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Correlation of prostaglandin E2 production and gastric acid secretion in infants with hypertrophic pyloric stenosis.

PURPOSE: The aim of this study was to evaluate the relationship between gastric acid secretion and prostaglandin E2 (PGE2) generation in gastric mucosa and the role of PGE2 in the pathogenesis of hypertrophic pyloric stenosis (HPS). METHODS: The authors measured the levels of PGE2 and gastric acidity in the gastric juice of HPS patients before and after a Ramstedt operation. RESULTS: The PGE2 concentration in the gastric juice of nine HPS patients before the Ramstedt operation was significantly higher than that of eight controls, and the concentration significantly decreased after the operation. A significant inverse correlation between the PGE2 concentration and the pH level in the gastric juice was shown in HPS patients before and after the operation. CONCLUSION: These results suggest that the enhanced generation of PGE2 in gastric mucosa in cases of HPS is a secondary phenomenon caused by hyperacidity and is not responsible for the pathogenesis of HPS.

Dinoprostone↗

Infantile hypertrophic pyloric stenosis after prenatal exposure to thalidomide.

In a retrospective study of 832 cases of thalidomide embryopathy (ThE) between October 1, 1959 and July 31, 1962, a highly significant accumulation of cases with infantile hypertrophic pyloric stenosis (IHPS) was registrated. Clinical course, X-ray and surgical findings and the sex ratio (male preponderance) were identical to IHPS occurring spontaneously. In the order of frequency of defects in ThE, IHPS is on position 11; among inner organ abnormalities, IHPS is on position 3 after heart and kidney defects. Thus, IHPS is the predominant gastrointestinal abnormality in ThE. For the first time, a substance (thalidomide) could be identified which obviously is able to influence manifestation of IHPS. There is a remarkable coincidence of IHPS and malformations such as hiatus hernia and tracheo-oesophageal fistula with or without oesophagus atresia. From the male preponderance, which is also observed in the ThE type of IHPS, it is concluded that thalidomide is not a primary cause in this process, but that disease manifestation is decisively influenced by thalidomide on the base of a genetic or familiar predisposition. Cause and development of IHPS are multifactorial; the mode of inheritance is polygenic. Probably, other substances may replace thalidomide.

Female↗

Sonographic diagnosis of infantile hypertrophic pyloric stenosis: critical appraisal of reliability and diagnostic criteria.

Critical review of the current medical literature shows lack of complete evaluation of the parameters of "medical decision making" in the sonographic diagnosis of infantile hypertrophic pyloric stenosis (IHPS). A computer program was specially written to calculate these parameters on three published series of patients with IHPS, using originally advocated and modified sonographic criteria. The sensitivity and specificity of the sonographic tests are influenced by the chosen diagnostic criteria, while predictive values are affected by the prevalence of IHPS in the particular population studied. A change in the ventral-dorsal diameter criterion from 1.2 cm to 1.5 cm increased the sensitivity (from 0.52 to 0.97, p less than 0.0001) and negative predictive value (0.60 to 0.95, p less than 0.01) in the population of Wilson et al (9), changing the prevalence of IHPS from 0.58 to 0.12, (sensitivity, specificity and sonographic criterion kept constant) reduced the positive predictive value from 0.90 to 0.48 (p less than 0.0001). The term "accuracy" has little scientific meaning and should be discarded in comparative evaluation. These results indicate that the real contribution of sonography in decision making in patients with suspected IHPS (but without a palpable mass) has not been properly evaluated by research workers.

Evaluation Studies as Topic↗

[Wernicke's encephalopathy in chronic gastropancreatic disease with pyloric stenosis].

The acute symptomatology of Wernicke's syndrome was observed in a 17-year-old female patient and a 48-year-old male patient. They were both affected by chronic gastropancreatic disease with pyloric stenosis and showed already subclinical thiamine deficiency. Symptoms occurred within 4 weeks of an infusion regime rich in carbohydrates. Parenteral administration of high doses of thiamine (200 mg/d and 360 mg/d) led to clear-cut regression of symptoms. Due to the unfavourable prognosis of Wernicke's encephalopathy prophylactic administration of thiamine in a daily dosage of 50-100 mg is recommended in patients with chronic intestinal disease and parenteral nutrition.

