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1.
Pediatr Blood Cancer ; 71(6): e30975, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38556718

ABSTRACT

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative. METHODS: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively. RESULTS: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]. CONCLUSION: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

2.
J Surg Res ; 280: 396-403, 2022 12.
Article in English | MEDLINE | ID: mdl-36037617

ABSTRACT

INTRODUCTION: Inferior vena cava (IVC) thrombus is an uncommon and challenging complication of abdominal malignancies in the pediatric population, which significantly influences the treatment options and clinical outcomes in this population. METHODS: In this review, we present the presentation, treatments, interventions, and outcomes with this clinically and technically challenging oncological finding from a free-standing children's hospital from 2006 to 2017. RESULTS: Fourteen patients with IVC thrombus were identified as having an associated abdominal malignancy. The abdominal malignancies consisted of eight Wilms tumors (63% stage III and 37% stage IV), and one spindle cell sarcoma, neuroblastoma (stage III), kidney clear cell sarcoma (stage III), sclerosing epithelioid fibrosarcoma, hepatoblastoma-epithelial (stage IV), and hepatic embryonal sarcoma (stage IV). 50% of patients were male, 71% White, 29% Black, 7% Hispanic; mean age at diagnosis was 4.09 (SD 2.43) years. CT imaging identified IVC tumor thrombus for 79% of patients, US abdomen complete recorded 14%, and MRI lumbar 7%. 3Out of 14 patients, 13 patients were taken to the operating room with 12 patients undergoing concurrent tumor resection and IVC thrombectomy. Of the remaining patients, one had IVC thrombectomy via femoral cutdown by interventional radiology, and one was noted to have resolution of IVC thrombus with neoadjuvant chemotherapy. Of patients who underwent resection, one required IVC ligation, and one patient required IVC interposition vein graft reconstruction using a right IJ conduit. 60% of patients undergoing thrombectomy received neoadjuvant chemotherapy. Mean time from the diagnosis of IVC tumor thrombus to surgical thrombectomy was 46 (SD 44) days. No operative mortalities were reported. There were five major complications (hemothorax, pulmonary embolisms, seroma, and sepsis) and two minor complications (pneumonia and UTI). With exclusion of patient who underwent IVC ligation, no patients developed signs of IVC compression or recurrent thrombosis after thrombectomy. CONCLUSIONS: IVC tumor thrombus can significantly alter the clinical treatment, surgical options, and outcomes of malignant abdominal tumors. Treatment of IVC tumor thrombus included adjuvant chemotherapy, segmental IVC resection with or without reconstruction, thrombectomy with intimal stripping, or resection of the thrombus with part of the IVC wall. Evidence for standard treatment practices for IVC tumor thrombus in the setting of abdominal malignancy is lacking due to the rarity of this finding and the varied clinical presentations.


Subject(s)
Abdominal Neoplasms , Kidney Neoplasms , Thrombosis , Venous Thrombosis , Humans , Child , Male , Child, Preschool , Female , Vena Cava, Inferior/surgery , Kidney Neoplasms/diagnosis , Kidney Neoplasms/surgery , Venous Thrombosis/etiology , Thrombosis/etiology , Abdominal Neoplasms/complications , Abdominal Neoplasms/diagnosis , Abdominal Neoplasms/surgery , Abdomen/pathology , Retrospective Studies , Nephrectomy/methods
3.
Surgery ; 172(4): 1251-1256, 2022 10.
Article in English | MEDLINE | ID: mdl-35933175

