Infantile Hypertrophic Pyloric Stenosis, the Cause of Non-bilious Vomiting of a 3-day-old Male Infant with Situs Inversus Totalis; A Case Report

Infantile Hypertrophic Pyloric Stenosis, the Cause of Non-bilious Vomiting of a 3-day-old Male Infant with Situs Inversus Totalis; A Case Report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Infantile Hypertrophic Pyloric Stenosis, the Cause of Non-bilious Vomiting of a 3-day-old Male Infant with Situs Inversus Totalis; A Case Report Ali Samady Khanghah, Khashayar Atqiaee, Ali Tabatabaey This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-1353951/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background: The Synchronicity of Situs Inversus totalis (SIT) and infantile hypertrophic pyloric stenosis (IHPS) is a rare phenomenon with only 2 reported cases in the literature. SIT is a mirror transposition of the major visceral organs, arranged along a left-right axis. Its prevalence is 0.1—0.6 per 10,000 live births. Rarely it is accompanied by neonatal intestinal obstruction, congenital heart defects, primary ciliary dyskinesia, renal disorders, biliary atresia, skeletal dysplasia, and mental retardation. IHPS is muscular hypertrophy of the pylorus, which results in gastric outlet obstruction leading to post-feeding non-bilious projectile vomiting. Its common age of involvement is three weeks to 3 months and is rarely reported in newborns. Case presentation: We have reported a case of synchronous SIT and IHPS in a 3-day-old newborn with classic symptoms. Following hemodynamic resuscitation, he underwent pyloromyotomy. The recovery and follow-up periods were uneventful, and the child's growth has resumed naturally. Conclusions: We present a patient with two extremely rare disorders leading to gastric outlet stenosis. In a newborn, even non-bilious vomiting can be a sign of a serious underlying condition and demands careful evaluation. Hypertrophic pyloric stenosis Pyloromyotomy Situs inversus totalis Vomiting Figures Figure 1 Figure 2 Figure 3 Background The Synchronicity of SIT and IHPS in an infant is a rare phenomenon that has only been reported in 2 cases in the literature [ 1 , 2 ]. SIT occurs when the major visceral organs are completely mirror-imaged, arranged along a left-right axis. The prevalence of this rare congenital condition is estimated at 0.1—0.6 per 10,000 live births [ 3 , 4 ]. SIT does not usually cause complications and may be incidentally diagnosed in later decades of life. IHPS is a less rare condition in infants characterized by an acquired narrowing of the pylorus. A near-complete obstruction of the pylorus is created by progressive hypertrophy of the pyloric muscle. The typical symptom is postprandial forceful projectile vomiting leading to dehydration and a hypochloremic metabolic alkalosis in infants [ 5 ]. Its incidence is estimated between 2 and 5 per thousand live births in western countries but is less common in other parts of the world [ 6 ]. Epidemiologically, infants get symptomatic between 3 weeks and three months of age [ 7 ]. We are reporting a case of IHPS who presented on day three of life. Case Presentation A 3-day-old term infant product of an uncomplicated pregnancy and normal vaginal delivery with a birth weight of 2.2 kg was referred to the pediatric surgery service on his second day of life because of non-bilious vomiting. The vomiting was progressive, usually followed breastfeeding and was not accompanied by abdominal distention or defecation problems. Findings on ultrasonography revealed that the pyloric canal is 22 mm in length and 4 mm in width. No other abdominal anomalies were reported. An incidental finding was the mirror displacement of the abdominal viscera. In clinical examination, the “olive sign” was absent. Preoperative echocardiography and upper gastrointestinal (UGI) barium contrast series were requested for preoperative preparation, which demonstrated dextrocardia [Figure 1 ]. Given typical imaging findings, the diagnosis of situs inversus totalis was confirmed. Since IHPS does not usually present this early in life, an upper Gastrointestinal series was also performed to rule out other possible causes. No evidence of malrotation or midgut volvulus was seen. Barium enema did not reveal evidence of intestinal atresia. Fluid Resuscitation was continued for 24 hours to normalize acid-base disturbances, electrolytes, and bicarbonate levels. After stabilization patient underwent