Bowel Lengthening Procedures in Children with Short Bowel Syndrome: A Systematic Review.

INTRODUCTION: The aims of the study are to systematically assess and critically appraise the evidence concerning two surgical techniques to lengthen the bowel in children with short bowel syndrome (SBS), namely, the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP), and to identify patient characteristics associated with a favorable outcome. MATERIALS AND METHODS: MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception till December 2019. No language restriction was used. RESULTS: In all, 2,390 articles were found, of which 40 were included, discussing 782 patients. The median age of the patients at the primary bowel lengthening procedure was 16 months (range: 1-84 months). Meta-analysis could not be performed due to the incomparability of the groups, due to heterogeneous definitions and outcome reporting. After STEP, 46% of patients weaned off parenteral nutrition (PN) versus 52% after LILT. Mortality was 7% for STEP and 26% for LILT. Patient characteristics predictive for success (weaning or survival) were discussed in nine studies showing differing results. Quality of reporting was considered poor to fair. CONCLUSION: LILT and STEP are both valuable treatment strategies used in the management of pediatric SBS. However, currently it is not possible to advise surgeons on accurate patient selection and to predict the result of either intervention. Homogenous, prospective, outcome reporting is necessary, for which an international network is needed.

Experience with fully covered self-expandable metal stents for anastomotic stricture following esophageal atresia repair.

There is a lack of experience with fully covered self-expandable metal stents (SEMSs) for the treatment of benign esophageal conditions in the pediatric population. This is the evaluation of our institutional experience of placing SEMSs for anastomotic stricture (AS) formation following esophageal atresia (EA) repair. Patients were jointly managed from the Department of Pediatric Surgery and Central Interdisciplinary Endoscopy at our institution. Thirteen children (8 male, 5 female) with a median age of 4 months (range: 1-32 months) who underwent treatment with SEMSs for a postoperative AS following EA repair between February 2006 and April 2016 were recruited into this retrospective study. SEMSs that are originally designed for other organs such as trachea, bronchus, biliary tract, or colon were inserted under general anesthesia via endoscopic guidance. Simultaneous fluoroscopy was not required in any case. In five infants, the stents were inserted primarily without previous therapy. Seven patients underwent stenting following dilatation with or without adjuncts (e.g. Mitomycin C, Triamcinolone). In one case with an AS and a simultaneous persistent tracheoesophageal fistula (TEF), multiple SEMSs were applied after failure to close the fistula with fibrin glue.The median duration of individual stent placement was 30 days (range: 5-91 days). In five children up to four different biliary, bronchial or colonic SEMSs were placed successively over time. There were no problems noted at stent insertion or removal. Eight children (62%) developed complications associated with stenting. At follow-up, in eight patients (62%) AS was resolved, including all of those five cases, who had their stents inserted without previous therapy. Five children (38%), who underwent dilatation prior to stenting did not improve their AS and required further intervention. Overall, the cohort exhibited a slight, but not significant weight gain between stent insertion and (final) stent removal.Insertion of SEMSs for AS following EA repair is safe and often successful with only one single application. It can be used as a primary procedure (without previous therapy) or after failed dilatations.There was one death in this study that was unrelated to stenting and occurred 12 months after stent removal. Because of the absence of manufactured, age-related devices, SEMSs that are originally designed for other organs can be applied. Establishment of a standardized management including stent placement for the treatment of AS following EA repair in the pediatric population is required.

Treatment outcome in 60 children with pathological fractures of the humerus caused by juvenile or aneurysmal bone cysts.