ABSTRACT

BACKGROUND: Ewing sarcoma, a malignancy originating from the bone or soft tissues most commonly diagnosed in adolescents, requires multimodality therapy. Although both surgical resection and radiation therapy are effective local control modalities, there are limited data comparing outcomes in patients treated with surgery versus radiation. We sought to determine whether there were differences in 5-year local failure-free survival, event-free survival, and overall survival based on the modality used for local control. METHODS: Patients treated for Ewing sarcoma at a single tertiary pediatric hospital between 2010 and 2020 were included for retrospective analysis. Patient and tumor demographics, treatment information, and patient response to therapies were collected from the medical record. Outcome measures were local failure-free survival, event-free survival, and overall survival at 5 years from diagnosis. RESULTS: Sixty-one patients met inclusion criteria. All patients received chemotherapy, and 68.9% of patients presented with localized disease. Of these, 23.8% were treated with radiation alone; the remaining 76.2% underwent resection ± radiation. A total of 52.4% of patients with localized disease achieved R0 resection. Only 3 patients experienced local progression; there was no difference between treatment groups. There was no significant association between local control modality and event-free survival or overall survival in patients with localized disease, regardless of margin status. CONCLUSION: There was no significant difference in 5-year local failure-free survival, event-free survival, or overall survival in Ewing sarcoma patients treated with radiation versus surgery ± radiation, regardless of whether or not R0 resection was achieved. Future directions include a multi-institutional study to allow for further subgroup analysis and increased sample size.


Subject(s)
Bone Neoplasms , Sarcoma, Ewing , Sarcoma , Adolescent , Bone Neoplasms/therapy , Child , Combined Modality Therapy , Humans , Retrospective Studies , Sarcoma, Ewing/therapy
4.
Pediatr Surg Int ; 38(10): 1427-1434, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35867126

ABSTRACT

PURPOSE: Resection of pediatric posterior thoracic tumors (PTTs) can be complicated by Artery of Adamkiewicz (AKA) injury. Post-op spinal ischemia occurs in approx. 3.2% of patients, typically due to iatrogenic vascular injury. Pre-op angiography (PSA) may help to avoid this complication. Herein, we aim to evaluate outcomes after initiation of routine PSA prior to PTT resection. METHODS: A single-institution retrospective review identified 25 children (< 18 years) treated for PTTs from 2009 to 2021. PTTs included: posterior mediastinum, paraspinal thorax and posterior chest wall tumors. PSA patients were compared to those without pre-operative angiography (NA). Demographics, perioperative and long-term outcomes and event-free survival (EFS) were assessed. RESULTS: Prior to 2012, eleven patients were treated without PSA. However, the last developed post-operative paraplegia secondary to spinal ischemia. Since this event, PSA has become routine for all PTTs (n = 14) identifying six AKAs and nine accessory spinal arteries. Resection was performed in ten (90.1%) NA patients and eight (57.1%) PSA patients. Based on PSA findings, resection was not offered to six patients and planned partial resection was performed in three patients. Five PSA patients required radiation therapy for local control vs two NA patients. There were no differences in recurrence or overall EFS. CONCLUSION: PSA aids in identifying patients with high-risk thoracic vascular anatomy and may prevent risk of post-operative paraplegia associated with PTT resection.


Subject(s)
Angiography , Thoracic Neoplasms , Child , Humans , Ischemia , Paraplegia/etiology , Paraplegia/prevention & control , Retrospective Studies , Thoracic Neoplasms/diagnostic imaging , Thoracic Neoplasms/surgery
5.
Cancer ; 128(14): 2786-2795, 2022 07 15.
Article in English | MEDLINE | ID: mdl-35561331

ABSTRACT

BACKGROUND: Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis. METHODS: A multi-institutional review of children with hepatocellular neoplasms was performed, including demographic, staging, treatment, and outcomes data. Patients were categorized as having conventional HCC (cHCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), and hepatoblastoma with HCC features (HB-HCC). Univariate and multivariate analyses identified predictors of mortality and relapse. RESULTS: In total, 262 children were identified; and an institutional histologic review revealed 110 cHCCs (42%; 69 normal background liver, 34 inflammatory/cirrhotic, 7 unknown), 119 FLCs (45%), and 33 HB-HCCs (12%). The authors observed notable differences in presentation and behavior among tumor subtypes, including increased lymph node involvement in FLC and higher stage in cHCC. Factors associated with mortality included cHCC (hazard ratio [HR], 1.63; P = .038), elevated α-fetoprotein (HR, 3.1; P = .014), multifocality (HR, 2.4; P < .001), and PRETEXT (pretreatment extent of disease) stage IV (HR, 5.76; P < .001). Multivariate analysis identified increased mortality in cHCC versus FLC (HR, 2.2; P = .004) and in unresectable tumors (HR, 3.4; P < .001). Disease-free status at any point predicted survival. CONCLUSIONS: This multi-institutional, detailed data set allowed a comprehensive analysis of outcomes for children with these rare hepatocellular neoplasms. The current data demonstrated that pediatric HCC subtypes are not equivalent entities because FLC and cHCC have distinct anatomic patterns and outcomes in concert with their known molecular differences. This data set will be further used to elucidate the impact of histology on specific treatment responses, with the goal of designing risk-stratified algorithms for children with HCC. LAY SUMMARY: This is the largest reported granular data set on children with hepatocellular carcinoma. The study evaluates different subtypes of hepatocellular carcinoma and identifies key differences between subtypes. This information is pivotal in improving understanding of these rare cancers and may be used to improve clinical management and subsequent outcome in children with these rare malignancies.


