Abstract
We report a case of prenatally diagnosed congenital perineal mass which was combined with anorectal malformation. The mass was successfully treated with posterior sagittal anorectoplasty postnatally. On ultrasound examination at a gestational age of 23 weeks the fetal perineal mass were found on the...
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PMID: 20592907
PDF is available here.
Abstract
Anorectal malformations (ARMs) and Hirschsprung disease (HD) are the most common congenital colorectal defects in the newborn. The outcomes of HD and ARMs have improved significantly because of improved understanding of the pathologic anatomy and physiology of these defects and of the modern surgica...
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PMID: 20307853
PDF is available here.
Abstract
Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malfor...
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PMID: 20307850
PDF is available here.
Abstract
The management of patients with colorectal disease in the pediatric population is challenging. Such management is all the more challenging when facing the constraints imposed by an environment with limited clinical resources. Three types of colorectal problems are highlighted in this article: anorec...
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PMID: 20307848
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Abstract
We and others have shown that genetic factors play an important role in the pathogenesis of ARMs, and many mouse genetic models suggest molecular pathways that may be altered in ARMs.
Copyright 2010 Elsevier Inc. All rights reserved....
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PMID: 20307847
PDF is available here.
Abstract
Normal clinical and radiologic examination predicted absence of a significant cardiac abnormality on echocardiography in 100% of cases. We conclude that routine echocardiography before embarking on surgical intervention may not always be necessary but should be reserved for infants with abnormal cli...
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PMID: 20438917
PDF is available here.
Abstract
Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with s...
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PMID: 20385284
PDF is available here.
Abstract
The aberrations in spatiotemporal expression pattern of Hoxd-13 on gD13 to gD16 suggested that Hoxd-13 may be an essential inductive signal for normal development of the hindgut, and altered expression may contribute to the abnormal development of the hindgut and accordingly lead to ARMs.
Copyright...
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PMID: 20385283
PDF is available here.
Abstract
Spinal dysraphism is common in patients with ARM, and its prevalence is higher in patients with complex ARM. Spinal anomalies can occur even with benign types of ARM and, therefore, that all patients should be screened. Magnetic resonance imaging is useful in detecting occult SD that may be missed b...
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PMID: 20385285
PDF is available here.
Abstract
This study revealed potential risk factors for ARM, including fever during pregnancy, maternal overweight, use of multivitamins, paternal smoking, and occupational exposures, but a familial component seems important as well.
Copyright 2010 Wiley-Liss, Inc....
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PMID: 20073076
PDF is available here.
Abstract
Longitudinal studies are needed to clarify whether these results can be attributed to improvements in surgical techniques that have contributed to improved QL in younger cohorts, or if, instead, quality of life in patients with ARM decreases over time. Intervention efforts should focus on bowel mana...
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PMID: 20128108
PDF is available here.
Abstract
We aim to present our experience, management challenges and patterns of ED with a review of the literature.
The study was carried out at a Nigerian Tertiary Hospital (2005-2008 inclusive).
We retrospectively analyzed seven patients with ED managed in our hospital for sex, age, clinical presentations...
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PMID: 20339180
PDF is available here.
Abstract
We report a case of VACTERL complex which had concomitant horseshoe lung, laryngeal cleft, and hypertrophic pyloric stenosis, which has not been previously reported.
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PMID: 20964119
PDF is available here.
Abstract
When recognized in the extremely premature infant in the delivery room, ambiguous genitalia is a devastating diagnosis. It can be even more so, if the diagnosis is made several weeks later. The case study reported here illustrates this point. A 24-week Twin B infant born with multiple congenital ano...
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PMID: 20211828
PDF is available here.
Abstract
The allele distributions of all five RET SNPs in the HD patients deviated from those in the normal population (p < 0.05), whereas those of the ARM patients did not. The allele distributions of these RET SNPs in the IPO patients were all significantly different from those in the HD patients. Allele d...
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PMID: 20123584
PDF is available here.
Abstract
Our experience with these patients is similar to that provided in the literature. We consider that it is important to identify any associated anomalies, especially heart disease, craniofacial anomalies and other gastrointestinal malformations, because they condition the patient's management. Patholo...
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PMID: 20578578
PDF is available here.
Abstract
The features of CT manifestation in 16 cases were as follows: (1) Horizontal part of duodenum could not reach medioventral line or could reach it but encircled right-down behind the superior mesenteric artery(SMA). (2) Ectopic ileocecal junction. (3) Jejunum located in right-middle abdomen while ile...
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PMID: 19921570
PDF is available here.
Abstract
For the 39 institutions that responded to the questionnaire, prevalence of eversion syndrome ranged from 0% to 30%. For Aurelia aurita, eversion was more common at institutions with only captive-raised and no wild-caught jellyfish. Eversion was most common among young (approx 1- to 2-month-old) grow...
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PMID: 19719423
PDF is available here.
Abstract
Our experience here suggests that a PSARP provided satisfactory results in adults who present late with an anorectal malformation....
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PMID: 19710959
PDF is available here.
