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Clinical case
Hypertrophic pyloric stenosis in a preterm infant: a case report
Estenosis hipertrófica de píloro en un prematuro: a propósito de un caso
L.P. Islas-Domíngueza,d,
Corresponding author
luneonatos@hotmail.com

Corresponding author at: Hospital General de México Dr. Eduardo Liceaga, Dr. Balmis 148, Colonia Doctores, Delegación Cuauhtémoc, C.P. 06720 Mexico City, Mexico.
, J.R. Jiménez-Jiménezb,d, J. Cruz-Díazc,d, K. Castellanos Reyesc,d
a Head of Paediatrics Department, Hospital General de México “Dr. Eduardo Liceaga”, Mexico City, Mexico
b Neonatologist, Neonatal Intensive Care, Hospital General de México “Dr. Eduardo Liceaga”, Mexico City, Mexico
c Registered Nurse, Hospital General de México, “Dr. Eduardo Liceaga”, Mexico City, Mexico
d Neonatologist, Instituto Mexicano del Seguro Social, Centro Médico Nacional la Raza, Mexico City, Mexico
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Hypertrophic pyloric stenosis &#40;HPS&#41; is a disease which occurs in the second week of life&#44; of unknown origin&#44; which consists of the narrowing of the pylorus due to concentric muscular hypertrophy&#44; causing gastric outlet obstruction with progressive vomiting that leads to malnutrition&#44; dehydration and serious metabolic disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Prompt diagnosis prevents complications&#44; reduces the morbidity rate and enables surgical treatment with an excellent prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The origin is unknown&#44; but the most accepted hypotheses suggest the use of concentrated baby formulas&#44; lack or reduction of pyloric muscle innervation&#44; elevation of gastrin and gastric somatostatin and even allergy&#46;<a class="elsevierStyleCrossRefs" href="#bib0355"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Repeated vomiting favours oedema of the pyloric mucous membrane&#44; which exacerbates the symptoms&#44; leading to loss of fluids&#44; hydrogen ions and chlorine&#44; all of which leads to hypochloraemic alkalosis&#46; The lack of actual ingestion leads to malnutrition and greater sensitivity to metabolic&#44; haemodynamic and infectious complications&#46;<a class="elsevierStyleCrossRefs" href="#bib0185"><span class="elsevierStyleSup">2&#44;5&#44;6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Some patients present jaundice derived from elevated indirect bilirubin&#44; from a not completely understood mechanism that reduces glucuronyl transferase and increases enterohepatic bilirubin circulation&#59; this is corrected when the patient undergoes surgery&#46;<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">6&#44;7</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The symptoms do not usually appear before the second or third week of life&#44; and earlier onset is exceptional&#46;<a class="elsevierStyleCrossRefs" href="#bib0215"><span class="elsevierStyleSup">8&#8211;11</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0035" class="elsevierStylePara elsevierViewall">A male newborn &#40;NB&#41;&#44; from the second pregnancy of a 27-year-old mother&#46; She denies drug addiction&#46; Three normal obstetric ultrasounds&#46; Discharge of clear transvaginal fluid for 30<span class="elsevierStyleHsp" style=""></span>h&#46; Baby born vaginally with Apgar 8-9&#44; Capurro &#8220;B&#8221; 35 weeks&#44; gestation&#44; weight 2550<span class="elsevierStyleHsp" style=""></span>g&#44; height 48<span class="elsevierStyleHsp" style=""></span>cm&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Management starts with fasting&#44; oxygen therapy&#44; ampicillin and amikacin for seven days due to premature membrane rupture&#46; Insidious respiratory and infectious evolution&#46; Sudden onset of postprandial non-biliary vomiting and increase of indirect bilirubin&#46; Given the possibility of nosocomial infection&#44; he is started on cefepime plus dicloxacillin&#46; X-ray of chest and abdomen shows gastromegaly &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Oral ingestion is resumed&#44; with enteral stimulation&#44; but vomiting persists so he continues to fast&#44; a central line is installed and parenteral nutrition is started&#46; Gastric hyperperistalsis and palpation of pyloric mass&#46; Blood gas shows normochloraemic metabolic alkalosis&#46; Ultrasound shows pylorus 18&#46;8<span class="elsevierStyleHsp" style=""></span>mm long&#44; with slices showing muscle wall thickness of up to 4&#46;3<span class="elsevierStyleHsp" style=""></span>mm &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; confirming the diagnosis of HPS&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall">Surgical finding&#58; 2<span class="elsevierStyleHsp" style=""></span>cm pylorus with pearly appearance&#46; Oral intake resumed 24<span class="elsevierStyleHsp" style=""></span>h after surgery&#44; well tolerated and accepting milk increments&#46; Antibiotics suspended as there is no evidence of infection&#46; Discharged home with follow-up as outpatient&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0050" class="elsevierStylePara elsevierViewall">Hypertrophic pyloric stenosis is a very