Pyloric Stenosis and Nonbilious Vomiting in Infants: Negative Base Excess and Hypercapnia—Two Opposing Points of One Scale a Comparative Case Series

Background: Blood pH and bicarbonate estimations are basal acid-base laboratory tests that are performed in infants with infantile hypertrophic pyloric stenosis (IHPS). This study aimed to define the clinical value of pCO2 and BE in infants suspected to have IHPS. Methods: We collected data from 80 “surgical” infants younger than 100 days with prolonged nonbilious vomiting who were suspected to have IHPS. In 65 infants, pyloric stenosis was confirmed, and 15 infants had nonsurgical conditions. Capillary blood was tested for standard acid-base parameters and lactate. The two groups were compared. Results: Eighty-eight percent of the IHPS infants had elevated standard bicarbonate levels (st bicarb) > 25 mmol/l, and 60% had BE > 3.5 mmol/l;12% of the infants showed hypercapnia (pCO2 ≥ 50 mmHg) associated with markedly increased standard bicarbonate and BE. Infants with nonsurgical vomiting were older at admission (p = 0.002), had a longer duration of vomiting (p < 0.001), were older (p = 0.002) and weighted more at admission (p = 0.004), had lower pCO2 (p = 0.021), lower st bicarb (p < 0.001) and lower BE (p = 0.001). In addition, nonsurgical infants showed a trend to anemia (p = 0.002). Conclusions: In infants with IHPS/nonbilious vomiting, acid-base analysis (ABA) is equivocal or inconclusive. These findings may be misleading and could result in a false clinical decision. Nonsurgical vomiting is associated with a lower degree of alkalosis, normocapnia to slight hypercapnia and a base deficit. However, even infants with IHPS may present with a negative BE. In infants with IHPS and severe alkalosis, hypercapnia carries a risk for respiratory depression. Monitoring the infant’s respiration allows for the early detection of respiratory deterioration.

Case Report: Four Siblings with Osteopetrosis and Pyloric Stenosis and Three Cousins with Osteopetrosis

Osteopetrosis incidence is less than 1:200,000 births in most populations. It’s more common in consanguineous people as it’s unusual for two members of the same family. The incidence of Hypertrophic pyloric stenosis is 1 in 300 - 900 newborns. Hypertrophic pyloric stenosis is due to hypertrophy of the smooth muscle of the pyloric sphincter. The classic age of occurrence is the first few months of life, and the classic presentation is non-bilious projectile vomiting after feeding. We report a rare association of osteopetrosis and pyloric stenosis in four siblings and osteopetrosis in three cousins. All four patients were operated on and followed by nephrology and metabolic departments for osteopetrosis and metabolic acidosis.

Rapid right ventricular pacing for balloon valvuloplasty in congenital aortic stenosis:A systematic review

BACKGROUND Balloon aortic valvuloplasty(BAV)is a well-established treatment modality for congenital aortic valve stenosis.AIM To evaluate the role of rapid right ventricular pacing(RRVP)in balloon stabilization during BAV on aortic regurgitation(AR)in pediatric patients.METHODS A systematic review of the MEDLINE,Cochrane Library,and Scopus databases was conducted according to the PRISMA guidelines(end-of-search date:July 8,2020).The National Heart,Lung,and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment.RESULTS Five studies reporting on 72 patients were included.The studies investigated the use of RRVP-assisted BAV in infants(>1 mo)and older children,but not in neonates.Ten(13.9%)patients had a history of some type of aortic valve surgical or catheterization procedure.Before BAV,58(84.0%),7(10.1%),4(5.9%)patients had AR grade 0(none),1(trivial),2(mild),respectively.After BAV,34(49.3%),6(8.7%),26(37.7%),3(4.3%),patients had AR grade 0,1,2,and 3(moderate),respectively.No patient developed severe AR after RRVP.One(1.4%)developed ventricular fibrillation and was defibrillated successfully.No additional arrhythmias or complications occurred during RRVP.CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV,which could potentially decrease AR rates.

