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[Pyloric stenosis with rare etiology].

The authors report on a 30 month-old boy who experienced pyloric stenosis related to an hamartoma. This case shows the difficulties in such a clinical and histological diagnosis.

Child, Preschool↗

A rational approach to the diagnosis of hypertrophic pyloric stenosis: do the results match the claims?

The currently accepted premise that the diagnosis of hypertrophic pyloric stenosis (HPS) should be made on clinical grounds, with ultrasound (US) and upper gastrointestinal series (UGIS) reserved for those with a negative clinical examination, was tested. Variable clinical skills of initial examiners, including pediatric surgeons, made abdominal palpation no more sensitive or specific than US or UGIS. For those with a negative clinical examination, proceeding directly to a UGIS will result in monetary savings, especially if good clinical performance decreases the probability of HPS among those without palpable pyloric "tumors." The benefits of a "US first" approach (no radiation, better patient and parent acceptance, no contrast medium) are less apparent but no less important and increase as clinical experience declines and performance of US improves. Criteria for the clinical or sonographic diagnosis of HPS should be kept strict to avoid false-positive results; false-negatives and other causes of vomiting should be identified by UGIS.

Diagnosis, Differential↗

Hypertrophic pyloric stenosis associated with hiatal hernia.

From 1988 to 1994 the authors treated three patients with hypertrophic pyloric stenosis (HPS) who presented with hiatal hernia (HH). Two of the cases had been diagnosed preoperatively. HPS should be considered in HH cases in which a significant delay of gastric output is suspected in the early neonatal period.

Female↗

More infantile hypertrophic pyloric stenosis?

In 1978 there was a disproportionate increase in the number of cases of Infantile Hypertrophic Pyloric Stenosis (IHPS) admitted to the Cambridge Military Hospital Aldershot. This increase has not been sustained in subsequent years, although the mean incidence for the period 1979/82 was slightly higher than the preceding years 1975/77. Analysis of the cases occurring in 1978 did not reveal any unusual factor relating to the increased incidence.

Female↗

Anaesthesia for congenital hypertrophic pyloric stenosis. A review of 350 patients.

The anaesthetic management of 350 consecutive patients with congenital hypertrophic pyloric stenosis over an 8-year period is reviewed. The anaesthetic technique is discussed and the complications encountered reviewed. The morbidity rate was 15.9%. The anaesthetic related morbidity rate was 3.7%. One patient in the series died (0.27%) 8 weeks after pyloromyotomy, as a result of an underlying myopathy.

Anesthesia, Inhalation↗

Infantile hypertrophic pyloric stenosis in Dar Es Salaam.

A five-year review revealed that 15 infants with hypertrophic pyloric stenosis were managed in Muhimbili Medical Centre. All belonged to parents residing within Dar es Salaam city: 12 were African, two Arabs and one was an Indian. the M.F ratio was 6'5:1. The approximate incidence rate was found to be one in 5,500 births. The majority presented with the characteristic clinical picture of the surgical condition and all were managed by the standard Fredet-Ramstedt pyloromyotomy. Mortality was 6.7pc.

Birth Order↗

Structural immaturity of the pylorus muscle in infantile hypertrophic pyloric stenosis.

Recent reports indicate that extracellular matrix and cytoskeleton plasmalemmal elements are altered in infantile hypertrophic pyloric stenosis (IHPS). Desmin is a cytoskeletal protein that is important for the organization and function of muscular fibers. It has been found to be increased in the smooth muscle in chronic intestinal pseudo-obstruction and in skeletal muscle in some forms of myopathies as well as in unexplained hypertrophic cardiomyopathies. The aim of this study was to analyze the expression of desmin in IHPS. Full-thickness muscle-biopsy specimens were obtained from 8 IHPS patients (age range 23 to 41 days) at pyloromyotomy, from 8 age-matched controls without evidence of gastrointestinal (GI) disease at autopsy, and from 2 stillborns who died at 27 and 30 weeks of gestation without evidence of GI disease. Indirect immunohistochemistry was performed using the avidin-biotin-peroxidase complex method with anti-desmin and visualized by development with 3-diaminobenzidine tetrahydrochloride. Pyloric muscle in IHPS demonstrated strong desmin immunoreactivity. The expression of desmin was also strong in the muscular layers of fetal pylorus. In the age-matched controls absent or weak desmin immunoreactivity was seen in the pyloric muscle layer. The increased amount of desmin in hypertrophied pyloric muscle in IHPS may result in inco-ordination of contraction and relaxation of the pylorus, thus causing motility dysfunction. The similar pattern of desmin expression in IHPS and fetal pylorus suggests that the organization of intermediate filaments in IHPS is in a fetal stage of development.

Desmin↗

Vomiting after pyloromyotomy for infantile hypertrophic pyloric stenosis.

An analysis of the factors which may predispose towards postoperative vomiting after pyloromyotomy for hypertrophic pyloric stenosis was carried out in 72 infants at this hospital. 26 (36%) infants experienced moderate to severe postoperative vomiting of sufficient intensity to cause the postoperative feeding regimen to be modified or interrupted. Only two parameters were found to be of statistical significance. These were the state of the oesophageal mucous membrane on endoscopical examination and the presence of haematemesis in the preoperative period. No evidence for a gastric mucosal lesion could be found. An advanced oesophageal mucosal lesion was found in 30% of patients, and this was the source of the haemorrhage in all 11 in whom haematemesis was noted postoperatively. The stay in hospital was prolonged (8 days) in those infants with troublesome vomiting postoperatively compared with those with lesser problems (3 days).

