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The neonatal obstacle caused by a pre-pyloric diaphragm represent a rare cause of high oclusion in new-born. We present the case of a 6 days old new-born admitted in our department for nonbilious vomiting and feeding intolerance in which the clinical exam and the imagistic explorations (plain and contrast abdominal X-Rays, and ultrasound) could not reveal an evident cause for the oclusion. The surgical intervention imposed by the simptoms revealed the existece of a complete diaphragm in the prepyloric region. The initial excision of the diaphragm was not sufficient, the patient undergoing a second surgical intervention of gastrojejunal anastromosis with favorable evolution this time. The authors are presenting diagnosis and theraputical management peculiarities of this rare condition.

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.

Purpose: The purpose of this study was to compare the incidence and type of technical complications seen in a concurrent series of pyloromyotomies done open and laparoscopically., Methods: The medical records of all patients who underwent pyloromyotomy for congenital hypertrophic pyloric stenosis over a 66-month period were reviewed (n = 457). Information obtained included age, sex, weight, operating time, and intraoperative and postoperative complications., Results: Four hundred fifty-seven pyloromyotomies were equivalently divided between the 2 techniques (232 laparoscopic, 225 open). Demographic characteristics and operating times were similar. There were no deaths in the series. The overall incidences of complications were similar in the 2 groups (open, 4.4%; laparoscopic, 5.6%). There was a greater rate of perforation with the open technique and a higher rate of postoperative problems including incomplete pyloromyotomy in the laparoscopic group., Conclusions: The open and laparoscopic approaches have similar overall complication rates. The distribution and the type of complications differ, however.

A neonate with penile agenesis and congenital hypertrophic pyloric stenosis is presented. The patterns of associated anomalies with penile agenesis, and those of congenital hypertrophic pyloric stenosis are discussed.

Idiopathic hypertrophic pyloric stenosis (IHPS) was thought to be a congenital disease traditionally, even though several published reports assumed IHPS was an acquired disease. The pathogenesis and inheritance patterns of IHPS are not fully understood. Except for the familial recurrence of IHPS, concordance of IHPS in monozygotic or dizygotic twins was also noted, but occurrence in female twins is rare. From July 1992 through June 2000, 130 patients were diagnosed with IHPS in our hospital including one pair of female twins. We present the finding in the twins and review the associated articles about the pathogenesis and inheritance patterns of IHPS.

We report a case of congenital pyloric atresia in which two cystic masses in the fetal upper abdomen and polyhydramnios were observed from 24 weeks' gestation to term. The two cystic masses were thought to indicate the 'double bubble' sign in utero, leading to a misdiagnosis of congenital duodenal obstruction. At 33 weeks' gestation, continuous ultrasound observation of the two cystic masses was made for 60 min. During the periods when gastric peristalsis was absent, the 'double bubble' sign was observed. However, during the periods when gastric peristalsis was present, the 'double bubble' sign disappeared. In retrospect, both cystic masses were stomach and the 'double bubble' sign disappeared with peristalsis of the antrum. In order to differentiate from congenital duodenal obstruction, whole stomach configuration should be delineated by continuous observation covering periods when gastric peristalsis is active as well as quiet., (Copyright 2002 S. Karger AG, Basel)

Results of treatment of congenital pylorostenosis in children during last 15 years were studied. There was established, that degree of hypotrophy increases proportionally to duration of vomitive period. Changes of clinical signs of illness due to depression of immune defence, observation by divisional pediatrician with administration of spasmolytic preparation constitute the causes of late hospitalization to unit of pediatric surgery. Late results of treatment witness, that timely performance of operation constitutes an effective method.

The authors present a case of an infant who was treated for recurrent vomiting following pyloromyotomy. Gastroscopic examination showed a polypoid tumour of 4-5 mm in diameter located at the antral region. The patient recovered following re-pyloromyotomy.

