Quality of Life of Children with Short Bowel Syndrome from Patients' and Parents' Points of View.

Despite limited research, existing studies using generic quality of life (QOL) tools indicate decreased physical health and compromised emotional functioning in children with IF. This study investigates QOL in children with short bowel syndrome (SBS) and its determinants. The study included 57 pediatric patients with SBS treated at Mannheim's University Hospital between 1998 and 2014. To evaluate QOL, the KINDL questionnaire was used. Three age-specific questionnaire variants were employed, and parental proxy reports were collected. Most patients underwent intestinal lengthening procedures, with varying primary diagnoses. A comparison with healthy children from the patient's perspective revealed no difference but from the parent's perspective showed lower QOL in SBS patients, especially regarding physical and mental well-being. QOL varied with age, with 7-10-year-olds reporting the lowest scores. Several factors, including independence from parenteral nutrition and the presence of a complete colon, positively influenced QOL. The independence of parenteral nutrition and the presence of a complete colon positively influenced QOL. The Bianchi technique for intestinal lengthening has also shown promise but needs further research. The observation sample in this study is too small to generalize about the whole population of SBS patients. However, this study shows that many health and treatment factors affect QOL, and a large multicenter study is necessary. Our findings underline the importance of appropriate psychological support for children with SBS and their families.

Long-Term Evaluation of the Shape of the Reconstructed Diaphragm in Patients with Left-Sided Congenital Diaphragmatic Hernia Using Serial Chest Radiographs and Correlation to Further Complications.

Background: Defining risk factors for long-term comorbidities in patients after neonatal repair of congenital diaphragmatic hernia (CDH) is an important cornerstone of the implementation of targeted longitudinal follow-up programs. Methods: This study systematically assessed serial chest radiographs of 89 patients with left-sided CDH throughout a mean follow-up of 8.2 years. These geometrical variables for the left and right side were recorded: diaphragmatic angle (LDA, RDA), diaphragmatic diameter (LDD, RDD), diaphragmatic height (LDH, RDH), diaphragmatic curvature index (LDCI, RDCI), lower lung diameter (LLLD, RLLD) and thoracic area (LTA, RTA). Results: It was demonstrated that the shape of the diaphragm in patients with large defects systematically differs from that of patients with small defects. Characteristically, patients with large defects present with a smaller LDCI (5.1 vs. 8.4, p < 0.001) at 6 months of age, which increases over time (11.4 vs. 7.0 at the age of 15.5 years, p = 0.727), representing a flattening of the patch and the attached rudimentary diaphragm as the child grows. Conclusions: Multiple variables during early follow-up were significantly associated with comorbidities such as recurrence, scoliotic curves of the spine and a reduced thoracic area. Some geometrical variables may serve as surrogate parameters for disease severity, which is associated with long-term comorbidities.

Muscle hypertrophy and neuroplasticity in the small bowel in short bowel syndrome.

Short bowel syndrome (SBS) is a severe, life-threatening condition and one of the leading causes of intestinal failure in children. Here we were interested in changes in muscle layers and especially in the myenteric plexus of the enteric nervous system (ENS) of the small bowel in the context of intestinal adaptation. Twelve rats underwent a massive resection of the small intestine to induce SBS. Sham laparotomy without small bowel transection was performed in 10 rats. Two weeks after surgery, the remaining jejunum and ileum were harvested and studied. Samples of human small bowel were obtained from patients who underwent resection of small bowel segments due to a medical indication. Morphological changes in the muscle layers and the expression of nestin, a marker for neuronal plasticity, were studied. Following SBS, muscle tissue increases significantly in both parts of the small bowel, i.e., jejunum and ileum. The leading pathophysiological mechanism of these changes is hypertrophy. Additionally, we observed an increased nestin expression in the myenteric plexus in the remaining bowel with SBS. Our human data also showed that in patients with SBS, the proportion of stem cells in the myenteric plexus had risen by more than twofold. Our findings suggest that the ENS is tightly connected to changes in intestinal muscle layers and is critically involved in the process of intestinal adaptation to SBS.

Impact of cryopreservation on viability, gene expression and function of enteric nervous system derived neurospheres.