Spectrum of magnetic resonance imaging findings in congenital lumbar spinal stenosis

AIM: To investigate whether congenital lumbar spinal stenosis(CLSS) is associated with a specific degenerative changes of the lumbar spine. METHODS: The lumbar spine magnetic resonance imaging studies of 52 subjects with CLSS and 48 control subjects were retrospectively evaluated. In each examination, the five lumbar levels were assessed for the presence or absence of circumferential or shallow annular bulges, annular tears, anterior or posterior disc herniations, epidural lipomatosis, Schmorl's nodes,spondylolisthesis, pars defects, and stress reactions of the posterior vertebral elements. RESULTS: Compared to control individuals, subjects with CLSS exhibited increased incidence of circumferential and shallow annular bulges, annular tears, discherniations and spondylolisthesis(P < 0.05). CONCLUSION: CLSS is associated with increased incidence of degenerative changes in specific osseous and soft-tissue elements of the lumbar spine.

Endoscopic magnetic compression stricturoplasty for congenital esophageal stenosis:A case report

BACKGROUND Congenital esophageal stenosis(CES)is a rare malformation of the digestive tract.Endoscopic dilation and thoracotomy have been the main treatments for CES.However,there is no well-defined management protocol.Magnetic compression stricturoplasty(MCS)has been used in refractory esophageal stricture in children after esophageal atresia.CASE SUMMARY We describe the first case of MCS for CES in one female child patient.The child(aged 3 years and 1 mo)was admitted due to frequent vomiting and choking after eating complementary food since 7 mo old.Esophagography and gastroendoscopy showed that there was stenosis in the lower esophagus,suggesting a diagnosis of CES.The patient did not receive any treatment for esophageal stricture including surgery or endoscopic dilation procedures before MCS.MCS procedure was smoothly conducted without complications.At 24 mo after MCS,durable esophageal patency without dysphagia was achieved.CONCLUSION MCS may serve as an alternative and efficient method for patients with CES.

Costello Syndrome with Congenital Pulmonary Valve Stenosis and Ventriculomegaly—A Case Report

Costello syndrome is an extremely rare genetic disorder with growth delay after birth and typically results in short stature during childhood. It is one of the RASopathy of Ras/MAPK pathway syndromes. It affects the transforming protein p21, an enzyme that in humans is encoded by the HRAS gene. H-Ras is a small G protein and once bound to Guanosine triphosphate, it will activate a Raf kinase like C-Raf, the next step in the MAPK/ERK pathway (mitogen-actvated protein kinase/extracellular signal-regulated kinase) i.e., MEK (mitogen-actvated ERK kinase), a protein that phosphorylate ERK which can directly and indirectly activate many transcription factors. This pathway is also known as Ras-Raf, MEK-ERK pathway, which is a chain of proteins on the cell that communicate a signal from a receptor on the surface of the cell to the DNA in the nucleus of the cell. Activation of ERK 1/2 is involved in signal transduction pathways associated with cardiac hypertrophy. The developmental syndromes caused by germline mutations in genes that alter the RAS components of the MAP/ERK signal transduction pathway are called “RASopathies”. Cardiovascular abnormalities are important features of Costello syndrome and other RASopathies such as Noonan syndrome. Background of this case report described the congenital valvular pulmonic stenosis and ventriculomegaly associated with Costello syndrome by transthoracic echocardiographic imaging in a 9-year-old male boy.

The Prognostic Value of Myocardial Deformation in Patients with Congenital Aortic Stenosis

Aims:To assess the prognostic value of left ventricular(LV)global longitudinal strain(GLS)and global longitudinal early diastolic strain rate(GLSre)with regard to cardiovascular events,as congenital aortic stenosis(AoS)is associated with significant mortality and morbidity but predictors for clinical outcome are scarce.Strain analysis provides a robust and reproducible method for early detection of LV dysfunction,which might be of prognostic value.Methods:This prospective study,included clinically stable patients with congenital AoS between 2011–2013.LV GLS and GLSre was performed in the apical 4,3 and 2-chamber views using Tomtec software.The endpoint was a composite of death,heart failure,hospitalization,arrhythmia,thrombo-embolic events and re-intervention.Results:In total 138 patients were included(33[26–43]years,86(62%)male),NYHA class I:134(97%).Mean LV GLS was–15.3±3.2%,GLSre 0.66±0.18 s–1.Both correlated with NT-proBNP,LV volumes and ejection fraction(strongest LV GLS with LV EF:r–0.539,p<0.001,strongest LV GLSre with age:r–0.376,p<0.001).During median follow-up of 5.9[5.5–6.2]years,the endpoint occurred in 53(38%)patients:4 patients died,9 developed heart failure,22 arrhythmias,8 thrombo-embolic events and 35 re-interventions.Both LV GLS(standardized HR(sHR 0.62(95%CI 0.47–0.81)and GLSre(sHR 0.62(95%CI 0.47–0.83)were associated with the endpoint.Additional multivariable analysis showed that both GLS and GLSre were associated independent of left atrial volume,NT-proBNP and prior re-interventions.Conclusion:Left ventricular GLS and GLSre are reduced in adult patients with congenital AoS.Both markers are associated with adverse cardiac events and have clear clinical relevance.

