Evaluation of a fluid resuscitation protocol for patients with hypertrophic pyloric stenosis

IntroductionWe previously developed an institutional, evidence-based fluid resuscitation protocol for neonates with infantile hypertrophic pyloric stenosis (HPS) based on the severity of electrolyte derangement on presentation. We aim to evaluate this protocol to determine its efficacy in reducing the number of preoperative lab draws, time to electrolyte correction, and overall length of stay.MethodsA single center, retrospective review of 319 infants with HPS presenting with electrolyte derangement from 2008 to 2020 was performed; 202 patients managed pre-protocol (2008–2014) and 117 patients managed per our institutional fluid resuscitation algorithm (2016–2020). The number of preoperative lab draws, time to electrolyte correction, and length of stay before and after protocol implementation was recorded.ResultsUse of a fluid resuscitation algorithm decreased the number of infants who required four or more preoperative lab draws (20% vs. 6%) (p < .01), decreased median time to electrolyte correction between the pre and post protocol cohorts (15.1 h [10.6, 22.3] vs. 11.9 h [8.5, 17.9]) (p < .01), and decreased total length of hospital stay (49.0 h [40.3, 70.7] vs. 45.7 h [34.3, 65.9]) (p < .05).ConclusionImplementation of a fluid resuscitation algorithm for patients presenting with hypertrophic pyloric stenosis decreases the frequency of preoperative lab draws, time to electrolyte correction, and total length of hospital stay. Use of a fluid resuscitation protocol may decrease discomfort through fewer preoperative lab draws and shorter length of stay while setting clear expectations and planned intervention for parents.Level of EvidenceIII – Retrospective comparative study.     

Glial-derived growth factor signaling pathway in infantile hypertrophic pyloric stenosis

BACKGROUND/PURPOSE: Glial-derived growth factor (GDNF), which is the ligand of RET is reported to be essential for the development of enteric nervous system. A GDNF knockout mouse model has shown that the gastric region is a critical passing site between GDNF-RET-independent neuroblasts (colonizing the esophagus) and GDNF-RET-dependent neuroblasts (colonizing the small and large bowel). The earliest GDNF site of production is the mesenchyme and the outer smooth muscle cell (SMC) layer of the developing bowel. In the mature gastrointestinal tract the presence of GDNF is restricted to enteric glial cells. The aim of this study was to investigate the expression of GDNF and RET in infantile hypertrophic pyloric stenosis (IHPS). METHODS: Full-thickness muscle biopsy specimens were obtained from 8 IHPS patients at pyloromyotomy and from 8 age-matched controls without gastrointestinal disease. Indirect immunohistochemistry was performed using avidin-biotin-peroxidase complex method with anti-GDNF and anti-RET antibodies. Quantitative analysis was performed using sandwich-type enzyme-linked immunosorbent assay (ELISA) for GDNF. RESULTS: GDNF- and RET-positive nerve fibers were absent or markedly reduced in IHPS compared with controls. GDNF was expressed strongly by smooth muscle cells of both muscular layers in IHPS, whereas no GDNF expression was detected in pyloric muscle of controls. The quantity of total GDNF in IHPS was significantly higher than in controls (P < .01). CONCLUSIONS: The lack or markedly decreased number of GDNF-positive nerve fibers in IHPS supports the hypothesis of a selective immaturity of the enteric glia in the muscular layers in IHPS. The strong expression of GDNF in smooth muscle cells in IHPS and the increased levels of GDNF in IHPS suggest a compensatory mechanism by which the smooth muscle cells continue to produce GDNF until maturation of the enteric glial cells occurs.     

Infantile hypertrophic pyloric stenosis: a review of 222 cases

The results of 222 cases of hypertrophic pyloric stenosis operated on at the Princess Margaret Hospital for Children in Perth, from 1979 to 1984, have been reviewed. There were no deaths, but there was a 7% incidence of wound infection and a 72% incidence of postoperative vomiting. Staphylococcus Aureus was cultured from 62% of the infected wounds. Prophylaxis against Staphylococcus Aureus infection and delayed introduction of feeding are suggested.     

Delayed presentation of hypertrophic pyloric stenosis: a rare case

Hypertrophic pyloric stenosis (HPS) is the most common cause of gastric outlet obstruction in infants, with an incidence of 1.5 to 3 per 1000 live births, and classically presents at 3 to 4 weeks of age. Delayed presentation of HPS is an extremely rare occurrence after early infancy. With the exclusion of congenital HPS, gastric outlet obstruction in childhood is a rare condition (1:100,000). We report a case of delayed presentation of HPS in a 4 1/2-year-old child in whom the pylorus was hypertrophied and appeared like an “olive,” and for which pyloromyotomy was curative.     

