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1.
Pediatr Blood Cancer ; : e31026, 2024 Apr 28.
Article in English | MEDLINE | ID: mdl-38679864

ABSTRACT

PURPOSE: Our objectives were to compare overall survival (OS) and pulmonary relapse between patients with metastatic Ewing sarcoma (EWS) at diagnosis who achieve rapid complete response (RCR) and those with residual pulmonary nodules after induction chemotherapy (non-RCR). PATIENTS AND METHODS: This retrospective cohort study included children under 20 years with metastatic EWS treated from 2007 to 2020 at 19 institutions in the Pediatric Surgical Oncology Research Collaborative. Chi-square tests were conducted for differences among groups. Kaplan-Meier curves were generated for OS and pulmonary relapse. RESULTS: Among 148 patients with metastatic EWS at diagnosis, 61 (41.2%) achieved RCR. Five-year OS was 71.2% for patients who achieved RCR, and 50.2% for those without RCR (p = .04), and in multivariable regression among patients with isolated pulmonary metastases, RCR (hazards ratio [HR] 0.42; 95% confidence interval [CI]: 0.17-0.99) and whole lung irradiation (WLI) (HR 0.35; 95% CI: 0.16-0.77) were associated with improved survival. Pulmonary relapse occurred in 57 (37%) patients, including 18 (29%) in the RCR and 36 (41%) in the non-RCR groups (p = .14). Five-year pulmonary relapse rates did not significantly differ based on RCR (33.0%) versus non-RCR (47.0%, p = .13), or WLI (38.8%) versus no WLI (46.0%, p = .32). DISCUSSION: Patients with EWS who had isolated pulmonary metastases at diagnosis had improved OS if they achieved RCR and received WLI, despite having no significant differences in rates of pulmonary relapse.

2.
J Pediatr Surg ; 59(5): 869-873, 2024 May.
Article in English | MEDLINE | ID: mdl-38402129

ABSTRACT

BACKGROUND: To review the outcomes of premature patients with type C esophageal atresia (EA). METHODS: In this retrospective cohort study, charts of patients of type C EA patients were reviewed from 1992 to 2022. Outcomes of premature patients were compared to term patients. Preterm patients were analyzed to compare outcomes of infants with very low birth weights (VLBW) to patients >1,500 g as well as primary versus delayed anastomosis. RESULTS: Among 192 type C EA, 67 were premature. Median and interquartile range (IQR) gestational age and birth weight of preterm patients were 34 [33-36] weeks and 1965 [1740-2290] g. Delayed anastomosis was performed in 12 (18%) preterm vs. 3 (2%) term patients (p = 0.0003). Short-term postoperative outcomes were similar between preterm and term patients, except for recurrent fistula (16% vs. 6%, p = 0.01). Prematurity was associated with an increased need for long-term enteral tube feeding (56% vs. 10%, p = 0.0001) and parenteral nutrition (10 days vs. 0 days, p = 0.0004). The length of stay was 3 times longer when patients were premature (50 days vs. 17 days, p = 0.002). Delayed surgery in preterm patients was associated with post-operative leaks, strictures, recurrent fistula, prolonged enteral tube feeding, and gastrostomy insertion. Patients with very low birth weight (VLBW) were compared to other preterm patients and showed no difference in terms of rate of delayed surgery, and post-operative outcomes. CONCLUSION: Despite increased prematurity-related comorbidities and low birth weight, premature infants with type C EA/TEF have similar post-operative outcomes to term patients though recurrent fistula was more frequent with prematurity. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: III.


