Presentation and outcomes in hypertrophic pyloric stenosis: An 11-year review.

AIM: To evaluate the trend in presentation and postoperative outcomes of infants with hypertrophic pyloric stenosis (HPS) over the last decade. METHODS: This was a multicentre retrospective study in two tertiary paediatric centres between 2005 and 2015 inclusive. Participants included 626 infants who underwent a pyloromyotomy for HPS. We collected data on presentation features (age, weight, clinical signs, blood gas results, ultrasound findings) and postoperative outcomes (length of stay (LOS), complications, time to first postoperative feed). RESULTS: No trend was identified during the study period with regards to age, weight, biochemical findings (pH, chloride, base excess) or pre-operative ultrasound measurements. There was a downtrend in the number of palpated tumours over time, with a mean of 36% of tumours clinically palpated. Pyloric wall thickness had a moderate association with LOS in patients admitted for >8 days (correlation = 0.4752) but had a weak negative association with shorter lengths of stay (≤8 day, correlation = -0.094). Overall, median time to first feed was 7.80 h and improved yearly during the study period (hazard ratio = 1.07). CONCLUSIONS: Patients presenting with HPS are not being identified at an earlier age or with fewer biochemical derangements, in contrast to our initial perceptions. Subsequently, biochemical derangements can still play an important role in the diagnosis of HPS, and attention needs to be given to fluid management and electrolyte correction in all patients with HPS.

Infantile Hypertrophic Pyloric Stenosis: An Epidemiological Review.

Infantile hypertrophic pyloric stenosis (IHPS) is the most common gastrointestinal disease among infants. IHPS occurs as an isolated condition or together with other congenital anomalies. Association with genetic and environmental factors, bottle feeding, younger maternal age, maternal smoking, and erythromycin administration in the first two weeks of life has been shown. Familial aggregation has been described and a family history is seen in 47.9 percent of siblings. Infants typically present with projectile vomiting associated with symptoms of failure to thrive. An olive-like mass palpable in the right upper abdominal quadrant is being reported less frequently because of earlier diagnosis by ultrasound. IHPS is generally corrected through laparoscopic pyloromyotomy. Endoscopic pyloric balloon dilation is a novel alternative. Further studies on different populations, the general population, and familial segregation to determine the prevalence, influence, and mode of familial aggregation and correlation with environmental factors are needed to determine the etiology of IHPS.

Hypertrophic pyloric stenosis following repair of oesophageal atresia and tracheo-oesophageal fistula in a neonate.

Development of hypertrophic pyloric stenosis (HPS) after a few weeks of repair of an oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) is a rare condition in early infancy. Although vomiting or feeding intolerance in operated cases of OA+TOF are attributed to oesophageal stricture, gastro-oesophageal reflux and oesophageal dysmotility, it may also be caused by HPS. Herein, we report a newborn infant who had OA and TOF operation on day 2 of life and diagnosed to have HPS at 15th day of age. Even though it is a rare anomaly, HPS should be kept on mind in the presence of persistent vomiting following repair of OA.

Optimizing peripheral venous pressure waveforms in an awake pediatric patient by decreasing signal interference.

The purpose of this technological notes paper is to describe our institution's experience collecting peripheral venous pressure (PVP) waveforms using a standard peripheral intravenous catheter in an awake pediatric patient. PVP waveforms were collected from patients with hypertrophic pyloric stenosis. PVP measurements were obtained prospectively at two time points during the hospitalization: admission to emergency department and after bolus in emergency department. Data was collected from thirty-two patients. Interference in the PVP waveforms data collection was associated with the following: patient or device motion, system set-up error, type of IV catheter, and peripheral intravenous catheter location. PVP waveforms can be collected in an awake pediatric patient and adjuncts to decrease signal interference can be used to optimize data collection.

[Analysis of hypertrophic pyloric stenosis: size does matter]. / Análisis de la estenosis hipertrófica del píloro: el tamaño sí importa.