Congenital hypertrophic pyloric stenosis, an important cause of intractable vomiting in infants is diagnosed clinically and confirmed ultrasonographically. Other useful interventions are plain radiography and barium study. Differential diagnosis includes pylorospasm and gastroesophageal reflux. Management protocol includes correction of dehydration and electrolyte imbalance and either Fredet Ramstedt pyloromyotomy or medical treatment with atropine sulphate. Atropine is initially given intravenously till vomiting is controlled and then orally at double the effective i.v. done for another 3 weeks. Atropine sulphate is generally well tolerated and side effects are few like tachycardia, raised SGPT and hyperthermia. Atropine sulphate is very effective, cheap, safe and perhaps more acceptable treatment option for CHPS.

Background/purpose: An increasing number of pediatric surgeons are using the laparoscopic approach to treat pyloric stenosis. The advantage of laparoscopic pyloromyotomy is uncertain and has not been evaluated in the setting of a pediatric surgery fellowship program., Methods: The authors retrospectively reviewed the medical records of all patients who underwent pyloromyotomy for congenital hypertrophic pyloric stenosis at their institution from January 1, 1997 through December 31, 2000 (n = 117). Information obtained included age, sex, weight, admission laboratory values, attending surgeon, resident surgeon and their level of training, operating time, intraoperative and postoperative complications, time to full feedings, incidence of postoperative emesis, duration of postoperative emesis, length of stay, and total hospital charges. These variables then were compared between the open (OPEN) and laparoscopic (LAP) groups., Results: From January 1, 1997 through December 31, 2000, 65 LAP and 52 OPEN pyloromyotomies were performed. Characteristics of patients in the OPEN and LAP groups were similar. The mean operating time was 33 +/- 2 minutes for OPEN versus 38 +/- 2 minutes for LAP (P =.07). The incidence of postoperative emesis (LAP, 68%, OPEN, 65%), duration of postoperative emesis (LAP, 7.3 +/- 1.2 hours; OPEN, 8.1 +/- 1.8 hours), and time to full feedings (LAP, 19.5 +/- 1.6 hours; OPEN, 19.5 +/- 1.3 hours) did not differ significantly between groups (P >.05). Mean postoperative length of stay in both groups was similar (LAP, 31 +/- 5; OPEN, 28 +/- 2 hours; P =.64). Mucosal perforation occurred in 5 patients (8%) in the Lap and 2 patients (4%) in the OPEN group (P =.39). Postoperative complications occurred in 12 LAP (18%) and 6 OPEN patients (12%, P =.31). Five LAP cases were converted to OPEN. In the LAP group there was one unrecognized mucosal perforation and one incomplete pyloromyotomy both of which required reoperation. As the laparoscopic approach was adopted, general surgery resident participation as operating surgeon in these cases decreased from 81% in 1997 to 19% in 2000. Hospital charges were higher in the LAP group, but not significantly (LAP, $6,676 +/- 1,005; OPEN, $5,292 +/- 306; P = 27)., Conclusions: Laparoscopic pyloromyotomy has progressively become the dominant surgical approach to pyloromyotomy at our institution. The LAP and OPEN approaches have similar outcomes. However, the Lap approach may be associated with increased complication rates, a reduction in general surgery resident operative experience, and higher hospital charges., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

This study aimed to quantify the neural changes in congenital pyloric stenosis in dogs and to study the comparative anatomy between this condition in dogs and that in infantile hypertrophic pyloric stenosis. Eight specimens from the pylorus of dogs with pyloric stenosis and six control specimens were examined using conventional histology and immunohistochemistry for a range of neural antigens. The changes in the proportion of nerves immunoreactive for each antigen were quantified and analysed statistically. The morphology of the nerves in the diseased dogs was similar to that in controls. Only vasoactive intestinal peptide was reduced in expression in dogs (median proportion in control dogs 0.57, in diseased dogs 0.17; P = 0.065). This study demonstrates both morphological similarities and significant differences between closely related conditions in dogs, humans and other species.

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.