Subject(s)
Esophageal Atresia , Infant, Newborn, Diseases , Tracheoesophageal Fistula , Infant, Newborn , Infant , Humans , Esophageal Atresia/surgery , Esophageal Atresia/complications , Tracheoesophageal Fistula/surgery , Retrospective Studies , Treatment Outcome , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Infant, Premature , Infant, Very Low Birth Weight
3.
Ann Surg ; 279(3): 528-535, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-37264925

ABSTRACT

OBJECTIVE: The purpose of this study was to describe management and outcomes from a contemporary cohort of children with Wilms tumor complicated by inferior vena caval thrombus. BACKGROUND: The largest series of these patients was published almost 2 decades ago. Since then, neoadjuvant chemotherapy has been commonly used to manage these patients, and outcomes have not been reported. METHODS: Retrospective review of 19 North American centers between 2009 and 2019. Patient and disease characteristics, management, and outcomes were investigated and analyzed. RESULTS: Of 124 patients, 81% had favorable histology (FH), and 52% were stage IV. IVC thrombus level was infrahepatic in 53 (43%), intrahepatic in 32 (26%), suprahepatic in 14 (11%), and cardiac in 24 (19%). Neoadjuvant chemotherapy using a 3-drug regimen was administered in 82% and postresection radiation in 90%. Thrombus level regression was 45% overall, with suprahepatic level showing the best response (62%). Cardiopulmonary bypass (CPB) was potentially avoided in 67%. The perioperative complication rate was significantly lower after neoadjuvant chemotherapy [(25%) vs upfront surgery (55%); P =0.005]. CPB was not associated with higher complications [CPB (50%) vs no CPB (27%); P =0.08]. Two-year event-free survival was 93% and overall survival was 96%, higher in FH cases (FH 98% vs unfavorable histology/anaplastic 82%; P =0.73). Neither incomplete resection nor viable thrombus cells affected event-free survival or overall survival. CONCLUSIONS: Multimodal therapy resulted in excellent outcomes, even with advanced-stage disease and cardiac extension. Neoadjuvant chemotherapy decreased the need for CPB to facilitate resection. Complete thrombectomy may not always be necessary.


Subject(s)
Kidney Neoplasms , Surgical Oncology , Venous Thrombosis , Wilms Tumor , Humans , Child , Kidney Neoplasms/surgery , Vena Cava, Inferior/surgery , Wilms Tumor/surgery , Wilms Tumor/drug therapy , Venous Thrombosis/pathology , Thrombectomy/methods , Retrospective Studies , Nephrectomy/methods
4.
Ann Surg ; 279(3): 536-541, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-37487006

ABSTRACT

OBJECTIVE: To determine the impact of nodal basin ultrasound (US) surveillance versus completion lymph node dissection (CLND) in children and adolescents with sentinel lymph node (SLN) positive melanoma. BACKGROUND: Treatment for children and adolescents with melanoma are extrapolated from adult trials. However, there is increasing evidence that important clinical and biological differences exist between pediatric and adult melanoma. METHODS: Patients ≤18 years diagnosed with cutaneous melanoma between 2010 and 2020 from 14 pediatric hospitals were included. Data extracted included demographics, histopathology, nodal basin strategies, surveillance intervals, and survival information. RESULTS: Of 252 patients, 90.1% (n=227) underwent SLN biopsy (SLNB), 50.9% (n=115) had at least 1 positive node. A total of 67 patients underwent CLND with 97.0% (n=65/67) performed after a positive SLNB. In contrast, 46 total patients underwent US observation of nodal basins with 78.3% (n=36/46) of these occurring after positive SLNB. Younger patients were more likely to undergo US surveillance (median age 8.5 y) than CLND (median age 11.3 y; P =0.0103). Overall, 8.9% (n=21/235) experienced disease recurrence: 6 primary, 6 nodal, and 9 distant. There was no difference in recurrence (11.1% vs 18.8%; P =0.28) or death from disease (2.2% vs 9.7%; P =0.36) for those who underwent US versus CLND, respectively. CONCLUSIONS: Children and adolescents with cutaneous melanoma frequently have nodal metastases identified by SLN. Recurrence was more common among patients with thicker primary lesions and positive SLN. No significant differences in oncologic outcomes were observed with US surveillance and CLND following the identification of a positive SLN.