abdominal exploration through a transverse left-leaning supraumbilical incision. No anomaly except SIT and a thickened pylorus located on the left side were detected. The thickness zone was visible in the pyloric region compatible with the ultrasonography report [Figure 2 ]. Classic Ramstedt pyloromyotomy was done [Figure 3 ]. After checking for a leak by insufflation of 60 ml of air through a nasogastric tube, the pylorus was returned to the abdominal cavity. Feeding with Pedialyte was initiated four hours postoperatively. He was discharged from the hospital the following day. Postoperative and 3-month follow-up visits were uneventful, and he remains within the normal range on growth charts. Discussions Situs inversus viscerum occurs when intra-abdominal organs are mirrored inside the abdomen. This is considered a rare phenomenon with a prevalence rate of 0.1—0.6 per 10,000 live births [ 1 , 2 ]. Diagnosis of SIT is usually delayed from the neonatal period to infancy only after radiographic evaluation for respiratory distress or another reason [ 8 ]. A radiographic examination reveals the stomach bubble in the right abdomen and the apex of the heart pointing to the right. In the majority of cases, SIT is not associated with any complications. However, neonatal intestinal obstruction [ 9 ], congenital heart defects, primary ciliary dyskinesia, renal disorders, biliary atresia, skeletal dysplasia, and mental retardation [ 10 ] are associated with this anomaly. SIT accompanying infantile hypertrophic pyloric stenosis in an infant is a rare phenomenon and has been reported in only 2 cases in the literature [ 1 , 2 ]. In infantile hypertrophic pyloric stenosis (IHPS), young infants of 3 weeks to 3 months suffer from the acquired hypertrophy of the muscular layer of the antropyloric portion of the stomach causing obstruction. Isolated IHPS is the most common diagnosis requiring surgery in infants [ 11 ]. Reports estimate an incidence of 2 to 5 per thousand live births in the Western world [ 12 ]. Male infants are more affected with a 4:1 male-to-female ratio [ 13 ]. Risk factors mentioned in the literature include family history, gender, younger maternal age, being a first-born infant, and maternal feeding patterns [ 14 ]. The infants at birth appear normal but within the first few weeks of life develop nonbilious forceful projectile vomiting. Pyloric muscle progressively gets hypertrophied and obstructs gastric emptying. Since sometimes the emesis is infrequent, it may be misdiagnosed as gastroesophageal reflux disease (GERD). If left untreated, there is a risk of death due to dehydration, malnutrition, and hypochloraemic, hypokalaemic metabolic alkalosis. The typical examination finding is palpation of the "olive" in the epigastrium, which is the thickened pylorus. However, it is not uniformly present and its recognition is hampered by the experience of the examiner, presence of gastric distention, and an unsettled infant. For these reasons, ultrasonography is the investigation of choice with a sensitivity and specificity of 100% in experienced hands [ 15 ]. In the treatment of IHPS, the priority is fluid resuscitation and electrolytes replacement followed by feedings cessation. The pyloromyotomy is not an emergent procedure and is considered the second line. Gastric decompression is controversial but is used in severe cases to reduce the risk of aspiration. There are several surgical approaches, from minimally invasive laparoscopy to open accessing the pylorus. The typical right upper quadrant transverse incision is used most commonly. In our patient, given the SIT we performed a left upper quadrant transverse incision instead and extended it to explore the entire intra-abdominal cavity for all possible anomalies. Conclusions Vomiting in a newborn is serious, even if it is non-bilious and needs careful evaluation. Although IHPS is a disorder of infants generally aged 3 weeks to 3 months, it may rarely present earlier. Very rarely as in our patient SIT can be associated with gastrointestinal disorders such as IHPS. Abbreviations SIT Situs inversus totalis IHPS Infantile hypertrophic pyloric stenosis UGI Upper gastrointestinal Declarations Funding The authors received no funds to conduct the study. It was self-funded. Competing interests The authors declare no competing interests relating to this original work. Ethics Approval For this type of study, formal consent is not required and