OBJECTIVES: To analyze the correlation between pyloric size and evolution of patients surgically treated for infantile hypertrophic pyloric stenosis (IHPS). PATIENTS AND METHODS: We realized a retrospective study of 109 patients undergoing IHPS in 5 years. We analyzed by χ2 test if a correlation between evolution time, age and postoperative vomiting and gastroesophageal reflux disease (GERD), and pyloric muscle thickness (group A > 4.5 mm and group B ≤ 4.5 mm) and pyloric channel length (group A ≥ 20 mm and group B < 20 mm) exists. RESULTS: There is a statistically significant correlation between longer evolution history (>48 h) and pyloric length ≥ 20 mm and between age (> 30 days) and muscle thickness > 4.5 mm (p= 0.022 and p= 0.009, respectively). Also, 38.5% of 109 patients had postoperative emesis 2 days after surgery and 13.8% had GERD for a median time of 8.27 months (1-12 months), showing that there is a statistically significant correlation (p= 0.007) between pyloric channel length ≥ 20 mm and postoperative emesis. It was not observed correlation between pyloric thickness and length and GERD. CONCLUSIONS: It has been observed that there is correlation between pyloric length ≥ 20 mm and postoperative emesis. However, it has not been observed regarding the pyloric muscle thickness. A longer preoperative evolution and age > 30 days are correlated to channel length ≥ 20 mm and muscle thickness > 4.5 mm, respectively.

OBJETIVOS: Analizar la relación entre el tamaño del píloro y la evolución de los pacientes intervenidos de estenosis hipertrófica del píloro (EHP). PACIENTES Y METODOS: Estudio retrospectivo de 109 pacientes intervenidos de EHP en 5 años. Se analiza mediante el test de ji al cuadrado si existe relación entre las horas de evolución, la edad y los vómitos y reflujo gastroesofágico (RGE) postoperatorios, con el grosor muscular pilórico (grupo A > 4,5 mm y grupo B ≤ 4,5 mm) y la longitud del canal pilórico (grupo A ≥ 20 mm y grupo B < 20 mm). RESULTADOS: Existe una relación estadísticamente significativa entre el mayor tiempo de evolución (> 48 h) y la longitud del píloro ≥ 20 mm y entre la edad (> 30 días) y el grosor muscular > 4,5 mm, con una p= 0,022 y p= 0,009, respectivamente. Asimismo, de los 109 pacientes, el 38,5% presentó algún vómito durante los 2 días posteriores a la intervención y el 13,8% presentó RGE durante un tiempo medio de 8,27 meses (1-12 meses), demostrándose que existe relación entre una longitud pilórica ≥ 20 mm y la presencia de vómitos postoperatorios, siendo estadísticamente significativo (p= 0,007). No encontramos una relación estadísticamente significativa entre el grosor y la longitud del píloro con el RGE. CONCLUSIONES: Se ha observado que existe relación entre una longitud del canal pilórico ≥ 20 mm y los vómitos postoperatorios, sin embargo, no se ha observado relación con el grosor muscular pilórico. Pese a ello, un mayor tiempo de evolución y una edad > 30 días sí están relacionados con la longitud ≥ 20 mm y el grosor muscular > 4,5 mm, respectivamente.

Does metabolic alkalosis influence cerebral oxygenation in infantile hypertrophic pyloric stenosis?

BACKGROUND: This pilot study focuses on regional tissue oxygenation (rSO2) in patients with infantile hypertrophic pyloric stenosis in a perioperative setting. To investigate the influence of enhanced metabolic alkalosis (MA) on cerebral (c-rSO2) and renal (r-rSO2) tissue oxygenation, two-site near-infrared spectroscopy (NIRS) technology was applied. MATERIALS AND METHODS: Perioperative c-rSO2, r-rSO2, capillary blood gases, and electrolytes from 12 infants were retrospectively compared before and after correction of MA at admission (T1), before surgery (T2), and after surgery (T3). RESULTS: Correction of MA was associated with an alteration of cerebral oxygenation without affecting renal oxygenation. When compared to T1, 5-min mean (± standard deviation) c-rSO2 increased after correction of MA at T2 (72.74 ± 4.60% versus 77.89 ± 5.84%; P = 0.058), reaching significance at T3 (80.79 ± 5.29%; P = 0.003). Furthermore, relative 30-min c-rSO2 values at first 3 h of metabolic compensation were significantly lowered compared with postsurgical states at 16 and 24 h. Cerebral oxygenation was positively correlated with levels of sodium (r = 0.37; P = 0.03) and inversely correlated with levels of bicarbonate (r = -0.34; P = 0.05) and base excess (r = -0.36; P = 0.04). Analysis of preoperative and postoperative cerebral and renal hypoxic burden yielded no differences. However, a negative correlation (r = -0.40; P = 0.03) regarding hematocrite and mean r-rSO2, indirectly indicative of an increased renal blood flow under hemodilution, was obtained. CONCLUSIONS: NIRS seems suitable for the detection of a transiently impaired cerebral oxygenation under state of pronounced MA in infants with infantile hypertrophic pyloric stenosis. Correction of MA led to normalization of c-rSO2. NIRS technology constitutes a promising tool for optimizing perioperative management, especially in the context of a possible diminished neurodevelopmental outcome after pyloromyotomy.

