Failed preoperative vacuum bell therapy does not affect outcomes following minimally invasive repair of pectus excavatum.

PURPOSE: It is unknown if failed preoperative vacuum bell (VB) treatment in patients undergoing minimally invasive repair of pectus excavatum (MIRPE), delays repair and/or affects postoperative outcomes. METHODS: A retrospective data analysis including all consecutive patients treated at one single institution undergoing MIRPE was performed between 2000 and 2016. Patients were stratified into preoperative VB therapy versus no previous VB therapy. RESULTS: In total, 127 patients were included. Twenty-seven (21.3%) patients had preoperative VB treatment for 17 months (median, IQR 8-34). All 27 patients stopped VB treatment due to the lack of treatment effect. Eight (47.1%) of 17 assessed VB patients showed signs of skin irritation or hematoma. VB treatment had no effect on length of hospital stay (p = 0.385), postoperative complications (p = 1.0), bar dislocations (p = 1.0), and duration of bar treatment (p = 0.174). Time spent in intensive care unit was shorter in patients with VB therapy (p = 0.007). Long-term perception of treatment including rating of primary operation (p = 0.113), pain during primary operation (p = 0.838), own perspective of look of chest (p = 0.545), satisfaction with the procedure (p = 0.409), and intention of doing surgery again (p = 1.0) were not different between groups. CONCLUSIONS: Failed preoperative VB therapy had no or minimal effect on short-term outcomes and long-term perceptions following MIRPE.

Intestinal IMINO transporter SIT1 is not expressed in human newborns.

The expression of amino acid transporters in small intestine epithelia of human newborns has not been studied yet. It is further not known whether the maturation of imino acid (proline) transport is delayed as in the kidney proximal tubule. The possibility to obtain small intestinal tissue from patients undergoing surgery for jejunal or ileal atresia during their first days after birth was used to address these questions. As control, adult terminal ileum tissue was sampled during routine endoscopies. Gene expression of luminal imino and amino acid transporter SIT1 (SLC6A20) was approximately threefold lower in newborns versus adults. mRNA levels of all other luminal and basolateral amino acid transporters and accessory proteins tested were similar in newborn mucosa compared with adults. At the protein level, the major luminal neutral amino acid transporter B0AT1 (SLC6A19) and its accessory protein angiotensin-converting enzyme 2 were shown by immunofluorescence to be expressed similarly in newborns and in adults. SIT1 protein was not detectable in the small intestine of human newborns, in contrast to adults. The morphology of newborn intestinal mucosa proximal and distal to the obstruction was generally normal, but a decreased proliferation rate was visualized distally of the atresia by lower levels of the mitosis marker Ki-67. The mRNA level of the 13 tested amino acid transporters and accessory proteins was nonetheless similar, suggesting that the intestinal obstruction and interruption of amniotic fluid passage through the small intestinal lumen did not affect amino acid transporter expression. NEW & NOTEWORTHY System IMINO transporter SIT1 is not expressed in the small intestine of human newborns. This new finding resembles the situation in the proximal kidney tubule leading to iminoglycinuria. Lack of amniotic fluid passage in small intestinal atresia does not affect amino acid transporter expression distal to intestinal occlusion.

Comparison of environmental risk factors for esophageal atresia, anorectal malformations, and the combined phenotype in 263 German families.