Subject(s)
Carcinoma, Hepatocellular , Liver Neoplasms , Surgical Oncology , Carcinoma, Hepatocellular/pathology , Child , Humans , Liver Neoplasms/pathology , Neoplasm Recurrence, Local/pathology , Neoplasm Staging , Retrospective Studies
6.
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
7.
A A Pract ; 15(11): e01542, 2021 Nov 02.
Article in English | MEDLINE | ID: mdl-34735416

ABSTRACT

Nociception is the detection of noxious stimulation by the nervous system. The PMD-200 monitor is a validated, emerging technology for intraoperative monitoring using the nociception level (NOL) index. We describe a pediatric case of an open resection of paraganglionic masses during which episodic increases in NOL index and blood pressure coincided with tumor manipulation, presumably due to a catecholamine surge. Since the patient was under stable and adequate analgesia, the increases in NOL index likely reflected the physiologic effects of tumor handling rather that the presence of a true noxious stimulus. Clinicians should consider this limitation when using this monitor.


Subject(s)
Nociception , Paraganglioma , Child , Humans , Monitoring, Intraoperative , Pain Measurement , Paraganglioma/surgery , Remifentanil
8.
Pediatrics ; 147(2)2021 02.
Article in English | MEDLINE | ID: mdl-33504609

ABSTRACT

BACKGROUND: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration. METHODS: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period. RESULTS: Sixty-six children, with a median age of 11 years (range 1-17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; P = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% (n = 11) and count recovery in 35% (n = 6). Cancer therapy was delayed in 35% (n = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, P = .05) and longer LOS (29 vs 12 days; P = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications (P < .01). CONCLUSIONS: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.


Subject(s)
Appendectomy/trends , Appendicitis/therapy , Chemotherapy-Induced Febrile Neutropenia/therapy , Neoplasms/therapy , Watchful Waiting/trends , Adolescent , Appendicitis/diagnosis , Appendicitis/epidemiology , Chemotherapy-Induced Febrile Neutropenia/diagnosis , Chemotherapy-Induced Febrile Neutropenia/epidemiology , Child , Child, Preschool , Female , Humans , Infant , Male , Neoplasms/diagnosis , Neoplasms/epidemiology , Retrospective Studies , Watchful Waiting/methods
9.
Pediatr Blood Cancer ; 67(9): e28425, 2020 09.
Article in English | MEDLINE | ID: mdl-32658372

ABSTRACT

BACKGROUND: To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD). METHODS: Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected. RESULTS: Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival. CONCLUSION: This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.


Subject(s)
Exocrine Pancreatic Insufficiency/mortality , Pancreatic Neoplasms/mortality , Pancreatic Neoplasms/surgery , Pancreaticoduodenectomy/adverse effects , Postoperative Complications/mortality , Adolescent , Adult , Child , Child, Preschool , Exocrine Pancreatic Insufficiency/etiology , Female , Humans , Infant , Male
11.
J Pediatr Hematol Oncol ; 41(8): 596-600, 2019 11.
Article in English | MEDLINE | ID: mdl-31135714

