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ABSTRACT: Operative treatment of giant omphalocele (OC) is still a challenge for pediatric surgeons. We were interested to ascertain whether published operative techniques for giant OC once advocated by their authors were still being used by these authors and whether the techniques had been modified or even abandoned for other techniques.
Relevant studies concerning the treatment of giant OC were identified by an electronic search. Publication date of the articles was from 1967 to 2009. A questionnaire was sent to the first author or coauthor, unless contact details were unavailable. The described surgical techniques were categorized into primary closure, staged closure, and delayed closure.
Almost half of the authors (42%), independent of the initial technique used (primary, staged, or delayed closure), changed or stopped using their technique after the publication of the article. The change was not to one particular proven better technique. Herniation rate was lower in delayed closure (9% delayed vs 18% staged vs 58% primary).
The results of the questionnaire did not show a generally accepted method of treatment after more than 30 years of innovations in managing patients with a giant OC. There are generally 2 main treatment modalities: staged closure and delayed closure. Because of the lack of large patient numbers and late follow-up, long-term results of the published techniques are needed, and randomized multicenter trials based on these outcomes are recommended. Until then, we remain dependent on expert opinions.
Journal of Pediatric Surgery 03/2011; 46(3):482-8. · 1.45 Impact Factor
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Charlotte H W Wijers,
Ivo de Blaauw,
Carlo L M Marcelis, Rene M H Wijnen,
Han Brunner,
Paola Midrio,
Piergiorgio Gamba,
Maurizio Clementi,
Ekkehart Jenetzky,
Nadine Zwink, [......],
Stefan Holland-Cunz,
Stuart Hosie,
Stefanie Märzheuser,
Eberhard Schmiedeke,
Célia Crétolle,
Sabine Sarnacki,
Marc A Levitt,
Nine V A M Knoers,
Nel Roeleveld,
Iris A L M van Rooij
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ABSTRACT: The recently established International Consortium on Anorectal Malformations aims to identify genetic and environmental risk factors in the etiology of syndromic and nonsyndromic anorectal malformations (ARM) by promoting collaboration through data sharing and combined research activities.
The consortium attempts to recruit at least 1,000 ARM cases. DNA samples are collected from case-parent triads to identify genetic factors involved in ARM. Several genetic techniques will be applied, including SNP arrays, gene and whole exome sequencing, and a genome-wide association study. Questionnaires inquiring about circumstances before and during pregnancy will be used to obtain environmental risk factor data.
Currently, 701 ARM cases have been recruited throughout Europe. Clinical data are available from all cases, and DNA samples and questionnaire data mainly from the Dutch and German cases. Preliminary analyses on environmental risk factors in the Dutch and German cohort found associations between ARM and family history of ARM, fever during first trimester of pregnancy and maternal job exposure to cleaning agents and solvents.
First results show that both genetic and environmental factors may contribute to the multifactorial etiology of ARM. The International Consortium on Anorectal Malformations will provide possibilities to study and detect important genes and environmental risk factors for ARM, ultimately resulting in better genetic counseling, improved therapies, and primary prevention.
Pediatric Surgery International 11/2010; 26(11):1093-9. · 1.25 Impact Factor
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ABSTRACT: After 25 years of practice and positive results of the Rehbein-procedure (RB) for children with Hirschsprung Disease (HD), we changed to the less invasive transanal endorectal pull through (TERPT). The aim of this study was to compare short- and mid-term complications of these two procedures in our patients with HD.
Retrospective data of 50 HD patients were analyzed. Of these patients, 25 underwent RB (2000-2006) and in 25 the TERPT was performed (2005-2009). Medical records were reviewed to score complications and outcomes. Differences were analyzed using Chi-Square and Mann-Whitney U tests.
All RB patients (100%) were given a colostomy compared with four patients (16%) in the TERPT group (p < 0.001). The average age at surgery in the RB group was 191 days whereas this was 72 days in the TERPT group (p < 0.01). The mean length of time of surgery in the RB group (158 min) was not significantly different from that in the TERPT group (183 min). Ganglion cells were located in all specimens at the proximal end of the specimens. The median time to first feeding significantly decreased from 2 days (range 1-11) in the RB group to 1 day (range 1-3) in the TERPT group (p < 0.01). The median length of hospital stay decreased in the TERPT group (8 days) compared with the RB group (10 days) (p < 0.001). There was a significant reduction in postoperative obstructive symptoms during the first 6 months in the TERPT group (48%) compared with the RB group (84%) (p = 0.016). Postoperative enterocolitis decreased from 40% in the RB group to 24% in the TERPT group although this was not statistically significant.