Adolescent↗

A novel mutation of WT1 exon 9 in a patient with Denys-Drash syndrome and pyloric stenosis.

We report a novel mutation in WT1 exon 9 (1214 A>G) resulting in an amino acid change from H to R at codon 405 in a 46 XY female patient who had congenital hypertrophic pyloric stenosis, pseudohermaphroditism masculinus, renal failure, and Wilms tumor, and died at the age of 22 months. The patient demonstrated the difficulty in diagnosing a patient with intersex before conclusive genetic characterization.

Denys-Drash Syndrome↗

Diagnostic aids in the differentiation of pyloric stenosis from severe gastroesophageal reflux during early infancy: the utility of serum bicarbonate and serum chloride.

This study evaluated whether serum bicarbonate levels, serum chloride levels, and other diagnostic criteria could be used to differentiate pyloric stenosis (PS) from severe gastroesophageal reflux (GER) during early infancy. The investigation was a retrospective, case-control study conducted in the emergency department of a large, academic children's hospital. Cases were 75 consecutive infants with PS confirmed in the operating room. Controls were 75 consecutive infants 12 weeks of age or younger with the diagnosis of GER whose serum electrolytes had been examined. Projectile vomiting was sensitive (0.93) but not specific (0.39) for PS. The mean serum bicarbonate level was 27.2 mmol/L for PS patients and 22.3 mmol/L for GER patients (P < .00001), and the mean serum chloride level was 95.7 mmol/L and 103.6 mmol/L for PS patients and GER patients, respectively (P < .00001). Serum bicarbonate levels of > or =29 mmol/L and serum chloride levels of < or =98 mmol/L had high positive predictive values (0.96 and 0.97, respectively) and were specific (0.99 for both) but not very sensitive (0.36 and 0.50, respectively) in identifying patients with PS. Only one patient would have been misclassified (false positive) as having PS using either of these cutoff values. These laboratory tests can also help discriminate between PS and GER when the history and physical examination fail to do so. For example, of the 20 patients with PS who did not have a pyloric mass palpated, 3 (15%) had serum bicarbonate levels of > or =29 mmol/L, and 6 (30%) had serum chloride levels of < or =98 mmol/L. In conclusion, the serum bicarbonate or serum chloride level offers a useful additional diagnostic tool in the evaluation of children presenting during early infancy with vomiting of uncertain etiology.

Bicarbonates↗

Streamlining the care of patients with hypertrophic pyloric stenosis: application of a clinical pathway.

PURPOSE: The purpose of this study was to evaluate the impact of a clinical pathway on infants admitted to a pediatric tertiary care center with the diagnosis of hypertrophic pyloric stenosis (HPS). METHODS: The records of 132 HPS patients were evaluated before and after implementation of a clinical pathway. Infants were excluded for prematurity, admission to nonsurgical services, or multiple diagnoses requiring prolonged hospitalization, resulting in 83 patients for analysis. Group I (prepathway, n = 40) and group II (postpathway, n = 43) infants were analyzed for time from admission to operation, operation to first feeding, operation to discharge, total length of stay, hospital charges, metabolic status at time of admission, and postoperative complications. The Mann-Whitney test was performed (statistical significance at P <.05). RESULTS: There was no significant difference between group I and group II patients in the length of preoperative hospitalization or metabolic status at the time of hospital admission. In comparison with group I patients, there was a significant reduction in time to resumption of oral feedings (4.6 +/- 1.9 hours v 7.5 +/- 3.2 hours; P <.001) for group II infants and a significantly earlier discharge (26.7 +/- 6.8 hours v 38.0 +/- 11.7 hours; P <.001). This resulted in a shortened length of stay (41.8 +/- 9.7 hours v 57.8 +/- 14.3 hours; P <.001) with an associated decrease in hospital charges ($4,555 +/- $464 v $5,400 +/- $1,017; P <.001). CONCLUSIONS: Elimination of practice variability by the use of a clinical pathway for HPS resulted in significant reduction of hospital stay and related charges. The impact of the pathway occurred in the postoperative period and is a consequence of a rapid and systematic return to oral feedings.