Subject(s)
Melanoma , Sentinel Lymph Node , Skin Neoplasms , Adult , Humans , Adolescent , Child , Melanoma/diagnostic imaging , Melanoma/surgery , Melanoma/pathology , Skin Neoplasms/diagnostic imaging , Skin Neoplasms/surgery , Sentinel Lymph Node/pathology , Neoplasm Recurrence, Local/pathology , Lymph Node Excision , Sentinel Lymph Node Biopsy , Retrospective Studies
5.
6.
Pediatr Transplant ; 28(1): e14674, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38054589

ABSTRACT

INTRODUCTION: In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction. CASE PRESENTATION: We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank. The living donor underwent a laparoscopic-assisted left lateral hepatectomy. The reconstruction of the vena cava took place on the back table and the liver was implanted en bloc with the reconstructed IVC in the recipient. Anticoagulation was immediately restarted after the surgery because of her pro-thrombotic state. Her postoperative course was complicated by a biliary anastomotic leak and an infected biloma. The patient recovered progressively and remained well on outpatient clinic follow-up 32 weeks after the procedure. CONCLUSION: IVC reconstruction using a cryopreserved pulmonary vein graft is a valid option during LDLT for pediatric patients with BCS where reconstruction of the IVC entails considerable challenges. Early referral to a pediatric liver transplant facility with a multidisciplinary team is also important in the management of pediatric patients with BCS.


Subject(s)
Budd-Chiari Syndrome , Liver Transplantation , Pulmonary Veins , Female , Humans , Child , Budd-Chiari Syndrome/complications , Budd-Chiari Syndrome/surgery , Liver Transplantation/methods , Hepatic Veins/surgery , Living Donors , Vena Cava, Inferior/surgery
7.
J Pediatr Surg ; 58(1): 146-152, 2023 Jan.
Article in English | MEDLINE | ID: mdl-37306366

ABSTRACT

PURPOSE: The optimal approach for pediatric inguinal hernia repair continues to be debated. We conducted a regional retrospective study to assess rates of recurrence and metachronous hernias after open repair (OPEN) and laparoscopic repair (LAP) METHODS: A retrospective cohort study was conducted at two children's hospitals that serve a region of approximately 4 million people. All patients < 14 years old undergoing OPEN or LAP by pediatric surgeons during a 5-year period (2011 - 2015) were analyzed after a minimum follow up of 4 years. Cox proportional regression was used to compare the effect of surgical approach on hernia recurrence and metachronous contralateral hernias. RESULTS: A total of 1,952 patients, 587 female (30%) and 1365 male (70%), had 2305 hernias repaired. Median post operative follow up time was 6.6 years (range 4-9 years). OPEN and LAP were performed for 1827 (79%) and 478 (21%) hernias, respectively. There were no significant differences in rate of prematurity, age at repair, or frequency of emergent repair. LAP was associated with a lower incidence of metachronous contralateral hernias compared to OPEN (1.4% vs 3.8%, p = 0.047), and a higher incidence of recurrence (9% vs 0.9%, p < 0.001). After adjusting for confounders, LAP had a higher rate of recurrence than OPEN (hazard ratio 10.4, 95% CI 6-18.1).The recurrence rate did not decrease over the study period (p = 0.731). CONCLUSION: Laparoscopic inguinal hernia repair in children resulted in a modest decrease in the incidence of metachronous hernias, at the cost of a significant increase in recurrence. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: Level III.


Subject(s)
Hernia, Inguinal , Laparoscopy , Humans , Child , Female , Male , Adolescent , Hernia, Inguinal/surgery , Retrospective Studies , Hospitals, Pediatric , Postoperative Period
9.
J Pediatr Pharmacol Ther ; 28(1): 20-28, 2023.
Article in English | MEDLINE | ID: mdl-36777982

ABSTRACT

Acetaminophen is one of the oldest medications commonly administered in children. Its efficacy in treating fever and pain is well accepted among clinicians. However, the available evidence supporting the use of acetaminophen's different modes of administration remains relatively scarce and poorly known. This short report summarizes the available evidence and provides a framework to guide clinicians regarding a rational use of acetaminophen in children.

