Digital platforms to facilitate physical activities for people with physical or sensory disabilities: A scoping review.

BACKGROUND: People with disabilities (PWD) commonly experience difficulties in accessing their environments, which can lead to restricted participation in outdoor leisure-time physical activity. Participating in outdoor leisure-time physical activity (OLTPA) provides health and social benefits to PWD and benefits to the communities in which they live. OBJECTIVE: The aim of the study was to identify features existing in digital platforms that facilitate access to OLTPA for PWD. METHODS: A scoping review was conducted in four library databases and in Google advance search to identify relevant scientific and grey literature, and websites. Each step of the review was independently conducted by two co-authors who confirmed consensus of results. Descriptive data analyses were performed. RESULTS: Seven scientific studies and ten websites were included in the scoping review. Seven presented mobile apps, nine presented a website and one presented an online database. Sources reported five main obstacles to using digital platforms that support access to physical activities (e.g., lack of digital literacy, technical issues, unintuitive design), and 10 facilitators (e.g., possibility to personalize your online space, accessibility features of the navigation). Among these sources, a trend emerged in the most important factors and features to consider for the visuals and navigation of the platforms. CONCLUSION: The features of digital platforms that facilitate access to OLTPA include intuitive design compliant with accessibility guidelines and supported by navigation tools, personalization of the online space, and features for social interactions.

Evaluation of aortopexy in the management of severe tracheomalacia after esophageal atresia repair.

Severe tracheomalacia (TM) is a difficult problem in esophageal atresia (EA) patients. We reviewed our experience with aortopexy and other interventions for severe TM in this population. With review ethics board approval, a retrospective review of TM in postoperative EA patients was conducted (1989-2010). Demographics, perinatal, and surgical information regarding EA repair was collected. TM infants were analyzed for symptomatology, clinical severity, investigations, interventions, and outcomes. Data are presented as proportions or median(range). One hundred and thirty-two EA patients were reviewed. Most had type C atresia (87.3%), and 18 patients (13.6%) died. Twenty-five patients (18.9%) had TM of whom five (20%) died. Median symptom onset was 18 days (0-729) after EA repair, with stridor (64%) or retractions/distress (44%) being most frequent. Four and two patients had airway obstruction or cardiorespiratory arrest, respectively. Median time from symptom onset to investigations was 11 days; these were most commonly rigid bronchoscopy (56%) and fluoroscopy (36%). Ten patients (40%) had severe TM on bronchoscopy. Six underwent aortopexy, one fundoplication, and three were treated medically. Length of hospital stay (LOS) post-aortopexy was 13 days (5-60), and ventilation time was 2 days (0-9). LOS was 60.5 (1-69) days postdiagnosis in non-aortopexy patients. Readmission rates for respiratory issues were significantly less in the aortopexy (median 0 vs. 5; P = 0.048) group over 2-year follow up after discharge. Complications of aortopexy included transfusion (1) and temporary diaphragmatic paresis (1), and one mortality secondary to severe congenital cardiac anomalies. Our experience suggests that aortopexy is safe and effective for the treatment of severe TM. It is associated with reduced LOS compared with other treatment strategies and few complications or long-term sequelae.

Thoracotomy for repair of esophageal atresia: not as bad as they want you to think!