Breast Feeding↗

Hypertrophic pyloric stenosis: volumetric measurement of nasogastric aspirate to determine the imaging modality.

The authors postulated that volumetric measurement of residual gastric aspirate in neonates and infants with nonbilious projectile vomiting could enable differentiation between patients with hypertrophic pyloric stenosis (HPS) and those with gastroesophageal reflux (GER) and help to determine whether ultrasound (US) or fluoroscopy of the upper gastrointestinal tract would best confirm the diagnosis. In the 38 patients (all but two of whom had been fasting for 3-4 hours), 10 mL or more of nasogastric aspirate was considered indicative of obstruction. HPS occurred in 91.7% of patients with 10 mL of aspirate or more, whereas GER occurred in 85.7% of patients with less than 10 mL. The differences between the two groups were statistically significant. Solely on the basis of residual volume (greater than or equal to 10 mL), the cause of vomiting could be differentiated, prior to standard radiologic studies, 89.4% of the time. It is concluded that patients with projectile vomiting who have 10 mL or more of residual aspirate in the stomach should undergo US for confirmation of HPS; those with less than 10 mL should undergo fluoroscopy for confirmation of GER.

Diagnosis, Differential↗

Duplication of the hepatopancreatic bud presenting as pyloric stenosis.

Duplication of systemic organs is not a frequent finding. We report the occurrence of duplication of the hepatopancreatic bud with presence of mature hepatic and pancreatic tissue in a nine-day-old girl who presented with intestinal obstruction mimicking infantile hypertrophic pyloric stenosis.

Diagnosis, Differential↗

A statistical analysis of birth-order effects with application to data on pyloric stenosis.

A method is presented for correcting incomplete data for birth-order analysis and variations in the ascertained incidence of affected individuals are allowed. The statistical analysis involves application of a test developed by Wilcoxon, Mann and Whitney, and Haldane and Smith. Examples are detailed for data correction, and the analysis of data for pyloric stenosis is discussed.

Birth Order↗

Adult hypertrophic pyloric stenosis--a description in 1834?

John Peacock MD was in practice in Darlington when he published his Practical Hints on the Treatment of Several Diseases in 1834. It is suggested that his cases described therein of ;scirrhous pylorus' are adult hypertrophic pyloric stenosis. If authenticated, his work would predate the earliest description of this condition by the French pathologist, Professor Jean Cruveilhier in 1835.

England↗

Endoscopic hydrostatic balloon dilation of ulcer-induced pyloric stenosis in rheumatoid arthritis and secondary amyloidosis.

We describe a 50-year-old Japanese woman with rheumatoid arthritis who presented with near-complete gastric outlet obstruction. The patient also suffered from secondary gastrointestinal and cardiac amyloidosis. Gastroscopy revealed multiple huge gastric antral ulcers in which amyloid deposits were identified on histologic examination. The ulcers became scars after treatment with omeprazole, which cause in severe pyloric stenosis. Endoscopic hydrostatic balloon dilation under fluoroscopic guidance was performed twice for 10 min. The pyloric outlet remained sufficiently patent 22 months later.

Amyloidosis↗

Serum electrolytes and capillary blood gases in the management of hypertrophic pyloric stenosis.

The purpose of this study was to assess whether the concentration of serum chloride and other variables, namely serum sodium, potassium and bicarbonate, can be used to predict metabolic acid-base status in infants with hypertrophic pyloric stenosis (HPS) and to assess whether such a prediction is influenced by the state of hydration. One hundred and sixty-three infants with HPS who had at least one set of serum electrolyte and capillary blood gas estimations performed after admission were studied retrospectively. A further 25 infants who had their electrolyte and blood gases measured after at least 12 h of rehydration and correction of the serum sodium and chloride abnormalities were studied prospectively. Stepwise multiple regression analysis, using standard bicarbonate as the dependent variable, revealed serum chloride concentration to be the most powerful independent predictor of standard bicarbonate level (r = -0.69, P less than 0.0001). Other variables did not improve the correlation significantly. In the retrospective study of untreated patients, the calculation of a 90 per cent prediction interval for the model indicated that if the serum chloride level is less than 96 mmol/l, one could be 95 per cent confident (one tail) that the patient was alkalaemic. To be 95 per cent confident that the patient was not alkalaemic, the serum chloride level would have to be greater than 121 mmol/l. The prospective study found that following rehydration a serum chloride level greater than or equal to 106 mmol/l more accurately predicted absence of alkalaemia. We conclude that predictability of acid-base status from measurement of serum chloride depends on the state of hydration of the patient.