was performed under a waiver of informed consent by the Institutional Review Board, ARUMS, and Iran. Consent to Participate The authors confirmed that they have gotten all proper patient written consent formats. The parents have given their consent for their images and other clinical information to be reported in the form. The parents were informed about the confidentiality of the names and initials, and due efforts would be made to hide their identity. Consent for publication Parents have given written informed consent for publication Availability of data and materials Not applicable. Code availability Not applicable Authors' contributions Planning: Khashayar Atqiaee, Ali Tabatabaey Conduct: Khashayar Atqiaee, Ali Samady Khanghah Reporting: Khashayar Atqiaee, Data acquisition; Ali Samady Khanghah Data interpretation: Ali Samady Khanghah Manuscript preparation, editing, and review: Ali Tabatabaey Acknowledgements The authors would like to acknowledge all the hospital staff who assisted us in treating and studying the patient. References Gupta AK, Yadav L, Pathak M. A rare association of pyloric stenosis and situs inversus: Impact on diagnosis and treatment. J Clin neonatology. 2014;3(1):51. Harrington B, Chambers T, Grier D. A diagnosis obscured: pyloric stenosis with situs inversus. Arch Dis Child. 1997;76(4):385. Evans WN, Acherman RJ, Restrepo H. Heterotaxy in southern Nevada: prenatal detection and epidemiology. Pediatr Cardiol. 2015;36(5):930–4. Lin AE, et al. Laterality defects in the national birth defects prevention study (1998–2007): birth prevalence and descriptive epidemiology. Am J Med Genet Part A. 2014;164(10):2581–91. Applegate MS, Druschel CM. The epidemiology of infantile hypertrophic pyloric stenosis in New York State: 1983 to 1990. Archives of paediatrics & adolescent medicine. 1995;149(10):1123–9. MacMahon B. The continuing enigma of pyloric stenosis of infancy a review . Epidemiology, 2006: p. 195–201. Taylor ND, Cass DT, Holland AJ. Infantile hypertrophic pyloric stenosis: has anything changed? J Paediatr Child Health. 2013;49(1):33–7. Izpisua BJ. How the Body Tells Left from Right. Sci Am. 1999;280(6):46–51. Gupta M, et al. Ileal atresia associated with Hirschsprung disease (total colonic aganglionosis). J Pediatr Surg. 2005;40(9):e5–7. Chen W, et al. Comorbidities in situs inversus totalis: A hospital-based study. Birth defects research. 2020;112(5):418–26. Dalton BG, et al. Optimizing fluid resuscitation in hypertrophic pyloric stenosis. J Pediatr Surg. 2016;51(8):1279–82. To T, et al. Population demographic indicators associated with the incidence of pyloric stenosis. Archives of paediatrics & adolescent medicine. 2005;159(6):520–5. Holcomb GW, Murphy JP, St SD, Peter. Holcomb and Ashcraft's pediatric surgery E-Book. Elsevier Health Sciences; 2019. White MC, et al. Sensitivity and cost minimization analysis of radiology versus olive palpation for the diagnosis of hypertrophic pyloric stenosis. J Pediatr Surg. 1998;33(6):913–7. Hernanz-Schulman M. Pyloric stenosis: role of imaging. Pediatr Radiol. 2009;39(2):134–9. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-1353951","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":98844698,"identity":"b55448b4-8e82-488e-80ab-659a7d6b0d41","order_by":0,"name":"Ali Samady Khanghah","email":"","orcid":"","institution":"Ardabil University of Medical Sciences: Ardebil University of Medical Sciences","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ali","middleName":"Samady","lastName":"Khanghah","suffix":""},{"id":98844699,"identity":"29eb5c8c-8088-4f4d-85b0-4b11a690e573","order_by":1,"name":"Khashayar Atqiaee","email":"data:image/png;base64,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","orcid":"https://orcid.org/0000-0003-0421-1334","institution":"Mashhad University of Medical Sciences Faculty of Medicine","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Khashayar","middleName":"","lastName":"Atqiaee","suffix":""},{"id":98844700,"identity":"3379841b-12dc-46b8-8e4c-138911c16b4f","order_by":2,"name":"Ali Tabatabaey","email":"","orcid":"","institution":"University of Toronto Faculty of Medicine: University of Toronto Temerty Faculty of Medicine","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ali","middleName":"","lastName":"Tabatabaey","suffix":""}],"badges":[],"createdAt":"2022-02-12 19:57:50","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-1353951/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-1353951/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":20459555,"identity":"f3146ea0-b127-4a22-8a06-324b2913100e","added_by":"auto","created_at":"2022-04-18 14:53:45","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":39803,"visible":true,"origin":"","legend":"\u003cp\u003eUGI barium contrast was showing situs inversus totalis.