ABSTRACT

INTRODUCTION: The diagnosis of pulmonary invasive fungal infection (IFI) in the pediatric oncology patient is challenging. Consensus criteria developed in 2008 state that bronchioalveolar lavage (BAL) results cannot confirm this diagnosis. A video-assisted thoracoscopic biopsy (VATS-biopsy) of lungs has been increasingly used to assist in evaluating these children for IFI. Our goal was to evaluate the impact of BAL and VATS-biopsy results on the management of IFI among pediatric oncology patients. METHODS: A retrospective review of all oncology patients evaluated for IFI with VATS-biopsy and/or BAL over 9 years was carried out at a single free-standing children's hospital. The primary outcome was management changes in the use of antifungal therapy on the basis of diagnostic procedure, fungal culture results, lung imaging, and serological markers. RESULTS: A total of 102 patients underwent 122 diagnostic evaluations for IFI. Ninety-one workups included only BAL, 17 evaluations involved only VATS-biopsy, and 14 cases involved both BAL and VATS-biopsy. The diagnostic yield of VATS-biopsy (38.7%) was superior to that of BAL (27.6%). There was poor concordance between VATS-biopsy and BAL results in the 14 cases where both were performed. Upon workup completion, IFI was proven in 12 children, probable in 29, and possible in 52. The odds of continuing antifungals increased 3-fold for patients with probable IFI and 12.7 times for those with the proven disease. DISCUSSION: On the basis of the inferior diagnostic yield of BAL, we believe that VATS-biopsy may be a more useful diagnostic adjuvant in the diagnosis of IFI in the immunocompromised pediatric oncologic patient population.


Subject(s)
Antifungal Agents/administration & dosage , Invasive Fungal Infections , Lung Diseases, Fungal , Neoplasms , Thoracic Surgery, Video-Assisted , Adolescent , Biopsy , Bronchoalveolar Lavage , Child , Child, Preschool , Female , Humans , Invasive Fungal Infections/diagnosis , Invasive Fungal Infections/drug therapy , Invasive Fungal Infections/pathology , Lung Diseases, Fungal/diagnosis , Lung Diseases, Fungal/drug therapy , Lung Diseases, Fungal/pathology , Male , Neoplasms/diagnosis , Neoplasms/drug therapy , Neoplasms/microbiology
12.
J Pediatr Surg ; 50(6): 992-5, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25837269

ABSTRACT

PURPOSE: Pediatric surgeons routinely use fluoroscopy for central venous line (CVL) placement. We examined radiation safety practices and patient/surgeon exposure during fluoroscopic CVL. METHODS: Fluoroscopic CVL procedures performed by 11 pediatric surgeons in 2012 were reviewed. Fluoroscopic time (FT), patient exposure (mGy), and procedural data were collected. Anthropomorphic phantom simulations were used to calculate scatter and dose (mSv). Surgeons were surveyed regarding safety practices. RESULTS: 386 procedures were reviewed. Median FT was 12.8 seconds. Median patient estimated effective dose was 0.13 mSv. Median annual FT per surgeon was 15.4 minutes. Simulations showed no significant difference (p=0.14) between reported exposures (median 3.5 mGy/minute) and the modeled regression exposures from the C-arm default mode (median 3.4 mGy/minute). Median calculated surgeon exposure was 1.5 mGy/year. Eight of 11 surgeons responded to the survey. Only three reported 100% lead protection and frequent dosimeter use. CONCLUSION: We found nonstandard radiation training, safety practices, and dose monitoring for the 11 surgeons. Based on simulations, the C-arm default setting was typically used instead of low dose. While most CVL procedures have low patient/surgeon doses, every effort should be used to minimize patient and occupational exposure, suggesting the need for formal hands-on training for nonradiologist providers using fluoroscopy.


Subject(s)
Catheterization, Central Venous/methods , Occupational Exposure , Patient Safety/statistics & numerical data , Practice Patterns, Physicians'/statistics & numerical data , Radiation Exposure , Radiography, Interventional/methods , Adolescent , Child , Child, Preschool , Female , Fluoroscopy , Health Care Surveys , Humans , Infant , Infant, Newborn , Male , Occupational Exposure/prevention & control , Occupational Exposure/statistics & numerical data , Radiation Exposure/prevention & control , Radiation Exposure/statistics & numerical data , Retrospective Studies , Surgeons , Young Adult
13.
J Pediatr Surg ; 49(3): 469-73, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24650480