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and anorectal malformations (ARM) represent the severe ends of the fore- and hindgut malformation spectra. Previous research suggests that environmental factors are implicated in their etiology. These risk factors might indicate the influence of specific etiological mechanisms on distinct developmental processes (e.g. fore- vs. hindgut malformation). The present study compared environmental factors in patients with isolated EA/TEF, isolated ARM, and the combined phenotype during the periconceptional period and the first trimester of pregnancy in order to investigate the hypothesis that fore- and hindgut malformations involve differing environmental factors. Patients with isolated EA/TEF (n = 98), isolated ARM (n = 123), and the combined phenotype (n = 42) were included. Families were recruited within the context of two German multicenter studies of the genetic and environmental causes of EA/TEF (great consortium) and ARM (CURE-Net). Exposures of interest were ascertained using an epidemiological questionnaire. Chi-square, Fisher's exact, and Mann-Whitney U-tests were used to assess differences between the three phenotypes. Newborns with isolated EA/TEF and the combined phenotype had significantly lower birth weights than newborns with isolated ARM (P = 0.001 and P < 0.0001, respectively). Mothers of isolated EA/TEF consumed more alcohol periconceptional (80%) than mothers of isolated ARM or the combined phenotype (each 67%). Parental smoking (P = 0.003) and artificial reproductive techniques (P = 0.03) were associated with isolated ARM. Unexpectedly, maternal periconceptional multivitamin supplementation was most frequent among patients with the most severe form of disorder, i.e. the combined phenotype (19%). Significant differences in birth weight were apparent between the three phenotype groups. This might be attributable to the limited ability of EA/TEF fetuses to swallow amniotic fluid, thus depriving them of its nutritive properties. Furthermore, the present data suggest that fore- and hindgut malformations involve differing environmental factors. Maternal periconceptional multivitamin supplementation was highest among patients with the combined phenotype. This latter finding is contrary to expectation, and warrants further analysis in large prospective epidemiological studies.

European consensus meeting of ARM-Net members concerning diagnosis and early management of newborns with anorectal malformations.

The ARM-Net (anorectal malformation network) consortium held a consensus meeting in which the classification of ARM and preoperative workup were evaluated with the aim of improving monitoring of treatment and outcome. The Krickenbeck classification of ARM and preoperative workup suggested by Levitt and Peña, used as a template, were discussed, and a collaborative consensus was achieved. The Krickenbeck classification is appropriate in describing ARM for clinical use. The preoperative workup was slightly modified. In males with a visible fistula, no cross-table lateral X-ray is needed and an anoplasty or (mini-) posterior sagittal anorectoplasty can directly be performed. In females with a small vestibular fistula (Hegar size <5 mm), a primary repair or colostomy is recommended; the repair may be delayed if the fistula admits a Hegar size >5 mm, and in the meantime, gentle painless dilatations can be performed. In both male and female perineal fistula and either a low birth weight (<2,000 g) or severe associated congenital anomalies, prolonged preoperative painless dilatations might be indicated to decrease perioperative morbidity caused by general anesthesia. The Krickenbeck classification is appropriate in describing ARM for clinical use. Some minor modifications to the preoperative workup by Levitt and Peña have been introduced in order to refine terminology and establish a comprehensive preoperative workup.

Models of short bowel syndrome in pigs: a technical review.

BACKGROUND: Short bowel syndrome (SBS) is still a life-threatening disease in both children and adults. Although the therapeutic options are improving, challenges still remain, and to overcome these challenges is a major focus of SBS research today. In order to simulate anatomical and physiological conditions similar to those in humans for research, porcine models of SBS are often used. Various approaches for generating SBS models have been described in the literature. METHODS/RESULTS: In this work, we present a review of different types of porcine models of SBS and outline the differences between those models regarding types of animals, surgical procedures, monitoring, and methods of assessment. CONCLUSION: The aim of this study was to select the most suitable SBS model regarding the purpose of the research.

Urinary matrix metalloproteinases-2/9 in healthy infants and haemangioma patients prior to and during propranolol therapy.

UNLABELLED: The mechanism of therapeutic success of propranolol for severe infantile haemangioma remains unclear. Propranolol was shown to modify matrix metalloproteinase (MMP) levels, which are associated with tumour pathogenesis. We hypothesized that urinary MMP2/9 is higher in patients with infantile haemangioma compared to healthy infants and that propranolol reduces MMP2/9 levels and thus leads to an involution of the haemangioma. In this case, MMP2/9 could be used as a marker of indicated therapy or therapeutic success. Urinary samples were taken before, 2 weeks after, and 2 months after the beginning of propranolol treatment in haemangioma patients and once in healthy controls. Activity of MMP2/9 was determined by commercially available activity kits. Urine of 22 haemangioma patients and 21 control subjects was obtained. Propranolol therapy had significant success in all patients. MMP2/9 was present in most samples, the younger the children the higher the MMP2 levels. Haemangioma patients showed lower levels of MMP2. The MMP2 levels were significantly higher after 2 weeks of propranolol than prior to therapy. There were no differences in MMP9 levels. CONCLUSIONS: Presence of MMP2/9 in the urine of infants <1 year can be explained by high rate of physiological tissue remodelling. Unexpectedly, MMP2 was lower in the urine of haemangioma patients and higher 2 weeks after propranolol treatment. Taking this and the diverse results in literature into account, the correlation between MMPs, proliferation, and regression of haemangiomas and propranolol remains unclear.