Immunohistochemical study of hypertrophic pyloric stenosis

In spite of the clinical experience about infantil Hypertrophic Pyloric Stenosis (IHPS), its etiopathology remains unknown. Recent studies have been focussed in immunohistochemistry techniques for valuing the neuronal development in the pyloric muscle.     

Primary hypertrophic pyloric stenosis in the adult

Analysis of 100 consecutive patients with pyloric outlet obstruction revealed that 37% of the obstructions were secondary to peptic ulcer disease and 42% were caused by malignant neoplasm. Only a single patient with primary hypertrophic pyloric stenosis was identified, and whether this lesion is a cause or effect of peptic ulcer disease remains unclear. Similarly, the association of this entity with congenital pyloric stenosis is unknown.     

Infantile hypertrophic pyloric stenosis: a comparative study of pyloric traumamyoplasty and Fredet-Ramstedt pyloromyotomy.

PURPOSE: The aim of this study was to compare the incidence of surgical complications (duodenal perforation, postoperative vomiting, wound infection or dehiscence, incisional hernia) between 2 different surgical techniques for the resolution of hypertrophic pyloric stenosis in children. METHODS: A clinically controlled, randomized study with follow-up from 24 to 36 months was conducted. One hundred children between 15 days and 2 months old, who underwent surgical resolution of hypertrophic pyloric stenosis, were put randomly into 2 groups: I, pyloric traumamyoplasty (n = 43); II, Fredet-Ramstedt pyloromyotomy (n = 57). Both groups were controlled for the main demographic variables. Postoperative follow-up was blind for the surgical team. Statistical analysis was done with simple frequencies, percentages, Student's t test, and chi(2). RESULTS: There was not a single case of duodenal perforation, incomplete pyloromyotomy, wound infection, dehiscence, or incisional hernia in any group (P value, not significant). Postoperative emesis was present in 8 patients, uniformly distributed between groups. The operating room time for traumamyoplasty was 39.3 +/- 16.4 minutes versus 54 +/- 16.4 minutes for pyloromyotomy (P =.0003). CONCLUSIONS: This controlled study proves that traumamyoplasty is a simple procedure, quicker to perform, and as safe as pyloromyotomy for the treatment of infantile hypertrophic pyloric stenosis in children. For these reasons, the authors believe it should be considered as an alternative.     

Investigation and diagnosis of hypertrophic pyloric stenosis

Forty-nine infants who underwent investigation or surgery for hypertrophic pyloric stenosis (HPS) over a 30-month period were reviewed. Significant weight loss was present in 18 infants, of whom 16 had HPS. A test feed was performed in 46 infants with 1 of 27 false positive and 6 of 29 false negatives. An ultrasound examination was performed in 34 infants with 4 of 23 false positives and 1 of 11 false negatives. It is recommended that if the test feed is positive then surgery should be performed, otherwise an ultrasound examination can be used as a screening test but a positive result should be confirmed by other means before surgery.     

Extraordinary hyperbilirubinemia in a neonate with idiopathic hypertrophic pyloric stenosis.

This case describes an extraordinarily elevated total bilirubin level that reverted to normal 9 1/2 wk after Fredet-Ramstedt pyloromyotomy. Although the etiology of jaundice occurring in patients with IHPS remains uncertain, theories implicating inhibition of the glucuronyl transferase system have been proposed. Infants with IHPS have a documented hypergastrinemia. An hypothesis is offered, illustrated by this case, to explain the inhibition of the glucuronyl transferase system with resultant hyperbilirubinemia by the hypergastrinemia of idiopathic hypertrophic pyloric stenosis.     

先天性肥厚性幽门狭窄的超声诊断

先生性肥厚性幽门狭窄(congential hypertrophic pyloricstenosis,CHPS)是新生儿常见多发的消化道畸形,临床主要表现为呕吐、胃蠕动波、右上腹包块三大症状。若不早期诊断及时手术,最后患儿常因严重营养不良而衰竭死亡;如能早期诊断及时手术,预后良好。以往诊断以X线钡餐检查为     

Infantile hypertrophic pyloric stenosis in a Third-World environment.

Sixty-two consecutive patients with hypertrophic pyloric stenosis, presenting over a 6-year period, are reported. The sequelae of late presentation and diagnostic delay are outlined. Clinical diagnosis may be difficult. Barium meal examination has proved a more useful single investigation than ultrasonography, although these methods of investigation are complementary. Management difficulties include severe biochemical derangement, correction of which delays surgery. In black communities pyloric stenosis remains a rare condition and heightened clinical awareness remains the most important means of achieving earlier diagnosis.