Surgical repair of congenital tracheal stenosis associated with complex congenital heart disease in infants

Objective To evaluate results of one stage repair of congenital tracheal stenosis associated with complex congenital heart disease in infants. Methods Two infants with congenital stenosis associated with Tetralogy of Fallot were operated on at the age of 1. 5 and 3 years respectively. In the younger child, the 1. 2 cm long of tracheal stenosis was excised and anastomosed. The other case with 3 cm in length of stenosis at the mid-segment of trachea was excised and repaired with a patch. Results The postoperative progress was uneventful. The patients were followed-up for 6 months to 1 year without any complications. Conclusion One stage repair of congenital tracheal stenosis associated with complex congenital heart disease in infants has got satisfactory results. With cardiopulmonary bypass, the operation can be carried out safely. 6 refs,2 figs.

Video capsule endoscopy and CT enterography in diagnosing adult hypertrophic pyloric stenosis

Primary adult hypertrophic pyloric stenosis is a rare but important cause of gastric outlet obstruction that may be misdiagnosed as idiopathic gastroparesis.Clinically,patients present with early satiety,abdominal fullness,nausea,epigastric discomfort and eructation.Permanent gastric retention of a video capsule endoscope is diagnostic in differentiating between the two diseases,in the absence of an organic gastric outlet obstruction.This case presents the longest video capsule retention in the medical literature to date.It is also the first case report of adult hypertrophic pyloric stenosis diagnosed with video capsule endoscopy or a computed tomography scan.Finally,an unusual"plugging"of the gastric outlet with free floating capsule has an augmented effect on disease physiology and on patient’s symptoms.

Idiopathic non-hypertrophic pyloric stenosis in an infant successfully treated via endoscopic approach

Non-peptic, nonhypertrophic pyloric stenosis has rarely been reported in pediatric literature. Endoscopic pyloric balloon dilation has been shown to be a safe procedure in treating gastric outlet obstruction in older children and adults. Partial gastric outlet obstruction (GOO) was diagnosed in an infant by history and confirmed by anupper gastrointestinal series (UGI). Abdominal ultrasonography and computed tomography scan excluded idiopathic hypertrophic pyloric stenosis, abdominal tumors, gastrointestinal and hepato-biliary-pancreatic anomalies. Endoscopic findings showed a pinhole-sized pylorus and did not indicate peptic ulcer disease, Helicobacter pylori infection, antral web, or eviden-ce of allergic and inflammatory bowel diseases. Three sessions of a step-wise endoscopic pyloric balloon dilation were conducted under general anesthesia and a fluoroscopy at two week intervals using catheter balloons (Boston Scientific Microvasive?, MA, USA) of increasing diameters. Repeat UGI after the first session revealed normal gastrointestinal transit and no intestinalobstruction. The patient tolerated solid food without any gastrointestinal symptoms since the first session. The endoscope was able to be passed through the pylorus after the last session. Although the etiology of GOO in this infant is unclear (proposed mechanisms are herein discussed), endoscopic pyloric balloon dilation was a safe procedure for treating this young infant with non-peptic, non-hypertrophic pyloric stenosis and should be considered as an initial approach before pyloroplasty in such presentations.

Antral web associated with distal antral hypertrophy and prepyloric stenosis mimicking hypertropic pyloric stenosis

A 3-year-old boy presented with postprandial vomiting and epigastric pain for 3 wk. Barium meal study suggested hypertrophic pyloric stenosis. Ultrasound of the stomach after water loading revealed an echogenic antral web with an eccentric aperture and distal antral hypertrophy.Subsequent endoscopy confirmed the ultrasound findings.Web resection and antropyloroplasty resulted in excellent recovery. To our knowledge, the barium meal and ultrasound findings of an antral web-associated distal antral hypertrophy and prepyloric stenosis has not previously been described.