The introduction of TERPT reduced the need for colostomies; it shortened days to first feeding after surgery and reduced hospital stay. It also improved short-term outcome with less obstructive symptoms. We recommend TERPT surgery as a first choice in children with HD. we consider the RB now to be obsolete.
Pediatric Surgery International 11/2010; 26(11):1117-20. · 1.25 Impact Factor
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Nynke A Hosper,
Alex J Eggink,
Luc A J Roelofs, Rene M H Wijnen,
Marja J A van Luyn,
Ruud A Bank,
Martin C Harmsen,
Paul J Geutjes,
Willeke F Daamen,
Toin H van Kuppevelt,
Dorien M Tiemessen,
Egbert Oosterwijk,
Jane J Crevels,
Willeke A M Blokx,
Fred K Lotgering,
Paul P van den Berg,
Wout F J Feitz
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ABSTRACT: In spina bifida the neural tube fails to close during the embryonic period and it is thought that prolonged exposure of the unprotected spinal cord to the amniotic fluid during pregnancy causes additional neural damage. Intra-uterine repair might protect the neural tissue from exposure to amniotic fluid and might reduce additional neural damage. Biodegradable collagen scaffolds may be useful in case of fetal therapy for spina bifida, but biochemical properties need to be studied. The aim of this study was to investigate whether biodegradable collagen scaffolds can be used to treat full-thickness fetal skin defects. We hypothesized that the pro-angiogenic growth factors VEGF and FGF2 would enhance vascularization, epidermialization and lead to improved wound healing. To investigate the effect of these two growth factors, a fetal sheep model for skin defects was used. Compared to wounds treated with bare collagen scaffolds, wounds treated with growth factor-loaded scaffolds showed excessive formation of capillaries and less myofibroblasts were present in these wounds, leading to less contraction. This study has demonstrated that collagen scaffolds can be used to treat fetal skin defects and that the combination of collagen scaffolds with VEGF and FGF2 had a beneficial effect on wound healing.
Biomaterials 02/2010; 31(14):3910-9. · 7.40 Impact Factor
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ABSTRACT: Long-term outcome and quality of life in omphalocele (OC) studies are mainly focused on cosmetic disorders with the abdominal scar and gastrointestinal disorders. The aim of this study was to compare long-term mortality, morbidity, and quality of life between patients with minor and giant OCs.
Records of 89 minor and 22 giant OC children were reviewed. A questionnaire on general health was sent to all patients. A second questionnaire concerning quality of life and functional status; Darthmouth COOP Functional Health Assessment Charts/WONCA (COOP/WONCA) was sent to all patients aged 18 years or older and a peer control group.
Of the surviving patients (69 minor OC, 20 giant OC), 12 were lost to hospital follow-up. The first questionnaire was returned by 64 (83%) of 77 patients. There were no significant differences in gastrointestinal disorders. Cosmetic problems were experienced significantly more in giant OC. The results of the COOP/WONCA charts indicated a good to very good quality of life in both groups comparable to the control group.
Our study indicates that after a high level of medical intervention perinatally, quality of life is good to very good in both groups and comparable to healthy young adults.
Journal of Pediatric Surgery 08/2009; 44(7):1355-9. · 1.45 Impact Factor
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ABSTRACT: The aim of the study was to evaluate patient demographics, classification and location of the atresia, operative management, postoperative care, and outcome in 114 infants with jejunoileal atresia (JIA) over a period of more than 3 decades.
This was a retrospective case series in a tertiary care teaching hospital. Records of all patients with JIA treated at the authors' institution between 1971 and 2004 were examined.
Sixty-two percent of atresia and stenosis was noted in the jejunum, 30% in the ileum, and 8% in both the jejunum and the ileum. Atresias and stenosis were classified as follows: 7% type 0, 16% type I, 21% type II, 24% type IIIa, 10% type IIIb, 22% type IV. Gastrointestinal anomalies were encountered in 24% of patients, genitourinary malformations in 9%, cystic fibrosis in 9%, neurologic anomalies in 6%, and congenital heart disease in 4%. Operative management included resection with primary anastomosis in 69% of all patients and temporary enterostomies in 26%. After operative management, 15% of children had resultant short bowel syndrome. Oral feeding was allowed on median day 7, and full energy expenditure via the enteric route was reached on median day 20. Forty-seven percent of infants required central venous line placement for total parenteral nutrition. Early postoperative complications occurred in 28% of patients with JIA and late postoperative complications in 17%. We observed a mortality rate of 11%.
