Establishing disability weights for congenital pediatric surgical conditions: a multi-modal approach.

BACKGROUND: Burden of disease (BoD) as measured by Disability-Adjusted Life Years (DALYs) is one of the criteria for priority-setting in health care resource allocation. DALYs incorporate disability weights (DWs), which are currently expert-derived estimates or non-existent for most pediatric surgical conditions. The objective of this study is to establish DWs for a subset of key pediatric congenital anomalies using a range of health valuation metrics with caregivers in both high- and low-resource settings. METHOD: We described 15 health states to health professionals (physicians, nurses, social workers, and therapists) and community caregivers in Kenya and Canada. The health states summaries were expert- and community-derived, consisting of a narrated description of the disease and a functional profile described in EQ-5D-5 L style. DWs for each health state were elicited using four health valuation exercises (preference ranking, visual analogue scale (VAS), paired comparison (PC), and time trade-off (TTO)). The PC data were anchored internally to the TTO and externally to existing data to yield DWs for each health state on a scale from 0 (health) to 1 (dead). Any differences in DWs between the two countries were analyzed. RESULTS: In total, 154 participants, matched by profession, were recruited from Kijabe, Kenya (n = 78) and Hamilton, Canada (n = 76). Overall calculated DWs for 15 health states ranged from 0.13 to 0.77, with little difference between countries (intra-class coefficient 0.97). However, DWs generated in Kenya for severe hypospadias and undescended testes were higher than Canadian-derived DWs (p = 0.04 and p < 0.003, respectively). CONCLUSIONS: We have derived country-specific DWs for pediatric congenital anomalies using several low-cost methods and inter-professional and community caregivers. The TTO-anchored PC method appears best suited for future use. The majority of DWs do not appear to differ significantly between the two cultural contexts and could be used to inform further work of estimating the burden of global pediatric surgical disease. Care should be taken in comparing the DWs obtained in the current study to the existent list of DWs because methodological differences may impact on their compatibility.

Do intramedullary implants improve survival in elderly patients with trochanteric fractures? A retrospective study.

BACKGROUND AND HYPOTHESIS: There is currently no consensus regarding superiority of the intramedullary fixation over the sliding hip screw. Regional variation remains high and not backed up by solid evidence. Given these premises we aimed to analyze weather implant preference can influence the postoperative survival. Secondary objectives were determining the trend for implant choice and confounding factors associated with intramedullary nails compared to sliding hip screws. PATIENTS AND METHODS: Retrospective data was obtained from patient charts with the main diagnosis of extracapsular/ trochanteric fractures, corresponding to ICD S72.1 codes. Between 2008-2012, 441 patients underwent osteosynthesis with a dynamic hip screw and 155 with intramedullary nail respectively. The living status was determined by comparing the patient identification number against the national population evidence records. RESULTS: The lifetable shows similar survival for both implants over the 5 year period. The yearly mortality was 19.4% for the dynamic hip screw and 21.8% for the intramedullary implant respectively, even though the later were used predominantly in older patients. This age difference is significant according to both parametric and non-parametric tests whereas duration of hospital stay are similar. We found a clear increase in the proportion of intramedullary implants, for a total of 11.2% over the 5 year period. There is no difference for the one year mortality and overall survival between sliding screw plates and intramedullary constructs. CONCLUSIONS: A clear increase in the use of intramedullary implants for trochanteric fractures was observed. This is even more apparent for older ages, presumably due to an higher surgeon confidence with the biomechanical stability of the intramedullary constructs.

Quantifying the Disability from Congenital Anomalies Averted Through Pediatric Surgery: A Cross-Sectional Comparison of a Pediatric Surgical Unit in Kenya and Canada.