Introduction: Impairment of both the central and peripheral nervous system is a major cause of mortality and disability. It varies from an affection of the brain to various types of enteric dysganglionosis. Congenital enteric dysganglionosis is characterized by the local absence of intrinsic innervation due to deficits in either migration, proliferation or differentiation of neural stem cells. Despite surgery, children's quality of life is reduced. Neural stem cell transplantation seems a promising therapeutic approach, requiring huge amounts of cells and multiple approaches to fully colonize the diseased areas completely. A combination of successful expansion and storage of neural stem cells is needed until a sufficient amount of cells is generated. This must be combined with suitable cell transplantation strategies, that cover all the area affected. Cryopreservation provides the possibility to store cells for long time, unfortunately with side effects, i.e., upon vitality. Methods: In this study we investigate the impact of different freezing and thawing protocols (M1-M4) upon enteric neural stem cell survival, protein and gene expression, and cell function. Results: Freezing enteric nervous system derived neurospheres (ENSdN) following slow-freezing protocols (M1-3) resulted in higher survival rates than flash-freezing (M4). RNA expression profiles were least affected by freezing protocols M1/2, whereas the protein expression of ENSdN remained unchanged after treatment with protocol M1 only. Cells treated with the most promising freezing protocol (M1, slow freezing in fetal calf serum plus 10% DMSO) were subsequently investigated using single-cell calcium imaging. Freezing of ENSdN did not alter the increase in intracellular calcium in response to a specific set of stimuli. Single cells could be assigned to functional subgroups according to response patterns and a significant shift towards cells responding to nicotine was observed after freezing. Discussion: The results demonstrate that cryopreservation of ENSdN is possible with reduced viability, only slight changes in protein/gene expression patterns and without an impact on the neuronal function of different enteric nervous system cell subtypes, with the exception of a subtle upregulation of cells expressing nicotinergic acetylcholine receptors. In summary, cryopreservation presents a good method to store sufficient amounts of enteric neural stem cells without neuronal impairment, in order to enable subsequent transplantation of cells into compromised tissues.

Use of Temporary Double-J Stent Placement for Children With Congenital Hydronephrosis: A Long-Term Single-Center Cohort Study.

OBJECTIVE: To evaluate the utilization of double-J stents in children with congenital hydronephrosis in order to avoid or postpone more invasive surgical intervention. Numerous studies have demonstrated that congenital hydronephrosis caused by ureteropelvic junction obstruction (UPJO) or primary obstructive megaureter (POM) may require a surgical correction in up to 20% of cases. METHODS: All infants with severe hydronephrosis and/or an obstructive pattern on renal scintigraphy that received double-J stent placement between 2010 to 2015 in our center were analyzed. Children were followed regularly with ultrasound and received antibiotic metaphylaxis. Urinary tract infection (UTI) and double-J dislocation were defined as complications. Treatment success was defined as avoidance of surgery and reduction of hydronephrosis to grade 1 or 0 during the observation period. RESULTS: 29 children were included, in these, 34 (23 UPJO, 7 POM, 4 UPJO and POM) treatment attempts were performed. Stent implantation failed in six cases, resulting in 28 double-J stent treatments, of which 19 (69%) were successful within the follow-up period of 20 to 104 months. The most common complications were febrile UTI in 6 of29 cases and double-J dislocation in 3 of 29 cases. CONCLUSION: During the observation period, the success rate of temporary double-J in urodynamically relevant obstruction was relatively high. However, urinary tract infections and a complex disease course due to renal stent dislocation, as well as the need for repeated anesthesia and radiation exposure, should be taken into account. Hence, we do not recommend double-J stents placement in all children with congenital hydronephrosis; it may be useful in selected cases.

[Recommendations of the S3 Guideline "Use of Extracorporeal Circulation (ECLS/ ECMO) for Cardiac and Circulatory Failure" of the Association of Scientific Medical Societies in Germany]. / Empfehlungen der S3-Leitlinie (AWMF) "Einsatz der extrakorporalen Zirkulation (ECLS/ECMO) bei Herz- und Kreislaufversagen".