\u0026nbsp;\u003c/p\u003e","description":"","filename":"Figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1353951/v1/a00a428ae59eb680c00fff08.jpg"},{"id":20459557,"identity":"3a474173-01ec-416f-94df-dc0c92d9760d","added_by":"auto","created_at":"2022-04-18 14:53:46","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":3860961,"visible":true,"origin":"","legend":"\u003cp\u003eleft upper quadrant transverse incision of the liver (L) appeared in the left and hypertrophied pylorus (P) with duodenum (D) in the right.\u0026nbsp;\u003c/p\u003e","description":"","filename":"Figure2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1353951/v1/b1e6022a72363756af76db03.jpg"},{"id":20459556,"identity":"c60ec64a-af09-47ba-88d4-e5e3c14ff9d4","added_by":"auto","created_at":"2022-04-18 14:53:45","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":1978875,"visible":true,"origin":"","legend":"\u003cp\u003ePyloromyotomy procedure.\u0026nbsp;\u003c/p\u003e","description":"","filename":"Figure3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1353951/v1/0ab52627f0b1cf6fd6f1647a.jpg"},{"id":21708744,"identity":"709a26ad-40f3-4451-9025-821e7bf9e250","added_by":"auto","created_at":"2022-05-20 14:00:13","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":559001,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-1353951/v1/6a62ece0-359f-41aa-8120-b0834751fa72.pdf"},{"id":20459558,"identity":"02f0b479-49aa-446b-bdee-0524e46c8a80","added_by":"auto","created_at":"2022-04-18 14:53:46","extension":"pdf","order_by":7,"title":"","display":"","copyAsset":false,"role":"supplement","size":690800,"visible":true,"origin":"","legend":"","description":"","filename":"CAREchecklist.pdf","url":"https://assets-eu.researchsquare.com/files/rs-1353951/v1/2e7a250d90d4116c25ba695c.pdf"}],"financialInterests":"","formattedTitle":"Infantile Hypertrophic Pyloric Stenosis, the Cause of Non-bilious Vomiting of a 3-day-old Male Infant with Situs Inversus Totalis; A Case Report","fulltext":[{"header":"Background","content":"\u003cp\u003eThe Synchronicity of SIT and IHPS in an infant is a rare phenomenon that has only been reported in 2 cases in the literature [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. SIT occurs when the major visceral organs are completely mirror-imaged, arranged along a left-right axis. The prevalence of this rare congenital condition is estimated at 0.1\u0026mdash;0.6 per 10,000 live births [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. SIT does not usually cause complications and may be incidentally diagnosed in later decades of life. IHPS is a less rare condition in infants characterized by an acquired narrowing of the pylorus. A near-complete obstruction of the pylorus is created by progressive hypertrophy of the pyloric muscle. The typical symptom is postprandial forceful projectile vomiting leading to dehydration and a hypochloremic metabolic alkalosis in infants [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Its incidence is estimated between 2 and 5 per thousand live births in western countries but is less common in other parts of the world [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Epidemiologically, infants get symptomatic between 3 weeks and three months of age [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. We are reporting a case of IHPS who presented on day three of life.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 3-day-old term infant product of an uncomplicated pregnancy and normal vaginal delivery with a birth weight of 2.2 kg was referred to the pediatric surgery service on his second day of life because of non-bilious vomiting. The vomiting was progressive, usually followed breastfeeding and was not accompanied by abdominal distention or defecation problems. Findings on ultrasonography revealed that the pyloric canal is 22 mm in length and 4 mm in width. No other abdominal anomalies were reported. An incidental finding was the mirror displacement of the abdominal viscera. In clinical examination, the \u0026ldquo;olive sign\u0026rdquo; was absent. Preoperative echocardiography and upper gastrointestinal (UGI) barium contrast series were requested for preoperative preparation, which demonstrated dextrocardia [Figure \u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e]. Given typical imaging findings, the diagnosis of situs inversus totalis was confirmed.