The treatment of pathological fractures of the humerus caused by juvenile or aneurysmal bone cysts (JBC/ABC) should be a single approach with a high success rate and low complication rate. This study evaluates how day by day treatment concepts fulfil these aims. Children below 15 years of age with a pathological fracture of the humerus caused by a JBC or ABC between 01.01.2001 and 31.12.2010, were investigated by chart review in four major paediatric trauma centres. Age, gender, fracture localisation, X-ray findings, treatment and outcome - assessed by the Capanna classification (I to IV), were analysed. 60 children [41male, 19 female; mean age: 9 years (4-14 years)] with 43 JBC and 12 ABC were included as well as five cysts, who could not be classified definitively. First treatment was non-operatively in 33 children. Of these 27 cysts did not improve; likewise the supportive installation of cortisone in six patients did not change the outcome. The first treatment consisted of elastic stable intramedullary in 13 children; up to three nail exchanges included. But only six of these reached (nearly) complete resolution (I/II). Overall the combined mechanical and biological treatment with curettage, elastic stable intramedullary nailing, (artificial) bone substitute and in some cases growth factors was performed as the 1st-line treatment in nine patients and further in 2nd or 3rd-line treatments in 13 humeral cysts. More than half of these reached a complete or nearly complete resolution of the cyst (12x I, 5x II, 1x III, 4x IV). Major complications in all operated patients were six nails not removable and two children with upper extremities length differences. Healing rates are low for non-operative treatment, elastic stable intramedullary nailing alone and by using cortisone for cysts resolution in pathological fractures of the humerus. Data support a combined mechanical and biological treatment with curettage, elastic stable intramedullary nailing, (artificial) bone substitute and the use of growth factors.

[LiLa classification for paediatric long bone fractures. Intraobserver and interobserver reliability]. / LiLa-Klassifikation für Frakturen der langen Röhrenknochen im Wachstumsalter. Intra- und Interobserverreliabilität.

BACKGROUND: There are two child-specific fracture classification systems for long bone fractures: the AO classification of pediatric long-bone fractures (PCCF) and the LiLa classification of pediatric fractures of long bones (LiLa classification). Both are still not widely established in comparison to the adult AO classification for long bone fractures. METHODS: During a period of 12 months all long bone fractures in children were documented and classified according to the LiLa classification by experts and non-experts. Intraobserver and interobserver reliability were calculated according to Cohen (kappa). RESULTS: A total of 408 fractures were classified. The intraobserver reliability for location in the skeletal and bone segment showed an almost perfect agreement (K = 0.91-0.95) and also the morphology (joint/shaft fracture) (K = 0.87-0.93). Due to different judgment of the fracture displacement in the second classification round, the intraobserver reliability of the whole classification revealed moderate agreement (K = 0.53-0.58). Interobserver reliability showed moderate agreement (K = 0.55) often due to the low quality of the X-rays. Further differences occurred due to difficulties in assigning the precise transition from metaphysis to diaphysis. CONCLUSIONS: The LiLa classification is suitable and in most cases user-friendly for classifying long bone fractures in children. Reliability is higher than in established fracture specific classifications and comparable to the AO classification of pediatric long bone fractures. Some mistakes were due to a low quality of the X-rays and some due to difficulties to classify the fractures themselves. Improvements include a more precise definition of the metaphysis and the kind of displacement. Overall the LiLa classification should still be considered as an alternative for classifying pediatric long bone fractures.

[Therapy of paediatric pancreatic trauma]. / Therapie traumatischer Pankreasverletzungen im Kindesalter.

BACKGROUND: The goal of this clinical study is to evaluate diagnostics and therapeutical strategies for paediatric pancreatic trauma. It is assumed that conservative treatment is at least as good as operative treatment. PATIENTS / MATERIAL AND METHODS: In a retrospective study 36  children with pancreatic trauma treated between the years 1987 and 2010 in the paediatric surgery department of the University Medical Centre Mannheim were included. Injury grades, diagnostics and treatment were evaluated. RESULTS: In a collective of 36  patients with an average age of seven years the following injuries were found: 26  grade 1, three grade  2, five grade  3 and two grade  4 injuries. Eight patients presented with polytrauma, 20 showed additional injuries. Initial levels of amylase and lipase and screening ultrasound were not always sensitive. 18  patients received conservative treatment. The remaining 18  were operated: either exploratory laparotomy and drainage or pancreas reconstruction with inner stenting or distal pancreatic resection were performed. Pancreatic pseudocysts were drained by transgastric stenting. CONCLUSION: 20 years ago blunt pancreatic trauma was usually treated operatively. Today grade 1 and grade 2 injuries should be treated conservatively. Higher graded pancreatic ductal injuries can be managed operatively with ductal stenting and organ conservation. Pancreatic resections are not always necessary. Endoscopic transgastric drainage of persisting pancreatic pseudocysts is also successful in children.