In recent years, the use of mechanical support for patients with cardiac or circulatory failure has continuously increased, leading to 3,000 ECLS/ECMO (extracorporeal life support/extracorporeal membrane oxygenation) implantations annually in Germany. Due to the lack of guidelines, there is an urgent need for evidence-based recommendations addressing the central aspects of ECLS/ECMO therapy. In July 2015, the generation of a guideline level S3 according to the standards of the Association of the Scientific Medical Societies in Germany (AWMF) was announced by the German Society for Thoracic and Cardiovascular Surgery (GSTCVS). In a well-structured consensus process, involving experts from Germany, Austria and Switzerland, delegated by 16 scientific societies and the patients' representation, the guideline "Use of extracorporeal circulation (ECLS/ECMO) for cardiac and circulatory failure" was created under guidance of the GSTCVS, and published in February 2021. The guideline focuses on clinical aspects of initiation, continuation, weaning and aftercare, herein also addressing structural and economic issues. This article presents an overview on the methodology as well as the final recommendations.

Long-Term Evaluation of Gastroesophageal Reflux in Neonates with and without Preventive Anti-reflux Surgery at the Time of Congenital Diaphragmatic Hernia Repair.

One potential comorbidity after congenital diaphragmatic hernia (CDH) is gastroesophageal reflux (GER), which can have a substantial effect on patients' quality of life, thriving, and complications later in life. Efforts have been made to reduce gastroesophageal reflux with a preventive anti-reflux procedure at the time of CDH repair. In this follow-up study of neonates participating in a primary RCT study on preventive anti-reflux surgery, symptoms of GER were assessed longitudinally. Long-term data with a median follow-up time of ten years was available in 66 patients. Thirty-one neonates received an initial fundoplication. Secondary anti-reflux surgery was necessary in 18% and only in patients with large defects. It was required significantly more often in patients with intrathoracic herniation of liver (p = 0.015) and stomach (p = 0.019) and patch repair (p = 0.03). Liver herniation was the only independent risk factor identified in multivariate regression analysis. Primary fundopexy and hemifundoplication did not reveal a protective effect regarding the occurrence of GER symptoms, the need for secondary antireflux surgery or the gain of body weight regardless of defect size neither in the short nor in the long term. Symptoms of GER must be assessed carefully especially in children with large defects, as these are prone to require secondary anti-reflux surgery in the long-term. Routine evaluation of GER including endoscopy and impedance measurement should be recommended especially for high-risk patients.

Small Bowel Obstruction After Neonatal Repair of Congenital Diaphragmatic Hernia-Incidence and Risk-Factors Identified in a Large Longitudinal Cohort-Study.

Objective: In patients with a congenital diaphragmatic hernia (CDH), postoperative small bowel obstruction (SBO) is a life-threatening event. Literature reports an incidence of SBO of 20% and an association with patch repair and ECMO treatment. Adhesions develop due to peritoneal damage and underly various biochemical and cellular processes. This longitudinal cohort study is aimed at identifying the incidence of SBO and the risk factors of surgical, pre-, and postoperative treatment. Methods: We evaluated all consecutive CDH survivors born between January 2009 and December 2017 participating in our prospective long-term follow-up program with a standardized protocol. Results: A total of 337 patients were included, with a median follow-up of 4 years. SBO with various underlying causes was observed in 38 patients (11.3%) and significantly more often after open surgery (OS). The majority of SBOs required surgical intervention (92%). Adhesive SBO (ASBO) was detected as the leading cause in 17 of 28 patients, in whom surgical reports were available. Duration of chest tube insertion [odds ratio (OR) 1.22; 95% CI 1.01-1.46, p = 0.04] was identified as an independent predictor for ASBO in multivariate analysis. Beyond the cut-off value of 16 days, the incidence of serous effusion and chylothorax was higher in patients with ASBO (ASBO/non-SBO: 2/10 vs. 3/139 serous effusion, p = 0.04; 2/10 vs. 13/139 chylothorax, p = 0.27). Type of diaphragmatic reconstruction, abdominal wall closure, or ECMO treatment showed no significant association with ASBO. A protective effect of one or more re-operations has been detected (RR 0.16; 95% CI 0.02-1.17; p = 0.049). Conclusion: Thoracoscopic CDH repair significantly lowers the risk of SBO; however, not every patient is suitable for this approach. GoreTex®-patches do not seem to affect the development of ASBO, while median laparotomy might be more favorable than a subcostal incision. Neonates produce more proinflammatory cytokines and have a reduced anti-inflammatory capacity, which may contribute to the higher incidence of ASBO in patients with a longer duration of chest tube insertion, serous effusion, chylothorax, and to the protective effect of re-operations. In the future, novel therapeutic strategies based on a better understanding of the biochemical and cellular processes involved in the pathophysiology of adhesion formation might contribute to a reduction of peritoneal adhesions and their associated morbidity and mortality.