rare condition in preterm infants&#46; The literature describes an incidence rate of 1 to 2&#46;9 cases per 1000 pre-term births&#46;<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">10&#44;13&#44;14</span></a> It is a very common cause of surgery in the first month of life&#46; Despite the radical change of this condition&#39;s presentation in the last 10 years&#44;<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">23</span></a> the rarity of its presentation in preterm infants is still emphasised&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">12</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">In full-term newborns it appears from the second to the seventh week after birth&#44; and its incidence rate is much higher than in pre-terms&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">10</span></a> This year&#44; Stark et al&#46; reported a series of 2466 newborns with HPS&#44; 208 &#40;8&#46;43&#37;&#41; of whom were premature&#44; compared with 2258 &#40;91&#46;57&#37;&#41; full-term babies with the problem&#44; showing how rare this condition is in babies with less than 37 weeks of gestation &#40;wog&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">10</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The literature describes factors associated to preterm newborns with HPS&#58; mothers with multiple pregnancies&#44; exposure to macrolides and male gender<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">10</span></a>&#59; however&#44; the greater the prematurity&#44; from 28 to 31 wog&#44; a ratio of 1&#58;1 F&#8211;M is reported&#46; This ratio increases to 2&#58;1 F&#8211;M from 32 to 36 wog&#44; and for full-term NBs&#44; the F&#8211;M ratio is 5&#58;1&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">12</span></a> Our patient was of the gender primarily reported in the literature&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Predominance of the male gender has a genetic component&#44; as the greater risk of gastrointestinal malformations in that gender is well documented&#44; as is the risk of rotavirus infections in childhood&#46; This suggests considerable differences in the development&#44; maturity and function of the gastrointestinal tract between men and women&#46;<a class="elsevierStyleCrossRefs" href="#bib0255"><span class="elsevierStyleSup">15&#8211;19</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Mutations in chromosomes 2q&#44; 3p&#44; 5q&#44; 7p&#44; 11q&#44; 16p and even in chromosome x play an important role in the development of HPS&#46;<a class="elsevierStyleCrossRefs" href="#bib0245"><span class="elsevierStyleSup">14&#44;17&#8211;21</span></a> However&#44; despite a large variety of studies&#44; it has not been possible to document a Mendelian inheritance pattern&#44; not even when present in monozygotic twins&#44; which supports the theory that environmental factors are fundamental in its presentation&#46;<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">22</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Other risk factors associated to HPS are&#58; caesarian birth and mothers smoking during pregnancy&#46;<a class="elsevierStyleCrossRefs" href="#bib0235"><span class="elsevierStyleSup">12&#44;13</span></a> However&#44; caesarian birth as a risk factor is not fully clear&#44; as in some series the birth method is consistent with maternal infection&#46; Vaginal birth has also been described as beneficial&#44; as it prevents hormonal cascades and stress factors in newborns&#46; Our patient was born vaginally and therefore not affected by such a risk factor&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">The association with smoking has also been attributed to tobacco substances that cause pyloric spasm and muscular hypertrophy&#46;<a class="elsevierStyleCrossRefs" href="#bib0235"><span class="elsevierStyleSup">12&#44;13</span></a> The mother of the NB in question denied any toxic habits&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Mothers under 20 years of age&#44; diabetic and with a low education level have also been associated to a greater risk of HPS&#44; as are women exposed to pesticides&#44; as they act as endocrine disruptors&#46;<a class="elsevierStyleCrossRefs" href="#bib0280"><span class="elsevierStyleSup">20&#44;23</span></a> None of these factors were present in this case&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">On the other hand&#44; prostaglandin infusion at standard doses has also been reported as a cause of the condition&#44; due to direct pharmacological effects on the pyloric muscle&#46;<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">24</span></a> This drug was not administered to our patient&#44; as he did not present a ductus arteriosus-dependent cardiopathy&#44; but it is important to consider this undesirable effect in patients who do&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">The clinical presentation often consists of vomiting that could initially be mistaken for reflux&#44; yet episodes often lead to metabolic alkalosis due to loss of hydrogen ions and chlorine&#46; This is consistent with our patient&#39;s symptoms&#46; This presentation in pre-term newborns is