Congenital hypertrophic pyloric stenosis (CHPS) is a common condition in infancy associated with smooth muscle hypertrophy and resulting in pyloric outlet obstruction. The final diagnosis of CHPS is based on precise ultrasonographic measurements of length and width of the pyloric muscle. Based on our clinical and sonographic experience, we observed that smaller measurements of the pyloric muscle were obtained in dehydrated infants than in children examined after proper fluid restoration. The clinical importance of these observations was evident because false-negative results could be obtained. An experimental animal work followed, proving our clinical observation to be true. A significant difference of about 30% to 50% was found between measurements of the muscle thickness of the gastric and pyloric muscles in a state of water deprivation, as compared with a state of full hydration (p < 0.05). Based on our preliminary results, we suggest that children with suspected CHPS should be well hydrated before the ultrasound (US) examination is performed, to avoid false-negative results and a consequent delay in treatment.

The population prevalence of congenital hypertrophic pyloric stenosis in Donetsk region in the course of 11 years (1989-1999) has been descriptively studied. Its frequency is 0.73:1000 or 1:1370 newborn infants annual with range of 0.54 to 1.01:1000. It is somewhat lower than that of more wide spread congenital diseases: labial cleft and hemochromatoses. Pyloric stenosis is detected in 42 of 43 populations of Donetsk region. There is a population nonuniformity of the case rate: from 0.19 to 1.9:1000 newborn infants, that the authors are inclined to explain by dissimilarity of a gene pool in the populations due to random migration of the population.

Objective: Quinolones, mainly nalidixic acid, are frequently used in Hungary to treat bacterial infections in pregnant women, but so far no controlled epidemiological studies of congenital abnormalities among infants born to women treated with nalidixic acid during pregnancy have been reported., Methods: The analysis of oral nalidixic acid use during pregnancy in the mothers of cases with congenital abnormalities and in their matched population controls without congenital abnormality was carried out in the population-based dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities between 1980 and 1996. The study included 22865 women who had newborns or fetuses with congenital abnormalities, and 38151 pregnant women who had newborn infants without any defects (controls)., Results: In the case group, 242 (1.1%) and in the control group, 377 (1.0%) pregnant women were treated with nalidixic acid (crude OR: 1.1 with 95% CI: 0.9-1.3). Seventeen different congenital abnormality groups were evaluated and a higher prevalence of pyloric stenosis was found in seven case infants born to mothers who received nalidixic acid treatment during the last months of pregnancy (adjusted OR: 11.0 with 95% CI: 1.3-91.4)., Conclusions: Treatment with nalidixic acid during pregnancy may increase the risk of pyloric stenosis, though the chance effects cannot be excluded.

A 9-week-old boy developed massive postoperative abdominal distension following a Fredet-Ramstedt pyloromyotomy for hypertrophic pyloric stenosis. A plain abdominal X-ray film was suggestive of a tension pneumoperitoneum. Passage of a cannula into the peritoneal space resulted in forceful expulsion of air, resulting in relief of the distension, which recurred after a few hours. At laparotomy, the tension pneumoperitoneum was due to a pyloric fistula at the proximal end of the pyloromyotomy, although a mucosal breach at the first operation had been at the distal end. Closure of the fistula resulted in complete resolution. A limited review of the literature is undertaken.

Between 1919 and 1941, 71 infants suffering from pyloric hypertrophy were operated on by Ramstedt performing an extramucosal pyloromyotomy. Of these patients, we could identify and investigate 41. Four out of 31 long-term surviving patients have been Billroth II-resected (BII). One of these needed re-resection because of an anastomotic ulcer. None of all the long-term survivors developed a carcinoma. Two patients were treated conservatively because of gastritis and one because of esophagitis. All patients, except the one requiring re-resection and one suffering from maldigestion, were absolutely free of complaints. The average time between operation and re-checking was 57 years. The oldest patient was examined 72 years after the operation.

The current report has been devoted to the presentation of the diagnosis and operative treatment of congenital hypertrophic pyloric stenosis in monozygotic twins. Special attention has been paid to a number of theories aiming to explain the aetiology and the mechanisms contributing to the development of congenital hypertrophic pyloric stenosis.