Biomechanical analysis of a synthetic femur spiral fracture model: Influence of different materials on the stiffness in flexible intramedullary nailing.

BACKGROUND: Flexible intramedullary nail fixation of dislocated diaphyseal femur fractures has gained wide acceptance for children and adolescents with open physes. Studies with a special emphasis on complications reveal frequent problems regarding stability, usually in complex fracture types such as spiral fractures and in older children weighing >40kg. This biomechanical study analyses how much the material of the nails influences the stiffness in a synthetic bone model. METHODS: Twenty-four composite grafts (Sawbones®, 4th generation, medullar canal of 10mm) with an identical spiral fracture were used in three configurations of eight grafts. Elastic stable intramedullary nailing was performed in a retrograde C-shaped manner with two nails of equal size (2×3.5mm). Close contact of the fragments could be achieved. We compared Group A (steel nails) with Group B and C (two types of titanium nails). All specimens underwent 4-point bending, torsion and axial compression in the 0° and 9° positions, and the results were analysed. FINDINGS: Group A (steel nails) revealed a significantly higher stiffness in all directions than Group B. Apart from compression in the 9° position this steel nail fixation showed significant higher stiffness than titanium nails of Group C. Comparing Group B and C did not show an systematic difference. INTERPRETATION: In this biomechanical study with composite artificial bones the use of steel Nails demonstrated the highest stiffness in our model when compared to two different titanium nail configurations. Apart from in cases of known allergy or planned MRI-examinations our results and data from the literature question the use of titanium nails.

[Dislocated midclavicular fractures in children and adolescents: who benefits from operative treatment?]. / Dislozierte Klavikulafrakturen im Kindes- und Jugendalter: Wer profitiert von einer Operation?

BACKGROUND: Displaced midclavicular fractures in children are generally treated non-operatively. But there is a lack of age-related data concerning the functional and cosmetic results following conservative treatment and patients' content with the treatment. PATIENTS AND METHODS: In patients with displaced midclavicular fractures complications and pain was evaluated in relation to treatment option and age. Outcome was measured with the Constant Shoulder Score, a client satisfaction questionnaire (ZUV-8) about the satisfaction with the treatment and the cosmetic result and ultrasound of both clavicles. RESULTS: A review of emergency room and fracture clinic records revealed 101 children and adolescents in whom displaced midclavicular fracture had been treated between 1/1997 and 12/2007. The follow-up included 59 patients with 60 fractures 1-10 years after the accident. Of those, 50 fractures were treated with a sling and 10 by operation. All patients under the age of ten were treated conservatively and reached very good functional and cosmetic results. Only an eight-year-old girl suffered from a painful pseudarthrosis. After resection and stabilisation by flexible intramedullary nailing, the outcome was excellent. Patients over the age of ten received a conservative treatment in 28 cases and ten had an operation (K-wires fixation n=2, elastic stable intramedullary nail n=8 in 7 patients). Functional outcome independent of treatment method was as good as in younger children, but the global and cosmetic satisfaction score was much lower. Older patients with a non-operative treatment suffered from more pain and were dissatisfied with the long immobilisation. CONCLUSION: The method of choice in children under the age of ten with a displaced clavicular fracture is the non-surgical treatment supported by sufficient pain medication. Older children reach good results but suffer from more pain and are dissatisfied by the cosmetic results and immobilisation. Because of this, active older children and adolescents with a displaced clavicular fracture benefit from elastic stable intramedullary nailing.

The chronic liver disease in patients with short bowel syndrome: etiology and treatment.

