central, Dunedin 9016, New Zealand
c
Paediatric Surgery Department, Waikato Hospital, Elizabeth Rothwell Building, Pembroke Street, Private Bag 3200, Hamilton 3240, New Zealand

a r t i c l e i n f o a b s t r a c t

Article history: Pyloric obstruction (PO) in a newborn is a rare condition which occurs in about 1 in 100,000 neonates.
Received 10 October 2013 This is often associated with Epidermolysis bullosa and it often shows a familial pattern with an autosomal
Received in revised form recessive inheritance. A definite etiology of the condition has not been identified, however, the etiologies
20 November 2013
have been proposed such as genetic predisposition, familial inheritance and intrauterine incidents such
Accepted 20 November 2013
Available online 9 December 2013
as failure of canalization or a vascular event. Pyloric atresia (PA) is classified into 3 anatomic variations
which often warrant different surgical procedures. Excisions of the web, standard pyloroplasty, mucosal
advancement anastomosis or gastroduodenostomy have been recommended depending on the type
Key words:
Pyloric obstruction
of atresia. We present a rare case of PA from the Fiji Island in a healthy newborn baby whose
Epidermolysis bullosa surgical management was challenging as in most developing countries with limited resources. A trans-
Trans-anastomotic feeding tube anastomotic nasojejunal feeding tube through type A pyloric atresia in the absence of parental nutrition
can allow time for feeding prior to any definitive pyloroplasty.
Ó 2014 The Authors. Published by Elsevier Inc. Open access under CC BY-NC-SA license.

1. Case report
A term female newborn baby presented to the main referral
hospital in Suva, Fiji with non-bilious vomiting, failure to pass
meconium with an upper abdominal distension. Other systemic
examinations were grossly normal. Abdominal x-ray (Fig. 1) dem-
onstrates a dilated stomach with no distal bowel gas. An upper
gastrointestinal contract study (Fig. 2) demonstrates the pyloric
obstruction with a thickened and distended stomach and failure of
contrast to reach the first and second part of the duodenum. A
proximal duodenal obstruction was suspected initially since it was
more common and surgical repair was indicated.
At laparotomy, the stomach and the pylorus were much thick-
ened; with a very thin-walled duodenum. A longitudinal incision
over the pylorus confirmed a 0.5 cm thick pyloric web (type A
This paper was presented at the RACS Annual Congress in Kuala Lumpur e 6th
May 2012.
Abbreviations: PO, pyloric obstruction; PA, pyloric atresia; NJ, nasojejunal;
CWMH, Colonial War Memorial Hospital.
* Corresponding author. Tel.: þ64 7 83988716 (B), þ64 21408780 (M); fax: þ64 7
8398765.
E-mail addresses: Jitoko.Cama@waikatodhb.health.nz, jitokocama@yahoo.co.nz
(J.K. Cama).
pyloric atresia) which was then partially excised as shown in Fig. 3.
Having not had a similar case before, the pyloric web was only partly
excised to avoid going through the wall of the pylorus and duo-
denum which could potentially cause leakage. It was deemed unsafe
to do a HeinekeeMikulicz pyloroplasty in the first laparotomy as
that would mean mobilizing the duodenum and anastomosing the
very thin-walled first part of the duodenum to the thickened py-
lorus. The duodenum was so thin that the suturing needle could be
clearly seen through the wall of the duodenum probably due to the
lack of gastric juice peristalsis through the duodenum in utero.
However, we are not certain as to the cause. It was felt at the time of
surgery that kocherisation of the duodenum was unsafe. Having
confirmed the absence of a second atresia distally with saline
infusion, the pyloric web was incompletely excised to allow a trans-
anastomotic nasojejunal (NJ) feeding tube to be inserted through
and the longitudinal incision across the pylorus and duodenum was
then approximated primarily without any tension. A separate oro-
gastric tube was also inserted to decompress the stomach; she was
later commenced on NJ feeds on Day 2 post-operative which she
had tolerated quite well. Given the lack of total parental nutrition
(TPN), the lack of a proper softer NJ feeding tube and the thin du-
odenum, it was deemed safer to close the duodenum and the
thickened pylorus primarily without having to close the incision
transversely (pyloroplasty). The sutures would possibly have torn
through the duodenum when sutured to the pylorus and any
anastomotic leakage would have a detrimental outcome in Fiji due
to the resource stricken neonatal unit and no TPN.

2213-5766 Ó 2014 The Authors. Published by Elsevier Inc. Open access under CC BY-NC-SA license.
http://dx.doi.org/10.1016/j.epsc.2013.11.009
 S. Nagra, J.K. Cama / J Ped Surg Case Reports 2 (2014) 12e14 13

Fig. 1. Plain chest and abdominal x-rays showing the dilated stomach without any
bowel gas distally. Fig. 2. Upper gastrointestinal contrast study demonstrating the pyloric obstruction
with a thickened and distended stomach.

