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Clinical presentation of pyloric stenosis: the change is in our hands.

BACKGROUND: Hypertrophic pyloric stenosis classically presents as projectile vomiting during the third to fourth week of life, associated with good appetite. Additional classical presenting findings include palpation of the pyloric tumor, described as olive-shaped; a visible gastric peristaltic wave after feeding; and hypochloremic, hypokalemic metabolic alkalosis. It was recently claimed that this presentation has changed due to the easier access to gastrointestinal imaging. OBJECTIVE: To validate this contention and discuss possible reasons. METHODS: We conducted a retrospective chart review of all patients who underwent pyloromyotomy for HPS between 1990 and 2000. Only patients with confirmed HPS at the time of surgery were included. We also performed a comprehensive review of older studies for comparison. RESULTS: Seventy patients underwent pyloromyotomy over the 10 year period. Overall, 81% of patients were male infants and the mean age at diagnosis was 40 days. The mean duration of symptoms was 8 days. A firstborn child was noted in 43% of the cases. The classical symptom of projectile vomiting was absent in one-third of the patients, a pyloric tumor was not palpated in one-half of the cases, bicarbonate was higher than 28 mEq/L in 20%, and a pH of above 7.45 was present in 25% of patients. Hypochloremia was noted in about one-third. We found a good correlation between ultrasonographic width and length of the pylorus and the intraoperative findings. Pylorus length +/- 24 mm correlated with significantly longer duration of symptoms. When compared with previous studies, the main findings were not significantly different; namely, mean age at diagnosis, percentage of male gender, and duration to diagnosis. The decrease in the number of pyloric tumors palpated paralleled the increase in the use of upper gastrointestinal series and ultrasonography in particular. CONCLUSIONS: The clinical presentation of HPS has not actually changed despite the easier accessibility of GI imaging studies. However, the one significant change is the low percentage of pyloric tumors palpated, probably due to declining clinical skills accompanied by earlier utilization of imaging studies. The use of imaging and laboratory studies did not change the age at diagnosis but may have shortened the time for diagnosis and reduced the postoperative stay. Imaging and laboratory studies may be helpful for the subgroup with a non-classical clinical presentation.

Digestive System Surgical Procedures↗

Management and ultrasonographic appearance of infantile hypertrophic pyloric stenosis with intravenous atropine sulfate.

Some infants with hypertrophic pyloric stenosis (HPS) have responded to oral atropine treatment. To achieve sufficient effect of atropine, it must be administered intravenously (i.v.). Therefore, with ultrasonography, we studied the changes in the pyloric muscle in HPS during and after intravenous administration of atropine. Twenty-three infants were studied. Atropine sulfate was initially administered at a dose of 0.04 mg/kg day i.v., and the dose was increased by 0.01 mg/kg/day until vomiting ceased. When vomiting ceased after administration of intravenous atropine sulfate, the infants received oral atropine sulfate at twice the effective intravenous dose; this was continued for 2 weeks. Ultrasonography was repeated until pyloric muscles normalized. Twenty-two infants were free from vomiting after 1-8 days of intravenous atropine sulfate (dosages of 0.04-0.11 mg/kg/day). In 21 infants, weight gain continued after atropine treatment even though no change in thickness of the pyloric muscles was demonstrated ultrasonographically. Only 2 infants required pyloromyotomy because of prolonged treatment or a mistake in underdosing of oral atropine. All of the 21 infants who recovered after intravenous atropine without surgery had normalization of pyloric muscle caliber, as shown by ultrasonography 4-12 months after treatment. Atropine is an effective medicine for HPS. Regression of pyloric thickening after vomiting has been controlled implies that pyloric muscle hypertrophy could be worsened by the spasm that occurs in HPS.

Administration, Oral↗

Junctional epidermolysis bullosa with pyloric stenosis presenting with electron microscopic findings suggestive of epidermolysis bullosa simplex.

We present an infant girl who was born with pyloric stenosis and epidermolysis bullosa (EB). Electron microscopy of a skin biopsy specimen showed findings suggestive of EB simplex, but immunofluorescence (IF) mapping of the same specimen established the diagnosis of junctional EB. Because electron microscopy findings may sometimes be misleading, an EB patient with pyloric stenosis and electron microscopy findings suggestive of EB simplex should have a biopsy specimen examined by immunofluorescence mapping, which may confirm that the patient in fact has junctional EB.

Biopsy↗

Fluid resuscitation in infantile hypertrophic pyloric stenosis.

UNLABELLED: The purpose of this analysis was to investigate biochemical disturbances at presentation and initial fluid resuscitation before surgery in infantile pyloric stenosis. The charts of 139 consecutive infants (113 boys and 26 girls) between 7 d and 20 wk of age with hypertrophic pyloric stenosis were reviewed. The infants were treated at the Department of Pediatric Surgery, University of Bern, Switzerland, in the period between 1987 and 1997. A trend towards hypokalaemia (13 of the 139 patients), hypochloraemia (39 patients) and especially metabolic alkalosis (98 patients) was frequently noted on admission. In 84 patients, data on fluid management and on circulating sodium, potassium, chloride and the acid-base balance immediately before surgery were also available. In these patients a significant correlation was found between the parenteral chloride dose given for fluid repair (y = 0.310 x; r = 0.54; p < 0.001) and the changes in plasma bicarbonate. The equation indicates that a chloride dose of 10 mmol/kg body weight is required to reduce plasma bicarbonate on average by 3 mmol/. CONCLUSION: Since assessment of the fluid volume stated by physical examination and history is inaccurate in infants with vomiting, the severity of metabolic alkalosis helps to define the amount of fluid required for repair.

