Treatment of Congenital Gastric Outlet Obstruction due to a Web: A Retrospective Single-center Review.

BACKGROUND: Congenital gastric antral web and congenital pyloric atresia are rare reasons of congenital gastric outlet obstruction (CGOO). Depending on the different forms, the symptoms vary from unspecific epigastric pain to nonbilious "projectile" vomiting, ileus and failure to thrive. Throughout the years, many different treatment options are described, like simple surgical dilatation, incision or excision of the membrane, possibly combined with a pyloroplasty. PATIENTS AND METHODS: In this retrospective single-center observation study, we present 9 cases with different kinds of webs leading to CGOO. In a time period of 45 years (1970 to 2015) different treatment strategies and limitations of minimal invasive endoscopic treatment on the basis of complications and outcome are discussed. RESULTS: Six patients had an incomplete antrum web. One patient suffered from a complete antrum web. In addition, in 2 patients a complete pyloric web, as well as an incomplete pyloric web was found. During the past 45 years, the treatment options for CGOO due to webs changed, and with the development of smaller and more flexible video endoscopes an endoscopic treatment has become feasible. The treatment strategy and follow-up examination was specific to every patient. CONCLUSION: On the basis of our review, minimal endoscopic procedures in small newborns and toddlers require a high level of experience. They are technically challenging and need suitable equipment. Overall, endoscopists and pediatric surgeons should equally be prepared for all possible complications in every treatment step. The endoscopic approach to managing this unusual disease may require multiple interventions and is associated with a risk of perforation. Therefore, we are convinced that centers undertaking this treatment modality should be equipped with the proper instrumentation, prepared to perform multiple endoscopic interventions, and supported with an ability to emergently convert to surgery if required.

Congenital pyloric atresia: Early and delayed presentations-A single centre experience of a rare anomaly.

Pyloric atresia may occur as an isolated or pure atresia or may be associated with other anomalies like those involving skin, renal or gastrointestinal systems etc. The most frequent association is with junctional epidermolysis bullosa, a rare autosomal recessive disorder affecting the skin and mucosa. We present a prospective evaluation of three patients of pyloric atresia at our institute over last 2 years (2012-2014)-one of them presenting at 5th day of life and the other two at 5 and 6 years of age, respectively. Two of them did not have any other associated anomaly whereas one patient had incidental finding of Meckel's diverticulum at the time of surgery for pyloric atresia plus he had been operated upon for right-sided pelvioureteric junction obstruction at 1 year of age. All patients underwent laparotomy after stabilization. The two patients presenting late had type 1 pyloric atresia with a pyloric membrane having a central hole in both the patients. So, excision of the membrane with Heineke Mikulicz pyloroplasty was done in both of them. The neonate who presented early had type 2 pyloric atresia and underwent Finney's pyloroplasty.

Duplicación quística intraluminal de píloro asociada a hernia diafragmática congénita. Presentación de un caso / Intraluminal pyloric duplication cyst associated with congenital diaphragmatic hernia. A case report

La duplicación pilórica extraluminal representa la localización menos frecuente de todas las duplicaciones del tubo digestivo; sin embargo, la duplicación pilórica intraluminal (DPI) se ha documentado de manera excepcional como una anomalía congénita de extrema rareza en recién nacidos y pacientes pediátricos. Nosotros aportamos el primer caso de DPI asociada a hernia diafragmática congénita. Presentamos el caso clínico de un niño de 29 días de vida con sospecha de síndrome pilórico debido a una obstrucción mecánica asociada a hernia diafragmática congénita. El diagnóstico de quiste de DPI fue confirmado por los hallazgos intraoperatorios y un posterior examen histológico. La duplicación pilórica intraluminal es una malformación o anomalía (mejor que enfermedad) rara, causante de la obstrucción mecánica del canal pilórico, y debería tenerse en cuenta en el diagnóstico diferencial del síndrome de obstrucción pilórica neonatal (AU)

The extraluminal pyloric duplication represents the least common location of all the digestive tract duplications. However, intraluminal pyloric duplication has been exceptionally reported as an extremely rare congenital anomaly in newborns and pediatric patients. We report the first case of intraluminal pyloric duplication associated with congenital diaphragmatic hernia. We present a 29 days old boy with suspected pyloric syndrome due to mechanical obstruction associated with congenital diaphragmatic hernia. The diagnosis of intraluminal pyloric duplication cyst was confirmed by intraoperative findings and subsequent histological examination. Intraluminal pyloric duplication is a rare disease causing intrinsic mechanical obstruction of the pyloric channel. It should be taken into account in the differential diagnosis of neonatal pyloric obstruction síndrome (AU)

Congenital pyloric atresia, presentation, management, and outcome: a report of 20 cases.