Short bowel syndrome is a life threatening disease with a high mortality and morbidity. Since home parenteral nutrition (PN) has been established, there is an increasing number of patients surviving the acute loss of bowel function. But on the long-time these patients suffer from different complications of PN, with loss of central venous access, recurrent sepsis and finally the syndrome of progressive cholestatic liver disease. Both loss of central venous access and especially the progressive cholestatic liver disease are the limiting factor for the long-term survival of patients suffering from intestinal failure. Interestingly, the pathophysiologic mechanisms of PN induced intrahepatic cholestasis have not been dissolved yet and seem to be of multifactorial genesis. Cholestasis has shown to be associated with prematurity, recurrent sepsis, enteral and PN, especially with lipid emulsions. Enteral feeding and a well-controlled regime of PN lower the incidence of end-stage liver disease and, therefore, has to be optimized in the therapy of these patients.

[The laparoscopic spleen-preserving approach to splenic cysts: advantages and limitations]. / Laparoskopische organerhaltende operationen bei nicht-parasitären milzzysten: möglichkeiten und grenzen.

INTRODUCTION: Due to the multiple functions of the spleen the preferred management of cystic non-parasitic lesions is nowadays laparoscopic partial splenectomy or decapsulation with preservation of the spleen. We have analysed our patients with non-parasitic cystic lesions and the current literature to weigh the benefits and complications of these methods in children and adults. PATIENTS AND METHODS: Laparoscopic partial splenectomy was performed in three children with dysontogenetic cysts. Laparoscopic marsupialisation was performed in one child and in three adults. RESULTS: Follow-up in the true cysts showed no recurrence in one patient and one residual cyst in the second. In the third patient, splenectomy was performed because there was not enough residual spleen to warrant preservation. In the group with post-traumatic cysts, no complications or recurrences were observed. DISCUSSION: The laparoscopic spleen-preserving approach for the treatment of non-parasitic cysts is feasible, but challenging. The main problem is a recurrence rate of more than 20 %. The reason for recurrence remains uncertain: it is probably due to different operative strategies, the morphology of the cysts or the presence of residual cysts. The recurrence rate in post-traumatic cysts is low on the basis of our own experience and a literature survey. CONCLUSION: The benefit of laparoscopic treatment in true non-parasitic splenic cysts has to be weighed against the rate of recurrence. A complete resection of the cysts should be attempted. In post-traumatic cysts, laparoscopy offers a good minimally invasive treatment option.

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.

[Predicting growth patterns after supracondylar fracture of the humerus in childhood]. / Wie lässt sich die Wachstumsprognose nach kindlicher suprakondylärer Humerusfraktur erfassen?

Supracondylar fracture of the humerus is the most frequently investigated fracture in children. However, systematic studies about postoperative growth patterns cannot be found in the literature. In this retrospective study, the authors attempt to delineate the configuration of supracondylar fractures which allow spontaneous correction of a malalignment or cause post-traumatic growth disturbances. The study included 256 children with supracondylar fracture of the humerus: 184 of them (71.9%) were available for longterm follow-up. The plain films of these children were reviewed and the Baumann and shaft-capitulum angles recorded. Spontaneous correction of a primarily displaced fracture was found in 13.0% ( n=21) of all fractures in the sagittal plane. Spontaneous correction in the frontal plane could not be shown. Growth disturbance was discovered in 10.5% ( n=16) in the frontal plane. No growth disturbance was demonstrated in the sagittal plane. The relatively small number of growth disturbances can be attributed to the low growth potential of the distal physeal plate of the humerus. Growth disturbances with secondary rotational errors were not observed in this study. Spontaneous corrections of alignment in the sagittal plane are only possible under a certain age. Spontaneous correction in the frontal plane, however, is inadequately assessed with this study. The design of a prospective study was formulated to assess how to improve the therapeutic management of supracondylar fractures and to answer additional open questions. Follow-up radiographs and standardized clinical evaluations have to be performed at the time of first free range of movement and 2 years after the trauma. This study will investigate whether therapeutic progress at follow-up is better evaluated with radiological or clinical means.