Application of bacterial nanocellulose-based wound dressings in the management of thermal injuries: Experience in 92 children.

BACKGROUND: Management of pediatric thermal injuries involves a high standard of care in a multidisciplinary setting. To avoid physical and psychological sequelae, wound dressings should minimize hospitalization time and anesthesia while maximizing patient comfort. PATIENTS AND METHODS: 190 children with thermal injuries of the torso, arms and legs were treated with polyurethane foam dressings or bacterial nanocellulose sheets. Data were analyzed retrospectively regarding hospitalization, procedures with general anesthesia, scar formation, rate of infection and need for skin grafting. RESULTS: The groups did not differ significantly concerning age, gender distribution or percentage of injured total body surface area. Statistical analysis showed that length of hospitalized care and procedures undergoing anesthesia were significantly reduced in the nanocellulose group (each p < 0.0001). There was no significant difference in rate of complications, wound healing and rate of skin grafting between the two subgroups. DISCUSSION: Acting as a temporary epidermal substitute, bacterial nanocellulose enables undisturbed reepithelialization without further wound dressing changes. In children, no additional topical antimicrobial agents are indicated for unimpaired wound healing. CONCLUSIONS: Bacterial nanocellulose is superior to polyurethane foam regarding length of hospitalization and number of interventions under anesthesia. It offers a safe, cost-effective treatment option and provides excellent comfort in pediatric patients.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Diagnostics, Preoperative, Operative, and Postoperative Management.

INTRODUCTION: Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature. MATERIALS AND METHODS: Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9. RESULTS: Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1-9). Eight of these (62%) did not reach consensus. CONCLUSION: Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework.

INTRODUCTION: Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. MATERIALS AND METHODS: The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. RESULTS: Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. CONCLUSION: Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

Is there a standard treatment for displaced pediatric diametaphyseal forearm fractures?: A STROBE-compliant retrospective study.

To review our institutional results and assess different surgical and non-surgical techniques for the treatment of displaced diametaphyseal forearm fractures in children and adolescents.Thirty-four children (25M, 9F) with a total of 36 diametaphyseal forearm fractures who underwent treatment under general anesthesia between July 2010 and February 2016 were recruited to this retrospective study. From October 2016 until March 2018 patients and/or parents were contacted by telephone and interviewed using a modified Pediatric Outcomes Data Collection Instrument (PODCI).Median age at the time of injury was 9.1 years (range, 1.9-14.6 years). Initial treatment included manipulation under anesthesia (MUA) and application of plaster of Paris (POP) (n = 9), elastic stable intramedullary nailing (ESIN) (n = 10), percutaneous insertion of at least one Kirschner wire (K-wire) (n = 16), and application of external fixation (n = 1). Eleven children (32%) experienced a total of 22 complications. Seven complications were considered as major, including delayed union (n = 1) and extensor pollicis longus (EPL) tendon injury (n = 1) following ESIN, as well as loss of reduction (n = 2) and refractures (n = 3) after MUA/POP. The median follow-up time was 28.8 months (range, 5.3-85.8 months). In 32 out of 34 cases (94%) patients and/or parents were contacted by telephone and a PODCI score was obtained. Patients who experienced complications in the course of treatment had a significantly lower score compared with those whose fracture healed without any sequelae (P = .001). There was a trend towards an unfavorable outcome following ESIN compared with K-wire fixation (P = .063), but not compared with POP (P = .553). No statistical significance was observed between children who were treated initially with a POP and those who had K-wire fixation (P = .216).There is no standard treatment for displaced pediatric diametaphyseal forearm fractures. Management with MUA/POP only is associated with an increased refracture rate. Based on our experience K-wire fixation including intramedullar positioning of at least one pin seems to be favorable compared with ESIN.