Current practice and future perspectives in the treatment of short bowel syndrome in children--a systematic review.

PURPOSE: Short bowel syndrome (SBS) is a malabsorption disorder of the intestine, which leads to an inadequate alimentary supply. A number of therapeutic approaches are already in use, but research advances may provide new options in the future. The purpose of this paper was to provide an overview of the established therapeutic approaches together with a discussion of the future perspectives in the treatment of patients with SBS. We review those studies dealing with the treatment of SBS patients and discuss both surgical and non-surgical approaches together with tissue engineering. METHODS: A systemic review of Medline-cited studies dealing with current practice and future perspectives in the treatment of short bowel in children was performed. RESULTS: Surgical approaches, non-surgical approaches, and tissue engineering which was used in the treatment of SBS were analyzed. Among the surgical approaches, the bowel lengthening procedures and small bowel transplantation are prevalent. Stimulants are most important concerning non-surgical approaches. Tissue engineering seems to be more experimental and was also evaluated. CONCLUSION: The treatment of SBS patients remains very complex. It is eminent to find the best therapeutic option for each patient and to individualize and modify the different possible types of applied techniques frequently.

Practice of dilatation after surgical correction in anorectal malformations.

BACKGROUND: In order to prevent stricture of the neoanus after surgical correction, regular dilatation is recommended. There is a lack of knowledge about the performance of anal dilatation and the occurrence of pain. The aim of our investigation was to describe the practice of dilatation and to identify possible risk factors for painful procedures. METHODS: Congenital Uro-Rectal Malformations Network is a German interdisciplinary multicenter research network. With standard questionnaires, physicians interviewed 243 patients and/or their parents at home, additional 103 patients born since 2009 were assessed through their treating physicians. RESULTS: In total, 88 % of the patients received dilatations. Treatment lasted for 7 months in median (range 1-156 months), until the age of 13 months (range 1-171 months). In 69 % painful dilatation was reported; without a significant differences in age or gender. In 32 % bleeding was reported. In 30 % at least one dilatation was performed under general anesthesia. In 11 % some kind of analgesia was used. Type of fistula, dilatations lasting longer than 10 months and Hegar size above 15 were relevant factors for experience of pain. There were about 16 % postoperative strictures of the neoanus, without reported differences in dilatation procedures; but there was a relation to type of malformation. CONCLUSION: Considering the high number of painful treatments, predictors for painful dilatations should be further clarified through standardized documentation and prospective evaluation in order to improve follow-up.

[Elbow dislocation in childhood. Long-term observational study]. / Ellenbogenluxationen im Kindesalter. Eine Langzeitbeobachtung.

Traumatic dislocation of the elbow is rare in children with an incidence of 3-6% of all elbow injuries. In the literature the outcome after elbow dislocation in childhood is rarely discussed. In the present study 33 children treated in our clinic from 2001 to 2008 with an acute traumatic dislocation of the elbow were retrospectively included. All events were unilateral whereby 1 child (3%) showed a recurrence of elbow dislocation after 9 weeks, 30% had a pure dislocation, 70% had a concomitant fracture, 55% showed a fracture of the medial epicondyle, 6% a fracture of the lateral epicondyle and 9% a further fracture. Of the fractures 83% required open reduction with osteosynthesis. After an average of 4.5 years 20 children (61%) were clinically examined. There were no instabilities of the joint and only minor clinical limitations of the range of motion. The established Mayo elbow performance score showed good to excellent results for all children. Despite severe joint trauma with frequently accompanying fractures, post-traumatic functional deficits are rarely limiting, independent of the accompanying fracture. The frequency of recurrence is low and instabilities were not seen.

Revision surgery in pectus excavatum after failure of Nuss technique.

