Abstract:
:Congenital pyloric atresia (CPA) is a very rare condition that was first described by Calder in 1749. Commonly, CPA occurs as an isolated lesion, which has an excellent prognosis, but it can also be seen in association with other malformations, which can have a negative impact on the final outcome. The medical records of all patients with the diagnosis of CPA treated at our hospital were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, history of polyhydramnios, diagnosis, associated anomalies, operative findings, treatment and outcome. Eleven cases with the diagnosis of CPA were treated at our hospital. There were five males and six females. Seven were products of full term normal vaginal delivery and the remaining four were prematures. Their birth weights ranged from 1.2 to 3.9 kg (mean 2.2 kg). Polyhydramnios was seen in seven (63.6%). Associated anomalies were seen in six (54.5%). These included epidermolysis bullosa (EB) in three, hereditary multiple intestinal atresias (HMIA) in two including a duplication cyst in one of them, esophageal atresia in one, Down's syndrome in one, congenital heart disease in one, cleft palate in one and aplasia cutis congenital (ACC) in one. Intraoperatively, five had pyloric diaphragms, in two of them there were double diaphragms, three had pyloric atresia with a gap between the two ends and two had pyloric atresia with no gap. One of them had duodenal perforation as well as ileal perforation. The patients with pyloric diaphragms had excision of diaphragms and Heineke-Mickulicz pyloroplasty. Four of the other five patients had gastro-duodenostomy. One of them also had duodeno-jejunostomy due to associated distal duodenal atresia and excision of duplication cyst. The patient with duodenal and ileal perforation and because of the marked distension of the duodenum had reduction duodenoplasty, gastroduodenostomy, and duodeno-jejunostomy. The area of ileal perforation was resected and end-to-end anastomosis was done after correction of the associated malrotation. One patient with associated esophageal atresia had gastrostomy and gastrojejunostomy. Post-operatively, all did well initially, but subsequently, six of them died giving an overall survival of 45.5%. Sepsis was the cause of death in all of them. CPA is very rare and when it occurs in isolation it has a good prognosis. The association of CPA with HMIA is universally fatal. CPA in association with EB has a high mortality but there are encouraging results with the use of steroids and phenytoin. Sepsis continues to be the main cause of death and an associated combined immunodeficiency should be excluded.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Al-Salem AHdoi
10.1007/s00383-007-1903-0subject
Has Abstractpub_date
2007-06-01 00:00:00pages
559-63issue
6eissn
0179-0358issn
1437-9813journal_volume
23pub_type
杂志文章abstract:BACKGROUND:Variation exists in the diagnostic testing for gastroesophageal reflux (GER) in infants and in the application of surgical therapy. There has been an increase in antireflux surgery (ARS) since the development of laparoscopy but the outcomes in high-risk infants is unclear. This study examines the results of ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-018-4281-x
更新日期:2018-07-01 00:00:00
abstract::Adrenocortical tumors (ACT) are rare in childhood. Determination of malignant potential is difficult. To assess the presentation, clinical behaviour, and histology of these tumors in our center, the records of nine patients with ACTs between 1989 and 2000 were analyzed. The age, sex, clinical presentation, investigati...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0727-1
更新日期:2003-08-01 00:00:00
abstract:PURPOSE:Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practi...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-017-4062-y
更新日期:2017-06-01 00:00:00
abstract::In severe vaginal malformations, when the distance between the upper vaginal pouch and perineum is too long (6 cm or more), reconstruction of the vagina can be performed by colonic interposition or by long cutaneous flaps obtained by the tissue expansion technique. Two female adolescents were treated using expanded la...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:
更新日期:1997-02-01 00:00:00
abstract::Pullthrough procedures for Hirschsprung diseases typically have favorable results. However, some children experience long-term postoperative complications comprising stooling disorders, such as intermittent enterocolitis, severe stool retention, intestinal obstruction, as well as incontinence. Reoperative Hirschsprung...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-016-4045-4
更新日期:2017-04-01 00:00:00
abstract:INTRODUCTION:Objectively evaluating the lack of bowel control (fecal incontinence) continues to be a challenge. Many have attempted to measure the severity of fecal incontinence and to evaluate its impact on the quality of life by developing standardized scoring systems. Some of these systems have been validated but no...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-016-3909-y