Gastrojejunostomy for pyloric stenosis after acute gastric dilatation due to overeating

A 34-year-old woman presented at our hospital withabdominal distention due to overeating.Acute gastric dilatation was diagnosed.The patient was hospitalized,and nasogastric decompression was initiated.On hospitalization day 3,she developed shock,and her respiratory state deteriorated,requiring intubation and mechanical ventilation.Nasogastric decompression contributed to the improvement in her clinical condition.She was discharged 3 mo after admission.During outpatient follow-up,her dietary intake decreased,and her body weight gradually decreased by 14 kg.An upper gastrointestinal series and endoscopy revealed pyloric stenosis; therefore,we performed gastrojejunostomy 18 mo after her initial admission.The patient was discharged from the hospital with no postoperative complications.Gastric necrosis and perforation due to overeating-induced gastric dilatation are life-threatening conditions.Surgical intervention may be required if delayed pyloric stenosis occurs after conservative treatment.We report a case of pyloric stenosis due to overeating-induced gastric dilatation treated by gastrojejunostomy 18 mo after the initial presentation.

Pathophysiology of Hypertrophic Pyloric Stenosis Revisited: The Use of Isotonic Fluid for Preoperative Infusion Therapy Is Supported

Background: The aim of this study was to elucidate the preoperative clinical and biochemical profile of infants with IHPS to optimize infusion therapy. Patients and Method: We retrospectively analyzed data from 56 infants who were operated for IHPS. Our study includes growth and laboratory data prior to the initiation of therapy. Results: Median duration of propulsive vomiting was 4 d;the median age was 37 d (18 - 108), and the median body weight was 3840 g (2760 -5900). Metabolic alkalosis (MAlk) with a pH of 7.45 ± 0.06 and an stHCO3- of 28.7 ± 4.5 mmol/l was found. In a subgroup of the infants, negative base excess (BE) was observed. The sodium concentration was normal or reduced (mean/median of 137 mmol/l). There was a strong negative correlation between stHCO3- and K+. The carbon dioxide partial pressure tended to increase (5.72 ± 0.84 kPa). Calculations of osmolality revealed a normal osmolarity. Hypoglycemia did not occur. The creatinine clearance according to the Schwartz formula remained at a normal level (85.3 ± 24.3 ml/min/1.73 m2). Discussion: The presented case series is characterized by a short duration of preoperative vomiting. MAlk can be classified as a chloride deficiency syndrome. It is accompanied by normo- or hyponatremic dehydration with normal osmolality. Partial respiratory compensation occurred. A normal creatinine clearance indicated good glomerular renal function. Conclusion: The presented study supports the use of an isotonic infusion fluid with a low glucose concentration for preoperative infusion therapy.

Pyloric stenosis associated Crohn's disease responding to adalimumab therapy

Gastroduodenal Crohn’s disease (CD) is rare and the response to standard medical therapy is often poor. Anti-tumor necrosis factor therapy has revolutionised the treatment of CD. We present a patient with pyloric stenosis associated with CD which improved with Adalimumab therapy. We recommend considering antitumor necrosis factor therapy in symptomatic gastroduodenal CD.

New insights on the pathogenesis of pyloric stenosis of infancy. A review with emphasis on the hyperacidity theory

A review is presented on the theories concerning the cause of pyloric stenosis with emphasis on the primary position of inherited hyperacidity in pathogenesis. Existing theories are critically analysed and the hyperacidity theory is precisely defined in the light of recent physiological insights into the gastrointestinal hormone motilin. The progressive fixed fasting hypergastrinaemia within the first few weeks of life will, in the baby who inherits acid secretion at the top of the normal range, produce hyperacidity of sufficient severity to trigger the process of acid-induced work hypertrophy of the pylorus. The potential contribution of motilin is discussed. The baby who inherits a normal gastric acidity will not reach acid levels severe enough to trigger sphincter hypertrophy despite the early gastrin stimulus. The potential threat will cease when gastrin naturally declines with age and the pyloric canal becomes wider. Genetic factors clearly must also be involved and these are separately discussed.