BACKGROUND: Pediatric surgical practice is different in low- and middle-income countries as compared to North America. While resources are limited, the impact of pediatric surgical procedures is significant. The objective of this study was to calculate and compare disability-adjusted life years (DALYs) averted in a Kenyan and Canadian surgical unit for a subset of pediatric congenital anomalies. METHODS: Medical records of children having undergone surgical procedures for 13 congenital conditions in both surgical units were collected over 12 months. DALYs for each condition were calculated using previously obtained disability weights derived in each country. Age-adjusted life expectancy rates from the WHO were used to determine years of life lost. Risk of permanent disability without surgery and probability of successful treatment values were obtained from the literature and included in the DALY calculation. RESULTS: The conditions accounting for the largest total number of averted DALYs in Kenya were hydrocephalus (60.8%) and spina bifida (18.1%), whereas in Canada they were hydrocephalus (24.2%) and undescended testes (19.2%). A total of 23,169 DALYs were averted through 1042 surgical procedures (22.2 DALYs per procedure) during the study period in Kenya, compared to 5497 DALYs through 373 procedures (14.7 DALYs per procedure) in Canada. CONCLUSIONS: Using recent developments in burden of disease measurement, the results point to the significant impact of pediatric surgical centers in addressing the global burden of congenital surgical disease. The study carries significant implications for resource allocation and training.

Risks and challenges of orthopaedic invasive interventions in haemophilia in a low-resource country. A single-center experience.

UNLABELLED: Haemophilic arthropathy is a defining feature and a debilitating condition of persons with haemophilia (PwH) in low resource countries. Orthopaedic surgery is unavoidable for patients with high occurrence of joint damage. AIMS: We aimed to evaluate the spectrum and outcome of invasive orthopaedic therapies in PwH and von Willebrand diseases (VWD). PATIENTS AND METHODS: Our descriptive observational retrospective study included 131 invasive surgical procedures, performed on 76 consecutive patients, most of them (93.4%) with severe disease, treated in Timisoara's Haemophilia Center over a period of 12 years; 17.1% had pre-operation anti-FVIII inhibitors. Invasive elective procedures were predominant (90.8%) as compared to emergency measures (9.2%); according to their invasiveness, 20.6% of interventions were major, 44.3% intermediate and 35.1% minor. Results were good in the majority of cases; significantly reduced joint bleed rate and pain score were the most consistent achievements. The greatest proportion of complications occurred after major (66.7%), compared to moderate (25.6%) and minor (7.7%) interventions. The main threatening complication was the development (3.8%) or increase (4.6%) of inhibitor titer. Local bacterial infections and wound dehiscence complicated the evolution in 4.6% and 0.8 % of cases, respectively; we noticed no blood-borne infections or thrombotic accidents. Low dosage (10.7%) and short duration of substitution (21.4%) led to increased post-surgical bleeding and post-haemorrhagic anaemia. CONCLUSIONS: Surgery is a highly demanding intervention in haemophilia, which cannot be ignored in a low resource country. It represents a life or limb-saving and quality of life-improving measure.

Clinical relevance of altered bone immunopathology pathways around the elbow.

Normal healing of fractures is a complex process that relies heavily on a cascade of consecutive activations of immune cells and mediators. This mechanism somewhat overlaps with all processes related to bone metabolism, from the absence of unions to heterotopic ossifications and osteoporosis. We aimed to review and describe this intricate process of bone metabolism with particular focus on abnormal function and to exemplify it with a series of clinical cases which could justify their practical importance. The elbow has great potential for fracture healing but it is very sensitive to prolonged immobilization which can easily lead to intra-articular adherences and stiffness. In addition, the interosseus membrane facilitates communication between the regenerative environments when both radius and ulna are fractured. Such extensive injuries, around the proximal forearm, can lead to heterotopic ossifications and synostosis, which decrease sagittal range of motion through impingement and even block rotational movement through bone bridges. Increased knowledge and awareness of the biological mechanism of fracture healing, will have great improvement in the pharmacological adjuvant treatment of elbow injuries.

Getting the job done: analysis of the impact and effectiveness of the SmileTrain program in alleviating the global burden of cleft disease.