Abstract
Neonatal surgical care requires a current understanding of pre- and postnatal intervention for a myriad of congenital anomalies. This article includes an update of the recent information on commonly encountered fetal and neonatal surgical problems, highlighting specific areas of controversy and chal...
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PMID: 19501697
PDF is available here.
Abstract
Management of anorectal malformations, especially that of proximal and more severe forms have developed significantly in the last 25 years. Echography and magnetic resonance imaging have improved the diagnostic accuracy, especially in terms of associated anomalies that are the main cause of fataliti...
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PMID: 19300133
PDF is available here.
Abstract
Here, we report a case of intrapancreatic accessory spleen confirmed by pathologic diagnosis and discuss its differential diagnosis and surgical management with a review of the literature.
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PMID: 19266611
PDF is available here.
Abstract
We found a common bile duct that was divided into two distinct ducts, one the main and the other the accessory duct, during its course downwards. The two bile ducts had a parallel course emerging from the common bile duct after its formation and reuniting just above the head of the pancreas. Finally...
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PMID: 19803266
PDF is available here.
Abstract
Two patients are presented with intestinal pseudo-obstruction and malformations of their intestinal muscularis propria. Histopathological findings in each case indicate a distinct alteration of intestinal muscle architecture. The 1st patient, a teenager with lifelong constipation, had an additional...
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PMID: 18788889
PDF is available here.
Abstract
We suppose that the difference of blood flow resulting from this time lag is one of the reasons why the inferior epigastric artery from the obturator artery is very rare in comparison to the obturator artery from the inferior epigastric artery....
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PMID: 18706793
PDF is available here.
Abstract
When permitted by intestinal size in infants younger than 1 year, stapled anastomoses were safe and effective and significantly reduced operative time....
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PMID: 19040941
PDF is available here.
Camilla Salvestrini,
John A McGrath,
Linda Ozoemena,
Khaled Husain,
Eman Buhamrah,
Nasim Sabery,
Alan Leichtner,
Paul A Rufo,
Antonio Perez-Atayde,
Cate H Orteu,
Franco Torrente,
Robert B Heuschkel,
Michael A Thomson and
Simon H Murch
Abstract
ITGB4 mutation may induce a desquamative enteropathy in infancy without significant skin disease. A history of pyloric atresia is important in infants with severe chronic diarrhoeal disease and should prompt investigation for JEB-PA associated mutations. Acquired immune responses may exacerbate prim...
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PMID: 18955862
PDF is available here.
Abstract
In ARM patients self-efficacy and parents self efficacy are correlated to important outcomes, respectively body image and sexual functioning in adults and postoperative care satisfaction in parents of those under 16 years old. Future longitudinal studies are needed to evaluate causal relations betwe...
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PMID: 18797885
PDF is available here.
Abstract
Increased risk of associated malformations in children with clefts must to be highlighted and disseminated to health professionals involved in the management of children with clefts. Routine screening for other malformation, especially cardiac, skeletal, and central nervous system malformations, may...
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PMID: 18848099
PDF is available here.
B Goldschmidt,
C A Lopes,
M Moura,
D M Fasano,
M C R Andrade,
L B Cysne,
M B Gonçalves,
J S Bravin,
T Kugelmeier,
C F Viana,
F A Silva and
A M Marinho
Abstract
The association between the craniofacial and other corporal anomalies is unclear....
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PMID: 18773507
PDF is available here.
Abstract
We report on a 7-week-old girl who was found to have a rectal duplication cyst. The rectal duplication cyst was successfully excised laparoscopically. Rectal duplication cysts are rare alimentary tract anomalies generally discovered during childhood. Complications include symptoms arising from the c...
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PMID: 18675659
PDF is available here.
Abstract
This study illustrated the development of the SMC in normal and ARM rats. On E16, the location and appearance of SMC in ARM rats were similar to the normal rats, and at this time, the ectopic rectal orifice could be noted. From E18 on, the maldevelopment of SMC could be observed in ARM rats. These o...
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PMID: 18675634
PDF is available here.
Abstract
This report describes a case of congenital thickening and shortening of the round ligament of the liver, a rare cause of gastric outlet obstruction. There are few published cases in the literature and these were generally diagnosed at a late stage and were mostly among adults. The patient underwent...
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PMID: 18675654
PDF is available here.
Abstract
Nerve fibers are decreased in the proximal ganglionated colon in patients with HD without associated gut dysmotility. Poor clinical outcome was noted only in a patient with normal innervation and markedly decreased ICC. Collection of data from a much larger number of patients with poor clinical outc...
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PMID: 18675631
PDF is available here.
Abstract
Meckel's diverticulum and duplication of the alimentary tract are very important as the congenital anomalies of ileum. As these two diseases have the common clinical characteristics such as sex, age, symptoms and complications, it is often difficult to diagnose before surgery. This report describes...
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PMID: 18616126
PDF is available here.
Abstract
This large case series of intestinal malrotation in the nonpediatric age group suggests that Ladd's procedure can be performed very safely. Moreover, the results suggest that patients with known malrotation should have Ladd's procedure performed electively rather than urgently....