11.
J Pediatr Surg ; 58(1): 82-88, 2023 Jan.
Article in English | MEDLINE | ID: mdl-36357227

ABSTRACT

BACKGROUND: The aim of this study was to establish the feasibility and safety of the use of indocyanine green technology during pediatric intestinal resections. While indocyanine green fluorescence angiography (ICG-FA) has been advocated as an imaging technique to assess bowel perfusion in adults, few studies have evaluated this technology in a pediatric context. METHODS: A prospective clinical trial was conducted. Patients 16 years old or younger undergoing a surgery potentially requiring an intestinal resection were eligible. Patients received a standardized intravenous injection of indocyanine green and intestinal perfusion was evaluated. The study endpoints included safety, impact on bowel resection and feasibility and acceptance of ICG-FA in this population. RESULTS: From May 2020 to March 2021, 30 consecutive patients were included in this trial. Final analysis was done on 28 patients with a median age of 15.00 [6.36,85.00] weeks and weight of 5.58 [3.64,11.70] kg at surgery. Adequate fluorescence was achieved in less than one minute for all cases with an average dose of 0.14 mg/kg. No adverse event related to indocyanine green occurred. ICG-FA versus standard assessment of potential resection sites differed in 62% (95% IC 0.41-0.82) of our cases. Qualitative analysis demonstrated that 95% of the surgical team agreed that ICG-FA was safe. CONCLUSIONS: The use of ICG-FA is feasible and safe for pediatric intestinal resections. Introduction of ICG-FA was simple and acceptance rates were high within the surgical team. This fluorescence imaging may be a valuable imaging technology for intestinal resections in pediatric surgery.


Subject(s)
Digestive System Surgical Procedures , Fluorescein Angiography , Indocyanine Green , Adolescent , Child , Humans , Infant , Digestive System Surgical Procedures/methods , Fluorescein Angiography/adverse effects , Fluorescein Angiography/methods , Prospective Studies , Child, Preschool
12.
Ann Surg ; 277(5): e1130-e1137, 2023 05 01.
Article in English | MEDLINE | ID: mdl-35166261

ABSTRACT

OBJECTIVE: We conducted a multicenter study to assess treatments and outcomes in a national cohort of infants with congenital ovarian cysts. SUMMARY BACKGROUND DATA: Wide variability exists in the treatment of congenital ovarian cysts. The effects of various treatment strategies on outcomes, specifically ovarian preservation, are not known. METHODS: Female infants diagnosed with congenital intra-abdominal cysts between 2013 and 2017 at 10 Canadian pediatric surgical centers were retrospectively evaluated. Sonographic characteristics, median time to cyst resolution, incidence of ovarian preservation, and predictors of surgery were evaluated. Subgroup analyses were performed in patients with complex cysts and cysts ≥40 mm in diameter. RESULTS: The study population included 189 neonates. Median gestational age at diagnosis and median maximal prenatal cyst diameter were 33 weeks and 40 mm, respectively. Cysts resolved spontaneously in 117 patients (62%), 14 (7%) prenatally, and the remainder at a median age of 124 days. Intervention occurred in 61 patients (32%), including prenatal aspiration (2, 3%), ovary sparing resection (14, 23%), or oophorectomy (45, 74%). Surgery occurred at a median age of 7.4weeks. Independent predictors of surgery included postnatal cyst diameter ≥40 mm [odds ratio (OR) 6.19, 95% confidence interval (CI) 1.66-35.9] and sonographic complex cyst character (OR 63.6, 95% CI 10.9-1232). There was no significant difference in the odds of ovarian preservation (OR 3.06, 95% CI 0.86 -13.2) between patients who underwent early surgery (n = 22) and those initially observed for at least 3 months (n = 131). CONCLUSIONS: Most congenital ovarian cysts are asymptomatic and spontaneously resolve. Early surgical intervention does not increase ovarian preservation.