Alkalosis↗

Laser recanalization of pyloric stenosis: a guidewire-directed contact probe technique.

A variety of techniques has been used to relieve the obstructive symptoms of pyloric stenosis. Endoscopic dilatation using hydrostatic balloon dilators, multiple-diameter bougies, and electrocautery with a sphincterotome have been described. The neodymium yttrium aluminum garnet laser also has been used, with both noncontact and contact probes. We describe a new technique using a guidewire-directed contact probe for laser recanalization of pyloric stenosis in a patient with radiation-induced gastric outlet obstruction.

Aged↗

Infantile hypertrophic pyloric stenosis: a fresh approach to the diagnosis.

Infantile hypertrophic pyloric stenosis is a condition occurring in early infancy. The traditional approach to the diagnosis has been clinical, relying on the palpation of a 'tumour' caused by the muscular thickening of the pylorus. In doubtful cases a barium meal is diagnostic. Ultrasound, with the lack of hazards associated with radiation, provides an additional method of investigation which is accurate and will diagnose some cases in which no pyloric tumour is palpable. This will spare infants from a barium meal but there are still cases in which a barium meal will be necessary because a tumour cannot be palpated or demonstrated by ultrasound.

Barium Sulfate↗

Endoscopic findings in hypertrophic pyloric stenosis: appearance in classic and evolving disease.

BACKGROUND: Hypertrophic pyloric stenosis (HPS) is the most common abdominal surgical disorder in infants. Although the majority of cases are diagnosed by ultrasound, equivocal cases may require endoscopy. This study was performed to assess the various endoscopic appearances of HPS in infants. METHODS: A prospective study comparing the endoscopic appearance of the antrum and pylorus of 18 children with HPS to 21 children in a normal control group. RESULTS: Antral or pyloric mucosal hypertrophy was visualized endoscopically in all 18 study patients. The degree of mucosal thickening varied depending on the age of presentation and duration of symptoms. Antral fold hypertrophy was first noted at 10 days of age, and in the oldest patient (4 months of age) a pyloric mass was noted. By comparison, 21 control infants had no evidence of antral or pyloric narrowing or mucosal thickening. CONCLUSIONS: Upper endoscopy can be a valuable adjunctive diagnostic tool in select cases of HPS when imaging tests are inconclusive or when infants present with clinical symptoms outside the typical age-time frame for HPS. Because HPS may evolve over time, it is important that the endoscopist recognize the different appearances of HPS.

Disease Progression↗

[Sonography of hypertrophic pyloric stenosis. Statistical analysis of the values and the diagnostic criteria].

On the basis of 149 patients with the clinical suspicion of hypertrophic pyloric stenosis, sonographic diagnostic criteria were defined retrospectively. The normal ranges for the various criteria were calculated on the basis of 90 patients in whom hypertrophic pyloric stenosis had been excluded. No correlation could be found between these normal ranges and chronological age. Cut-off values between normal (n = 90) and abnormal (n = 59) were calculated by means of ROC analysis. The cut-off value of 14 mm resulted in a sensitivity of 76.9% and a specificity of 97.8% for the diameter, while the cut-off value of 18 mm gave a sensitivity of 80.3% and a specificity of 100% for the canal length. The muscle wall thickness had a sensitivity of 84.4% and a specificity of 95.5% when 4 mm was taken as the cut-off value. Combination of several parameters did not improve the results. The influence of the prevalence on the positive and negative predictive values is calculated by applying the above-mentioned cut-off values and the corresponding sensitivities and specificities. A diagnostic procedure is recommended; this is shown in the form of a flow chart for various possible prevalences.

Humans↗

Ultrastructural observations of the myenteric plexus of the pylorus in infantile hypertrophic pyloric stenosis.

Myenteric plexuses and smooth muscle of the pylorus obtained by biopsy at pyloro myotomy in 5 infants with hypertrophic pyloric stenosis were studied by electron microscopy. Animal controls consisted of pyloruses from 2 infant and 2 adult rabbits and 2 adult rats. The principal findings in the plexuses of the patients were moderate numbers of variably sized, swollen degenerating axons that contained dense bodies, lamellated figures, vacuoles, granular or fibrillar material, and swollen mitochondria. The significance of this alteration in the etiology and pathogenesis of this disorder is not clear. Neurons in the plexuses showed no definite abnormalities. Although these findings do not confirm previous light microscopic observation of neuronal changes in the pyloric myenteric plexuses, they do not exclude a neurogenic mechanism for this disorder. The presence of small immature neurons and large mature neurons suggests that neuronal maturation and development in infantile hypertrophic pyloric stenosis are not impaired as previously reported. No ultrastructural changes were found in interstitial cells of the myenteric plexuses. Except for hypertrophy of the circular smooth muscle layers, no specific alterations were found in muscle fibers of the pylorus.