A 17-year-old female had undergone two failed pectus excavatum repairs using the Nuss technique. Each time the bar had shifted, one time displacing the heart. Revision surgery was carried out using the Erlangen technique, which proved to be a safe and successful method to remove the displaced bar and correct the pectus excavatum.

Magnetic Double-J-Stent Removal Without General Anaesthesia in Children.

OBJECTIVES: To show the feasibility of magnetic double-J-stent (mDJS) removal without general anaesthesia in infants and children. METHODS: A retrospective analysis of mDJS removals was conducted between February 2018 and July 2020 in a cohort of 32 consecutive paediatric patients. Only patients with unilateral ureteric stenting were included. Stent retrieval was performed in an outpatient setting. In males the junction of the retrieval-catheter and the mDJS was confirmed by transabdominal ultrasound. All patients were followed-up for 4-12 weeks after stent removal. RESULTS: Thirty-two patients (54% males) were included. Median age was 3.8 years (range 1 month - 15 years). Ureteral stents remained in place for a median of 67.5 days (range 2 days - 6 months). General anaesthesia was necessary in one single patient due to expressed patient's and caregiver's wish. Thirty-one patients had stent removal without need for general anaesthesia. Thereof, nitrous oxide was used in 12 patients (37.5%), fentanyl in 3 patients (9.4%), midazolam in 3 patients (9.4%), and 17 patients (53.1%) did not need sedation at all. Seven patients (21.9%) being 8 months or younger had received peroral saccharosis. No complications occurred during stent removal. Retrieval was successful at first catheterization in 30 patients (93.8%). Two male patients needed a second catheterization (6.3%). CONCLUSION: The use of magnetic DJS is safe in the paediatric population and spares general anaesthesia during removal in almost all patients.

3D perfusion mapping and virtual surgical planning in the treatment of pediatric embryonal abdominal tumors.

INTRODUCTION: 3D imaging and surgical planning for the treatment of embryonal tumors using different techniques (CT versus MRI) are presently under discussion. Up to now, the main focus has been on visualizing the anatomy. Contrast medium dynamics have not been taken into consideration. The aim of the present study was to establish the technical means of integrating the 3D images from functional MRI data into the anatomical images and to determine clinical applications for this approach. MATERIAL AND METHODS: In 11 patients (mean age: 2.4 years) with solid tumors, 26 diagnostic MRI examinations were performed for primary diagnosis, treatment monitoring, or as part of the surgical planning. Seven children presented with neuroblastomas, three with Wilms' tumor, and one with advanced bilateral nephroblastomatosis. The MRI data were acquired using a 1.5-T system. For post-processing, we used volume rendering software, including an evaluation of perfusion. By using color-coded parametric images and integrating functional information, perfusion could be visualized and used for interactive surgical planning. Macroscopic and microscopic sections served as the gold standard for assessing tissue viability. RESULTS: We were able to integrate the dynamic data into the anatomical images for all patients. A good agreement was found between the results of surgical planning, including perfusion mapping, with the surgical site, subsequently produced macroscopic sections and the results of random microscopic examinations. CONCLUSIONS: Perfusion mapping using color-coded parametric images of pediatric abdominal tumors extends the diagnostic techniques currently available. We provide first proof of the possibility of integrating functional information into 3D MR images in children. Monitoring the treatment of nephroblastoma and surgical planning for pediatric embryonal tumors represent potential applications of this technique.

Chance fracture--a rare injury in pediatric patients?

We report on a 9-year-old girl who was involved in a car accident. She suffered severe polytrauma with torn abdominal muscles, rupture of the mesenteric arteries, bowel and bladder, hematoma at the right colonic flexure and disruption of the intervertebral ligaments L2/L3, including the intervertebral disc, typical of Chance fracture. The abdominal bleeding was stopped, the bowel resected and the fracture fixed by internal fixation. The patient developed a postoperative enterocutaneous fistula in the right flank and paraplegia. She underwent three laparotomies with ileostomy and closure of the fistula. Two years later, she has normal bowel movement, the wounds are closed, the internal fixation has been removed, she is able to walk with crutches and suffers from a mild bladder dysfunction. Chance fracture is a typical fracture in adults involved in motor vehicle accidents. In the last 10 years, there have only been four case reports describing this fracture in children under the age of 10. All of these individuals were involved in a car accident and had been fixed with a lap belt. With the increasing use of lap belts, this fracture has to be considered even in young children. Mild clinical symptoms can be associated with severe intra-abdominal injuries.