[Corrective interventions after elbow para-articular fractures in childhood]. / Korrektureingriffe nach ellenbogennahen Frakturen im Kindesalter.

Besides supracondylar fracture of the humerus there are several injuries of the elbow joint, which may lead to major disability. In this study 5 cases of corrective procedures are described after elbow fractures. Initially the lesions were overlooked. These were a fracture of the radial condyle, producing a pseudarthrosis, three cases of Monteggia fractures with persisting dislocation of the radial head in young children and a periarticular calcification issuing from an avulsion of the radial epicondyle and the radial capsule in a 13-year-old. All children had marked functional limitation of the elbow joint. The primarily overlooked fractures were corrected early. Various osteosynthesis procedures including movement and distraction extend fixator were employed. Overall, in all patients an almost complete movement of the joint at existing stability could be achieved. Transcondylar and Monteggia fractures should not be overlooked at the initial diagnosis as secondary operations for correction always have a less favorable outcome than the primary one. For the management of ankylosis of the elbow a movement extend fixator after distraction is a useful additional management.

Congenital epulis: a rare diagnosis in paediatric surgery.

Congenital epulis of the newborn is a rare tumour which is usually benign. The first description of a case is attributed to Neumann in 1871. The word "epulis" is derived from Greek and means "on the gum" or "gum boil". Epulis is also known as a congenital gingival granular cell tumour because of its histological features. Since 1871, 216 cases have been reported. Female babies are affected 8-10 times more often than males. Epulis is located on the maxillary ridge twice as often as on the mandible, mostly as single tumours but rarely as multiple tumours. Macroscopically, epulis is a pedunculated tumour with a smooth or lobulated surface. The basis of the tumour is the alveolar mucosa. The size varies from a few millimetres to 9 cm in diameter. After birth, the tumour normally does not increase in size. Microscopic examination shows a central mass of granular cells. This mass is surrounded by a stratified squamous mucosa. The histogenesis of the tumour is unknown. Spontaneous regression of congenital epulis has been reported in four cases. However, surgical excision is generally indicated due to interference with feeding or respiration. Recurrence of the tumour after surgery has not been reported yet.

Hemarthrosis after trauma to the pediatric knee joint: what is the value of magnetic resonance imaging in the diagnostic algorithm?

In children, compulsory arthroscopy for hemarthrosis after knee trauma is not justified because ligamentous and meniscal damage is rare. In a prospective study, we analyzed the diagnostic value of radiography, magnetic resonance imaging (MRI), and arthroscopy in 51 patients up to 14 years of age with acute knee trauma. Plain radiography revealed 16 osseous lesions (5 metaphyseal, 3 patellar, 4 physeal fractures, 3 avulsions of the tibial spine, and 1 osseous ligamentous tear). In 29 patients, the cause of hemarthrosis remained unclear. All patients were evaluated by MRI. A diagnosis could be assigned to all 29 patients. MRI demonstrated lesions in 38 patients. In addition, the following lesions were discovered: 8 patellar dislocations, 13 bruises, 1 rupture of the anterior cruciate ligament, 1 osteochondritis dissecans, and 13 joint effusions. In 13 patients, MRI was followed by arthroscopy to confirm the diagnosis. Both, MRI and arthroscopy missed two osteochondral fractures. In addition, three chondral lesions were not picked up by MRI. MRI is a reliable tool for assessing the extent of knee lesions in children.

Characteristic pattern and management of intra-articular knee lesions in different pediatric age groups.