Subject(s)
Cysts , Fetal Diseases , Ovarian Cysts , Female , Humans , Infant , Infant, Newborn , Pregnancy , Canada , Fetal Diseases/diagnosis , Fetal Diseases/surgery , Ovarian Cysts/diagnostic imaging , Ovarian Cysts/surgery , Retrospective Studies , Treatment Outcome , Ultrasonography, Prenatal
14.
Int J Cancer ; 151(10): 1696-1702, 2022 11 15.
Article in English | MEDLINE | ID: mdl-35748343

ABSTRACT

Wilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi-institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P < .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event-free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage-specific treatment regimens.


Subject(s)
Kidney Neoplasms , Pleural Effusion, Malignant , Pleural Effusion , Surgical Oncology , Wilms Tumor , Child , Humans , Incidence , Kidney Neoplasms/epidemiology , Kidney Neoplasms/surgery , Pleural Effusion/epidemiology , Pleural Effusion/etiology , Pleural Effusion, Malignant/epidemiology , Pleural Effusion, Malignant/etiology , Pleural Effusion, Malignant/surgery , Retrospective Studies , Wilms Tumor/epidemiology , Wilms Tumor/surgery
15.
Int J Cancer ; 151(7): 1059-1067, 2022 10 01.
Article in English | MEDLINE | ID: mdl-35604778

ABSTRACT

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy. We describe the largest cohort of IMT patients to date, aiming to further characterize this rare, poorly understood tumor. This is a multi-institutional review of IMT patients ≤39 years, from 2000 to 2018, at 18 hospitals in the Pediatric Surgical Oncology Research Collaborative. One hundred and eighty-two patients were identified with median age of 11 years. Thirty-three percent of tumors were thoracic in origin. Presenting signs/symptoms included pain (29%), respiratory symptoms (25%) and constitutional symptoms (20%). Median tumor size was 3.9 cm. Anaplastic lymphoma kinase (ALK) overexpression was identified in 53% of patients. Seven percent of patients had distant disease at diagnosis. Ninety-one percent of patients underwent resection: 14% received neoadjuvant treatment and 22% adjuvant treatment. Twelve percent of patients received an ALK inhibitor. Sixty-six percent of surgical patients had complete resection, with 20% positive microscopic margins and 14% gross residual disease. Approximately 40% had en bloc resection of involved organs. Median follow-up time was 36 months. Overall 5-year survival was 95% and 5-year event-free survival was 80%. Predictors of recurrence included respiratory symptoms, tumor size and distant disease. Gross or microscopic margins were not associated with recurrence, suggesting that aggressive attempts at resection may not be warranted.


Subject(s)
Surgical Oncology , Child , Humans , Neoplasm Recurrence, Local , Protein Kinase Inhibitors , Receptor Protein-Tyrosine Kinases
16.
Pediatr Surg Int ; 38(7): 1041-1045, 2022 Jul.
Article in English | MEDLINE | ID: mdl-35429262

ABSTRACT

INTRODUCTION: Pediatric bowel obstruction after intra-abdominal cancer surgery is relatively frequent. Few publications have specifically addressed this significant complication. The purpose of this study was to assess the frequency, etiology and treatment options of bowel obstructions following abdominal cancer surgery in children using our institutional database. MATERIALS AND METHODS: We retrospectively analyzed a single tertiary pediatric hospital database over a 10-year period. The clinical characteristics of patients with and without bowel obstruction were compared using bivariate analyses. The details of the conservative and operative management of bowel obstructions were evaluated. RESULTS: Out of 130 eligible patients, 18 (13.8%) developed bowel obstruction in a mean follow-up of 5.7 years. Patients who developed bowel obstruction were more likely to have received preoperative radiation therapy (16.7 vs 2.7%, p = 0.036) and had longer operative time (398 vs 268 min, p = 0.022). Non-operative management was successful in 39% of patients (7/18). When patients needed surgical intervention, minimally invasive approach was attempted and successfully performed in 36% of cases (4/11), none of which required conversion to laparotomy nor presented with recurrent bowel obstruction. CONCLUSION: Bowel obstruction is a frequent complication after abdominal cancer surgery in children. Conservative management is frequently successful. For patients requiring surgical treatment, laparoscopy remains a valuable option and should be considered in selected cases.