BACKGROUND: The study measured the success of SmileTrain, the largest cleft charity globally, in alleviating the global burden of disease (GBD). It was done by estimating averted disability-adjusted life years (DALYs) and delayed averted DALYs because of the global backlog in cleft procedures. METHODS: Anonymized data for all procedures in the SmileTrain global database were analyzed by age, sex, country, region, and surgery type. DALYs averted were calculated using life expectancy tables and established and estimated disability weights. The cost-effectiveness analysis used mean SmileTrain procedural disbursement figures. Sensitivity analysis was performed using various cleft incidence rates, life expectancy tables, and disability weights. RESULTS: During 2003-2010 a total of 536,846 operations were performed on 364,467 patients-86 % in Southeast Asia and the western Pacific region. Procedure numbers increased yearly. Mean age at primary surgery-6.2 years (9.8 years in Africa)-remained fairly constant over time in each region. Globally, 2.1-4.7 million DALYs were averted through the operations at a total estimated cost of US$196 M. Mean DALYs per patient were 3.8-9.0, and mean cost per DALY was $72-$134. Total delayed GBD due to advanced age at surgery was 191,000-457,000 DALYs. CONCLUSIONS: Despite an unparalleled number of surgeries performed and yearly increase by one charity, the unmet and delayed averted cleft GBD remains significant in all regions. Large geographic disparities reflect varied challenges regarding access to surgery. Cleft surgeries are cost-effective interventions to reduce the global burden of disease (GBD). Future challenges include increased collaboration among cleft care providers and a focus on remote global areas by building infrastructure and local training.

Heavy-particle radioactivity of superheavy nuclei.

The concept of heavy-particle radioactivity (HPR) is changed to allow emitted particles with Z(e) > 28 from parents with Z > 110 and daughter around (208)Pb. Calculations for superheavy (SH) nuclei with Z = 104-124 are showing a trend toward shorter half-lives and larger branching ratio relative to α decay for heavier SHs. It is possible to find regions in which HPR is stronger than alpha decay. The new mass table AME11 and the theoretical KTUY05 and FRDM95 masses are used to determine the released energy. For 124 we found isotopes with half-lives in the range of ns to ps.

Cluster-randomized, crossover trial of the efficacy of plain soap and water versus alcohol-based rub for surgical hand preparation in a rural hospital in Kenya.

BACKGROUND: The aim of this cluster-randomized, crossover trial was to compare the efficacy of plain soap and water with an alcohol-based handrub for surgical hand preparation and prevention of surgical-site infection (SSI) in a Kenyan rural hospital. METHODS: A total of 3317 patients undergoing clean and clean-contaminated surgery were included. Follow-up data 30 days after discharge were available for 3133 patients (94.5 per cent). RESULTS: SSI occurred in 255 patients (8.1 per cent), with similar rates for both study arms: 8.3 per cent for alcohol-based handrub versus 8.0 per cent for plain soap and water (odds ratio 1.03, 95 per cent confidence interval 0.80 to 1.33). After adjustment for imbalances between study arms and clustering effects, the main outcome measure remained unchanged (adjusted odds ratio 1.06, 0.81 to 1.38). The duration of surgery and wound contamination class independently predicted SSI. The cost difference between the methods was small (€4.60 per week for alcohol-based handrub compared with €3.30 for soap and water). CONCLUSION: There was no statistically or clinically significant difference in SSI rates, probably because more important factors contribute to SSI development. However, this study demonstrated the feasibility and affordability of alcohol-based handrubs for hand preparation before surgery in settings without continuous, clean water. REGISTRATION NUMBER: NCT00987402 (http://www.clinicaltrials.gov).

Repair of a post-traumatic cartilage defect with a cell-free polymer-based cartilage implant: a follow-up at two years by MRI and histological review.

Microfracture is frequently used as the first line of treatment for the repair of traumatic cartilage defects. We present the clinical and histological results 18 months to two-years after treatment in a 26-year-old male with a post-traumatic chondral defect of the medial femoral condyle managed by microfracture covered with chondrotissue, a cell-free cartilage implant made of a resorbable polyglycolic acid felt and hyaluronic acid.

Encephalocele--a single institution African experience.

OBJECTIVE: This study seeks to find out the presentation, management and complications of encephaloceles in an African setting. DESIGN: a retrospective study reviewing the age and sex of the patients, type and contents of encephaloceles, associated anomalies, preoperative evaluation and investigations, surgical approaches, intra- and post-operative complications as well as follow-up outcomes. SETTING: Bethany Crippled Children's centre and Bethanykids at Kijabe Hospital (BKKH), between January 1998 and August 2006. PATIENTS: Of the 53 patients seen, 23 were males and 30 females. The median age at presentation was four months. RESULTS: The follow-up period extended to eight years. Twenty nine patients had occipital encephaloceles, and 39 were operated using the direct external approach. Cererobrospinal fluid leak was the most common post-operative complication. Recurrence occurred in four patients and death in six. CONCLUSIONS: Most of the encephalocele patients manage d at BKKH had good outcomes and proceeded to live normal or near-normal lives. Our study confirms that even in resource-constrained areas, children with encephaloceles can be successfully managed with acceptable outcomes.