ABSTRACT

PURPOSE: While obesity is associated with increased mortality and decreased functional outcomes in adult burn patients, the ramifications of larger than average body size in the pediatric burn population are less well understood. The present study examines whether obesity was associated with poor outcomes following pediatric burn injuries. METHODS: Thermal injury data for patients ≤ 18 years of age admitted to a Level III burn center over ten years (n=536) was analyzed. Obesity was defined as ≥ 95 th percentile of weight for height according to the WHO growth charts (<2 years of age) or BMI for age according to the CDC growth charts (2-18 years of age). Outcomes were compared between thermally injured obese (n=154) and non-obese (n=382) children. All data was collected in accordance with IRB regulations. RESULTS: Obese and non-obese thermally-injured children did not differ in TBSA, percentage of full thickness burn, or overall mortality. However, these groups were significantly different with respect to age (obese=7.16 ± 0.46 years, non-obese=9.38 ± 0.32 years, p<0.001) and days requiring mechanical ventilation (obese=4.89 ± 1.3 days, non-obese=2.67 ± 0.49 days, p<0.05). For thermally injured children admitted to the BICU without inhalation injury (n=175); the obese (n=46) and non-obese (n=129) did not differ significantly with respect to age, TBSA, percentage of full thickness burn or other outcome measures. However, significant differences between these groups were noted for ICU LOS (obese=18.59 ± 5.18 days, non-obese=9.51 ± 1.82 days, p<0.05) and number of days requiring mechanical ventilation (obese=11.65 ± 3.91 days, non-obese=3.92 ± 0.85 days, p<0.05). CONCLUSION: These data show thermally-injured obese pediatric patients required longer and more intensive medical support in the form of BICU care and respiratory intervention. Counter to findings in adult populations, differences in mortality were not observed. Collectively, these findings suggest obesity as a risk factor for increased morbidity in the pediatric burn population.


Subject(s)
Burns/epidemiology , Obesity/epidemiology , Adolescent , Anti-Infective Agents/therapeutic use , Burn Units/statistics & numerical data , Burns/therapy , Burns, Inhalation/epidemiology , Burns, Inhalation/therapy , Child , Child, Preschool , Comorbidity , Female , Humans , Infant , Infections/drug therapy , Infections/epidemiology , Length of Stay/statistics & numerical data , Male , Respiration, Artificial/statistics & numerical data , Respiratory Insufficiency/epidemiology , Respiratory Insufficiency/etiology , Retrospective Studies , Risk Factors , Smoke Inhalation Injury/epidemiology , Smoke Inhalation Injury/therapy , Texas/epidemiology , Trauma Severity Indices , Treatment Outcome
14.
J Pediatr Hematol Oncol ; 36(8): 641-5, 2014 Nov.
Article in English | MEDLINE | ID: mdl-24065046

ABSTRACT

Juvenile xanthogranulomas (JXGs) are benign cutaneous lesions of childhood that often spontaneously involute. They rarely present as a noncutaneous tumors. However, JXG tumors have been described in numerous noncutaneous anatomic sites, presenting with a variety of symptoms. The severity of symptoms and accurate preoperative diagnosis of JXG should determine operative and nonoperative treatment options of these uncommon, benign, and self-limiting tumors. We report 3 cases of symptomatic, noncutaneous JXG from disparate anatomic sites all treated with aggressive surgical resection.


Subject(s)
Lung Diseases/diagnostic imaging , Pancreatic Diseases/diagnostic imaging , Trigeminal Nerve Diseases/pathology , Xanthogranuloma, Juvenile/diagnostic imaging , Xanthogranuloma, Juvenile/pathology , Adolescent , Child , Female , Humans , Infant , Lung Diseases/surgery , Magnetic Resonance Imaging , Male , Pancreatic Diseases/surgery , Severity of Illness Index , Tomography, X-Ray Computed , Trigeminal Nerve Diseases/surgery , Xanthogranuloma, Juvenile/surgery
15.
J Pediatr Surg ; 48(4): 893-8, 2013 Apr.
Article in English | MEDLINE | ID: mdl-23583154