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eSince IHPS does not usually present this early in life, an upper Gastrointestinal series was also performed to rule out other possible causes. No evidence of malrotation or midgut volvulus was seen. Barium enema did not reveal evidence of intestinal atresia.\u003c/p\u003e \u003cp\u003eFluid Resuscitation was continued for 24 hours to normalize acid-base disturbances, electrolytes, and bicarbonate levels. After stabilization patient underwent abdominal exploration through a transverse left-leaning supraumbilical incision. No anomaly except SIT and a thickened pylorus located on the left side were detected. The thickness zone was visible in the pyloric region compatible with the ultrasonography report [Figure \u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e]. Classic Ramstedt pyloromyotomy was done [Figure \u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e]. After checking for a leak by insufflation of 60 ml of air through a nasogastric tube, the pylorus was returned to the abdominal cavity. Feeding with Pedialyte was initiated four hours postoperatively. He was discharged from the hospital the following day. Postoperative and 3-month follow-up visits were uneventful, and he remains within the normal range on growth charts.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussions","content":"\u003cp\u003eSitus inversus viscerum occurs when intra-abdominal organs are mirrored inside the abdomen. This is considered a rare phenomenon with a prevalence rate of 0.1\u0026mdash;0.6 per 10,000 live births [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Diagnosis of SIT is usually delayed from the neonatal period to infancy only after radiographic evaluation for respiratory distress or another reason [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. A radiographic examination reveals the stomach bubble in the right abdomen and the apex of the heart pointing to the right. In the majority of cases, SIT is not associated with any complications. However, neonatal intestinal obstruction [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e], congenital heart defects, primary ciliary dyskinesia, renal disorders, biliary atresia, skeletal dysplasia, and mental retardation [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e] are associated with this anomaly. SIT accompanying infantile hypertrophic pyloric stenosis in an infant is a rare phenomenon and has been reported in only 2 cases in the literature [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. In infantile hypertrophic pyloric stenosis (IHPS), young infants of 3 weeks to 3 months suffer from the acquired hypertrophy of the muscular layer of the antropyloric portion of the stomach causing obstruction. Isolated IHPS is the most common diagnosis requiring surgery in infants [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Reports estimate an incidence of 2 to 5 per thousand live births in the Western world [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. Male infants are more affected with a 4:1 male-to-female ratio [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Risk factors mentioned in the literature include family history, gender, younger maternal age, being a first-born infant, and maternal feeding patterns [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. The infants at birth appear normal but within the first few weeks of life develop nonbilious forceful projectile vomiting. Pyloric muscle progressively gets hypertrophied and obstructs gastric emptying. Since sometimes the emesis is infrequent, it may be misdiagnosed as gastroesophageal reflux disease (GERD). If left untreated, there is a risk of death due to dehydration, malnutrition, and hypochloraemic, hypokalaemic metabolic alkalosis. The typical examination finding is palpation of the \"olive\" in the epigastrium, which is the thickened pylorus. However, it is not uniformly present and its recognition is hampered by the experience of the examiner, presence of gastric distention, and an unsettled infant. For these reasons, ultrasonography is the investigation of choice with a sensitivity