This is one of the largest series of neonates with JIA described. Short bowel syndrome seems to be the biggest problem resulting in longer hospital stay, more feeding problems, and higher morbidity and mortality rates. Management of children with short bowel syndrome has improved because of the use of total parenteral nutrition, new operative techniques, and better intensive care. In the last 15 years, survival has increased at the cost of the surviving children as we noted a higher percentage of late complications.
Journal of Pediatric Surgery 02/2009; 44(1):217-21. · 1.45 Impact Factor
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ABSTRACT: We evaluated the regeneration of the abdominal wall using a dual-layer collagen biomatrix, and the protective effect on the bowel of fetal abdominal wall repair in a fetal sheep model for gastroschisis. In 14 fetal lambs, the abdominal wall was opened at 79 days' gestation, creating a gastroschisis. In group 1, the gastroschisis was left uncovered. In group 2, the bowel was repositioned, and the defect was closed by suturing a collagen biomatrix into the abdominal wall. A cesarean section was performed at 140 days' gestation, and macroscopic and histological evaluation was performed. In the five lambs with a gastroschisis, the eviscerated part of the bowel was coalescent, showed extensive adhesions, and was covered by fibrous peel. In group 2, the abdominal wall had closed, with a firm connection to the native abdominal wall. The biomatrix was largely degraded and replaced by connective tissue with collagen and fibroblasts, neovascularisation, and scattered muscle cells. Minor or no adhesions of the bowel and no peel formation were observed. Abdominal wall tissue replacement using a collagen biomatrix was feasible in fetal lambs, resulting in a closed abdominal wall at birth. Immediate closure of the gastroschisis strongly diminished or prevented bowel adhesions and peel formation.
Tissue Engineering Part A 12/2008; 14(12):2033-40. · 4.64 Impact Factor
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ABSTRACT: Treatment of blunt injury of the pancreas in children remains controversial. Some prefer nonoperative treatment, whereas others prefer operative management in selected cases. This report reviews the treatment of patients with blunt pancreatic trauma admitted to a level I pediatric trauma center in The Netherlands.
Medical records of all children less than 15 years with blunt pancreatic trauma admitted to the University Medical Center St Radboud in the period 1975 to 2003 were retrospectively analyzed.
Thirty-four children were included, age 3 to 14 years. Most injuries were because of bicycle accidents (58%). On admission, amylase was raised in 90% of the patients. Five patients had pancreatic duct injuries identified by imaging (endoscopic retrograde cholangiopancreaticography was used once, magnetic resonance cholangiopancreaticography twice) or at surgery. Thirty-one children were initially managed nonoperatively. Pancreatic surgery was performed in 3 children (1 Roux-Y, 2 drainage only). Mean hospital stay was 29 days in the operative group and 24 days in the nonoperative group. Fluid collections developed in 2 operated patients. Both resolved spontaneously. In 14 of the 31 nonoperated patients, a pseudocyst developed. Only 6 of these needed secondary intervention. Of these, 3 were drained percutaneously. There was no mortality and no long-term morbidity in both groups.
Nonoperative management of pancreatic injury in children has good clinical outcome. Only 10% need secondary surgery. In 50%, pseudocysts develop of which half can be managed nonoperatively. The reliability of computed tomographic scan grading is of limited value to decide whether to operate primarily. There is little to gain with ERCP and stenting. The place of MRCP as a noninvasive diagnostic tool remains to be determined.
Journal of Pediatric Surgery 10/2008; 43(9):1640-3. · 1.45 Impact Factor
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ABSTRACT: Adhesive small bowel obstruction (SBO) is a feared complication after correction of abdominal wall defects in neonates. Knowledge of its incidence and potential risk factors in a well-documented group with strict follow-up is needed to guide preventive measures.
Records of 170 neonates with abdominal wall defects, 59 gastroschisis (GS) and 111 omphalocele (OC), were reviewed focusing on SBO. Risk of SBO was calculated, and potential risk factors were analyzed. Long-term complaints possibly associated with adhesions were assessed through questionnaire.