Is it outdated now to do a thoracotomy to repair esophageal atresia (EA)? Our practices and the literature on the subject of thoracoscopic and open thoracotomy repair of EA were reviewed, seeking answers to the following questions: Is it correct to compare the new thoracoscopic approach for the repair of EA against the thoracotomy techniques of 15-30 years ago? Should post-thoracotomy scoliosis/thoracic deformity reported in up to 56% of patients be a significant current concern? Are the clips used to close the fistula in thoracoscopic repairs as safe as open suture closures? Is the leak and stricture rate similar with thoracoscopic surgery? Are the anesthesia, period of ventilation, pain, time to first feeding, and the length of hospital stay significantly different with current thoracotomy techniques compared with thoracoscopic methods? Is the cosmetic result of a thoracoscopic repair significantly better? Is the learning curve for EA thoracoscopic repair harming patients for minimal long-term benefit? These questions were scientifically unanswerable at this time. The limited EA thoracotomies currently performed have a track record of proven safety and minimal morbidity. The results published by surgeons who are pioneers in thoracoscopy may not be generalizable, and the complication rate from teams with less experience is likely underreported. In selected patients and with experienced teams, thoracoscopic EA repair is appropriate. However, EA repair via thoracotomy should, for now, remain as the 'gold standard'. Further registry-based, multicenter, comparative studies on EA repair methodologies and outcomes should provide important answers.

Refractory strictures post-esophageal atresia repair: what are the alternatives?

Esophageal strictures remain the most frequent complication after esophageal atresia (EA) repair despite refinements in operative techniques. With an incidence of anastomotic stricture between 8% and 49%, EA is the most frequent cause of benign esophageal stricture in children. The mainstay of treatment for esophageal stricture is dilatation with a 58-96% success rate. In order to relieve dysphagia, between 1 and 15 dilatations will be required in each EA patient with an esophageal stricture. However dilatations may lead to complications including perforation (0.1-0.4% of all esophageal benign strictures) and sociopsychological morbidity. Fifty percent of EA strictures will improve in 6 months. However, 30% will persist and require repeat dilatations. The present article explores the variety of non-surgical alternative treatments for anastomotic strictures after EA repair, focusing on triamcinolone acetonide, mitomycin C and esophageal stents. We propose an algorithm for a more standardized therapeutic approach, with the hope that an international panel of experts could meet and establish a consensus.

[Focal dermal hypoplasia (Goltz syndrome) in the neonate: report of a case presenting with cleft lip and palate]. / Syndrome de Goltz chez un nouveau-né avec fente labiopalatine.

Focal dermal hypoplasia (Goltz syndrome) is a rare congenital dysplasia of the mesoectodermal derived tissues. It is an X-linked inheritance syndrome caused by mutations in the PORCNgene mapped on Xp11.23. The condition is characterized by cutaneous lesions distributed in linear areas associated with diverse congenital deformities. Given the rarely described neonatal features, we report a case of Goltz syndrome in a black female newborn. This is the first case known in Burkina Faso. The cutaneous, hair, and nail lesions usually observed were present, characterized by their preponderance on the left side of the body with exclusive ipsilateral skeletal abnormalities (cleft lip and palate, agenesis of the metatarsals and toes of the foot, syndactyly, lobster claw, and absence of a rib). The limits in the management and the negative social and cultural perception of the deformities in the context of a developing country did not favor the child's survival.

Large hepatic mesenchymal hamartoma leading to mid-trimester fetal demise.

This report describes a fetus with a large multiloculated cystic liver mass. Two small abdominal cysts were seen on ultrasound at 19 weeks of gestation but the patient was referred to us at 23 weeks, after the mass had grown to 8.0 x 5.6 x 7.0 cm, displacing intra-abdominal organs, heart and diaphragm. There was a small amount of ascites but no hydrops. There was polyhydramnios and a thick hyperechoic placenta. After detailed sonograms and MRI suggested the diagnosis of cystic mesenchymal hamartoma of the liver, cyst decompression was favored and consent was obtained. Unfortunately, absence of fetal cardiac activity was noted on the day of the planned intervention. Autopsy confirmed the diagnosis and demonstrated placental changes consistent with mesenchymal stem villous hyperplasia of the placenta. Large fetal cystic abdominal masses that compress the heart, lungs and other organs may benefit from prenatal decompression. This is the first report of cystic hamartoma of the liver apparent on second-trimester sonography, and the fourth time such a lesion is associated with fetal or neonatal death out of 11 cases diagnosed prenatally.

Incidence of important hemobilia following transhepatic biliary drainage: left-sided versus right-sided approaches.