Feminising genitoplasty: one-stage genital reconstruction in congenital adrenal hyperplasia: 30 years' experience.

The study objective is to evaluate the results of our surgical technique for children with congenital adrenal hyperplasia and ambiguous genitalia at the University Hospital of Heidelberg, Department of Paediatric Surgery. The records of 19 patients with congenital adrenal hyperplasia treated between 1972 and 2004 were reviewed with respect to age at surgery, operative procedures and outcome. We describe the recession clitoroplasty technique currently used in our hospital and highlight the importance of short and long-term follow-up results with respect to appearance, position and size of the clitoris and quality of the vagina. One-stage recession clitoroplasty and vaginoplasty gives very satisfactory cosmetic and functional results, with few complications and a reduced need for secondary surgical interventions. The results of this study support the assumption that total correction can be achieved through a single-stage operation, performed in infancy.

[Fetal surgery]. / Fetale Chirurgie.

Fetal surgery is a newly evolving multidisciplinary medical field, being performed in specialized centers. It deals with the treatment of selected fetuses with congenital anomalies that cause high morbidity and mortality. The aim of the surgical treatment is to avoid the development of progressive and eventually irreversible damage at birth. Examples of entities treated are obstructive uropathy leading to renal insufficiency, lung hypoplasia in severe congenital diaphragmatic hernia, severe congenital cystic adenomatoid malformation of the lung, and sacrococcygeal teratoma. This review describes principles of fetal surgery, physiopathologic processes of some of the anomalies treated in this way, and diagnostic and therapeutic approaches. Recently published results are presented and discussed.

Long-term effects of fundoplication in children with chronic airway diseases.

BACKGROUND: Association between chronic airway diseases (CAD) and gastroesophageal reflux disease (GERD) is well described, but causality has not yet been conclusively established. This study evaluates the therapeutic significance of laparoscopic Thal fundoplication in children with CAD and diagnosed GERD. METHODS: We performed a retrospective analysis of 182 neurologically nonimpaired children, all with medically refractory CAD and GERD undergoing laparoscopic Thal fundoplication. The clinical response, ability to wean oral and inhaled medication and satisfaction with postoperative results were evaluated. RESULTS: Main symptoms disappeared completely in 68.7% of patients and were markedly improved in a further 22% of patients following surgery. Complete discontinuation of medication was achieved in 70.1-96.4% of cases and reduced in a further 1.8-23.5%. One intraoperative complication occurred (gastric perforation), however no conversion to laparotomy was necessary. Postoperative Dumping Syndrome occurred in 1% of cases and was managed dietetically. Prolonged postoperative dysphagia occurred in 4.3% of patients, but disappeared within 8 weeks in all but one case. CONCLUSIONS: Our study suggests that Thal fundoplication in neurologically nonimpaired children with CAD and documented GERD is effective and safe. Children unresponsive to preoperative medical management showed significant improvement in airway symptoms together with a marked reduction in the need for medication. We conclude that laparoscopic Thal fundoplication represents a significant treatment worthy of consideration in this group of patients.

What do knockout models teach us about the enteric nervous system?

Since the first histological studies, enormous strides have been made in understanding the genetics and cell biology of enteric nervous system (ENS) formation. Several mitogenic and trophic factors have been implicated in the process of neural cell proliferation and differentiation. A number of natural (piebald-lethal mice [s l], lethal spotting mice [ls], spotting lethal rats [sl]) or target (Gfralpha1-deficient mice, ret.k - mice, and NT-4 knockout mice) mutations have been reported to produce developmental defects in neural crest cell migration, differentiation or survival. Study of these mutations continues to provide new insights into this complex system. In the present investigation, we showed that a lack of basic fibroblast growth factor (FGF) or growth hormone (GH) leads to morphological abnormalities of the enteric nervous system. Because knockouts, neither of FGF nor of GH, produce enteric nervous system defects substantial enough to compromise the ability of the gut to support life, we postulate that FGF and GH affect only a relatively small subset of neurons and/or that compensatory effects of other growth factors might occur.