Surgery--a challenge in haemophiliacs with inhibitors.

UNLABELLED: Treatment of haemophiliacs with inhibitors is of great concern in low-income countries confronting shortage in substitutive treatment. Invasive interventions on these patients represent a major challenge due to the fact that costs are significantly higher in comparison to similar procedures conducted on patients without inhibitors. OBJECTIVE: In the context of insufficient availability of clotting factor, we aimed at highlighting the experience of surgical treatment in inhibitor patients. We analyzed the indications, types of performed interventions and outcomes. PATIENTS, METHODS: This single center, retrospective analysis has been conducted on 7 inhibitor patients registered and treated in Haemophilia Center of Timisoara over ten years (1997-2007): six patients with severe hemophilia A (3 - high titer, 3 - low titer), one patient with von Willebrand disease (low titer).Three patients developed inhibitors only after 2-5 days post surgery. RESULTS: A total of 15 invasive procedures were carried out: 2 orthopedic interventions (1 arthrodesis, 1 arthroscopic synovectomy), 2 urogenital interventions (1 surgical testicular detorsion, 1 orchiectomy), 4 limb amputations (2 bilateral upper and 2 lower limb amputation), 2 pseudotumour (PT) surgery interventions, 5 drainages (2 massive pyohaemothorax, 1 drainage of shank haematoma, 1 drainage of compressive forearm haematoma, 1 drainage of thigh haematoma). Haemostasis was achieved in patients with low level inhibitors (< 5 BU/ml) with high doses of FVIII concentrates; in those with high inhibitor level (> 5 BU/ml), surgery was managed using by-passing agents. Supplementation with local fibrin glue and intravenous or local antifibrinolytic agents was given in 68.75% of interventions. Postoperative complications consisted of haemorrhagic shock in 13.33% of interventions and infection in 6.66%. Haemostatic outcome was evaluated by blood loss and duration of treatment, compared to expectations for non-inhibitor patients. The outcome was excellent and good in 66.66% of interventions, and fair in 33.33%. Discussion, conclusion: Indication of invasive procedures in haemophiliacs with inhibitors was limited to life and/or limb-threatening situations. In low-income countries, inhibitor and recovery of FVIII monitoring is mandatory in the postoperative follow-up of patients with low or no substitution prior to surgery due to false negative results at the preoperative investigation.

Orthopedic approach of haemophiliacs. A single center experience in Romania.

Clinical expression of inadequately treated haemophilia is dominated by orthopedic complications, requiring invasive or non-invasive interventions. OBJECTIVE: In Romania, with under dosed and late introduced "on demand" substitution, we aimed at highlighting the experience of orthopedic treatment and its outcome. PATIENTS, METHODS: Single center retrospective analysis regarding orthopedic interventions and their outcomes was conducted on 59 hemophilia A, B, and von Willebrand disease patients, between 2002 and 2007. RESULTS: The majority of interventions, invasive (60.71%) and non-invasive (39.28%), were elective, only two being emergencies. Postoperative functional evolution after synovectomies was good in 68.28%, fair in 24.39%, satisfactory in 7.31%. Results of 33 non-invasive (extensive releases) procedures were very good in 27.27%, good in 63.63%, poor in 9.09%. DISCUSSION, CONCLUSIONS: The important number and complexity of orthopedic interventions are proving the precarious musculoskeletal state in persons with hemophilia, demonstrating the need of improving substitution, at least with discontinue prophylaxis in patients with severe forms.

Entero-enteric fistula following mild necrotizing enterocolitis.

We report a 26-week gestational age newborn who developed an entero-enteric fistula associated with a small bowel stricture following a mild episode of necrotizing enterocolitis. The fistula led to a delay in the diagnosis and surgical treatment of the stricture, and should therefore be considered in the setting of partial or intermittent obstruction in the newborn who has had necrotizing enterocolitis.

Sonographic pitfalls in the diagnosis of enteric duplication cysts.