Our purpose here is to describe our experience with important hemobilia following PTBD and to determine whether left-sided percutaneous transhepatic biliary drainage (PTBD) is associated with an increased incidence of important hemobilia compared to right-sided drainages. We reviewed 346 transhepatic biliary drainages over a four-year period and identified eight patients (2.3%) with important hemobilia requiring transcatheter embolization. The charts and radiographic files of these patients were reviewed. The side of the PTBD (left versus right), and the order of the biliary ductal branch entered (first, second, or third) were recorded. Of the 346 PTBDs, 269 were right-sided and 77 were left-sided. Of the eight cases of important hemobilia requiring transcatheter embolization, four followed right-sided and four followed left-sided PTBD, corresponding to a bleeding incidence of 1.5% (4/269) for right PTBD and 5.2% (4/77) for left PTBD. The higher incidence of hemobilia associated with left-sided PTBD approached, but did not reach the threshold of statistical significance (p = 0.077). In six of the eight patients requiring transcatheter embolization, first or second order biliary branches were accessed by catheter for PTBD. All patients with left-sided bleeding had first or proximal second order branches accessed by biliary drainage catheters. In conclusion, a higher incidence of hemobilia followed left-versus right-sided PTBD in this study, but the increased incidence did not reach statistical significance.

Gastrointestinal duplications.

BACKGROUND/PURPOSE: The aim of this study was to review the presentations of gastrointestinal duplication (GID) and to assess the influence of prenatal diagnosis on treatment. METHODS: Retrospective review of all GID at 2 pediatric hospitals from 1980-2002 was conducted. RESULTS: Seventy-three patients (M43:F30) were identified: 21 neonates, 28 infants (1 to 24 months), 15 children (1 to 10 years), 9 adolescents (>/=11 years). GID location by frequency was ileum (31.5%), ileocaecal valve (30.2%), duodenum (9.6%), stomach (8.2%), jejunum (8.2%), colon (6.8%), and rectum (5.5%). In neonates and infants, vomiting and distension were the most common presentations. Volvulus, caused by a duplication, occurred in 23.8% of neonates and caused the death of one neonate. Intussusception was identified in 10.9% of patients. In older children and adolescents, pain and vomiting were the most common associations. Six of these patients were being treated for Crohn's disease, with the diagnosis of duplication made at laparotomy. Eighteen patients had a prenatal diagnosis by ultrasound scan, with 77.2% of these asymptomatic after birth. Most prenatal diagnoses occurred after 1991 (77.8%). When comparing an earlier period (1980 to 1991; 29 patients) with the current (1992 to 2002; 44 patients), a greater proportion of the latter patients were asymptomatic (36.4 v 13.8%) and had a lower incidence of complications (volvulus/intussusception). CONCLUSIONS: GID can lead to life-threatening complications. Prenatal diagnosis should lead to expeditious postnatal investigation and treatment before the onset of symptoms or complications. GID in older children can mimic Crohn's disease. Laparoscopy/laparotomy should be considered in patients with atypical Crohn's disease or when the diagnosis of an intraabdominal mass is unclear.

Post-operative colocolic intussusception.

A 2-year-old boy presented with bowel obstruction 5 days after repair of a recurrent epigastric hernia. Pre-operative imaging implicated intussusception as a cause of the obstruction. At operation a gangrenous transverse colocolic intussusception was resected en masse. No clear lead point was seen. Recovery was uneventful.

Intraoperative ultrasound and preoperative localization detects all occult insulinomas; discussion 1025-6.