Excessive Bright Echoes Sign for Hypertrophic Pyloric Stenosis Suggest the Diagnosis: Gastric Pneumatosis and Portal Venous Gas in Infants Suggest HPS.

We describe a new finding, the "excessive bright echoes" sign, for the diagnosis of hypertrophic pyloric stenosis (HPS). Portal venous gas and gastric wall pneumatosis were noted in 4 vomiting infants proven to have HPS. Portal venous gas can be concerning for ischemic bowel. Gastric wall pneumatosis can be seen in association with necrotizing enterocolitis and has been associated with increased gastric pressure from severe, usually proximal, bowel obstruction. Our HPS cases had prominent bright punctate echoes on sonography of the liver, portal vein lumen, and gastric wall. Knowledge of this excessive bright echoes sign suggests the need for sonography of the antropyloric area. One should consider HPS as a differential diagnostic possibility when the combination of bright echoes within the liver parenchyma, consistent with portal venous gas, and bright echoes in the gastric wall, consistent with gastric pneumatosis, are seen.

Infantile hypertrophic pyloric stenosis with unusual presentations in Sagamu, Nigeria: a case report and review of the literature.

A 24-day old female Nigerian neonate presented with protracted vomiting, fever and dehydration but without palpable abdominal tumour or visible gastric peristalsis. There was no derangement of serum electrolytes. The initial working diagnosis was Late-Onset Sepsis but abdominal ultrasonography showed features consistent with the diagnosis of IHPS. This case report highlights the atypical presentation of this surgical condition and the need to investigate cases of protracted vomiting in the newborn with at least, ultrasonography to minimize complications and reduce the risk of mortality in a resource-poor setting.

PRIMARY HYPERTROPHIC PYLORIC STENOSIS IN ADULTS.

THE AIM: to present some information-about the rare primary hypertrophy of the pylorus in adults and a clinical case of a patient with this disease. MATERIALS AND METHODS: the patient A., 52 years of age, after which clinical-instrumental and laboratory research was diagnosed with infiltrative form of cancer of the pylorus with decompensated stenosis. The used: videothoracoscopy, fluoroscopy, peripheral computer electrogastrogram, local fluorescence spectroscopy. The General blood and urine tests, biochemical blood tests, PCR for the determination of tumor markers. RESULTS: The resection of 2/3 of the stomach Billroth I. histological examination of the pylorus diagnosed with hypertrophy of circular muscle fibers, to a lesser extent, hypertro- phy of the longitudinal fibers with inflammatory changes. CONCLUSION: in cases of suspected infiltrative form of cancer of the pylorus in adults differential diagnosis it is advisable to include the primary hypertrophy of his.

The use of ultrasonography in infantile hypertrophic pyloric stenosis: does the patient's age and weight affect pyloric size and pyloric ratio?