BACKGROUND: Congenital pyloric atresia (CPA) is a very rare anomaly. It is usually seen as an isolated condition with excellent prognosis. Few cases are familial. These are usually associated with other hereditary conditions and have a poor prognosis. This is a review of our experience with 20 patients with CPA outlining aspects of diagnosis, associated anomalies and management. PATIENTS AND METHODS: This is a retrospective analysis of 20 cases seen over a 22 year period (December 1990 to December 2012). Their records reviewed for: age, sex, presentation, prenatal history, associated anomalies, investigations, treatment, operative findings and the outcome. RESULTS: 20 cases (9 Males, 11 Females) were treated. 7 patients were full term and the remaining 13 were prematures. Their mean birth weight was 2.1 kg (1.1 kg to 3.9 kg). Polyhydramnios was seen in 13 patients (65%). Two were brothers and four were members of the same family. Isolated CPA was seen in 7 (35%); 13 had an associated conditions: epidermolysis bullosa (EB) in 8 (40%) and multiple intestinal atresias (MIA) in 5 (25%). Three patients had associated esophageal atresia. All were operated on except two who died early due to unrelenting sepsis. The variety of pyloric atresias encountered were as follows: pyloric diaphragm in 13 including double diaphragms in 2, pyloric atresia with a gap in 4 and pyloric atresia without gap in 3. Ten died postoperatively giving an overall survival of 40%. CONCLUSIONS: CPA is a very rare condition. Isolated CPA carries a good prognosis. Association of CPA with other familial and congenital anomalies like EB and MIA carries a poor prognosis.

A case of congenital pyloric atresia with dystrophic epidermolysis bullosa.

Pyloric atresia with epidermolysis bullosa (EB) dystrophica is a rare entity that may not be immediately recognized. We describe the fourth confirmed case of pyloric atresia associated with the dystrophic subtype of EB diagnosed by standard pathologic measures, and discuss the clinical disease features and recent advances in the pathophysiology.

Epidermolysis bullosa and congenital pyloric atresia.

The association between epidermolysis bullosa (EB) and pyloric atresia (PA) is rare but well documented. Herein, we report a case of EB associated with congenital PA. A female baby, weighing 1480 g, was born vaginally to a 31-year-old gravida 7 lady at 33 weeks of gestation. Polyhydramnios was detected on antenatal assessment. The parents were non-consanguineous Saudis with no family history of significant illness. At birth, well-demarcated areas of peeled skin were present over knees, left leg and periumbilical region. Systemic examination revealed no other abnormality. On second day, the patient developed recurrent vomiting and abdominal distension. An abdominal X-ray revealed a single gastric gas bubble suggesting pyloric obstruction. Following gastroduodenostomy, the baby developed severe sepsis with multiorgan dysfunction and expired on 25th day of life. Skin biopsy showed cleavage within lamina lucida.

Prenatal diagnosis of isolated congenital pyloric atresia in a sibling.

Although familial occurrence of congenital pyloric atresia (CPA) has been frequently reported in the past, many of these cases were associated with epidermolysis bullosa (EB), and familial isolated CPA was a relatively rare condition. We prenatally diagnosed and successfully treated a sibling of a subject with isolated CPA, who was diagnosed prenatally by fetal ultrasonography based on the findings of a distended stomach combined with polyhydramnios. The first case was a 2398-g female infant born at 36 weeks of gestation, who had been prenatally diagnosed as CPA. The second case, a younger sister of the first case, was a female infant weighing 2434 g, who had been also diagnosed as CPA by fetal ultrasonography at the check-up for the polyhydramnios of the same mother. Neither of the infants showed dermal lesions such as EB, and both underwent pyloroplasty with an excision of the pyloric membrane successfully after birth.