Cytomegalovirus-associated gastric ulcer:A side effect of steroid injections for pyloric stenosis

The local injection of triamcinolone acetonide(TA) is effective in preventing pyloric stenosis and deformity following large endoscopic submucosal dissection(ESD).However,because of its long-acting nature,TA can induce long-term local immunosuppression and subsequent adverse events.We report a case of a cytomegalovirus(CMV) ulcer that formed only at the TA local injection site.A 68-year-old man underwent ESD to treat early gastric cancer that formed over the pylorus.The lesion extended to the duodenum,and an artificial ulcer covered more than two-thirds of the circumference of the pylorus.To prevent pyloric stenosis,TA was locally injected into the ulcer floor.On day 12,a deeper ulcer 10 mm in diameter was discovered in the center of the post-ESD ulcer.Biopsies revealed large cells with intranuclear inclusion bodies,which stained positive for the anti-CMV antibody.Local TA injections are useful,however,CMV ulcer might occur as adverse events.

Double Incomplete Pyloromyotomy (A. Ezzat Technique): A New Technique for Infantile Hypertrophic Pyloric Stenosis: Preliminary Study

Background-Purpose: The study aimed to see the outcome of Double Incomplete Pyloromyotomy as new technique for surgical management of infantile hypertrophic pyloric stenosis (IHPS). Methods: This study was conducted in pediatric surgery unite, Zagazig University Hospital, Egypt. Fifteen patients were included in this study (11 male and 4 female) with IHPS from January 2012 to January 2013. Under general anesthesia, two longitudinal separated incisions at different planes as pyloromyotomy. Results: Postoperative vomiting and weight gain were recorded. Follow up period was 3 months. Vomiting improved within first 48 hours then stopped after that. Weight gain significantly increased after theoperation when compared preoperatively. Conclusion: Double Incomplete Pyloromyotomy is a new, safe and effective procedure for treatment of infantile hypertrophic pyloric stenosis.

Comparison of outcomes between complete and incomplete congenital duodenal obstruction

BACKGROUND Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes. AIM To quantify and compare the association between CCDO and ICDO with outcome parameters. METHODS We retrospectively reviewed all patients who underwent operative repair of CCDO or ICDO in our tertiary care institution between January 2004 and January 2017. The demographics, clinical presentation, preoperative diagnostics and postoperative outcomes of 50 patients were compared between CCDO (n = 27;atresia type 1-3, annular pancreas) and ICDO (n=23;annular pancreas, web, Ladd′s bands). RESULTS In total, 50 patients who underwent CDO repair were enrolled and followed for a median of 5.2 and 3.9 years (CCDO and ICDO, resp.). CCDO was associated with a significantly higher prenatal ultrasonographic detection rate (88% versus 4%;CCDO vs ICDO, P<0.01), lower gestational age at birth, lower age and weight at operation, higher rate of associated congenital heart disease (CHD), more extensive preoperative radiologic diagnostics, higher morbidity according to Clavien-Dindo classification and comprehensive complication index (all P≤0.01). The subgroup analysis of patients without CHD and prematurity showed a longer time from operation to the initiation of enteral feeds in the CCDO group (P<0.01). CONCLUSION CCDO and ICDO differ with regard to prenatal detection rate, gestational age, age and weight at operation, rate of associated CHD, preoperative diagnostics and morbidity. The degree of CDO in mature patients without CHD influences the postoperative initiation of enteral feeding.

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few centers.This study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary center.Methods:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May 2022.Technical success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new regurgitation.Follow-up clinical records and echocardiography were obtained during the prenatal and postnatal periods.Results:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 years.The median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful procedures.One patient had pulmonary valve perforation without balloon dilation.No procedure-related deaths or significant complications occurred.However,one neonatal death occurred due to heart and renal failure.The median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up visit.Conclusion:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases

Background： Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions. Methods： Clinical and hemodynamic data from 40 pediatric patients （24 boys and 16 girls） with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis （PVS）, valved conduit stenosis, pulmonary artery stenosis （PAS）, and supravalvular pulmonary stenosis （SVPS）. Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed. Results： Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best （100%）, followed by the PVS group （90.9%） and the PAS group （85.7%）. There were a total of two transient complications （5.0%）. Conclusions： Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.