Partial trisomy 9q represents a rare and heterogeneous group of chromosomal aberrations characterised by various clinical features including pyloric stenosis. Here, we describe the case of a 1 year old female patient with different dysmorphic features including pyloric stenosis and prenatally detected partial trisomy 9q. This partial trisomy 9q has been analysed in detail to determine the size of the duplication and to characterise the chromosomal breakpoints. According to the data gained by different molecular cytogenetic techniques, such as fluorescence in situ hybridisation (FISH) with whole and partial chromosome painting probes, yeast artificial chromosome (YAC) probes, and comparative genomic hybridisation (CGH), the derivative chromosome 9 can be described as dup(9)(pter-->q22. 1::q31.1-->q22.1::q31.1--> q22.1::q31.1-->qter). Four breakpoint spanning YACs have been identified (y806f02, y906g6, y945f5, and y747b3) for the proximal breakpoint. According to this new case and previously published data, the recently postulated putative critical region for pyloric stenosis can be narrowed down to the subbands 9q22.1-q31.1 and is the result of either partial trisomy of gene(s) located in this region or a gene disrupted in 9q31.

We report the occurrence of congenital nephrotic-range proteinuria secondary to focal segmental glomerulosclerosis in an infant with epidermolysis bullosa and pyloric atresia. A homozygous missense mutation, R1281W, in exon 31 of the beta4 integrin gene, ITGB4, was identified. By immunofluorescence, beta4 integrin expression was reduced in both dermal keratinocytes and glomerular podocytes. This is the first demonstration of beta4 integrin expression in human glomeruli. We postulate a role for altered beta4 integrin function in the mediation of the glomerular permeability defect.

In order to investigate an apparent decline in the incidence of infantile hypertrophic pyloric stenosis (IHPS), all cases of IHPS from 1973-1997 in the county of Viborg were studied retrospectively. One hundred and fourty-seven patients were found, with a significant decrease in incidence in the last five year period. Ninety-two percent were males, 14% had a family history of IHPS. Eleven percent had a birthweight < 2500 g, as opposed to 5-6% in the normal population. Pyloric stenosis presents in one to four weeks after birth in 70% and in 5-12 weeks in 28%. The diagnosis was reached solely through the the clinical picture in 105 cases. Twenty-three were examined with upper gastrointestinal radiography, six were false negative, abdominal ultrasound was performed in 21 cases and was false negative in four. Five patients were reoperated because of an inadequate first operation. Postoperative complications with wound infections were seen in eight patients. A high degree of accuracy of diagnosis can only be achieved through the clinical picture.

The aim of this study was to confirm a clinical suspicion that the frequency of this disease is in decline. A retrospective review of case notes of all children admitted and operated in all hospitals in Viborg County during the period of 1.1.1973 to 31.12.1997 was performed. The local survey was supplemented with a national survey. One hundred and forty-seven patients from Viborg County were operated for infantile hypertrophic pyloric stenosis during the period described. Divided into five year periods the number per 1000 living births was consecutively 1.6, 2.4, 2.0, 2.8 and 0.9. The same tendency was found nationally, Table 1. The decrease started in 1993 and has since strengthened. The reason is unknown, but there is a relation regarding to the timing of a change in recommendations concerning positioning of infants during sleep. However, a causal relation is unknown.

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.

Preoperative and postoperative serum samples of 35 patients with different congenital gastrointestinal anomalies were analyzed for the markers CEA, CA 125 and 19-9 by immunoradiometric assay during a period of three years. The majority of the anomalies were aganglionic megacolon and hypertrophic pyloric stenosis. CA 125 and CA 19-9 were likely to indicate logistic model probabilities for babies with anomalies, while CEA was not (F=35.78, p<0.05 for CA CA 125 and F=4.36, p<0.05 for CA 19-9). Probability of no congenital anomaly for babies was: p (Normal)=e4.41-0.13CA125 - 0.05CA19-9/1+e4.41-0.13CA125- 0.05CA19-9 Using CA 125 as a marker, babies with congenital anomalies were determined with 83.3 percent probability (F= 11.33, p<0.05). On the other hand, it was not possible to predict the type of anomaly with these three markers. CEA, CA 125 and CA 19-9 seem to be prognostic variables associated with congenital anomalies. These biological markers provide information that can be incorporated into the diagnosis of anomalies but without doubt results of markers should be supported by clinical findings.