Experience with Stent Placement for Benign Pancreaticobiliary Disorders in Children.

Purpose: There is a lack of experience with stenting for benign pancreaticobiliary disorders in children. Materials and Methods: Fifteen children (9 male and 6 female) with a median age of 7.1 years (range 0.7-14.2 years) who underwent treatment with a plastic stent for a benign disorder of the pancreaticobiliary system between May 2003 and September 2017 were recruited to this retrospective study. Results: Biliary and/or pancreatic plastic stents were inserted into 5 patients with congenital, 4 with post-traumatic, and 6 with idiopathic pathologies. Median duration of individual stent placement was 111 days (range 14-1569 days). Eleven children (73%) were treated with one stent only. In 4 cases, up to 22 stents were successively placed over time. There were no complications during stent insertion or stent removal. Seven patients (47%) experienced adverse effects during stenting, including choledocholithiasis, pancreaticolithiasis, cholangitis, acute pancreatitis, stent obstruction, and stent fracture. At follow-up, in 11 cases (73%), the underlying condition was resolved. In 4 children, all of whom suffered from congenital pancreaticobiliary disorders, stent therapy was considered as a temporary treatment before definite surgery. Conclusions: Patients with congenital anomalies of the pancreaticobiliary tree often require surgery for definitive management. However, temporary stent placement can be accomplished safely and successfully and this serves as a bridge to temporize their obstructive process while awaiting surgical intervention. Children with post-traumatic or idiopathic disorders can frequently be managed definitively by stenting alone and many of these require only one single stent insertion.

Use of covered self-expandable stents for benign colorectal disorders in children.

PURPOSE: There is a lack of experience with covered self-expandable stents for benign colorectal disorders in children. METHODS: Five children (4M, 1F) with a median age of 5years (range, 6months-9years) who underwent treatment with covered self-expandable plastic (SEPSs) or self-expandable metal stents (SEMSs) for a benign colorectal condition between April 2005 and November 2013 were recruited to this retrospective study. Etiologies included: anastomotic stricture with (n=1) or without (n=3) simultaneous enterocutaneous fistula, as well as an anastomotic leak associated with enterocutaneous fistula (n=1). All children suffered from either Hirschsprung's disease (n=3) or total colonic aganglionosis (Zuelzer-Wilson syndrome) (n=2). RESULTS: Median duration of individual stent placement was 23days (range, 1-87days). In all cases up to five different stents were placed over time. At follow-up two patients were successfully treated without further intervention. In another patient the anastomotic stricture resolved fully, but a coexisting enterocutaneous fistula persisted. Overall, three patients did not improve completely following stenting and required definite surgery. Stent-related problems were noted in all cases. There was one perforation of the colon at stent insertion. Further complications consisted of stent dislocation (n=4), obstruction (n=1), formation of granulation tissue (n=1), ulceration (n=1) and discomfort (n=3). CONCLUSIONS: Covered self-expandable stents enrich the armamentarium of interventions for benign colorectal disorders in children including anastomotic strictures and intestinal leaks. A stent can be applied either as an emergency procedure (bridge to surgery) or as an adjuvant treatment further to endoscopy and dilatation. Postinterventional problems are frequent but there is a potential for temporary or definite improvement following stent insertion.

The effect of intermittent intraabdominal pressure elevations and low cardiac output on the femoral to carotid arterial blood pressure difference in piglets.