Acid-Base Imbalance↗

Reliability of ultrasound for the diagnosis of hypertrophic pyloric stenosis.

An ultrasound (US) examination might be the best way to study the pylorus and its pathological states once the reliability of this technique has been demonstrated. In order to evaluate the reliability of a sonographic diagnosis of hypertrophic pyloric stenosis (HPS), 17 randomly chosen patients with HPS were examined with both ultrasonography and radiography and then operated on or followed up clinically. Rank correlation between the size and morphological features of the pylorus was found in babies with HPS using US. No false positives or negatives were obtained, and all true positives and negatives were detected. Because of this and the safety of the US technique we wish to emphasize that it should be considered the method of choice in screening infants with severe vomiting. Whenever HPS is suspected from clinical observation, ultrasonography should precede a radiological examination before surgery.

Female↗

Caudal block with 4 mg x kg-1 (1.6 ml x kg-1) of bupivacaine 0.25% in children undergoing surgical correction of congenital pyloric stenosis.

BACKGROUND: Since 1970, bupivacaine 0.25% in a dose of 4 mg x kg-1 (1.6 ml x kg-1) has been used at the Hospital Infantil de México for caudal block in children undergoing surgical correction of congenital pyloric stenosis (CPS). Although this dose is considered unsafe, in our experience, it has been associated with a high success rate and a low incidence of adverse events. This experience has not been previously documented. METHODS: A retrospective cohort of patients undergoing surgical correction of CPS was studied. Nineteen patients received general anaesthesia while 223 received caudal block. The latter were then grouped according to the sedation technique. The rate of successful caudal blocks and complications were considered the major outcomes of the study, whereas the postsurgical fasting period and hospital stay were considered secondary outcomes. RESULTS: The rate of success of caudal block was 96%. Anaesthetic complications related to bupivacaine were present in 1.3%. Mortality occurred in the postoperatory period in one septic patient who also was suffering from gastroschisis that required general anaesthesia. Postoperatory fasting period and hospital stay tended to be higher with general anaesthesia than caudal block. However, of the 19 patients receiving general anaesthesia, five suffered serious comorbidity and nine were failed caudal blocks. CONCLUSIONS: Caudal block with bupivacaine 0.25% (4 mg x kg-1) was associated with a low rate of anaesthetic complications. Further prospective studies to clarify the risks and benefits are required.

Anesthesia, Caudal↗

Changing trends in the management of infantile hypertrophic pyloric stenosis--an audit over 11 years.

BACKGROUND: This article is a follow-up to an audit performed by the Department of Surgery and published in the Irish Journal of Medical Science in 1996. This audit reviewed all cases of Infantile Hypertrophic Pyloric Stenosis (IHPS) operated on over 22 years up to 1991. AIMS: We aim to demonstrate that radiologic investigations, namely barium meal and ultrasound, have been increasingly employed in the diagnosis of IHPS. In addition, ultrasound is now the investigation of choice. METHODS: We have reviewed all cases of IHPS, at the same institution, over the subsequent 11 years, with reference to any radiological investigations performed. In the previous study, the diagnosis of IHPS was made clinically in 92.6% with the remainder diagnosed radiologically. RESULTS: Over 11 years, 157 patients were diagnosed with IHPS. Male to female ratio was 4.06:1. Median age was four weeks (range 1-18 weeks).Twenty-four per cent had a barium meal, 36% had an ultrasound and 13% had both performed. CONCLUSION: We conclude a change in practice in the management of IHPS with radiology, particularly ultrasound, playing an increasing role.

Female↗