Posttraumatic hemarthrosis of the knee joint usually leads to arthroscopy. In a retrospective study we defined an age-specific spectrum of injuries and, subsequently, assigned an age-dependent diagnostic management for children up to 16 years. From January 1986 to December 1996, 1,273 patients up to 16 years of age with knee trauma presented to the emergency department. Overall, soft-tissue lesions prevailed (82%). Hemarthrosis occurred in only 18.0%. The patients were classified in three groups: group 1 (528 children younger than 10 years of age) with hemarthrosis in only 5.7% (mainly metaphyseal fractures and patellar dislocations), group 2 (207 preadolescents from 11 to 12 years of age) with hemarthrosis in 17.9% (patellar dislocations and fractures), and group 3 (538 adolescents between the age of 13 and 16 years) with hemarthrosis in 30.3% (main findings were intra-articular fractures and patellar dislocations, additionally, 16 ligamentous and two meniscal lesions). Correspondingly, physical examination, conventional radiography, and magnetic resonance imaging (most appropriate diagnostic tool for patellar dislocation) were sufficient to reveal injuries in children up to 13 years of age. Arthroscopy was predominantly justified in those older than 13 years of age, but only if it leads to therapeutic consequences.

[Diagnostic and therapeutic consequences of kidney injuries in pediatric blunt abdominal trauma]. / Diagnostische und therapeutische Konsequenzen nach Nierenverletzungen bei kindlichem stumpfem Bauchtrauma.

Renal lesions are frequently encountered in blunt pediatric abdominal trauma. In this retrospective study, we analyzed the incidence of renal trauma in these trauma patients to determine which diagnostic and therapeutic approaches were most predictive. From 1976 to 1996, 308 children sustaining blunt abdominal trauma were admitted to our department. Patients were evaluated using abdominal paracentesis, ultrasonography, and urinary analysis. In specific cases, i.v. urography, CT scan, and/or angiography were applied. We used the Organ Injury Scale (OIS) for classification of renal trauma into five grades. We encountered 69 serious abdominal traumas. Thirty-six patients sustained renal lesions grade 2 (G2) or higher; 20 children were polytraumatized. There were 67 renal lesions with 28 G1, 22 G2, 8 G3, 5 G4, 1 G5, and 3 lesions of the urinary tract. Ultrasonography and urinary analysis were found to be optimal diagnostic means for screening and observing the course of renal lesions. For lesions G2 or higher, CT scan was the most reliable in classifying and diagnosing renal lesions. This superseded i.v. urography. If no contrast medium was excreted in the CT scan, angiography was indicated. Only ten patients proceeded to operative therapy. During the period reviewed, a shift from operative to conservative treatment was notable with a tendency toward minimally invasive therapy. If lesions were G4 or G5, operative treatment was always indicated.

Management of kidney injuries in children with blunt abdominal trauma.

BACKGROUND/PURPOSE: The authors analyzed the incidence and the course of renal injuries encountered in a cohort of pediatric patients with blunt abdominal trauma. This review focuses on the early diagnostic and therapeutic approach rather than the long-term outcome and draws conclusions for an effective initial management. METHODS: From 1976 to 1996, the charts of 308 children with blunt abdominal trauma that were admitted to the authors' department were reviewed. The patients initially were evaluated using urinalysis, ultrasonography, and abdominal paracentesis (until 1984) and in specific cases iv-urography, computed tomography (CT), and angiography. The authors retrospectively classified the renal trauma after the widely used Organ Injury Scaling (OIS) into 5 grades and correlated the diagnostic value of various techniques as well as the diagnostic approach. RESULTS: Sixty-nine serious abdominal traumas were encountered. Thirty-six patients suffered renal lesions grade 2 (G2) or higher; 20 children were polytraumatized. There were 67 renal lesions including 28 G1, 22 G2, 8 G3, 5 G4, 1 G5, and 3 lesions of the lower urinary tract. Ultrasonography and urinalysis were found to be the optimal diagnostic methods for screening and following the course of renal injury. CT scan proved to be most reliable for detecting and exactly classifying renal lesions grade 2 or higher and superseded consecutively iv-urography. In cases in which CT scan failed to show renal excretion of contrast agent, angiography was performed. Ten patients proceeded to operative therapy. CONCLUSIONS: Ultrasonography and urinalysis proved to be the optimal initial evaluation tool for excluding renal injury both as a screening method and for further controls. Exact classification was possible by CT scan. During the reviewed time period a shift from surgical to conservative management was notable. If lesions were G4 or G5, surgical treatment with tendency toward minimally invasive therapy always was indicated.