Congenital hypertrophic pyloric stenosis is widely believed to be uncommon in premature infants, and is known to present after the corrected age for a 'term baby'. This cautionary tale highlights the fact that this condition can occur in a premature infant (and may present) in an atypical way well before the infant becomes a 'term baby'.

Werner in 1915, described a patient is characterized by a tibial bilateral aplasia or hypoplasia, polydactyly and absent thumbs. Autosomal dominant inheritance is demonstrated, with variable expressivity. The objective of this work is to describe a child with clinic and radiologic signs of Tibial Hypoplasia with Polydactyly. The genealogic study allowed us to suppose that the gene has a variable expressivity, since in the maternal branch, malformations such as syndactyly of hands, proximal implantation of thumbs and tibiae vara, have been found. The clinic, radiologic, and genetic aspects are discussed.

Congenital pyloric stenosis is rare in premature infants. This is a report of pyloric stenosis in a premature infant (35 weeks gestation) discovered on the 2nd day of life. There appears to be an association with polyhydramnios. Congenital hypertrophic pyloric stenosis should be considered in the differential diagnosis of premature infants with upper gastrointestinal symptoms. A review of the literature is included.

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.

Background: Few references exist regarding contemporary complications of pyloromyotomy (PM) for infantile hypertrophic pyloric stenosis (IHPS). Therefore, we reviewed the frequency and outcome of patients with IHPS who developed complications from PM., Method: A 25-year retrospective review was performed in two populations. The first group included all infants who had a PM for IHPS at two pediatric surgery centers. The second group included all infants referred from other institutions who developed complications following PM for IHPS., Results: Between 1969 and 1994, 901 PMs were performed. Intraoperative complications occurred in 40 patients (4%), including 39 duodenal perforations and 1 difficult intubation requiring prolonged ventilation. No unrecognized duodenal perforations or incomplete PMs were found. Postoperative complications developed in 52 patients (6%). The wound infection rate was less than 1%. Postoperative vomiting occurred in 31 infants (3%). The mortality rate was 0.1%, with 1 death due to sepsis from delayed diagnosis of Hirschsprung's disease. During the same study period, 11 patients were referred from other hospitals for postoperative complications. Five had persistent vomiting treated successfully with expectant management. Six infants needed reoperation: 3 for persistent IHPS, 1 for gastric outlet obstruction, and 1 for small bowel obstruction secondary to adhesions; 1 required wound abscess drainage., Conclusion: Pyloromyotomy is not without complications. Duodenal perforation should be infrequent, but when it occurs, it can usually be readily recognized and treated with minimal morbidity. Postoperative vomiting can be managed nonoperatively, but if it persists longer than 5 days, radiologic evaluation should be performed. Incomplete PM is uncommon and should not occur. A second myotomy is needed when the diagnosis of incomplete myotomy is established. A single standard of care should be expected of all surgeons who perform PM for IHPS.

Over a 34-month period, 51 open and 33 laparoscopic pyloromyotomies were performed for infantile hypertrophic pyloric stenosis. The patients' weight ranged from 1.2 to 5.3 kg. The mean operating time was 28 minutes for the open group and 41 minutes for the laparoscopic group. The average time to accept normal feeds was 41 hours in the open group and 32 hours in the laparoscopic group. There were three perforations, one inadequate pyloromyotomy, and one wound infection in the open group. In the laparoscopic group one patient underwent conversion to open surgery because of a duodenal perforation and three other patients required subsequent open procedures for duodenal perforation (n = 1), pyloric perforation (n = 1) and inadequate pyloromyotomy (n = 1). Additional complications in the laparoscopic group included two cases of omental extrusion through the umbilical port wound and one suture abscess. On the basis of these results we have modified our technique, and there have been no complications after laparoscopic pyloromyotomy in our last 10 patients. We believe laparoscopic pyloromyotomy requires further evaluation before it is accepted into common practice.

