Based on evidence from two collected and treated clinical observations of hypertrophic pyloric stenosis in children of 5 and 12 months of age, the authors give their point of view on the unresolved issue of the etiology of hypertrophic pyloric stenosis. They emphasize that there are more and more factors to prove this is an acquired condition

Faustin Mouafo Tambo

Gervais Andze Ondobo

Irène Kouna Tsala

Marcelin Ngowe Ngowe

Maurice Sosso

SpringerPlus

PubMed

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a SpringerOpen Journal
Mouafo Tambo et al. SpringerPlus 2014, 3:555
http://www.springerplus.com/content/3/1/555CASE STUDY Open AccessHypertrophic pyloric stenosis in infants: is it a
congenital or acquired disorder? Reflections on 2
cases
Faustin Félicien Mouafo Tambo*, Irène Nadine Kouna Tsala, Marcelin Ngowe Ngowe, Gervais Andze Ondobo
and Maurice Aurelien SossoAbstract
Based on evidence from two collected and treated clinical observations of hypertrophic pyloric stenosis in children
of 5 and 12 months of age, the authors give their point of view on the unresolved issue of the etiology of hypertrophic
pyloric stenosis. They emphasize that there are more and more factors to prove this is an acquired condition.
Keywords: Hypertrophic pyloric stenosis; EtiologyRésumé
À partir de 2 observations cliniques colligées et traitées de sténose hypertrophique du pylore chez les nourrissons
de 5 mois et 12 mois, les auteurs font part de leurs réflexions sur la question non encore résolue de l’étiologie de la
sténose hypertrophique du pylore. Ils soulignent l’existence de plus en plus de facteurs en faveur d’une affection
acquise.
Mots clés: Sténose hypertrophique du pylore; EtiologieIntroduction
Hypertrophic pyloric stenosis (HPS) is defined as an
hyperplasia of smooth muscle fibres of the pylorus that
is responsible for the narrowing of the pyloric canal and
obstruction of gastric emptying (Roquelaure and Sarles
2000; Li 2008). Its incidence is estimated at 1.5-3 per
1000 live births and the age of discovery is typically be-
tween the 3rd and 4th week of life (Mahalik et al. 2010;
Schwartz 2006). This condition is known to be rare in
Africa, and the cases of late discovery are even rarer in
this context because of the early involvement of life
prognosis, in the absence of resuscitation. Thus, the late
observation of 2 cases of hypertrophic pyloric stenosis in
infants of 5 months and 1 year in the paediatric surgery
department of the Obstetrics, Gynaecology and Paediat-
ric Hospital of Yaoundé (HGOPY) has prompted us to
reopen the debate on the etiology of hypertrophic* Correspondence: faustintambo@yahoo.fr
Obstetrics, Gynaecology and Paediatric Hospital, Yaoundé, Cameroon
© 2014 Mouafo Tambo et al.; licensee Springer
Commons Attribution License (http://creativeco
reproduction in any medium, provided the origpyloric stenosis which is still mysterious, quite known.
The aim of this study was to emphasize that there is
more and more evidence that prove that this is an ac-
quired condition whose mechanism is different from
that of a developmental abnormality.Observation N° 1
K.J, a 5-month old male infant and first born in a family
of one was admitted on 28.09. 06 for early non bilious
vomiting that had been lasting for 5 days. Upon interro-
gation, it appeared there had been no ingestion of any
caustic products, no gastro oesophageal reflux disease,
and no previous case of hypertrophic pyloric stenosis
was reported in the family. Physical examination per-
formed on this infant whose general state was preserved,
who had stained conjunctiva, was afebrile at 37° and
weighed 5 Kgs led to the discovery of peristaltic waves
spontaneously moving from left to right and, most im-
portantly, of a firm and moving oblong mass under the