OBJECTIVE: The sonographic double wall sign has been well described in the literature and is often the cornerstone in suggesting the diagnosis of an enteric duplication cyst. We report two cases with this sign that were erroneously diagnosed as enteric cysts and a third case without this sonographic feature that proved to be a duplication cyst. Histologic analysis of the specimens helps explain the cause of the sonographic pitfalls. CONCLUSION: The potential sonographic visualization of the split hypoechoic muscularis propria layer or identification of all five layers will increase the specificity in making the sonographic diagnosis of duplication cyst.

Training general surgery residents in pediatric surgery: A Canadian survey.

BACKGROUND/PURPOSE: The training of general surgeons in pediatric surgery is an important educational role of pediatric surgeons (PS). The authored surveyed this training process and the related expectations and perceptions of competence. METHODS: The authors surveyed all practicing members of the Canadian Association of Paediatric Surgeons (CAPS) in Canada, all general surgery program directors (PD), and all final year general surgery residents (GS). Questions included exposure to pediatric surgery, expected and perceived competence in managing common pediatric general surgical problems, and trainee practice intentions. RESULTS: Response rate to date was 51% from PS, 69% from PD, and 19% from GS. Sixty-seven percent of PS considered the exposure to pediatric surgery satisfactory, yet only 1 of 7 residents planning on pursuing general surgery felt adequately prepared. Trainees were expected to be competent in the conditions polled by 65% of PS and 74% of PD, yet only 38% of the trainees actually felt competent in them. The largest discrepancies were found for infant hernia, newborn colostomy, and cryptorchidism. Presence of a fellowship program and size of training program had no impact on perceived competence. CONCLUSIONS: Training of general surgeons in pediatric surgery varies across Canadian programs. Perceived resident competence often lags behind program and faculty expectations. These data can be used for directing educational priorities in general surgery programs.

Unilateral ovarian agenesis and fallopian tube maldescent.

Unilateral ovarian agenesis (UOA) and fallopian descent problems are very rare congenital defects. We present an unusual case of UOA associated with fallopian-tube maldescent discovered incidentally during a laparotomy in a female infant for a persistent heterogeneous right ovarian cyst refractory to needle aspiration. A necrotic, hemorrhagic paratubal cyst was found associated with a normal right adnexa. The left ovary was absent and a rudimentary left fallopian tube was found tightly stretched over the sigmoid colon to the left retroperitoneum. This previously unreported constellation of anomalies may carry significant potential risks for bowel obstruction and later fertility.

Cost factors in Canadian pediatric trauma.

OBJECTIVES: To estimate the costs of Canadian pediatric trauma and identify cost predictors. DESIGN: A chart review. SETTING: A regional trauma centre. STUDY MATERIAL: The charts of all 221 children who suffered traumatic injuries with an Injury Severity Score (ISS) of 4 or more seen over 6 years at a regional trauma centre. MAIN OUTCOME MEASURES: Patient data, injury data, all hospital-based costs, excluding nursing, food and medication costs. RESULTS: Mean (and standard deviation) patient age was 12.8 (5) years. Sixty percent were boys. Motor vehicle accidents (MVAs) accounted for 71% of the injuries, followed by falls (11%). The mean (and SD) total cost of care was Can$7,582 (Can$12,370), and the cost of media was Can$2,666. Total cost correlated directly with age (r = 0.29, p < 0.001) and Injury Severity Score (ISS) (r = 0.34, p < 0.001) and inversely with the Pediatric Trauma Score (PTS) (r = -0.20, p = 0.003). The presence of extremity injuries correlated significantly with total cost (r = 0.22, p = 0.001) and PTS (r = -0.25, p < 0.001) but not with the ISS. Logistic regression analysis identified runk injury, ISS and PTS as the main determinants of survival. CONCLUSIONS: The cost of pediatric trauma in Canada can be predicted from admission data and trauma scores. The cost of extremity injuries is significant and can be predicted by the PTS but not the ISS.

Congenital perineal lipoma presenting as "ambiguous genitalia": a case report.

A case is presented in which an unusual, phallic-shaped perineal lipoma raised the question of ambiguous genitalia following the delivery of an otherwise healthy female infant. The management of the case is described, and the critical features of the physical examination that contradicted that diagnosis are highlighted.