and specificity of 100% in experienced hands [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]. In the treatment of IHPS, the priority is fluid resuscitation and electrolytes replacement followed by feedings cessation. The pyloromyotomy is not an emergent procedure and is considered the second line. Gastric decompression is controversial but is used in severe cases to reduce the risk of aspiration. There are several surgical approaches, from minimally invasive laparoscopy to open accessing the pylorus. The typical right upper quadrant transverse incision is used most commonly. In our patient, given the SIT we performed a left upper quadrant transverse incision instead and extended it to explore the entire intra-abdominal cavity for all possible anomalies.\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eVomiting in a newborn is serious, even if it is non-bilious and needs careful evaluation. Although IHPS is a disorder of infants generally aged 3 weeks to 3 months, it may rarely present earlier. Very rarely as in our patient SIT can be associated with gastrointestinal disorders such as IHPS.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eSIT\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eSitus inversus totalis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eIHPS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eInfantile hypertrophic pyloric stenosis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eUGI\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eUpper gastrointestinal\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors received no funds to conduct the study. It was self-funded.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests relating to this original work.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics Approval\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFor this type of study, formal consent is not required and was performed under a waiver of informed consent by the Institutional Review Board, ARUMS, and Iran.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to Participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors confirmed that they have gotten all proper patient written consent formats. The parents have given their consent for their images and other clinical information to be reported in the form. The parents were informed about the confidentiality of the names and initials, and due efforts would be made to hide their identity.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003cstrong\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eParents have given written informed consent for publication\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCode availability\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003ePlanning:\u0026nbsp;Khashayar Atqiaee, Ali Tabatabaey\u003c/p\u003e\n\u003cp\u003eConduct:\u0026nbsp;Khashayar Atqiaee, Ali Samady Khanghah\u003c/p\u003e\n\u003cp\u003eReporting:\u0026nbsp;Khashayar Atqiaee,\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Data acquisition; Ali Samady Khanghah\u003c/p\u003e\n\u003cp\u003eData interpretation: Ali Samady Khanghah\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Manuscript preparation, editing, and review: Ali Tabatabaey\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors would like to acknowledge all the hospital staff who assisted us in treating and studying the patient.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eGupta AK, Yadav L, Pathak M. A rare association of pyloric stenosis and situs inversus: Impact on diagnosis and treatment. J Clin neonatology. 2014;3(1):51.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eHarrington B, Chambers T, Grier D. A diagnosis obscured: pyloric stenosis with situs inversus. Arch Dis Child. 1997;76(4):385.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eEvans WN, Acherman RJ, Restrepo H. Heterotaxy in southern Nevada: prenatal detection and epidemiology. Pediatr Cardiol. 2015;36(5):930\u0026ndash;4.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLin AE, et al. Laterality defects in the national birth defects prevention study (1998\u0026ndash;2007): birth prevalence and descriptive epidemiology. Am J Med Genet Part A. 2014;164(10):2581\u0026ndash;91.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eApplegate MS, Druschel CM. The epidemiology of infantile hypertrophic pyloric stenosis in New York State: 1983 to 1990. Archives of paediatrics \u0026amp; adolescent medicine. 