right costal margin which was strongly suggestive of a. This is an Open Access article distributed under the terms of the Creative
mmons.org/licenses/by/2.0), which permits unrestricted use, distribution, and
inal work is properly credited.
Mouafo Tambo et al. SpringerPlus 2014, 3:555 Page 2 of 3
http://www.springerplus.com/content/3/1/555pyloric olive. Abdominal sonography made after those
clinical observations highlighted a hypertrophic pylorus
with a 5.7 mm-thick wall associated with gastric stasis.
Therefore, surgery indication was made after a complete
pre surgery assessment, a short hydro electrolytic rebal-
ancing through a periumbilical approach and, an extra
mucosal Fredet-Ramstedt pyloromyotomy was carried
on 12.10.11 with no complications followed by a re-
sumption of feeding at H12. Five years later, no vomiting
has been reported, the weight curve of the child is nor-
mal and he tolerates a regular diet.
Observation N° 2
M.K., a 1-year old female infant was admitted in November
2008 for non bilious projectile vomiting that had been
lasting for two weeks. The anamnesis did not reveal any
abdominal distension, weight loss or previous medica-
tion. The medical history Chapter was not contributory.
The clinical examination performed on a dehydrated
infant revealed an epigastric arch. The pyloric olive was
not palpated. Abdominal sonography in this context
revealed a 25 mm-long and 10 mm-thick pyloric olive.
After resuscitation, the surgical indication was made
and an extra mucosal Fredet-Ramstedt pyloromyotomy
was carried out by periumbilical route. The immediate
post-surgery period was uneventful and there was a re-
sumption of feeding at H10. Three years later, no vomit-
ing has been reported and M.K. has a normal weight
gain.
Discussion
The condition described in 1887 by Hirschsprung has
been subjected to many pathogenic hypotheses, none of
which has been validated to date. Following the analysis
of the two presented clinical observations, the genetic
theory seems unlikely to explain the occurrence of
hypertrophic pyloric stenosis because no family case of
hypertrophic pyloric stenosis was reported in both cases.
That the patient in Observation No. 2 is a female is con-
trary to the literature data (Le Dosseur et al. 2009) which
posit that the disease is 3–4 times more common in
boys than in girls. Conversely the first born in a family
of one in Observation N°1 seems to have a multi gene
predisposition, one of the major genes being linked to
chromosome X, and is in the prevalence of the disease
in firstborn boys although that may be due to chance,
given the small size of our sample. The environmental
theory that tends to connect the occurrence of HPS to
stressful events during pregnancy has not been estab-
lished in our two cases. The neurogenic theory that
maintains that pyloric hypertrophy is the consequence
of an antropyloric dyssynergia which itself is a conse-
quence of the degeneration of the innervation did not
explain convincingly the occurrence of HPS in our 2cases. As a matter of fact, this theory cannot explain the
absence of obstructive events in degenerative diseases
like Chagas disease; another of its shortcomings is the
fact that no animal testing could reproduce the disease
by creating a barrier or hormonal destruction (Le Dosseur
et al. 2009). Late diagnosis, a common practice in our
under medicalised countries, is an argument in favor of a
congenital origin of the condition in two observed cases.
Indeed, the operator dependent nature of ultrasound, its
unavailability in emergency units and the inadequacy of
technical facilities in most developing countries combine
with the low socio-economic status of populations to ex-
plain the delay in the diagnosis of the condition in the two
reported cases. But more than anything else, it is the in-
complete and functional stenosis of the pyloric canal