Subject(s)
Abdominal Neoplasms , Intestinal Obstruction , Laparoscopy , Abdominal Neoplasms/surgery , Child , Humans , Intestinal Obstruction/etiology , Intestinal Obstruction/surgery , Laparoscopy/adverse effects , Laparotomy , Postoperative Complications/surgery , Postoperative Complications/therapy , Retrospective Studies , Treatment Outcome
17.
J Pediatr Surg ; 57(5): 877-882, 2022 May.
Article in English | MEDLINE | ID: mdl-35090716

ABSTRACT

PURPOSE: The origin of congenital abdominal cysts in the female fetus often dictates management. While most arise from the ovary and are often managed non-operatively, some are non-ovarian and are frequently removed. We analyzed a national sample of female infants with congenital abdominal cysts to elucidate prenatal and postnatal factors associated with the diagnosis of a non-ovarian cyst. METHODS: A retrospective cohort study of female infants who were prenatally diagnosed with abdominal cysts between 2013 and 2017 at 10 Canadian pediatric surgical centres was performed. Clinical characteristics, pre- and postnatal sonographic findings, and cyst trajectories were compared between patients with proven ovarian etiology and those with cysts arising from other organs. RESULTS: Of 185 infants with prenatally diagnosed abdominal cysts, 22 (12%) were non-ovarian, five of which had clear non-ovarian organ of origin on prenatal ultrasound. Comparison of the other 17 cysts with 163 congenital ovarian cysts showed the following factors to be associated with a non-ovarian origin: earlier gestational age at diagnosis (23.5 vs 33.5 weeks, p <0.001), smaller diameter on first prenatal ultrasound (15.8 vs. 39.7 mm, p <0.001), change in sonographic character from simple to complex (87% vs 22%, p <0.001), and postnatal sonographic characteristics of complex cyst (87% vs. 48%, p = 0.004). CONCLUSION: Clear organ of origin, diagnosis earlier in gestation, smaller initial prenatal cyst diameter, and sonographic cyst character change differentiate congenital non-ovarian cysts from their ovarian counterparts. These characteristics may be used to guide diagnosis and management.


Subject(s)
Cysts , Fetal Diseases , Neuroblastoma , Ovarian Cysts , Canada , Child , Cysts/diagnostic imaging , Cysts/surgery , Female , Fetal Diseases/diagnosis , Humans , Infant , Ovarian Cysts/diagnostic imaging , Ovarian Cysts/surgery , Pregnancy , Retrospective Studies , Ultrasonography, Prenatal
18.
J Pediatr Surg ; 57(5): 788-797, 2022 May.
Article in English | MEDLINE | ID: mdl-35063255

ABSTRACT

BACKGROUND: Effective shared decision-making in pediatric surgery requires clarity regarding which surgical outcomes are most important to patients and their families, and how they prefer to receive the information. Despite how essential this is for effective risk communication, little is known about the communication needs and preferences of patients and their families in elective pediatric surgery. METHODS: We administered a mailed and online cross-sectional survey in English and French to 548 families before or after surgery for hernia/hydrocele repair or tonsillectomy/adenoidectomy between July 2019 and February 2021. The survey consisted of 22 questions eliciting most valued patient-reported outcomes (PROs) across 4 domains: health-related quality of life (5), functional status (5), symptoms and symptom burden (5), health behaviours and patient experience (7), as well as overall impressions (3), surgical risks (5), communication preferences (4), and demographic questions (16). RESULTS: The survey was completed by 368 patient families (60 preoperative, 308 postoperative, response rate 67.2%). Most respondents (72%) indicated a significant desire to be informed on all listed PROs alongside surgical complications, and highly valued all functional and quality of life outcomes (92.9% & 89.8%, respectively). Preoperatively, patient families preferred to receive information in the form of pamphlets and websites, whereas postoperatively they preferred direct communication. CONCLUSION: Families value functional and quality of life PROs as much as clinical outcomes, and increasingly seek more contemporary (electronic) means of risk communication than we currently offer. This data will inform the development of mobile tools for personalized communication in pediatric surgery.


