[Clinical and therapeutical considerations regarding a rare case of pre-pyloric stenosis]. / Consideratii clinico-terapeutice privind un caz rar de stenoza prepilorica.

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.

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.

Comparison of the incidence of complications in open and laparoscopic pyloromyotomy: a concurrent single institution series.

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 quantitative study of the neural changes underlying pyloric stenosis in dogs.

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.

Intermittent 'double bubble' sign in a case of congenital pyloric atresia.

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.

[Emesis after pyloromyotomy in congenital hypertrophic pyloric stenosis: incomplete pyloromyotomy, recurring pyloric stenosis or gastric outlet obstruction?]. / Pyloromyotomia utáni hányás congenitalis hypertrophiás pylorusstenosisban: inkomplett pyloromyotomia, visszatéró pylorusstenosis vagy gyomorkimeneti obstructio?

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.

A comparison of laparoscopic and open pyloromyotomy at a teaching hospital.

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.

[Diagnosis and results of treatment of congenital pyloric stenosis in children]. / Diahnostyka ta rezul'taty likuvannia vrodzhennoho pilorostenozu u ditei.

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.

Postoperative tension pneumoperitoneum in an infant.

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.

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.

Long-term investigations after pyloromyotomy for infantile pyloric stenosis.

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.

CEA, CA125 and CA19-9 levels in congenital gastrointestinal anomalies.

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 with associated polyhydramnios in a premature infant.

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.

Complications of pyloromyotomy for infantile hypertrophic pyloric stenosis.

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.