which justifies, in our opinion, the late occurrence in both
reported cases. The unicist theory that relates infant HPS
to stenosing pyloric hypertrophy in adults was hard to
prove valid in the absence of esophageal pH monitoring
and upper gastro intestinal endoscopy (UGIE) to formally
eliminate the possible association between HPS, gastro
oesophageal reflux desease (GERD), peptic esophagitis
and ulcer. Similarly, hormonal theory that tends to explain
the occurrence of HPS by the hypergastrinemia in the
newborn has not been verified in our work, due to the
lack of laboratory equipped for such an assay.
According to (Mahalik et al. 2010), hypertrophic pyl-
oric stenosis is an acquired condition. Indeed, pyloric
stenosis is characterized by a hypertrophy of circular fi-
bers that would result in the narrowing and obstruction
of the pyloric canal due to the compression of the longi-
tudinal fibers of the mucosa. This acquired origin of
HPS had been identified and discussed since 2000 by
Paulozzi (Paulozzi 2000) who argued that HPS result
from a Helicobacter pylori infection.
That same year, (Hoey 2000) showed that the risk of
HPS is increased by a factor 8 for children who were ad-
ministered erythromycin between 3–13 days after birth.
The other antibiotics showed no risks of HPS. Similarly,
in 2002, (Sorensen et al. 2002) showed over a period of
10 years that the risk of the HPS was multiplied by 2 for
infants whose mother is a smoker.
Conclusion
No theory appears to explain all alone the Hypertrophic
Pyloric and its quite stereotypical period of occurrence. Its
etiology is probably multifactorial as the pylorus seems
sensitive to attack at certain times and in certain subjects.
Competing interests
The authors declare that they have no competing interests.
Authors’ contribution
FFMT: draft and revised; IKT: draft; MNN: revised; GAO: revised; AMS: revised.
All authors read and approved the final manuscript.
Mouafo Tambo et al. SpringerPlus 2014, 3:555 Page 3 of 3
http://www.springerplus.com/content/3/1/555Received: 18 November 2013 Accepted: 15 September 2014
Published: 24 September 2014
References
Hoey J (2000) Hypertrophic pyloric stenosis caused by erythromycin. CMA J 162
(8):1198
Le Dosseur P, Eurin D, Cellier C, Brasseur Doudry M (2009) Sténose
hypertrophique du pylore du nourrisson. J Ped Puer 22:315–325
Li BUK (2008) Hypertrophic pyloric stenosis. In: Kleinman R, Goulet OJ, Mieli-
Vergani G, Sanderson IR, Sherman P, Shneider BL (eds), 5th edn, Walkers
pediatric gastro intestinal disease. BC DECKER Inc, pp 134–135
Mahalik S, Prosad A, Sinha A, Kulshrestha R (2010) Delayed presentation of
hypertrophic pyloric stenosis: a rare case. J Ped Surg 45:E9–E11
Paulozzi LJ (2000) IS helicobacter pylori a cause of infantile hypertrophic pyloric
stenosis. Med Hypotheses 55(5):119–125
Roquelaure B, Sarles J (2000) vomissements du nourrisson. Orientation
diagnostique. Rev Prat 49:226
Schwartz MZ (2006) Hypertrophic pyloric stenosis. In: Grosfeld JL, O’Neill JA,
Fonkalsrud E (eds) Pediatric Surgery, 6th edn. Mosby – Elsevier, Philadelphia,
pp 1215–1223
Sorensen H, Norgard B, Pedersen L, Larsen H, Johnsen SP (2002) Maternal
smoking and risk of hypertrophic pyloric stenosis: 10 year population based
cohort study. BMJ 325(7371):1011–1012
doi:10.1186/2193-1801-3-555
Cite this article as: Mouafo Tambo et al.: Hypertrophic pyloric stenosis in
infants: is it a congenital or acquired disorder? Reflections on 2 cases.
SpringerPlus 2014 3:555.Submit your manuscript to a 
journal and beneﬁ t from:
7 Convenient online submission
7 Rigorous peer review
7 Immediate publication on acceptance
7 Open access: articles freely available online
7 High visibility within the ﬁ eld
7 Retaining the copyright to your article
    Submit your next manuscript at 7 springeropen.com