更新日期:2016-08-01 00:00:00
abstract::The morphogenesis of the external genitalia of human fetuses (16-250 mm crown-rump [CR] length, 6-26 weeks of gestation) obtained after medical termination of pregnancy were studied. Differential development (male/female) started after 50 mm CR length (9 weeks). At that time the external genitalia consisted of a cylin...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF01076950
更新日期:1997-07-01 00:00:00
abstract::Juvenile papillomatosis (JP) is a rare benign, proliferative breast tumor in children. We observed a 9-year-old girl with a large soft-tissue mass at the lower outer quadrant of the left breast. Ultrasonography showed an ill-defined, inhomogeneous mass with numerous small, hypoechoic areas. The tumor was completely ex...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830000462
更新日期:2001-03-01 00:00:00
abstract:PURPOSE:To identify potential metabolic biomarkers for distinguishing malignant and benign thyroid nodules in children and adolescents using a metabolomics approach. METHODS:A total of 96 consecutive patients (median age 14.29 ± 2.31 years, range 9-18 years) who underwent thyroidectomy and 40 healthy controls were enr...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-019-04584-0
更新日期:2020-02-01 00:00:00
abstract::A thoracic mass was identified on a routine ultrasound (US) examination in the 30th week of gestation. A postnatal chest radiograph, US, and computed tomography demonstrated a well-delineated, solid, cystic tumor in the left lower lobe. The mass was excised and a diagnosis of pulmonary blastoma (PB) was made on pathol...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF00497817
更新日期:1996-06-01 00:00:00
abstract::To determine the long-term outcome of congenital cystic adenomatoid malformation (CCAM), the records of all patients with CCAM treated in three Hungarian paediatric surgical centers between 1977 and 1996 were reviewed. Patients were followed for up to 20 years following diagnosis and treatment. In 20 patients CCAM was...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050593
更新日期:1999-07-01 00:00:00
abstract::Rhabdoid tumour of the kidney is not common. Presentation of such a lethal tumour along with blunt abdominal trauma is even rarer. We report such a case of Rhabdoid tumour of kidney which presented as renal trauma, and discuss the diagnostic difficulties and rapidly fatal outcome. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003839900290
更新日期:2000-01-01 00:00:00
abstract:PURPOSE:We evaluated the effectiveness of prophylactic mesenteric lymphatic ligation in preventing iatrogenic chylous fistulae in abdominal neuroblastoma surgery. METHOD:All patients who underwent resection of abdominal neuroblastoma between years 2002 and 2013 were included. A change in surgical strategy commenced fr...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-014-3581-z
更新日期:2014-10-01 00:00:00
abstract:AIM:The aim of this study was to examine the relationship between birth weight, pulmonary artery (PA) size, and outcome in congenital diaphragmatic hernia (CDH) to establish if PA size has prognostic value. METHODS:The subjects for this study were 39 consecutive left-sided CDH patients treated at our institution accor...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2899-z
更新日期:2011-08-01 00:00:00
abstract:PURPOSE:In order to evaluate the gubernaculum (GN) abnormalities quantitatively in patients with undescended testes (UDT), the area and attachment site of the gubernaculum were evaluated. PATIENTS AND METHODS:Sixty-seven testes from 61 patients with an undescended testis treated in the past 11 years at our institution...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-014-3597-4
更新日期:2014-11-01 00:00:00
abstract:PURPOSE:Chronological age has not always been an accurate predictor of gut motility in newborns. We hypothesized that the enteric plexus is immature at birth. We studied whole-mount preparations of the myenteric plexus in pigs from mid-point of gestation to adulthood. METHODS:Distal large bowel whole-mount preparation...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2265-y
更新日期:2008-12-01 00:00:00
abstract:PURPOSE:The aim of this study is to identify the risk factors for esophageal anastomotic stricture (EAS) and/or anastomotic leakage (EAL) after primary repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) in infants. METHODS:A retrospective chart review of 52 patients with congenital EA/TEF between Jan...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-016-3931-0
更新日期:2016-09-01 00:00:00
abstract:INTRODUCTION:The Morgagni hernia (MH) accounts for 3-4% of congenital diaphragmatic hernias. There is a paucity of data regarding this rare defect. The purpose of this study is to describe the characteristics of children with MH, surgical approaches for repair, and patient outcomes. METHODS:Pediatric patients (ages 0-...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-017-4078-3