Isolated intramuscular fibrous band masquerading antral web in a premature neonate. A case report.

Isolated fibrous gastric antral band in a neonate causing partial gastric obstruction and mimicking prepyloric antral web is not reported in the English literature, and we would like to report the first case. A premature neonate with feeding difficulties and bilious aspirate underwent upper gastrointestinal contrast examination. The upper gastrointestinal series showed a thin, radiolucent, circumferential band in the antrum with delayed gastric emptying. Radiographic diagnosis of prepyloric antral web with partial gastric obstruction was made. Intraoperatively, a circumferential fibrous cord was found in the muscle layer (tunica muscularis) of the antrum, which was completely divided. Clinically and radiographically, the 2 conditions could not be differentiated but require 2 separate surgical techniques for their management.

Pyloric atresia associated with Dieulafoy lesion and gastric dysmotility in a neonate.

In this case study, we report a male infant with pyloric atresia, extreme gastric distension, and a caliber-persistent gastric artery (Dieulafoy lesion) with massive gastric bleeding. After a transverse pyloroplasty and endoscopic hemoclip application to the caliber-persistent gastric artery, very slow gastric emptying developed, which required repeated surgical interventions. Gastroduodenostomy failed to promote gastric emptying. The intraoperative and postmortem histologic examinations of the gastric wall revealed a loss of interstitial cells of Cajal, which possibly explains the extreme motility disorder.

Gastric antrum hypertrophy causing outlet obstruction in an infant with congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is associated with multiple congenital anomalies affecting several organ systems, including the gastrointestinal system. Pyloric stenosis and bands are known and previously reported etiologies of gastric outlet obstruction in infants with CDH. We report the first case of gastric antrum hypertrophy causing gastric outlet obstruction in an infant with CDH.

An intraluminal pyloric duplication cyst in an infant.

An intraluminal pyloric duplication cyst is an extremely rare congenital anomaly. We report a case of an intraluminal pyloric duplication cyst in a 2-year-old girl with progressive increased vomiting. The patient had an ultrasonography, upper gastrointestinal series, and multiplanar reformatted computer tomography of the abdomen. The diagnosis was confirmed by surgery and histopathologic examination. The patient was asymptomatic at the 12-month follow-up.

Congenital gastric outlet obstruction and nonimmune hydrops fetalis: a prenatal sonographic diagnosis of a case with hydrothorax and ascites.

A case of a male fetus with sonographic diagnosis of hydrops fetalis at 19-week gestation is reported. The fetus had anasarca, bilateral massive pleural effusion, and ascites, in addition to cardiac arrhythmia and congenital gastric outlet obstruction. Mother's clinical history and laboratory workup excluded immune hydrops. The etiological dilemma and fetal outcome are discussed. We concluded, based on this case, that when fetal hydrops occurs early and is associated with multiple congenital anomalies, prolonging the pregnancy may be futile.

A rare gastric outlet anomaly: pyloric ostium on incissura angularis.

Congenital gastric outlet anomalies are rarely seen defects resulting from early embryologic development of foregut. We report a case of congenital pyloric outlet anomaly in a 60-year-old man with mild postprandial epigastric discomfort of approximately 3 months' duration. Endoscopic examination of the stomach showed a pyloric ostium on incissura angularis connecting the stomach to the duodenum. Usual site of pylorus at the end of antrum was closed like a sac. No signs of acute or chronic peptic ulcer were noted. A biopsy was taken from the region showed normal mucosal layers with a mild gastritis. There was no history of surgery, ulcer disease, or use of any medication. The case was considered to be congenital in origin and was successfully treated conservatively with proton-pump inhibitors and gastrokinetics.

Double gastric outlet obstruction with epidermolysis bullosa in a neonate.

We report a 2-day-old baby who had double gastric outlet obstruction, rectovaginal fistula and associated epidermolysis bullosa. She recovered after excision of diaphragms, pyloroplasty and pelvic colostomy.

Gastric duplication cyst causing gastric outlet obstruction.

We report a rare case of gastric outlet obstruction in a newborn infant caused by a gastric duplication cyst. Ultrasound provided a non-invasive and conclusive diagnostic technique, which should be used as a baseline investigation for cases of suspected gastric outlet obstruction.