HYPOTHESIS: Preoperative invasive localization procedures with intraoperative ultrasound (IOUS) can result in successful surgical treatment of occult insulinomas when noninvasive imaging study results are equivocal or negative. DESIGN: Prospective study. SETTING: Tertiary care university hospital. PATIENTS: Thirty-seven consecutive patients with a biochemical diagnosis of insulinoma without multiple endocrine neoplasia (MEN). INTERVENTION: All patients underwent portal venous sampling (PVS) (n = 22) or calcium angiogram (n = 15) followed by surgery with palpation and IOUS (n = 37). MAIN OUTCOME MEASURE: Portal venous sampling, calcium angiogram, palpation, and IOUS were compared for accurate localization of insulinoma. RESULTS: All patients were cured of hypoglycemia after surgery. Portal venous sampling correctly localized tumors in 17 (77%) of 22 patients. Calcium angiogram was correct in 13 (87%) of 15 patients. Palpation identified 24 (65%) of 37 tumors, and IOUS found 35 (95%) of 37 tumors. The 2 tumors missed by IOUS were located in the tail of the pancreas and were resected based on regional localization alone. CONCLUSIONS: Intraoperative ultrasound is the single best localization study, but it will miss some tumors that regional localization can identify. Combining both modalities allowed surgical cure of all insulinomas in our study. Therefore, we recommend both IOUS and regional localization for insulinoma when preoperative imaging studies are equivocal.

Use of antenatal steroids to counteract the negative effects of tracheal occlusion in the fetal lamb model.

Tracheal occlusion (TO) in fetal lambs induces pulmonary hyperplasia but has negative effects on type II cells. The purpose of this study was to determine whether antenatal steroids could reverse the adverse effects of TO on lung maturation in fetal lambs. Sixteen time-dated pregnant ewes (term, 145 d) and 24 of their fetuses were divided into six groups: 1) TO at 117 d gestation; 2) TO at 117 d with a single maternal intramuscular injection of 0.5 mg/kg betamethasone 24 h before delivery; 3) TO at 117 d and release of the occlusion 2 d before delivery; 4) TO and release of the occlusion with maternal steroids; 5) unoperated controls without antenatal steroid treatment; and 6) unoperated controls, littermates of groups 1-4, treated with antenatal steroids. All fetuses were killed at 137 d gestation. Outcome measurements consisted of lung weight-to-body weight ratio; lung morphometry determined by mean terminal bronchial density; and assessment of type II pneumocytes by in situ hybridization to the mRNA of surfactant proteins B and C. Lung weight-to-body weight ratio and mean terminal bronchial density were significantly different among groups with TO and controls, indicating increased lung growth and structural maturation. The density of type II pneumocytes was markedly decreased by TO. Release 2 d before sacrifice significantly increased the density and surfactant activity of type II pneumocytes, but to levels still far from controls. Steroids alone had an effect similar to release. An additive effect was noted with steroids and 2-d release resulting in type II cell density comparable to controls. After fetal TO, a single maternal intramuscular dose of 0.5 mg/kg of betamethasone 24 h before delivery allows partial recuperation of the type II pneumocytes, an effect that is potentiated by 2-d release.

Endoleak after endovascular repair of abdominal aortic aneurysm.

PURPOSE: We sought to assess the role of endovascular techniques in the management of perigraft flow (endoleak) after endovascular repair of an abdominal aortic aneurysm. METHOD: We performed endovascular repair of abdominal aortic aneurysm in 114 patients, using a variety of Gianturco Z-stent-based prostheses. Results were evaluated with contrast-enhanced computed tomography (CT) at 3 days, 3 months, 6 months, 12 months, and every year after the operation. An endoleak that occurred 3 days after operation led to repeat CT scanning at 2 weeks, followed by angiography and attempted endovascular treatment. RESULTS: Endoleak was seen on the first postoperative CT scan in 21 (18%) patients and was still present at 2 weeks in 14 (12%). On the basis of angiographic localization of the inflow, the endoleak was pure type I in 3 cases, pure type II in 9, and mixed-pattern in 2. Of the 5 type I endoleaks, 3 were proximal and 2 were distal. All five resolved after endovascular implantation of additional stent-grafts, stents, and embolization coils. Although inferior mesenteric artery embolization was successful in 6 of 7 cases and lumbar embolization was successful in 4 of 7, only 1 of 11 primary type II endoleaks was shown to be resolved on CT scanning. There were no type III or type IV endoleaks (through the stent-graft). Endoleak was associated with aneurysm dilation two cases. In both cases, the aneurysm diameter stabilized after coil embolization of the inferior mesenteric artery. There were two secondary (delayed) endoleaks; one type I and one type II. The secondary type I endoleak and the associated aneurysm rupture were treated by use of an additional stent-graft. The secondary type II endoleak was not treated. CONCLUSIONS: Type I endoleaks represent a persistent risk of aneurysm rupture and should be treated promptly by endovascular means. Type II leaks are less dangerous and more difficult to treat, but coil embolization of feeding arteries may be warranted when leakage is associated with aneurysm enlargement.