often diagnosed and treated as gastroesophageal reflux&#44; as premature babies have multiple risk factors such as the use of methyl-xanthines and immaturity of the upper oesophageal sphincter&#46; This makes it difficult to consider HPS as the primary origin of vomiting&#46;<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">25</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Our patient was initially treated with anti-reflux measures&#44; using widely recommended drugs at standard doses&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">25&#8211;27</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The patient was examined&#44; palpating a pyloric mass&#44; a pathognomic sign of the condition&#46;<a class="elsevierStyleCrossRef" href="#bib0365"><span class="elsevierStyleSup">28</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">Abdominal ultrasound is considered to be a highly sensitive &#40;91&#37;&#41; and specific &#40;100&#37;&#41; test&#44; which also makes it possible to distinguish duodenal membranes or other malformations as differential diagnoses&#46;<a class="elsevierStyleCrossRefs" href="#bib0365"><span class="elsevierStyleSup">28&#44;29</span></a> In our patient&#44; the ultrasound showed the findings described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#44; which are consistent with the literature&#46;<a class="elsevierStyleCrossRefs" href="#bib0365"><span class="elsevierStyleSup">28&#44;29</span></a> Three factors have been suggested for the condition&#39;s diagnosis&#58; the presence of delayed gastric output by ultrasound&#44; pyloric sphincter muscle thickness of &#8805;2&#46;5<span class="elsevierStyleHsp" style=""></span>mm and muscle length &#8805;14<span class="elsevierStyleHsp" style=""></span>mm&#46;<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">30</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0115" class="elsevierStylePara elsevierViewall">Other diagnostic tests mentioned in the literature are a gastroduodenal oesophageal series and endoscopy&#44; which can also have therapeutic purposes&#46;<a class="elsevierStyleCrossRefs" href="#bib0365"><span class="elsevierStyleSup">28&#44;30&#8211;32</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">Laparoscopic&#44; or minimally invasive&#44; surgery is increasingly recommended in the literature&#44; but requires appropriate medical instruments and equipment to tackle the physiology of pre-term infants and their reduced surgical fields&#44;<a class="elsevierStyleCrossRefs" href="#bib0335"><span class="elsevierStyleSup">30&#8211;33</span></a> with small incisions through the umbilical scar and excellent results&#44; starting food intake the following day&#46; However&#44; our hospital does not yet have the equipment required for this procedure in pre-term infants&#46; Even so&#44; the results were excellent in our patient&#44; operated on with a conventional technique&#44; enabling him to feed after 24<span class="elsevierStyleHsp" style=""></span>h&#44; with no complications and outpatient follow-up&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0125" class="elsevierStylePara elsevierViewall">The objective of presenting this case report is to consider HPS as a possible diagnosis in pre-term NB patients with food intolerance symptoms&#44;<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">10&#8211;13&#44;25&#44;29</span></a> even when gestational age and days of onset are not commonly reported in the literature&#44; as presentation has radically changed in the last 10 years and even male predominance is not common in such patients&#46; This could prevent said diagnosis&#44; delaying treatment&#44; increasing the duration of hospitalisation and inherent complications&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">It is therefore important to broaden our diagnostic approach and consider this condition in our patients&#44; as the tendency towards preterm birth in intensive or intermediate care units&#44; mothers aged 20 years or less&#44; with low educational levels&#44; smoking&#44; and the use of macrolides and prostaglandins for commonly associated conditions are increasingly common in our hospital population&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0140" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work centre on the publication of patient data&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0145" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflict of interest</span><p id="par0150" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interests&#46;</p></span></span>"
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                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">a&#41; Pyloric diameter from 15 to 18<span class="elsevierStyleHsp" style=""></span>mm&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">b&#41; Muscle thickness from 3 to 4<span class="elsevierStyleHsp" style=""></span>mm and length more than 17<span class="elsevierStyleHsp" style=""></span>mm&nbsp;\t\t\t\t\t\t\n
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Article information
ISSN: 01851063
Original language: English
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