AIMS: We aimed to obtain pyloric measurements of our patients with infantile hypertrophic pyloric stenosis (IHPS) using ultrasonography (US) and to evaluate the correlations between age, weight and pyloric size, pyloric ratio (PR). MATERIAL AND METHODS: We designed a retrospective study including 20 term infants with surgically proven IHPS and studied the ultrasonographically obtained pyloric muscle thickness (PMT), pyloric diameter (width) (PD), pyloric length (PL) and PR (PMT/PD) to determine if there were statistically significant associations between patient age/weight and pyloric measurements. RESULTS: The mean age of the infants was 38.7+/-17.3 days (range, 9-76 days) and their mean weight was 3688.5+/-772.7 g (range, 2810-6000 g), at referral. Mean PMT was 4.98+/-1.04 mm (range, 3.5-6.8 mm). Mean PD was 14.04+/-2.39 mm (range, 10-18 mm). Mean PL was 22.16+/-4.02 mm (range, 16-31.5 mm) and mean PR was 0.35+/-0.04 (range, 0.29-0.42). The correlation between age and PMT (r=0.654, p<0.05) and the correlation between age and PD (r=0.747, p <0.05) were significant. Age and weight were not significantly correlated with PR (p>0.05). CONCLUSIONS: The PMT and PD are age dependent parameters. The PR is age and weight independent and therefore, when combined with PMT, PD and PL, it can be useful in the diagnosis of IHPS in infants with early onset disease and/or in those with a lower weight.

Infantile hypertrophic pyloric stenosis (IHPS): a study of its pathophysiology utilizing the newborn hph-1 mouse model of the disease.

Infantile hypertrophic pyloric stenosis (IHPS) is a common disease of unknown etiology. The tetrahydrobiopterin (BH4)-deficient hyperphenylalaninemia-1 (hph-1) newborn mouse has a similar phenotype to the human condition. For hph-1 and wild-type control animals, pyloric tissue agonist-induced contractile properties, reactive oxygen species (ROS) generation, cGMP, neuronal nitric oxide synthase (nNOS) content, and Rho-associated protein kinase 2 (ROCK-2) expression and activity were evaluated. Primary pyloric smooth muscle cells from wild-type newborn animals were utilized to evaluate the effect of BH4 deficiency. One-week-old hph-1 mice exhibited a fourfold increase (P < 0.01) in the pyloric sphincter muscle contraction magnitude but similar relaxation values when compared with wild-type animals. The pyloric tissue nNOS expression and cGMP content were decreased, whereas the rate of nNOS uncoupling increased (P < 0.01) in 1-wk-old hph-1 mice when compared with wild-type animals. These changes were associated with increased pyloric tissue ROS generation and elevated ROCK-2 expression/activity (P < 0.05). At 1-3 days of age and during adulthood, the gastric emptying rate of the hph-1 mice was not altered, and there were no genotype differences in pyloric tissue ROS generation, nNOS expression, or ROCK-2 activity. BH4 inhibition in pyloric smooth muscle cells resulted in increased ROS generation (P < 0.01) and ROCK-2 activity (P < 0.05). Oxidative stress upregulated ROCK-2 activity in pyloric tissue, but no changes were observed in newborn fundal tissue in vitro. We conclude that ROS-induced upregulation of ROCK-2 expression accounts for the increased pyloric sphincter tone and nNOS downregulation in the newborn hph-1 mice. The role of ROCK-2 activation in the pathogenesis of IHPS warrants further study.

Transient hypertrophic pyloric stenosis due to prostoglandin infusion.

Prostaglandin E1 (PGE1) is widely used in ductus-dependant congenital heart disease to maintain the patency of ductus. Hypertrophic pyloric stenosis (HPS) due to gastric mucosal proliferation is a rare complication of prolonged PGE infusion. A male newborn who developed HPS during PGE1 infusion is presented to discuss the clinical features and treatment modalities of PGE-related transient HPS. The boy was 2500 g and born at 35 weeks of gestation from a 23-year-old mother. He was admitted to neonatal intensive care with breathing difficulty and cyanosis. His echocardiography revealed pulmonary atresia, ventricular septal defect and major aorta-pulmonary collateral (MAPCA). PGE infusion with a dose of 0.05 mcg kg⁻¹ was initiated. At the 8th day of infusion, he developed non-billous vomiting. Ultrasonographic evaluation revealed 1.9 cm length of pyloric channel and 0.5 cm of wall thickness on 11th day and diagnosed as HPS. On 42th postnatal day, he underwent MAPCA closure, right modified Blalock-Taussi shunt and repair of pulmonary artery bifurcation with bovine patch. PGE infusion was stopped and enteral nutrition was started on 8th postoperative day. Control ultrasonography on 12th postoperative day revealed normal pyloric channel length (0.9 cm) and wall thickness (0.3 cm). Prolonged use of PGE infusion in neonates with congenital heart disease may cause transient HPS. The clinical and radiological features of HPS relieves after stopping PGE infusion. It should be kept in mind that HPS due to PGE infusion can be transient and pyloromyotomy should be kept for patients with persistent findings.