Analysis of initial CT findings after endovascular repair of abdominal aortic aneurysm.

PURPOSE: To determine the spectrum and frequency of specific computed tomographic (CT) findings in the acute period after endovascular repair of abdominal aortic aneurysm (AAA). MATERIALS AND METHODS: CT images obtained 1--3 days after endograft placement were evaluated in 88 patients. The images were analyzed for stent position, appearance of endograft components, perigraft leak, and postoperative findings including air and acute thrombus within the aneurysm and air surrounding the femoral-femoral bypass graft. Findings that could be misinterpreted as perigraft leak were evaluated. RESULTS: Fifteen (17%) of 88 patients had perigraft leak in the acute postoperative period. The bare segment of the proximal self-expanding stent covered one or both renal arteries in 54 (61%) patients. One patient had CT evidence of renovascular compromise. Postoperative air was within the aneurysmal sac in 51 (58%) patients and surrounded the femoral-femoral bypass graft in 67 (94%) of 71 patients in whom the grafts were evaluated with CT. Mottled attenuation within the aneurysmal sac was seen in 50 (57%) patients. Forty-six (52%) patients had calcifications within longstanding thrombus. In 31 (35%) patients, findings that could have been misinterpreted as perigraft leak were identified. CONCLUSION: Accurate analysis of CT findings after endovascular AAA repair requires careful review of all available CT images (preprocedural and pre- and postcontrast) and clear understanding of specific stent-graft components and placement.

Laparoscopic excision of subdiaphragmatic epidermoid cyst: a case report.

Retroperitoneal epidermoid cysts are rare. The authors report a case of an 11-year-old boy with an asymptomatic subdiaphragmatic cyst, which was found incidentally during an investigation for hypertension. At laparoscopy, the cyst was densely adherent to the diaphragm, resulting in a pneumothorax during dissection. Nevertheless, the excision and the diaphragmatic repair could be completed laparoscopically without complication. Microscopic examination showed an epidermoid cyst. No similar case has been reported in the literature.

Endoscopic retrograde cholangiopancreatography in children: A surgeon's perspective.

PURPOSE: The aim of this study was to review the indications, success rate, and complications of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group. METHODS: From 1990 to 1999, 21 ERCP procedures were attempted in 20 patients. They consisted of 8 boys and 12 girls whose age ranged from 4 to 17 years (mean, 11.3 years). Fourteen were performed under deep sedation (mean age, 12.8 years), and 7 were done under general anesthesia (mean age, 7.6 years). All ERCP procedures were performed by experienced adult endoscopists. RESULTS: The indication for ERCP was biliary in 15 patients. Eleven had suspected choledocholithiasis by either ultrasound scan, intraoperative cholangiogram or magnetic resonance imaging (MRI). In 6 cases, the ERCP was done for pancreatic pathology. In 11 patients, the ERCP was diagnostic only, and in 10 a therapeutic procedure was done. The overall success rate was 90.5%. Post-ERCP complications consisted of 6 episodes of pancreatitis (28.5%), 4 of which followed a therapeutic procedure, and 1 episode of bleeding. Pancreatitis resolved 2 to 6 days post-ERCP. The patients underwent follow-up between 2 and 56 months after the ERCP (mean, 11 months). CONCLUSIONS: The authors conclude that even in experienced hands, ERCP in the pediatric population has a much higher complication rate than in adults (33.3%). We recommend that very specific indications be met before subjecting a pediatric patient to an endoscopic retrograde cholangiopancreatography.