ABSTRACT

INTRODUCTION: Spontaneous perforation of the bile duct (SPBD) is a rare abnormality of the extrahepatic biliary tree in infants. Limited porta hepatis exploration and drainage of the bile-soiled peritoneum are often sufficient treatment. Here, we describe three cases of SPBD, one of which required complex multi-disciplinary interventions for restoration of biliary continuity. METHODS: Three infants with bilious ascites from spontaneous biliary perforation were reviewed. Metrics included age, presenting symptoms, diagnostic tests, interventions performed, and outcomes. RESULTS: The presenting symptoms of all three infants were bilious ascites, sepsis, lethargy, anorexia, fever and persistent emesis. SPBD was confirmed pre-operatively by HIDA scan (hepatobiliary scintigraphy) in each case. Intra-operative, trans-cholecystic cholangiogram confirmed SBDP in two cases. Two of the infants were successfully treated with placement of a cholecystostomy tube and porta hepatis drains. The third infant, having failed multiple similar drainage procedures, required percutaneous transhepatic drainage of a persistent porta hepatis biloma. Ultimately percutaneous, transhepatic cannulation of the extra-hepatic biliary tree for prolonged stenting was required to successfully treat this biliary perforation. CONCLUSION: Complex spontaneous biliary perforation may require extensive interventions if the perforation fails to resolve with standard porta hepatis drainage. Access of the biliary tree via Interventional Radiology procedures for complex biliary disease of this type is novel and presents an alternative to traditional open surgical treatment and control of spontaneous biliary perforations.


Subject(s)
Bile Duct Diseases/diagnosis , Bile Duct Diseases/surgery , Cholangiography , Diagnosis, Differential , Drainage , Humans , Infant , Rupture, Spontaneous/diagnosis , Rupture, Spontaneous/surgery
16.
J Pediatr Surg ; 46(6): 1126-30, 2011 Jun.
Article in English | MEDLINE | ID: mdl-21683210

ABSTRACT

PURPOSE: Children treated for perforated appendicitis can have significant morbidity. Management often includes looking for and draining postoperative fluid collections. We sought to determine if drainage hastens recovery. METHODS: Children with perforated appendicitis treated with appendectomy from 2006 to 2009 were reviewed. Patients with postoperative fluid that was drained were compared with patients with undrained fluid with regard to preoperative features and postoperative outcomes. Statistical analyses included paired Student's t tests, Mann-Whitney U test, and linear regression. RESULTS: Five hundred ninety-one patients were reviewed. Seventy-one patients had postoperative fluid, of whom 36 had a drainage procedure and 35 did not. There was no significant difference in white blood cell count at the time of assessment for drainage (16.4 ± 4.0 vs 14.6 ± 4.9, P = .14), days with fever (3.5 ± 3.0 vs 2.9 ± 2.5, P = .35), or readmission rate (19% vs 31%, P = .28). After multivariate linear regression, larger fluid volumes were associated with prolonged length of stay (LOS) (P = .03). For fluid collections between 30-100 mL, there was no significant difference in LOS between the drain and no-drain groups (9.8 ± 3.5 vs 10.9 ± 5.2 days, P = .51). CONCLUSION: After appendectomy for perforated appendicitis, larger postoperative fluid collections are associated with prolonged LOS. Drainage of collections less than 100 mL may not hasten recovery.


Subject(s)
Appendectomy/methods , Appendicitis/surgery , Drainage/methods , Length of Stay/trends , Adolescent , Appendectomy/adverse effects , Appendicitis/diagnosis , Child , Child, Preschool , Cohort Studies , Drainage/adverse effects , Female , Follow-Up Studies , Humans , Incidence , Linear Models , Male , Multivariate Analysis , Postoperative Care/methods , Postoperative Complications/epidemiology , Postoperative Complications/therapy , Retrospective Studies , Risk Assessment , Statistics, Nonparametric , Treatment Outcome , Unnecessary Procedures
17.
J Pediatr Surg ; 45(7): 1413-9, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20638517