Reestenosis hipertófica de píloro tras piloromiotomía, ¿recurrencia verdadera o complicación posquirúrgica? / Recurrent Infantile hypertrophic pyloric stenosis after pyloromyotomy: progression of original disease or delayed surgical complication?

La recurrencia de estenosis hipertrófica de píloro es una entidad rara. Debe sospecharse ante la aparición de vómitos proyectivos tras un periodo postoperatorio sin incidencias por piloromiotomía previa. Suele obedecer a cirugía anterior insuficiente o a lesión duodenal intraoperatoria y habitualmente precisa nueva cirugía reparadora. Se presentan dos casos de reestenosis hipertrófica de píloro, y se discute la naturaleza de esta estidad (AU)

Recurrent Infantile hypertrophic pyloric stenosis is a rare condition. It must be suspected if new projectile vomiting reoccurs after an uneventful postoperative period. It is usually caused by incomplete previous surgery or accidental duodenal injury during Ramstedt’s procedure. In general a new pyloromyotomy is required. Two cases of hypertrophic pyloric restenosis are presented, and the nature of this condition is discussed (AU)

Evaluation of hypertrophic pyloric stenosis by pediatric emergency physician sonography.

OBJECTIVES: The objective was to evaluate the accuracy of pediatric emergency physician (EP) sonography for infants with suspected hypertrophic pyloric stenosis (HPS). METHODS: This was a prospective observational pilot study in an urban academic pediatric emergency department (PED). Patients were selected if the treating physician ordered an ultrasound (US) in the department of radiology for the evaluation of suspected HPS. RESULTS: Sixty-seven patients were enrolled from August 2009 through April 2012. When identifying the pylorus, pediatric EPs correctly identified all 10 positive cases, with a sensitivity of 100% (95% confidence interval [CI] = 62% to 100%) and specificity of 100% (95% CI = 92% to 100%). There was no statistical difference between the measurements obtained by pediatric EPs and radiology staff for pyloric muscle width or length (p = 0.5 and p = 0.79, respectively). CONCLUSIONS: Trained pediatric EPs can accurately assess the pylorus with US in the evaluation of HPS with good specificity.

Cambios epidemiológicos y diagnósticos en la estenosis hipertrófica de píloro / Changes in epidemiology and diagnosis of hypertrophic pyloric stenosis

Introducción: La estenosis hipertrófica de píloro es una de las causas más frecuentes de vómitos en los lactantes, con una importante repercusión clínica y metabólica, y más acentuados según progresa el tiempo de evolución. El objetivo de este estudio es detectar los cambios en su evolución epidemiológica y diagnóstica a lo largo de una década. Pacientes y métodos: Estudio descriptivo y retrospectivo de 141 pacientes tratados en nuestro hospital entre 2000 y 2009, ambos años inclusive. Se recogieron diversas variables epidemiológicas, clínicas y analíticas, y se compararon dos periodos elegidos de forma aleatoria, 2000-2004 y 2005-2009, para detectar posibles cambios. Resultados: La incidencia del 1,92‰ de recién nacidos vivos se mantuvo estable en el tiempo, con una relación entre varones y mujeres de 6:1. No se observaron cambios en el porcentaje de pérdida de peso de los pacientes. Sin embargo, se detectó un descenso del 33% en la edad en el momento del diagnóstico, así como un aumento de los pacientes diagnosticados con menos de 5 días de evolución. La disminución de las medidas ecográficas correspondientes a la longitud y el espesor de la pared indican un descenso en el diagnóstico de píloros de gran tamaño y, por tanto, más evolucionados. En cuanto a los parámetros analíticos estudiados, sólo la creatinina presenta una disminución de sus valores. Conclusiones: El diagnóstico de la estenosis hipertrófica de píloro ha experimentado una cierta anticipación. Los pacientes se diagnostican a una edad y con un tiempo de evolución menores y, por tanto, presentan una menor alteración clínica y analítica. El diagnóstico ecográfico ha desplazado de forma significativa al diagnóstico clínico (AU)