Pulmonary structural maturation and pulmonary artery remodeling after reversible fetal ovine tracheal occlusion in diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with thickened pulmonary arteries (PA) contributing to pulmonary hypertension. In the current study, the effects of antenatal glucocorticoids and reversible tracheal occlusion (TO) on PA structure were assessed in a hypoplastic lung model. METHODS: A left-sided CDH was created in fetal lambs at 80 days gestation, TO at 108 days, and release of the occlusion (TR) at 129 days. All were given 1 dose of maternal glucocorticoids at 135 days. At 136 days (term, 145 days), the fetus was delivered by cesarian section. CDH (n = 7), CDH + TO (n = 6), CDH + TO + TR (n = 6), and unoperated twin controls (n = 16) were compared. Outcome measurements were (1) lung growth, represented by lung weight to body weight ratio (LW/BW), (2) lung structural maturation, which is inversely proportional to mean terminal bronchiole density (MTBD), (3) PA medial and adventitial areas (square micrometers), (4) lung capillary load, which is the ratio of vessel surface area (SA) to tissue SA ratio. RESULTS: CDH lungs were hypoplastic with a low LW/BW and high MTBD. The small PAs (<75 microm) of CDH had an increased medial area, indicating increased muscle mass and an increased adventitial area. CDH + TO +/- TR increased LW/BW and achieved normal structural lung maturity with a low MTBD. Only CDH + TO thinned the PA medial area closer to control values. The adventitial area remained thick in CDH +/- TO +/- TR when compared with controls. All 4 groups had similar capillary load. CONCLUSIONS: TO may be especially important for PA remodeling in the latter part of gestation, because TR 1 week before delivery prevents thinning of the small PAs in CDH. The shaping achieved by TO in terms of lung growth, structural maturity, and pulmonary artery medial area thinning may prove beneficial in lessening the severity of the associated pulmonary hypertension in CDH.

The multiple facets of pulmonary sequestration.

PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.

Clinical versus sonographic evaluation of acute appendicitis in children: a comparison of patient characteristics and outcomes.

PURPOSE: Abdominal sonography has gained popularity in establishing the diagnosis of appendicitis in children with equivocal clinical presentations. However, no clear outcome benefits have been demonstrated to date. The authors conducted a retrospective study to compare the characteristics and outcomes of patients undergoing appendectomy after clinical evaluation only with those undergoing the procedure after sonography. METHODS: The charts of 454 consecutive patients undergoing appendectomy for acute appendicitis between January 1, 1998 and December 4, 1999 were reviewed. Patients operated on after clinical evaluation only were compared with patients operated on after abdominal sonography. RESULTS: Forty-two percent of patients (n = 191) constituted the sonography group. When compared with the clinical group, these patients had higher prevalence of female gender (52% v 38%; P =.004), longer symptom duration (2.2 +/- 2.5 v 1.6 +/- 1.6 days; P =.003), higher incidence of preoperative in-patient observation (19% v 4%; P <.001), longer duration between evaluation and operation (8.0 +/- 3.9 v 4.9 +/- 2.9 hours; P <.001), higher incidence of normal appendices on pathologic examination (13% v 6%; P =.006), and higher incidence of postoperative abscesses or phlegmons (4.4% v 1.2%; P =.04). The groups did not differ significantly in age, hospital stay, incidence of complicated appendicitis, or incidence of wound infection. CONCLUSIONS: Patients undergoing sonography before appendectomy have a longer delay before operation, a higher rate of misdiagnosis, and more postoperative complications. Limiting sonography to truly equivocal cases and using it early in the diagnostic workup may improve outcomes in this group of patients.