Three weeks later, after she had been tolerating the nasojejunal
feeding, we were hoping that the duodenum would be thicker. She
underwent a second laparotomy where the pylorus as expected
was stenosed but the first part of the duodenum had become quite
thick and safer to do a wide HeinekeeMikulicz pyloroplasty. Post-
operative recovery was uneventful and was commenced on
nasogastric feeds on day 2 and was later on full oral feeds by day 5
after the second laparotomy. Follow up at 6 months of age showed
she had remained asymptomatic and has been discharged without
any plan for a long term follow up.
intrauterine incidents such as failure of canalization or a vascular
event [3]. There is no sexual predilection but most PO can be
associated with low birth weight [4]. which was not seen in our
case.
Various classifications on the types of pyloric atresia have been
developed based on the 3 anatomic variations: Type A e pyloric

2. Discussion

Pyloric obstruction (PO) in a newborn is a rare congenital

problem, usually occurs as a solitary gastrointestinal lesion [1]. It
can however be associated with other atresias in esophagus, du-
odenum, jejunum, rectum and colon, or agenesis of the gall
bladder. Furthermore, Epidermolysis bullosa (EB), a rare skin dis-
ease is commonly associated with PO [1,2]. We reported a healthy
newborn baby with a solitary PO which was corrected surgically in
a two-stage procedure and avoiding a gastrostomy. This technique
is safe and can be done by general surgeons in developing coun-
tries, especially where the surgeons have not been exposed to this
condition or where parental nutrition and tertiary level neonatal
services is not readily available, such as Fiji.
Pyloric obstruction due to atresia shows a familial pattern with
an autosomal recessive inheritance [1]. The combination of Epi-
dermolysis bullosa (EB)/pyloric atresia (PA) is believed to be caused
by the pleiotropic expression of a single gene as there have
been no cases reported of a PA or EB occurring independently in
affected families [2]. A definite etiology of the PA has not been
identified, however literature suggests possibilities such as ge- Fig. 3. The right-sided debakey forceps demonstrates the 0.5cm thick pyloric web
netic predisposition, familial inheritance and potential causing the Type A pyloric atresia.
14 S. Nagra, J.K. Cama / J Ped Surg Case Reports 2 (2014) 12e14
membrane or web; Type B e pyloric channel is a solid cord; and
Type C e gap between the stomach and duodenum [4]. This case
report highlighted a type A PA which had occurred as a solitary
lesion in an otherwise healthy newborn.
Neonate with PA as previously reported are generally well and
would present with copious nonbilious frothy vomiting [5]. Often
due to the rarity of the disorder, the confirmation of the diagnosis
can be delayed. In this case, we had thought that this could be a
proximal duodenal atresia which are more common hence the
indication for surgery. Therefore, a high index of suspicion is
deemed necessary as delayed diagnosis may lead to pulmonary
aspiration, severe metabolic derangement, and gastric perforation,
which can be fatal [6]. One has to ensure that there is adequate
proximal decompression of the stomach with correction of the
fluid requirements and electrolytes prior to surgery.
The diagnosis of PO can now be detected on antenatal scan in
most developed countries. Sonographic features of polyhydramnios
with a dilated stomach but in the absence of a double bubble are
common features [7]. However, other causes of gastric distension
in-utero can give rise to similar ultrasound scan findings; therefore,
the newborn baby should have a plain abdominal x-ray which will
show a distended stomach bubble with paucity of gas distally. The
absence of double-bubble which is pathognomonic for duodenal
obstruction would often exclude that diagnosis.
Different types of pyloric atresia warrant different surgical pro-
cedures by pediatric surgeons. A type A defect will require an exci-
sion of the web, [8] whereas for a type B pyloric atresia, the standard
treatment is a Finney or HeinickeeMiculicz pyloroplasty. However, a
recent report by Dessanti et al., from Italy who have described a
much complex operation with a gastroduodenal mucosal advance-
ment anastomosis which can prevent reflux of bile [8], is something
that we would not recommend in all cases of PA. A type C lesion on
the other hand often requires a gastroduodenostomy [9], which can
be complicated later with peptic ulcers at the site of the anastomosis
or reflux of bile into the stomach. Formation of a gastrostomy in PO is
a controversial issue which is widely recommended in some centers
but we believe that it is not mandatory if one can achieve good
drainage and aspiration of the stomach with an orogastric tube [4].
General surgeons or pediatric surgeons in either developing or
developed countries should be aware that the duodenal wall could
be very thin as was seen in this case. We are not certain as to the
cause for this “thin” duodenum in a healthy term neonate. We had
assumed that this could be due to the lack of gastric juice or swal-
lowed liquor in-utero through the obstructed duodenum during
peritalsis. We have not found any literature available to explain this
finding.
General surgeons or young pediatric surgeons working in
developing countries who have not been exposed to this condition
can rely on this 2-stage procedure. With lack of parenteral support,
consumables, pediatric anesthetist and neonatal support, anasto-
motic leakage would be detrimental to these newborns. In this case,
given the thin duodenum, we believe that kockerization of the
duodenum should be avoided in the first laparotomy to prevent any
duodenal perforation if pyloroplasty is not feasible. A safer alter-
native would be to make a longitudinal incision over the pylorus
into the duodenum in a type A pyloric atresia with insertion of a
trans-anastomotic nasojejunal feeding tube to allow feeding,
especially when parental nutrition is not available. The stomach can
also be safely decompressed with a separate orogastric (OG) tube
rather than doing a gastrostomy to avoid its complications. This
technique we propose can be safely done by surgeons who might
encounter this condition, especially in the developing countries, if
the pyloroplasty is not feasible. With trans-anastomotic NJ feeding
and active peristalsis, a second stage laparotomy with pyloroplasty
after 2e3 weeks would be safer when the duodenal wall is thicker.
Some cases could even be considered for oral feeding if the trans-
anastomotic NJ feeding tube is removed but having pyloric stricture
in our opinion would require a re-laparatomy and pyloroplasty to
prevent further gastric outlet obstruction. As was noted in this case,
the site of the initial pyloric web was still quite stenosed and this
would have lead to ongoing obstruction. This two-stage technique
is safe and we recommend this in the type A PA but could even be
considered with the type B and Type C PA to avoid a gastrostomy or
a gastroduodenal anastomosis and its complications.
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