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PMID: 18347850
PDF is available here.
Abstract
We discuss the appropriate radiologic investigations and the necessity for surgical intervention. The possible embryogenesis is hypothesized....
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PMID: 18639672
PDF is available here.
Abstract
We report an intrathoracic gastric duplication cyst detected antenatally on obstetrical ultrasonography at 33 weeks of gestation. After birth, the newborn was directly intubated. At day 19, the intrathoracic cyst was removed, and the patient discharged on his 27th day of life. Histologic findings co...
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PMID: 18639707
PDF is available here.
Abstract
An association between the deletion of chromosome 22q11.2, microgastria, and impaired mucociliary function has never been observed before. A casual association seems highly unlikely and we can not exclude the possibility of genetic mechanisms that may link those syndromes....
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PMID: 18493898
PDF is available here.
Abstract
Mean age of the patients was 6.3 years (range 4 months to 16 years). In the preoperative barium enema study, a narrowed distal segment with proximal dilatation was noted in 8 patients, dilatation of the sigmoid or rectum without a narrowed distal segment was observed in 4 patients, and no specific f...
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PMID: 18493893
PDF is available here.
Abstract
Over a period of 25 years, preduodenal portal vein was diagnosed in five patients. The diagnosis was made during surgery performed because of symptoms of high intestinal obstruction. All five patients had intestinal malrotation as well and, in all patients, another cause for high intestinal obstruct...
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PMID: 18493888
PDF is available here.
Abstract
Duplications of the entire colon are very rare. An 18-month-old boy presented with symptoms of chronic constipation and was noted with a complete colonic duplication. The presentation and management are discussed.
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PMID: 18558157
PDF is available here.
Abstract
We offer an unusual and previously unreported presentation of a gastric duplication cyst....
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PMID: 18558208
PDF is available here.
Abstract
The laparoscopic approach to CDO repair using U-clips is safe and efficacious. In addition, patients undergoing laparoscopic repair of CDO had a shorter length of hospitalization and more rapid advancement to full feeding compared to babies undergoing the open approach. We feel that in the hands of...
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PMID: 18558173
PDF is available here.
Abstract
We describe 2 cases of children younger than 1 year who were dependent on a central venous catheter for total parenteral nutrition. They developed a chronic extensive obstruction of the right and left brachiocephalic veins with a superior vena cava syndrome. The patients' survival was dependent on t...
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PMID: 18558160
PDF is available here.
S W SW Moore,
A A Alexander,
D D Sidler,
J J Alves,
G P GP Hadley,
A A Numanoglu,
B B Banieghbal,
M M Chitnis,
D D Birabwa-Male,
B B Mbuwayesango,
A A Hesse and
K K Lakhoo
Abstract
We retrospectively collected data on 1,401 ARM patients from six South African Paediatric Surgical units plus representative samples from five other African countries from West, Central and Southern Africa. Data included ethnic group, age, gender as well as the anatomical pathology, classification a...
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PMID: 18386020
PDF is available here.
Abstract
We report an asymptomatic 62-year-old woman with complete agenesis of the dorsal pancreas. Abdominal computed tomography (CT) revealed a normal pancreatic head, but pancreatic body and tail were not visualized. Magnetic resonance imaging (MRI) findings were similar to CT. At magnetic resonance chola...
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PMID: 18473421
PDF is available here.
Nicholas J Zyromski,
John A Sandoval,
Henry A Pitt,
Alan P Ladd,
Evan L Fogel,
Wissam E Mattar,
Kumar Sandrasegaran,
David W Amrhein,
Fredrick J Rescorla,
Thomas J Howard,
Keith D Lillemoe and
Jay L Grosfeld
Abstract
Annular pancreas is associated with a spectrum of disease that differs in children and adults. Congenital anomalies are more common in children with annular pancreas; complex pancreatobiliary disorders and malignancy are more frequent in adults....
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PMID: 18471747
PDF is available here.
Abstract
Good longterm outcomes with low operative morbidity can be obtained with TS in selected patients, including those with postgastric bypass, but younger age and chronic pancreatitis appear to predict poorer outcomes....
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PMID: 18471721
PDF is available here.
Abstract
We present an interesting case in which we used a handheld gamma probe intraoperatively to localize two accessory spleens. The two accessory spleens were successfully retrieved and removed. A handheld gamma probe is used as a valuable tool in localizing residual splenic tissue following splenectomy....
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PMID: 18535886
PDF is available here.
Abstract
Intestinal malrotation in adults is often associated with a delay in diagnosis and increased morbidity. Enhanced awareness of this entity in adults may enhance patient counseling and improve therapeutic outcomes in these patients....
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PMID: 18387471
PDF is available here.
Abstract
Eight of 9 patients were followed up except for one patient who died in a car accident at the age of three. All patients had been weaned off parenteral nutrition for more than 2 years. The average residual small bowel length was 58.1 cm (range 35-70 cm), and the mean parenteral nutrition (PN) durati...
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PMID: 18437651
PDF is available here.