Female↗

Immature intrinsic nerve innervations of pyloric muscle in idiopathic hypertrophic pyloric stenosis.

BACKGROUND AND PURPOSE: Various etiologies have been suggested to be responsible for the development of idiopathic hypertrophic pyloric stenosis (IHPS) in the newborn. The purpose of this study was to determine the maturity of intrinsic nerves and nitrergic neurons in the pyloric muscle in IHPS. METHODS: Full thickness pyloric muscle specimens were obtained from 6 infants with IHPS with age ranging from 27 to 95 (mean 58) days old and subjected to immunohistochemical and double chemiluminescence staining for protein gene product 9.5 (PGP9.5) and neuronal nitric oxide synthase (nNOS). RESULTS: The results showed absence of myenteric plexus between the circular and longitudinal muscle layers in 2 patients, decrease in 1 patient, normal myenteric plexus in 1 patient, and absence of nNOS-containing neurons in 4 patients. All 6 patients had expression of markers for supporting nerve cells and myogenesis in the pyloric smooth muscle. CONCLUSIONS: These results suggest that absence or immaturity of intrinsic nerve and nNOS-containing neurons in the pyloric muscle is the cause of IHPS. The demonstrated lack of innervation or delayed innervation may be the responsible mechanism for the development of IHPS, but further study is needed to elucidate the pathogenesis.

Humans↗

Infantile hypertrophic pyloric stenosis: myopathic type.

Smooth muscle cell biopsies obtained at pyloromyotomy from 37 children with infantile hypertrophic pyloric stenosis (IHPS) were studied by light and electron microscopy and compared with 6 autopsy control cases without any clinical evidence of this disorder. In cases with IHPS an apparently irregular increase in the number of smooth muscle cells by mitosis was accompanied by an increase of the endoplasmic reticulum, proliferation of mitochondria and regressive changes, such as shrinkage, swelling, necrosis and apoptosis of smooth muscle cells. Other alterations, seen in some but not all cases consisted of large numbers of unusual dense granules some of which were clearly associated with actin filaments and, therefore, regarded as derivatives of the normally occurring dense bodies. Furthermore, intermyofibrillar and subsarcolemmal glycogen accumulations, various nuclear abnormalities and pleomorphic membranous cytoplasmic or nuclear bodies occurred. While smooth muscle cell abnormalities predominated in some cases of IHPS, in others there were more severe axonal changes in the myenteric plexus. It is suggested, therefore, that a primarily myogenic type of IHPS can be distinguished from a predominantly neurogenic type.

Biopsy↗

Infantile hypertrophic pyloric stenosis: a clinical review from a general hospital.

A review of 212 cases of infantile hypertrophic pyloric stenosis (IHPS) in a general hospital during an 8.5 year period documents clinical features and suggests refinement of treatment. Features such as sex, age at onset and presentation were similar to other studies. There was a preponderance of infants born in the summer. Premature infants represented 10% of the series, and the most premature (< 33 weeks gestation) presented later after birth (40 vs 25 days). Diagnosis was clinical in 65% of cases, but 4% presented before physical examination and diagnostic tests were positive. At the time of admission only 15% had significant abnormalities of serum electrolytes (chloride < 85 mmol/L). The periumbilical incision resulted in a hidden scar. Duodenal perforation (3.3%) and wound dehiscence (1.4%) are related to technical factors and can be avoided. The timing of commencement of postoperative feeds did not influence the rate of vomiting. The average length of stay was 3.5 days. These results are discussed with suggestions about how to minimize diagnostic tests and length of hospital stay.

Female↗

Congenital hypertrophic pyloric stenosis: A gastrin hypothesis pursued.

Pentagastrin and acetyl choline were shown to contract isolated strips of the circular pyloric muscle from baboons. These findings are compatible with the hypothesis that gastrin may have a part to play in the development or maintenance of congenital hypertrophiic pyloric stenosis of infancy.

Acetylcholine↗

Infantile hypertrophic pyloric stenosis: evaluation of sonographic criteria.

Sonography has become an important diagnostic adjunct in the evaluation of vomiting infants who are suspected to have infantile hypertrophic pyloric stenosis (IHPS). Two commonly used sonographic criteria, viz. pyloric diameter (D) and pyloric muscle wall thickness (T) were evaluated in 14 consecutive vomiting infants. The D and T were significantly larger in the IHPS group compared to the non IHPS group (1.40 +/- 0.34 cm vs 0.95 +/- 0.19 cm, p = 0.04; 4.4 +/- 1.1 mm vs 2.5 +/- 0.6 mm, p less than 0.01). By a combination of sonographic criteria (D greater than or equal to 1.2 cm & T greater than or equal to 3 mm), all the 4 non IHPS patients and 9 out of 10 IHPS patients were correctly diagnosed. The positive and negative predictive values were 1.00 and 0.80, respectively. However, using reported diagnostic criteria of D greater than or equal to 1.5 cm or T greater than or equal to 4 mm would have missed 60% and 20% of the IHPS cases, respectively. With proper choice of diagnostic criteria, sonography is a useful diagnostic tool in the evaluation of vomiting infants for IHPS patients.

Humans↗