1995;149(10):1123\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMacMahon B. \u003cem\u003eThe continuing enigma of pyloric stenosis of infancy a review\u003c/em\u003e. Epidemiology, 2006: p.\u0026nbsp;195\u0026ndash;201.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eTaylor ND, Cass DT, Holland AJ. Infantile hypertrophic pyloric stenosis: has anything changed? J Paediatr Child Health. 2013;49(1):33\u0026ndash;7.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eIzpisua BJ. How the Body Tells Left from Right. Sci Am. 1999;280(6):46\u0026ndash;51.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGupta M, et al. Ileal atresia associated with Hirschsprung disease (total colonic aganglionosis). J Pediatr Surg. 2005;40(9):e5\u0026ndash;7.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eChen W, et al. Comorbidities in situs inversus totalis: A hospital-based study. Birth defects research. 2020;112(5):418\u0026ndash;26.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDalton BG, et al. Optimizing fluid resuscitation in hypertrophic pyloric stenosis. J Pediatr Surg. 2016;51(8):1279\u0026ndash;82.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eTo T, et al. Population demographic indicators associated with the incidence of pyloric stenosis. Archives of paediatrics \u0026amp; adolescent medicine. 2005;159(6):520\u0026ndash;5.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eHolcomb GW, Murphy JP, St SD, Peter. Holcomb and Ashcraft's pediatric surgery E-Book. Elsevier Health Sciences; 2019.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eWhite MC, et al. Sensitivity and cost minimization analysis of radiology versus olive palpation for the diagnosis of hypertrophic pyloric stenosis. J Pediatr Surg. 1998;33(6):913\u0026ndash;7.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eHernanz-Schulman M. Pyloric stenosis: role of imaging. Pediatr Radiol. 2009;39(2):134\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Hypertrophic pyloric stenosis, Pyloromyotomy, Situs inversus totalis, Vomiting","lastPublishedDoi":"10.21203/rs.3.rs-1353951/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-1353951/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground:\u003c/strong\u003e The Synchronicity of Situs Inversus totalis (SIT) and infantile hypertrophic pyloric stenosis (IHPS) is a rare phenomenon with only 2 reported cases in the literature. SIT is a mirror transposition of the major visceral organs, arranged along a left-right axis. Its prevalence is 0.1—0.6 per 10,000 live births. Rarely it is accompanied by neonatal intestinal obstruction, congenital heart defects, primary ciliary dyskinesia, renal disorders, biliary atresia, skeletal dysplasia, and mental retardation. IHPS is muscular hypertrophy of the pylorus, which results in gastric outlet obstruction leading to post-feeding non-bilious projectile vomiting. Its common age of involvement is three weeks to 3 months and is rarely reported in newborns. \u003c/p\u003e\u003cp\u003e\u003cstrong\u003eCase presentation:\u003c/strong\u003e We have reported a case of synchronous SIT and IHPS in a 3-day-old newborn with classic symptoms. Following hemodynamic resuscitation, he underwent pyloromyotomy. The recovery and follow-up periods were uneventful, and the child's growth has resumed naturally.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eConclusions: \u003c/strong\u003eWe present a patient with two extremely rare disorders leading to gastric outlet stenosis. In a newborn, even non-bilious vomiting can be a sign of a serious underlying condition and demands careful evaluation.\u003c/p\u003e","manuscriptTitle":"Infantile Hypertrophic Pyloric Stenosis, the Cause of Non-bilious Vomiting of a 3-day-old Male Infant with Situs Inversus Totalis; A Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2022-04-18 14:53:44","doi":"10.21203/rs.3.rs-1353951/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"40192dc8-8176-43ab-a0a1-f343f0b6db37","owner":[],"postedDate":"April 18th, 2022","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2022-05-20T14:00:03+00:00","versionOfRecord":[],"versionCreatedAt":"2022-04-18 14:53:44","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-1353951","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-1353951","identity":"rs-1353951","version":["v1"]},"buildId":"WrCJVZZCHTDjtuVLN7oU0","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}