ABSTRACT

PURPOSE: Children requiring prehospital cardiopulmonary resuscitation (CPR) after traumatic injury have been shown to have poor survival. However, outcome of children still receiving CPR on-arrival by emergency medical service to the emergency department (ED) has not been demonstrated in a published clinical series. METHODS: An 11-year retrospective analysis from a level I pediatric trauma center of the outcomes of children requiring prehospital CPR after traumatic injury was undertaken. Outcome variables were stratified by survival, death, and CPR on-arrival. RESULTS: Of 169 children requiring prehospital CPR, there were 28 survivors and 141 deaths. Of 69 children requiring CPR on-arrival to the ED, there were no survivors. There were 70 females and 99 males. Mean age of survivors was 3.4 years; nonsurvivors, 8.8 years; and 4.6 years for CPR on-arrival. Thirty-nine percent of all injuries were sustained in motor vehicle collisions; 20%, motor pedestrian collisions; 19%, assaults; 7%, falls; 4%, all terrain vehicle/motorcycle/bicycle; and 4%, gunshot wounds. Forty-two percent of all patients expired in the ED, whereas 34% expired in the intensive care unit. Eighty-seven percent of CPR on-arrival patients expired in the ED. Fifty-five percent of survivors had full neurologic recovery. CONCLUSION: Although mortality was extremely high for children requiring CPR in the field After traumatic injury, it was absolute for those arriving at the ED still undergoing CPR.


Subject(s)
Cardiopulmonary Resuscitation , Emergency Medical Services , Wounds and Injuries/mortality , Wounds and Injuries/therapy , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Medical Futility , Retrospective Studies , Survival Rate , Time Factors , Treatment Outcome , United States/epidemiology
18.
J Pediatr Surg ; 45(3): 513-8, 2010 Mar.
Article in English | MEDLINE | ID: mdl-20223313

ABSTRACT

PURPOSE: Pediatric deep pelvic abscesses generally occur as a complication of perforated appendicitis or after laparoscopic appendectomy. We describe our technique and experience in imaging-guided transrectal drainage (TRD) of deep pelvic abscesses in children. METHODS: From January 2005 to November 2008, imaging-guided TRD was attempted in 29 children. The procedure records and medical records were reviewed retrospectively. RESULTS: Twenty-nine TRD procedures were performed in 17 males and 12 female patients. The mean age was 11.8 years (range, 3.8-15.9 years). Fourteen patients required TRD after an abdominal operative procedure: laparoscopic appendectomy (n = 9), open appendectomy (n = 3), exploratory celiotomy for gunshot wound (n = 1), and exploratory celiotomy with intestinal resection for adhesive bowel obstruction (n = 1). Fifteen patients were referred for TRD as initial therapy for deep pelvic fluid collections: perforated appendicitis (n = 14) and Crohn perforation (n = 1). Twenty-seven (93.1%) procedures were successful, described as either placement of a TRD catheter or aspiration of the deep pelvic fluid using transrectal approach. There were no procedure-related complications. CONCLUSIONS: Imaging-guided TRD in children is a safe and easy procedure that allows drainage of deep pelvic abscesses that are otherwise not easily accessible.


Subject(s)
Abscess/diagnostic imaging , Abscess/surgery , Pelvic Infection/diagnostic imaging , Pelvic Infection/surgery , Ultrasonography, Interventional/methods , Abscess/etiology , Adolescent , Appendectomy/adverse effects , Appendicitis/complications , Child , Child, Preschool , Cohort Studies , Digestive System Surgical Procedures/adverse effects , Drainage/methods , Female , Fluoroscopy/methods , Follow-Up Studies , Humans , Male , Probability , Radiography, Interventional , Rectum/diagnostic imaging , Risk Assessment , Severity of Illness Index , Tomography, X-Ray Computed/methods , Treatment Outcome
19.
J Trauma ; 63(5): 1127-31, 2007 Nov.
Article in English | MEDLINE | ID: mdl-17993961