Thirty-two patients underwent a pyloromyotomy via an umbilical incision; in 11 a modified umbilical approach was used to facilitate delivery of the pyloric mass. Incisions are made in the skin fold of the upper half of the umbilicus and at the midline, joining the two at the top. The skin incision is closed by upper umbilical translocation with a very good cosmetic result. This incision allows easy access to the pylorus and provides more convenient exposure. The absence of traction on the retractors avoids tissue ischemia, which leads to wound abscess development.

Background: The regulated ability of sphincters to relax allows adequate control of digestive transit. Relaxation of the lower esophageal sphincter (LES) is essential for esophageal emptying and, similarly, pyloric relaxation permits gastric emptying. When the relaxatory response of these sphincters is impaired, luminal transit is altered, as occurs in achalasia and hypertrophic pyloric stenosis. Nitric oxide (NO) has been identified as the main inhibitory neurotransmitter in both sphincteric regions. Moreover, the absence of NO synthase in the LES and the pylorus has been implicated in the pathogenesis of infantile hypertrophic pyloric stenosis (IHPS) and achalasia, respectively., Case Report: We present the case of a 12-year-old boy diagnosed with these two different conditions attributed to NO absence: IHPS and achalasia., Conclusion: To our knowledge this is the first time that such an association has been reported. Whether IHPS and achalasia have been associated in this patient by chance or because they share common pathophysiological mechanisms remains speculative, but is a tantalizing dilemma.

Hypertrophic pyloric stenosis (HPS) is very rare during the newborn period. Here we present a fullterm male neonate with abundant hematemesis 12 hours after birth which interrupted oral feeding. Bleeding subsided within three days after conservative measures, and oral feeding was restarted but not tolerated. The vomiting was effortless and nonbilious. An upper gastrointestinal series revealed gastric dilatation and partial obstruction of the gastric outlet. HPS was found by laparotomy on the fourth day and Fredet-Ramstedt pyloromyotomy relieved the gastric emptying. This is one of the few cases of HPS present at birth, which was diagnosed and surgically treated early, and we suggest a congenital etiology in previously reported cases of HPS. Hypertrophic pyloric stenosis (HPS) is a common cause of pediatric surgery. Usually young infants are involved; HPS is extremely rare in neonates and infants older than 6 months. Vomiting typically begins between the 3rd and 6th week of life, although some infants may have mild symptoms like regurgitation from birth.

We report a liveborn infant with severe intrauterine growth retardation and renal failure, delivered following detection of non-mosaic trisomy 2 by chorionic villus biopsy in the first trimester. Detailed analysis post-delivery indicated apparent complete trisomy 2 of the chorionic tissues, with a chromosomally normal infant demonstrating maternal uniparental disomy for chromosome 2.

Infants with esophageal atresia and tracheoesophageal fistula may have other associated anomalies. The development of infantile hypertrophic pyloric stenosis in the postoperative course of esophageal atresia with tracheoesophageal fistula is rarely reported. Because its symptoms may mimick postoperative complications such as gastroesophageal reflux or anastomotic stricture, the diagnosis may be delayed. We report an infant who had surgery for esophageal atresia with tracheoesophageal fistula at birth. The infant presented with nonbilious projectile vomiting at 4 weeks of age. Plain abdominal x-ray, barium upper gastrointestinal series and abdominal ultrasonography all supported the diagnosis of hypertrophic pyloric stenosis. The diagnosis was confirmed during surgery. After pyloromyotomy, the patient's condition improved.

Infants suffering from congenital heart anomalies who are receiving intravenous prostaglandins may present with gastric outlet obstruction. We describe a newborn with tetralogy of Fallot who was not being treated with prostaglandins and presented with gastric outlet obstruction. The typical clinical and radiological signs of infantile hypertrophic pyloric stenosis resolved spontaneously within several days. We suggest that there may be an association between cyanotic heart disease and gastric outlet obstruction unrelated to the use of prostaglandins.

We describe our experience with a supraumbilical skin-fold incision for pyloromyotomy in infants. Our technique uses a vertical fascial incision. This approach provides excellent exposure to the pylorus, allows greater operative flexibility, and results in a superior cosmetic outcome compared to the traditional right upper-quadrant incision.