Hypertrophic pyloric stenosis in infants: is it a congenital or acquired disorder? Reflections on 2 cases

Faustin Félicien Mouafo Tambo

Irène Nadine Kouna Tsala

Maurice Aurelien Sosso

Crossref

a SpringerOpen JournalMouafo Tambo et al. SpringerPlus 2014, 3:555http://www.springerplus.com/content/3/1/555CASE STUDY Open AccessHypertrophic pyloric stenosis in infants: is it acongenital or acquired disorder? Reflections on 2casesFaustin Félicien Mouafo Tambo*, Irène Nadine Kouna Tsala, Marcelin Ngowe Ngowe, Gervais Andze Ondoboand Maurice Aurelien SossoAbstractBased on evidence from two collected and treated clinical observations of hypertrophic pyloric stenosis in childrenof 5 and 12 months of age, the authors give their point of view on the unresolved issue of the etiology of hypertrophicpyloric stenosis. They emphasize that there are more and more factors to prove this is an acquired condition.Keywords: Hypertrophic pyloric stenosis; EtiologyRésuméÀ partir de 2 observations cliniques colligées et traitées de sténose hypertrophique du pylore chez les nourrissonsde 5 mois et 12 mois, les auteurs font part de leurs réflexions sur la question non encore résolue de l’étiologie de lasténose hypertrophique du pylore. Ils soulignent l’existence de plus en plus de facteurs en faveur d’une affectionacquise.Mots clés: Sténose hypertrophique du pylore; EtiologieIntroductionHypertrophic pyloric stenosis (HPS) is defined as anhyperplasia of smooth muscle fibres of the pylorus thatis responsible for the narrowing of the pyloric canal andobstruction of gastric emptying (Roquelaure and Sarles2000; Li 2008). Its incidence is estimated at 1.5-3 per1000 live births and the age of discovery is typically be-tween the 3rd and 4th week of life (Mahalik et al. 2010;Schwartz 2006). This condition is known to be rare inAfrica, and the cases of late discovery are even rarer inthis context because of the early involvement of lifeprognosis, in the absence of resuscitation. Thus, the lateobservation of 2 cases of hypertrophic pyloric stenosis ininfants of 5 months and 1 year in the paediatric surgerydepartment of the Obstetrics, Gynaecology and Paediat-ric Hospital of Yaoundé (HGOPY) has prompted us toreopen the debate on the etiology of hypertrophic* Correspondence: faustintambo@yahoo.frObstetrics, Gynaecology and Paediatric Hospital, Yaoundé, Cameroon© 2014 Mouafo Tambo et al.; licensee SpringerCommons Attribution License (http://creativecoreproduction in any medium, provided the origpyloric stenosis which is still mysterious, quite known.The aim of this study was to emphasize that there ismore and more evidence that prove that this is an ac-quired condition whose mechanism is different fromthat of a developmental abnormality.Observation N° 1K.J, a 5-month old male infant and first born in a familyof one was admitted on 28.09. 06 for early non biliousvomiting that had been lasting for 5 days. Upon interro-gation, it appeared there had been no ingestion of anycaustic products, no gastro oesophageal reflux disease,and no previous case of hypertrophic pyloric stenosiswas reported in the family. Physical examination per-formed on this infant whose general state was preserved,who had stained conjunctiva, was afebrile at 37° andweighed 5 Kgs led to the discovery of peristaltic wavesspontaneously moving from left to right and, most im-portantly, of a firm and moving oblong mass under theright costal margin which was strongly suggestive of a. This is an Open Access article distributed under the terms of the Creativemmons.org/licenses/by/2.0), which permits unrestricted use, distribution, andinal work is properly credited.Mouafo Tambo et al. SpringerPlus 2014, 3:555 Page 2 of 3http://www.springerplus.com/content/3/1/555pyloric olive. Abdominal sonography made after thoseclinical observations highlighted a hypertrophic pyloruswith a 5.7 mm-thick wall associated with gastric stasis.Therefore, surgery indication was made after a completepre surgery assessment, a short hydro electrolytic rebal-ancing through a periumbilical approach and, an extramucosal Fredet-Ramstedt pyloromyotomy was carriedon 12.10.11 with no complications followed by a re-sumption of feeding at H12. Five years later, no vomitinghas been reported, the weight curve of the child is nor-mal and he tolerates a regular diet.Observation N° 2M.K., a 1-year old female infant was admitted in November2008 for non bilious projectile vomiting that had beenlasting for two weeks. The anamnesis did not reveal anyabdominal distension, weight loss or previous medica-tion. The medical history Chapter was not contributory.The clinical examination performed on a dehydratedinfant revealed an epigastric arch. The pyloric olive wasnot palpated. Abdominal sonography in this contextrevealed a 25 mm-long and 10 mm-thick pyloric olive.After resuscitation, the surgical indication was madeand an extra mucosal Fredet-Ramstedt pyloromyotomywas carried out by periumbilical route. The immediatepost-surgery period was uneventful and there was a re-sumption of feeding at H10. Three years later, no vomit-ing has been reported and M.K. has a normal weightgain.DiscussionThe condition described in 1887 by Hirschsprung hasbeen subjected to many pathogenic hypotheses, none ofwhich has been validated to date. Following the analysisof the two presented clinical observations, the genetictheory seems unlikely to explain the occurrence ofhypertrophic pyloric stenosis because no family case ofhypertrophic pyloric stenosis was reported in both cases.That the patient in Observation No. 