Animals↗

Pyloric stenosis: is over-reliance on ultrasound scans leading to negative explorations?

AIM: To see whether increasing use of ultrasound scans in pyloric stenosis is leading to false-positive diagnoses, and even negative laparotomies. METHODS: Over a 2-year period, 76 neonates underwent laparotomy with a preoperative diagnosis of pyloric stenosis (PS). There were 57 males and 19 females, age ranged from 10 days to 7 weeks. RESULTS: Six patients proceeded straight to surgery after undergoing a test feed. The remaining 70 patients had one or more imaging investigations. Of these; 56 patients had an ultrasound only, 5 had a barium meal only while 9 patients had both investigations. Ultrasound showed evolving lesions in 2 patients. It was equivocal or falsely negative in 8 - all were diagnosed correctly after undergoing barium meals. Fifty-two patients were diagnosed correctly on ultrasound. There were, however, 3 false-positive ultrasonic diagnosis--i.e., at laparotomy the pylorus was found to be normal. One of these patients even had a "diagnostic" barium meal. The factors leading to these negative explorations are discussed. CONCLUSION: Pyloric "tumours" can be difficult to palpate early in the evolution of the disease. Reliance upon the ultrasound appearance of the pylorus without taking into account other important diagnostic evidence will increase the risk of false-positive diagnoses and unnecessary laparotomy. The importance of clinical examination and test feed is emphasised.

Barium Sulfate↗

Congenital hypertrophic pyloric stenosis in Nigeria.

Thirty one infants with congenital hypertrophic pyloric stenosis seen over a five year period (1970-1974) at the University College Hospital Ibadan are reported. Most of the patients presented late. There is a much higher male predominance but a lower prevalence among Nigerian first born infants when compared with Caucasian infants. It is suggested that the condition though uncommon is not rare in African infants, and that environmental rather than genetic or racial factors may be responsible for the apparent lower prevalence.

Female↗

Infantile hypertrophic pyloric stenosis: an infectious cause?

The aetiology of infantile hypertrophic pyloric stenosis (IHPS) remains unclear. The aim of this study was to test the hypothesis that a common bacterium, Helicobacter pylori (HP) may be implicated in the pathogenesis of IHPS. Thirty-nine consecutive infants with confirmed IHPS had their stool analysed with an enzyme immunoassay for the presence of HP. An age/sex-matched group of infants with unrelated surgical conditions were also tested. No positive results for the presence of HP stool antigen were obtained in the study nor the control group. The results of this study demonstrate no causative link between HP and IHPS. A genetic basis has been implicated for IHPS. However, evidence does exist that IHPS is a condition acquired after birth and that an infective agent may be involved in the pathogenesis. Further studies are required to elucidate perinatal factors that may induce the expression of this condition in a genetically sensitive individual.

Feces↗

Maternal and infant use of erythromycin and other macrolide antibiotics as risk factors for infantile hypertrophic pyloric stenosis.

OBJECTIVES: To evaluate the risk for infantile hypertrophic pyloric stenosis (IHPS) among infants prescribed systemic erythromycin, infants prescribed a course of erythromycin ophthalmic ointment, and infants whose mothers were prescribed a macrolide antibiotic during pregnancy. STUDY DESIGN: Retrospective cohort study of infants born at an urban hospital from June 1993 through December 1999. RESULTS: Of 14,876 eligible infants, 43 (0.29%) developed IHPS. Infants prescribed systemic erythromycin had increased risk of IHPS, with the highest risk in the first 2 weeks of age (relative risk = 10.51 for erythromycin in first 2 weeks, 95% CI 4.48, 24.66). Erythromycin ophthalmic ointment for conjunctivitis was not associated with increased risk of IHPS. Maternal macrolide antibiotics within 10 weeks of delivery may have been associated with higher risk of IHPS but the data were not conclusive. CONCLUSIONS: This study confirms an association between systemic erythromycin in infants and subsequent IHPS, with the highest risk in the first 2 weeks of age. No association was found with erythromycin ophthalmic ointment. A possible association with maternal macrolide therapy in late pregnancy requires further study. Systemic erythromycin should be used with prudence in early infancy.

Anti-Bacterial Agents↗

[Ultrasound diagnosis of pyloric stenosis in young children].

The results of observation of 12 children ranging in age from 1 to 4 mos with tentative diagnosis of pyloric stenosis are presented. The study was performed by special method, using "Aloka" SSD-280 (Japan) ultrasound apparatus. The characteristic symptoms of the disease were revealed in 9 children, diagnosis of pyloric stenosis was confirmed by means of endoscopy, palpation, and intraoperatively. The use of US is practically harmless, and can completely substitute for a roentgenologic method in detecting the given congenital pathology.

Humans↗