Introduction: Hypertrophic pyloric stenosis is one of the most common causes of vomiting in infants with significant clinical and metabolic impact, more pronounced as time progresses. The objective of this study is to detect epidemiological changes and diagnostic development of hypertrophic pyloric stenosis, over a decade. Patients and methods: Retrospective descriptive study of 141 patients treated in our hospital between 2000 and 2009, inclusive. Several epidemiological, clinical and laboratory variables were collected and compared between two periods of time chosen at random to detect changes: 2000-2004 and 2005-2009. Results: The incidence of 1.92 ‰ live births, remained stable over time, with a male: female ratio of 6:1. There were also no changes in the porcentage of patient weight loss. However, we detected a decrease of 33% in the age of infants at the time of diagnosis as well as an increase of patients with less than 5 days lasting symptoms. The decrease of ultrasound measurements, lenght and wall thickness lead to a decline in the diagnosis of large and therefore more evolved pylori. As for the analytical parameters studied, we only appreciated a decrease in creatinine values. Conclusions: The diagnosis of hypertrophic pyloric stenosis has experienced an anticipation. Patients are diagnosed younger with less hypertrophied pylorus and therefore less clinical and laboratory alteration. Diagnosis has shifted significantly from clinical to the ultrasound one (AU)

Infantile hypertrophic pyloric stenosis--genetics and syndromes.

Infantile hypertrophic pyloric stenosis (IHPS) is a common condition in neonates that is characterized by an acquired narrowing of the pylorus. The aetiology of isolated IHPS is still largely unknown. Classic genetic studies have demonstrated an increased risk in families of affected infants. Several genetic studies in groups of individuals with isolated IHPS have identified chromosomal regions linked to the condition; however, these associations could usually not be confirmed in subsequent cohorts, suggesting considerable genetic heterogeneity. IHPS is associated with many clinical syndromes that have known causative mutations. Patients with syndromes associated with IHPS can be considered as having an extreme phenotype of IHPS and studying these patients will be instrumental in finding causes of isolated IHPS. Possible pathways in syndromic IHPS include: (neuro)muscular disorders; connective tissue disorders; metabolic disorders; intracellular signalling pathway disturbances; intercellular communication disturbances; ciliopathies; DNA-repair disturbances; transcription regulation disorders; MAPK-pathway disturbances; lymphatic abnormalities; and environmental factors. Future research should focus on linkage analysis and next-generation molecular techniques in well-defined families with multiple affected members. Studies will have an increased chance of success if detailed phenotyping is applied and if knowledge about the various possible causative pathways is used in evaluating results.

Can the duration of vomiting predict postoperative outcomes in hypertrophic pyloric stenosis?

BACKGROUND AND OBJECTIVES: Hypertrophic pyloric stenosis (HPS) is a common cause of gastric outlet obstruction (GOO) in infants. Prolonged GOO is believed to result in acid and electrolyte disturbances, gastric atony, and delayed postoperative recovery. We studied the impact of prolonged vomiting as an indicator of GOO symptoms on the post-operative outcomes in HPS. DESIGN AND SETTING: A retrospective chart review of all patients who underwent pyloromyotomy at a tertiary care center between February 1997 and February 2009. PATIENTS AND METHODS: The duration of pre-operative vomiting was correlated with presenting electrolytes and acid-base balances, postoperative time to full feed, postoperative morbidity and duration of hospitalization. RESULTS: Forty-seven patients were identified. At presentation, the median (range) for duration of symptoms was 14 (3-60) days, and surgeries were performed at 2 (0-6) days after admission. Apart from one case of postoperative wound infection, all patients had an unremarkable recovery. The unusually prolonged duration of vomiting in our cohort did not correlate with the mean (SD) preoperative chloride level of 93.9 (8.8) mEq/L, mean (SD) pH level of 7.5 (0.9), mean postoperative time to full feeding of 31 (15.1) hours, or mean duration of hospitalization of 5.1 (2.2) days. CONCLUSION: Duration of vomiting in HPS at presentation does not seem to have a significant impact on the postoperative outcomes.