2 is a female is con-trary to the literature data (Le Dosseur et al. 2009) whichposit that the disease is 3–4 times more common inboys than in girls. Conversely the first born in a familyof one in Observation N°1 seems to have a multi genepredisposition, one of the major genes being linked tochromosome X, and is in the prevalence of the diseasein firstborn boys although that may be due to chance,given the small size of our sample. The environmentaltheory that tends to connect the occurrence of HPS tostressful events during pregnancy has not been estab-lished in our two cases. The neurogenic theory thatmaintains that pyloric hypertrophy is the consequenceof an antropyloric dyssynergia which itself is a conse-quence of the degeneration of the innervation did notexplain convincingly the occurrence of HPS in our 2cases. As a matter of fact, this theory cannot explain theabsence of obstructive events in degenerative diseaseslike Chagas disease; another of its shortcomings is thefact that no animal testing could reproduce the diseaseby creating a barrier or hormonal destruction (Le Dosseuret al. 2009). Late diagnosis, a common practice in ourunder medicalised countries, is an argument in favor of acongenital origin of the condition in two observed cases.Indeed, the operator dependent nature of ultrasound, itsunavailability in emergency units and the inadequacy oftechnical facilities in most developing countries combinewith the low socio-economic status of populations to ex-plain the delay in the diagnosis of the condition in the tworeported cases. But more than anything else, it is the in-complete and functional stenosis of the pyloric canalwhich justifies, in our opinion, the late occurrence in bothreported cases. The unicist theory that relates infant HPSto stenosing pyloric hypertrophy in adults was hard toprove valid in the absence of esophageal pH monitoringand upper gastro intestinal endoscopy (UGIE) to formallyeliminate the possible association between HPS, gastrooesophageal reflux desease (GERD), peptic esophagitisand ulcer. Similarly, hormonal theory that tends to explainthe occurrence of HPS by the hypergastrinemia in thenewborn has not been verified in our work, due to thelack of laboratory equipped for such an assay.According to (Mahalik et al. 2010), hypertrophic pyl-oric stenosis is an acquired condition. Indeed, pyloricstenosis is characterized by a hypertrophy of circular fi-bers that would result in the narrowing and obstructionof the pyloric canal due to the compression of the longi-tudinal fibers of the mucosa. This acquired origin ofHPS had been identified and discussed since 2000 byPaulozzi (Paulozzi 2000) who argued that HPS resultfrom a Helicobacter pylori infection.That same year, (Hoey 2000) showed that the risk ofHPS is increased by a factor 8 for children who were ad-ministered erythromycin between 3–13 days after birth.The other antibiotics showed no risks of HPS. Similarly,in 2002, (Sorensen et al. 2002) showed over a period of10 years that the risk of the HPS was multiplied by 2 forinfants whose mother is a smoker.ConclusionNo theory appears to explain all alone the HypertrophicPyloric and its quite stereotypical period of occurrence. Itsetiology is probably multifactorial as the pylorus seemssensitive to attack at certain times and in certain subjects.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionFFMT: draft and revised; IKT: draft; MNN: revised; GAO: revised; AMS: revised.All authors read and approved the final manuscript.Mouafo Tambo et al. SpringerPlus 2014, 3:555 Page 3 of 3http://www.springerplus.com/content/3/1/555Received: 18 November 2013 Accepted: 15 September 2014Published: 24 September 2014ReferencesHoey J (2000) Hypertrophic pyloric stenosis caused by erythromycin. CMA J 162(8):1198Le Dosseur P, Eurin D, Cellier C, Brasseur Doudry M (2009) Sténosehypertrophique du pylore du nourrisson. J Ped Puer 22:315–325Li BUK (2008) Hypertrophic pyloric stenosis. In: Kleinman R, Goulet OJ, Mieli-Vergani G, Sanderson IR, Sherman P, Shneider BL (eds), 5th edn, Walkerspediatric gastro intestinal disease. BC DECKER Inc, pp 134–135Mahalik S, Prosad A, Sinha A, Kulshrestha R (2010) Delayed presentation ofhypertrophic pyloric stenosis: a rare case. J Ped Surg 45:E9–E11Paulozzi LJ (2000) IS helicobacter pylori a cause of infantile hypertrophic pyloricstenosis. Med Hypotheses 55(5):119–125Roquelaure B, Sarles J (2000) vomissements du nourrisson. Orientationdiagnostique. Rev Prat 49:226Schwartz MZ (2006) Hypertrophic pyloric stenosis. In: Grosfeld JL, O’Neill JA,Fonkalsrud E (eds) Pediatric Surgery, 6th edn. Mosby – Elsevier, Philadelphia,pp 1215–1223Sorensen H, Norgard B, Pedersen L, Larsen H, Johnsen SP (2002) Maternalsmoking and risk of hypertrophic pyloric stenosis: 10 year population basedcohort study. BMJ 325(7371):1011–1012doi:10.1186/2193-1801-3-555Cite this article as: Mouafo Tambo et al.: Hypertrophic pyloric stenosis ininfants: is it a congenital or acquired disorder? Reflections on 2 cases.SpringerPlus 2014 3:555.Submit your manuscript to a journal and beneﬁ t from:7 Convenient online submission7 Rigorous peer review7 Immediate publication on acceptance7 Open access: articles freely available online7 High visibility within the ﬁ eld7 Retaining the copyright to your article    Submit your next manuscript at 7 springeropen.com

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Hypertrophic pyloric stenosis in infants: is it a congenital or acquired disorder? Reflections on 2 cases

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