BACKGROUND: Our previous work in a laparoscopic setting in piglets revealed that the systolic femoral artery pressure was approximately 5 % higher than its carotid counterpart, whereas the mean and diastolic values showed no significant difference. This remained idem when the intraabdominal pressure (IAP) was gradually increased. In this study, we aimed to investigate the effect of (1) intermittent IAP elevations and (2) a low cardiac output (CO) on the blood pressure (BP) difference cranially (carotid artery) and caudally (femoral artery) of a capnoperitoneum (ΔP = P a fem-P a carot). METHODS: A total of twenty-two piglets (mean body weight 11.0 kg; range 8.9-13.3 kg) were studied. Of these, 14 underwent intermittent IAP elevations at 8 and 16 mmHg, and ΔP was measured. In another 8 piglets, a model of reduced CO was created by introducing an air embolism (2 ml/kg over 30 s) in the inferior caval vein (VCI) at 12 mmHg IAP to further assess the influence of this variable on ΔP. RESULTS: Systolic ΔP remained at a mean of 5.6 mmHg and was not significantly affected by insufflation or exsufflation up to an IAP of 16 mmHg. Diastolic and mean values showed no differences between P a carot and P a fem. P a fem, systol remained higher than its carotid counterpart as long as the cardiac index (CI) was above 1.5 l/min/m2, but fell significantly below P a carot, systol at a low CI. There was no CO-dependent effect on diastolic and mean ΔP. Repeated IAP elevations do not significantly influence ΔP. CONCLUSIONS: Intermittent IAP elevations do not significantly influence ΔP. Despite of a CO-dependent inversion of systolic ΔP, mean BP measurements at the leg during laparoscopy remain representative even at low CO values.

The Surgical Treatment of Toxic Megacolon in Hirschsprung Disease.

OBJECTIVES: Enterocolitis remains the most significant cause of morbidity and mortality in Hirschsprung disease (HD). It could progress into toxic megacolon (TM)-acute dilatation of the colon as accompanying toxic complication of Hirschsprung enterocolitis. It is a devastating complication, especially in infants with so far undiagnosed HD. METHODS: A retrospective analysis of medical records of 4 infants with TM was performed. The diagnosis TM was determined on the basis of clinical information (abdominal pain or tenderness, abdominal distension, diarrhea, bloody diarrhea, and constipation), plain x-rays of the abdomen (segmental or total colonic dilation), and the presence of such criteria (fever, high heart rate, increased white blood cell count, C reactive protein, anemia, dehydration, electrolyte disturbances, hypotension). Surgical management and outcome was evaluated by retrospective chart review. RESULTS: The median duration of symptoms characteristic for TM was 3 days. Toxic megacolon was seen as the first manifestation of previously unknown HD in 3 patients; in 1 newborn, the contrast radiograph was suggestive of HD. In all patients, conservative treatment was failed. Three patients were treated with surgical decompression and ileostomy only. In all these cases, severe complications occurred, consequently 2 of them died. In 1 patient, a resection of the transverse dilated colon additionally was performed. This patient had no complications in postoperative period and survived. CONCLUSIONS: Because of the high mortality in patients with TM that were treated medically or with colonic decompression, a resection of massively distended part of the colon should be performed.

A comparison of intervention and conservative treatment for angulated fractures of the distal forearm in children (AFIC): study protocol for a randomized controlled trial.

BACKGROUND: Angulated fractures of the distal forearm are very frequent lesions in childhood. Currently, there are no standard guidelines on whether these children should be treated conservatively with a cast; with reduction and a cast; or with reduction, pinning and a cast under anesthesia. Minor prospective and retrospective studies have shown that the distal physis of the forearm possesses high remodeling capacity leading to reliable correction of malalignment. The aim of this trial is to answer the question about whether operative and conservative treatment show equivocal results. METHODS/DESIGN: This is a prospective, multinational, multicenter, randomized, observer-blinded, actively controlled, parallel group trial, with 24 months of observation. The primary objective of this trial is to assess whether or not the long-term functional outcome in remodeling patients is inferior to patients receiving closed reduction and K-wire pinning. The trial should include 742 patients with acute fracture. The patients will be included in 30 medical centers in Germany, Switzerland and Austria. All patients 5 to 11 years of age presenting at the emergency department with an angulated distal fracture of the forearm will be randomized online after informed consent. The primary endpoint is the Cooney Score after 24 months. The secondary endpoint is the grade of radiological displacement at 12/24 months. DISCUSSION: Therapy of angulated fractures is a matter of intensive debate. Primary manipulation and pinning under general anesthesia is recommended in order to avoid malalignment. No major study has proven the advantage of manipulation and pinning over immobilization alone. Should remodeling appear to be a safe alternative, manipulation under general anesthesia, K-wire pinning and removal of pins could be avoided, thus sparing significant costs. TRIAL REGISTRATION: DRKS00004874 , 30 October 2013.