One hundred forty-seven neonates were operated on, 12 were treated nonoperatively, and 11 patients died shortly after birth. Defects were primarily closed in 128, 7 neonates needed prosthetic mesh, and 12 had a silastic sac inserted. Twenty-six (18%) neonates had SBO, 14 (25%) of 55 with GS, and 12 (13%) of 92 with OC (P = .06). Of the 26 with SBO, 26 (88%) needed laparotomy. Four patients died because of SBO. Most episodes (85%) were in the first year. Sepsis and fascia dehiscence were predicting risk factors for SBO. Abdominal pain and constipation were frequent long-term complaints not significantly associated with SBO.
Adhesive SBO is a frequent and serious complication in the first year after treatment of congenital abdominal wall defects. Sepsis and fascial dehiscence are predictive factors.
Journal of Pediatric Surgery 04/2008; 43(3):479-83. · 1.45 Impact Factor
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ABSTRACT: Several techniques have been described to repair giant omphaloceles. There is no procedure considered to be the criterion standard worldwide. The aim of the present prospective study was to analyze the early and late results of secondary closure of giant omphaloceles using the component separation technique (CST) in infants.
From January 2004 to January 2007, 10 consecutive pediatric patients with a giant omphalocele were treated at our department. Initially, patients were treated conservatively. After epithelialization of the omphalocele, the abdominal wall was reconstructed using CST. Patients were monitored for complications during admission, and all patients were seen for follow-up.
Component separation technique was performed at median age of 6.5 months (range, 5-69 months). The median diameter of the hernia was 8 cm (range, 6-9 cm). There was no mortality. The postoperative course was uneventful in 7 patients. Complications were seen in 3 patients (infection, skin necrosis, and hematoma). Median hospital stay was 7 days. After median follow-up of 23.5 months (range, 3-39 month), no reherniations were found.
The CST is a safe 1-stage procedure for secondary closure in children with a giant omphalocele without the need for prosthetic material and with good clinical outcome.
Journal of Pediatric Surgery 02/2008; 43(1):246-50. · 1.45 Impact Factor
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Luc A J Roelofs,
Alex J Eggink,
Christina A Hulsbergen-van de Kaa, Rene M H Wijnen,
Toin H van Kuppevelt,
Herman T B van Moerkerk,
A Jane Crevels,
Alex Hanssen,
Fred K Lotgering,
Paul P van den Berg,
Wout F J Feitz
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ABSTRACT: To evaluate histological changes in an animal model for bladder exstrophy and fetal repair of the bladder defect with a molecular-defined dual-layer collagen biomatrix to induce fetal bladder wall regeneration.
In 12 fetal lambs the abdominal wall and bladder were opened by a midline incision at 79 days' gestation. In 6 of these lambs an uncorrected bladder exstrophy was created by suturing the edges of the opened bladder to the abdominal wall (group 1). The other 6 lambs served as a repair group, where a dual-layer collagen biomatrix was sutured into the bladder wall and the abdominal wall was closed (group 2). A caesarean section was performed at 140 days' gestation, followed by macroscopic and histological examination.
Group 1 showed inflammatory and maturational changes in the mucosa, submucosa and detrusor muscle of all the bladders. In group 2, bladder regeneration was observed, with urothelial coverage, ingrowth of fibroblasts and smooth muscle cells, deposition of collagen, neovascularization and nerve fibre formation. This tissue replaced the collagen biomatrix. No structural changes of the bladder were seen in group 2.
The animal model, as in group 1, for bladder exstrophy shows remarkable histological resemblance with the naturally occurring anomaly in humans. This model can be used to develop new methods to salvage or regenerate bladder tissue in bladder exstrophy patients. Fetal bladder wall regeneration with a collagen biomatrix is feasible in this model, resulting in renewed formation of urothelium, blood vessels, nerve fibres, ingrowth of smooth muscle cells and salvage of the native bladder.
Fetal Diagnosis and Therapy 02/2008; 24(1):7-14. · 1.05 Impact Factor
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ABSTRACT: The current report describes a case of an infant girl with a giant omphalocele in whom a new surgical technique was used for closing the abdominal wall after epithelialization of the omphalocele for 16 months. The technique used was translation of the muscular layers of the abdominal wall. The functional and cosmetic results appear superior compared with other suggested treatments used for this abdominal wall defect.
Pediatric Surgery International 06/2005; 21(5):373-6. · 1.25 Impact Factor