更新日期:2017-07-01 00:00:00
abstract::Congenital hepatic cysts are rare lesions of infancy. While operative management and outcomes have been extensively studied in adult patients with hepatic cysts, data in pediatric patients are limited. We discuss our experience in an infant and review relevant literature regarding operative technique and surgical outc...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-013-3312-x
更新日期:2013-08-01 00:00:00
abstract::A review of 100 consecutive cases of congenital diaphragmatic hernia (CDH) treated at our institute focusing on the efficacy of protocolized management (PM) was conducted. Of the 100 cases, 14 who became symptomatic more than 24 h after birth, and seven with fatal anomalies (four cardiac and three chromosomal) were ex...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1759-8
更新日期:2006-11-01 00:00:00
abstract::Sixty-seven patients with resected Wilms' tumour>1.0 kg are reported. Surgery in this group is difficult, may result in the sacrifice of adjacent structures, and frequently upstages the patient. Mortality is related to tumour burden. Whilst there is little agreement on the therapeutic implications of failure to respon...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1678-8
更新日期:2006-05-01 00:00:00
abstract::Female urethral injuries may cause serious morbidity with loss of the entire urethra in some cases. We review the results of construction of a neourethra by the flipped anterior bladder-tube technique in five girls aged 3-18 years (mean 8 years). All had pelvic fractures and had their initial management elsewhere; fou...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830000434
更新日期:2000-01-01 00:00:00
abstract:PURPOSE:The objective of this study was to characterize the clinical course and outcomes of children with pancreatic pseudocysts that were initially treated non-operatively or with percutaneous drainage. METHODS:A retrospective review of children with pancreatic pseudocysts over a 12-year period was completed. Categor...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-012-3236-x
更新日期:2013-03-01 00:00:00
abstract:PURPOSE:To evaluate early postoperative results in a case controlled study following clinical use of stem cells in extrahepatic biliary atresia (EHBA). METHODS:From July 2005 to March 2008, 30 cases of suspected EHBA were divided in two groups in an intervention study. Group A received autologous mononuclear bone marr...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-010-2712-4
更新日期:2011-01-01 00:00:00
abstract:INTRODUCTION:Applicants in the NRMP for pediatric surgery have little objective data available regarding factors predicting successful matching. We analyzed data from applicants at our institution to attempt to identify parameters correlated with three outcomes: successfully matching, or attaining either a top ten or t...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2912-6
更新日期:2011-11-01 00:00:00
abstract:BACKGROUND/AIM:Esophageal dilatation, gastroesophageal reflux, and intestinal obstruction have been demonstrated in CDH survivors. Abnormal esophageal and intestinal innervations were recently found in rats and babies with this disease. Our aim was to further characterize these malformations in embryos and fetal rats e...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-010-2788-x
更新日期:2011-02-01 00:00:00
abstract::Atypical mycobacterial disease is common in children in Australia. Over 22 years, records were kept prospectively by the senior author. The diagnosis was confirmed in 118 patients, either by culture or by the combination of a positive skin test plus typical histology. There were 46 boys and 72 girls with a median age ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0840-1
更新日期:2002-12-01 00:00:00
abstract::It is unknown whether noncomplicated acute appendicitis cause bacterial translocation. In this study, we aimed to test development of the bacterial translocation in the patients who were operated for acute appendicitis. In this prospective study, 10 control patients who underwent elective operations because of other r...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-007-1899-5
更新日期:2007-06-01 00:00:00
abstract::In contrast to adults, calcium carbonate gallstones are relatively common in children. Their pathogenesis is poorly understood. Cystic duct obstruction promotes calcium carbonate formation in bile and increases gallbladder mucin production. We tested the hypothesis that mucin producing epithelial cells would be increa...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1867-5
更新日期:2007-03-01 00:00:00
abstract::Negative pressure wound therapy (NPWT) is an accepted form of treatment in managing complex wounds in adults and children. It is not as widely used in the neonatal population due to limited clinical experience. We describe the application of the RENASYS™ system (Smith and Nephew, UK) in delivering NPWT to four neonate...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2868-6
更新日期:2011-08-01 00:00:00