The Surgical Correction of Congenital Deformities: The Treatment of Diaphragmatic Hernia, Esophageal Atresia and Small Bowel Atresia.

BACKGROUND: More than half of all congenital deformities can be detected in utero. The initial surgical correction is of paramount importance for the achievement of good long-term results with low surgical morbidity and mortality. METHODS: Selective literature review and expert opinion. RESULTS: Congenital deformities are rare, and no controlled trials have been performed to determine their optimal treatment. In this article, we present the prenatal assessment, treatment, and long-term results of selected types of congenital deformity. Congenital diaphragmatic hernia (CDH) affects one in 3500 live-born infants, while esophageal atresia affects one in 3000 and small-bowel atresia one in 5000 to 10,000. If a congenital deformity is detected and its prognosis can be reliably inferred from a prenatal assessment, the child should be delivered at a specialized center (level 1 perinatal center). The associated survival rates are 60-80% after treatment for CDH and well over 90% after treatment for esophageal or small-bowel atresia. Despite improvements in surgical correction over the years, complications and comorbidities still affect 20-40% of the treated children. These are not limited to surgical complications in the narrow sense, such as recurrence, postoperative adhesions and obstruction, stenoses, strictures, and recurrent fistulae, but also include pulmonary problems (chronic lung disease, obstructive and restrictive pulmonary dysfunction), gastrointestinal problems (dysphagia, gastro-esophageal reflux, impaired intestinal motility), and failure to thrive. Moreover, the affected children can develop emotional and behavioral disturbances. Minimally invasive surgery in experienced hands yields results as good as those of conventional surgery, as long as proper selection criteria are observed. CONCLUSION: Congenital deformities should be treated in recognized centers with highly experienced interdisciplinary teams. As no randomized trials of surgery for congenital deformities are available, longitudinal studies and registries will be very important in the future.

Single-staged surgical approach in congenital diaphragmatic hernia associated with esophageal atresia.

BACKGROUND: The coexistence of congenital diaphragmatic hernia (CDH) with esophageal atresia (EA) has only been reported occasionally in literature. Series of patients from a single institution with comparison of different postnatal therapeutic approaches have not been reported. We describe our management in this unique cohort of patients and discuss the procedures that can lead to successful outcomes in this association of congenital anomalies. METHODS: The surgical approaches and outcome of six neonates with CDH associated with EA and distal tracheo-esophageal fistula (TEF) are discussed. RESULTS: Five newborns were treated surgically, while one patient with trisomy 18 only received palliative treatment. In four patients TEF was ligated during laparotomy for CDH repair. Secondary surgery was performed for correction of EA via thoracotomy after 4-6 weeks (primary anastomosis in two patients, Foker's-technique in one patient, one patient deceased prior to secondary surgery). All three surviving patients required fundoplication due to severe gastro-esophageal reflux during the first year of life. Two patients also required dilatation for anastomotic stricture. In one preterm infant correction of both malformations was accomplished during one surgical intervention. The herniated organs were eventrated and temporarily placed into a silastic bag to allow a mediastinal shift to the left. Thus a continuous ventilation of the right lung with minimal compression and sufficient oxygenation was possible during esophageal repair via a right-sided thoracotomy and extrapleural approach. No further surgery was required so far. CONCLUSIONS: Definitive surgical correction in newborns with CDH and EA was so far accomplished with multiple surgical interventions. Ligation of TEF via an abdominal approach with repair of CDH followed by delayed repair of EA is prone to stenosis and gastro-esophageal reflux due to loss of esophageal length. With a new combination of established surgical methods a single-staged correction of both malformations is possible. This new approach might help to preserve sufficient length of esophagus to accomplish primary anastomosis without tension and therefore avoid long-term morbidity and repetitive surgeries.