ABSTRACT

BACKGROUND: Animal studies suggest that female gender imparts a protective effect on outcome after trauma, and implicate sex hormones as the cause. Human studies have yielded mixed results. These results are confounded by postmenopausal hormone replacement and the difficulty of controlling for pretrauma comorbidities. The pediatric population is a better model to determine the impact of gender and sex hormones on outcome after trauma. METHODS: The National Trauma Data Bank was queried for all patients from birth to 20 years of age. Age, gender, Injury Severity Score (ISS), mechanism of injury, mortality, intensive care unit days, and ventilator days were examined. To control for the effect of sex hormones, patients were divided into three groups by age: prepubertal (birth to 8 years), peripubertal (8.1-14.5 years), and postpubertal (14.6-20 years). We calculated survival rates for age group ISS subsets overall and by mechanism of injury. RESULTS: The prepubertal and peripubertal age groups had equivalent survival rates between genders across all severities of injury. The sex hormone-containing postpubertal cohort had a significantly improved survival rate for women across all ISS subgroups, and the effect was more pronounced with increasing ISS. This effect was despite a higher mean ISS for women at these greater magnitudes of injury. The cause of this effect could not be explained by mechanism of injury, ventilator days, or intensive care unit days. CONCLUSION: Female gender was associated with improved survival rates for patients demonstrating sex hormone production (i.e. postpubescent patients) in a manner that was directly proportional to their severity of injury. No protective effect of gender was seen in the prepubescent or peripubertal age groups.


Subject(s)
Gonadal Steroid Hormones/metabolism , Wounds and Injuries/metabolism , Wounds and Injuries/mortality , Adolescent , Burns/epidemiology , Burns/metabolism , Causality , Child , Child, Preschool , Cohort Studies , Female , Humans , Infant , Infant, Newborn , Injury Severity Score , Male , Outcome Assessment, Health Care , Sex Factors , Survival Analysis , United States/epidemiology , Wounds, Nonpenetrating/epidemiology , Wounds, Nonpenetrating/metabolism , Wounds, Penetrating/epidemiology , Wounds, Penetrating/metabolism
20.
JAMA ; 295(3): 285-92, 2006 Jan 18.
Article in English | MEDLINE | ID: mdl-16418463

ABSTRACT

CONTEXT: Many men with inguinal hernia have minimal symptoms. Whether deferring surgical repair is a safe and acceptable option has not been assessed. OBJECTIVE: To compare pain and the physical component score (PCS) of the Short Form-36 Version 2 survey at 2 years in men with minimally symptomatic inguinal hernias treated with watchful waiting or surgical repair. DESIGN, SETTING, AND PARTICIPANTS: Randomized trial conducted January 1, 1999, through December 31, 2004, at 5 North American centers and enrolling 720 men (364 watchful waiting, 356 surgical repair) followed up for 2 to 4.5 years. INTERVENTIONS: Watchful-waiting patients were followed up at 6 months and annually and watched for hernia symptoms; repair patients received standard open tension-free repair and were followed up at 3 and 6 months and annually. MAIN OUTCOME MEASURES: Pain and discomfort interfering with usual activities at 2 years and change in PCS from baseline to 2 years. Secondary outcomes were complications, patient-reported pain, functional status, activity levels, and satisfaction with care. RESULTS: Primary intention-to-treat outcomes were similar at 2 years for watchful waiting vs surgical repair: pain limiting activities (5.1% vs 2.2%, respectively; P = .06 [corrected]); PCS (improvement over baseline, 0.29 points vs 0.13 points; P = .79). Twenty-three percent of patients assigned to watchful waiting crossed over to receive surgical repair (increase in hernia-related pain was the most common reason offered); 17% assigned to receive repair crossed over to watchful waiting. Self-reported pain in watchful-waiting patients crossing over improved after repair. Occurrence of postoperative hernia-related complications was similar in patients who received repair as assigned and in watchful-waiting patients who crossed over. One watchful-waiting patient (0.3%) experienced acute hernia incarceration without strangulation within 2 years; a second had acute incarceration with bowel obstruction at 4 years, with a frequency of 1.8/1000 patient-years inclusive of patients followed up for as long as 4.5 years. CONCLUSIONS: Watchful waiting is an acceptable option for men with minimally symptomatic inguinal hernias. Delaying surgical repair until symptoms increase is safe because acute hernia incarcerations occur rarely.Clinical Trials Registration ClinicalTrials.gov Identifier: NCT00263250.


Subject(s)
Hernia, Inguinal/therapy , Adult , Aged , Disease Progression , Follow-Up Studies , Hernia, Inguinal/physiopathology , Hernia, Inguinal/surgery , Humans , Male , Middle Aged , Pain , Patient Satisfaction , Severity of Illness Index , Surgical Mesh
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