Subject(s)
Outpatients , Quality of Life , Child , Communication , Cross-Sectional Studies , Humans , Male , Surveys and Questionnaires
19.
Ann Surg ; 276(6): e969-e975, 2022 12 01.
Article in English | MEDLINE | ID: mdl-33156070

ABSTRACT

OBJECTIVE: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall. SUMMARY OF BACKGROUND DATA: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. METHODS: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. RESULTS: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status ( P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor(HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs resected (HR 1.78; P = 0.02). Overall, patient-reported quality of life is not impaired after chest wall tumor resection. CONCLUSIONS: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.


Subject(s)
Sarcoma , Scoliosis , Surgical Oncology , Thoracic Neoplasms , Thoracic Wall , Child , Humans , Adolescent , Thoracic Wall/surgery , Thoracic Wall/pathology , Quality of Life , Retrospective Studies , Thoracic Neoplasms/surgery , Thoracic Neoplasms/pathology , Sarcoma/surgery , Sarcoma/pathology
20.
Br J Surg ; 109(1): 129-135, 2021 12 17.
Article in English | MEDLINE | ID: mdl-34669930

ABSTRACT

BACKGROUND: Risk factors for paediatric inguinal hernia are poorly understood. This longitudinal cohort study assessed whether children with a maternal history of inguinal hernia or connective tissue disorders have a higher risk of developing inguinal hernias before 13 years of age. METHODS: The study included children followed up between birth and 13 years of age in Quebec, Canada, 2006-2019. Newborns whose mothers had inguinal hernias or connective tissue disorders were followed over time to identify future hospital admissions for inguinal hernia. Cox proportional hazards regression adjusted for patient characteristics was used to estimate hazard ratios (HRs) and 95 per cent confidence intervals for the association between maternal hernia or connective tissue disorders and future childhood hernias. Associations in girls and boys were examined separately. RESULTS: The study included 786 322 children with 6 186 448 person-years of follow-up. There were 6861 children with inguinal hernias, corresponding to an incidence of 11.1 per 10 000 person-years. Children with a maternal history of inguinal hernia had 2.92 (95 per cent c.i. 2.39 to 3.58) times the risk of having inguinal hernias relative to children whose mothers had no such history. Children with a maternal history of connective tissue disorders had 1.30 (1.00 to 1.68) times the risk. Maternal hernias were strongly associated with risk of inguinal hernias in girls (HR 5.34, 3.82 to 7.47), whereas maternal connective tissue disorders were associated with inguinal hernias in boys (HR 1.35, 1.02 to 1.79). CONCLUSION: Paediatric inguinal hernias may be associated with maternal inguinal hernias and connective tissue disorders, but the underlying reason for this relationship requires further investigation.


In this study of 786 322 children in Canada from 2006 to 2019, it was found that children whose mothers had an inguinal hernia or connective tissue disorder were more at risk of developing a hernia themselves. Repairs for inguinal hernia are among the most common operations performed in children. The results showed that girls whose mothers had an inguinal hernia had more than five times the risk of developing an inguinal hernia before 13 years of age. Boys whose mothers had connective tissue disorders had a 35 per cent greater risk of inguinal hernia. These findings suggest that inguinal hernias may be inherited.


Subject(s)
Hernia, Inguinal/etiology , Mothers/statistics & numerical data , Adolescent , Adult , Age Factors , Child , Child, Preschool , Connective Tissue Diseases/complications , Female , Humans , Infant , Infant, Newborn , Longitudinal Studies , Male , Proportional Hazards Models , Risk Factors , Sex Factors , Young Adult
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