Publications by authors named "HaiThuy N Nguyen"

13 Publications

  • Page 1 of 1

Angiosarcoma of the Pancreas in a Pediatric Patient With an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature.

J Pediatr Hematol Oncol 2021 Jul 1. Epub 2021 Jul 1.

Michael E. DeBakey Department of Surgery, Division of Pediatric Surgery, Texas Children's Surgical Oncology Program Texas Children's Department of Surgery, Dan L. Duncan Cancer Center, Baylor College of Medicine Department of Pathology and Immunology Division of Pediatric Gastroenterology Singleton Department of Radiology Department of Hematology Oncology Michael E. DeBakey Department of Surgery, Division of Vascular Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, TX.

Pancreatic angiosarcoma is an exceedingly rare malignancy accounting for <1% of pancreatic neoplasms. A very limited number of pancreatic angiosarcomas have been reported in the literature without any cases described in children. We present the case of a 17-year-old female diagnosed with angiosarcoma of the pancreas following pancreaticoduodenectomy for a pancreatic mass, initially presumed to be a solid pseudopapillary neoplasm of the pancreas. The angiosarcoma was found to have a novel activating internal tandem duplication in the KDR gene (KDR-internal tandem duplication). We discuss the current literature on this disease process. This is the first reported case of pancreatic angiosarcoma in a pediatric patient and the first with an activating KDR-internal tandem duplication.
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http://dx.doi.org/10.1097/MPH.0000000000002248DOI Listing
July 2021

Pathologic correlation with near infrared-indocyanine green guided surgery for pediatric liver cancer.

J Pediatr Surg 2021 Apr 25. Epub 2021 Apr 25.

Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Surgical Oncology Program, Texas Children's Liver Tumor Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX USA. Electronic address:

Purpose: Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common primary malignant tumors of childhood. Intraoperative indocyanine green (ICG) administration with near-infrared imaging (NIR) has emerged as a surgical technology that can be used to assist with localization of pulmonary metastases secondary to HB; however, there has been limited application as an adjunct for resection of the primary liver tumor and assessment of extrahepatic disease.

Methods: We present 14 patients treated for HB, HCC, and malignant rhabdoid tumor at our institution with the use of intraoperative NIR-ICG guidance. All patients were treated with 0.2-0.75 mg/kg IV ICG, 48-96 h prior to surgery. Intraoperative NIR-ICG guided imaging was performed with several commercial devices.

Results: Intraoperative NIR-ICG guidance allowed pulmonary metastasectomy in five patients using thoracoscopy or thoracotomy allowing for visualization of multiple nodules not seen on preoperative imaging most of which were positive for malignancy. NIR-ICG guidance allowed for assessment of extrahepatic extension in three patients; an HCC patient with extrahepatic lymph node extension of disease, an HB patient with extrapulmonary thoracic recurrence in the diaphragm and chest wall, and a patient with tumor rupture at diagnosis with peritoneal nodules at the time of surgery. This technique was used to guide partial hepatectomy in 11 patients for which the technique enabled successful identification of tumor and tumor margins. Three patients had nonspecific staining of the liver secondary to decreased timing from ICG injection to surgery or biliary obstruction. NIR-ICG enabled resection of satellite HB lesions in three multifocal patients and confirmed a benign satellite lesion in two additional patients.

Conclusions: Intraoperative use of NIR-ICG imaging during partial hepatectomy enabled enhanced identification and guidance for surgical resection of extrahepatic disease and multifocal liver tumors for the treatment of children with primary liver cancer.
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http://dx.doi.org/10.1016/j.jpedsurg.2021.04.019DOI Listing
April 2021

Ultrasound for the diagnosis of malrotation and volvulus in children and adolescents: a systematic review and meta-analysis.

Arch Dis Child 2021 Apr 20. Epub 2021 Apr 20.

Pediatrics, Baylor College of Medicine, Houston, Texas, USA

Context: Despite the advantages of ultrasound (US), upper gastrointestinal contrast series (UGI) remains the first-line diagnostic modality in the diagnosis of midgut malrotation and volvulus in children.

Objective: Evaluate the diagnostic accuracy of US in the diagnosis of malrotation with or without volvulus in children and adolescents aged 0-21 years, compared with the reference standard (diagnosis by surgery, UGI, CT, MRI, and clinical follow-up individually or as a composite).

Data Sources: We searched the electronic databases Ovid-MEDLINE, Embase, Scopus, CINAHL, and the Cochrane library in October 2019 and updated on 18 August 2020.

Study Selection: Studies evaluating the diagnostic performance of US for diagnosis of midgut malrotation with or without volvulus in children (0-21 years).

Data Extraction And Synthesis: The data were extracted independently by two authors and a bivariate model was used for synthesis.

Results: Meta-analysis of 17 cohort or cross-sectional studies and 2257 participants estimated a summary sensitivity of 94% (95% CI 89% to 97%) and summary specificity of 100% (95% CI 97% to 100%) (moderate certainty evidence) for the use of US for the diagnosis of malrotation with or without midgut volvulus compared with the reference standard. Subgroup analysis and meta-regression revealed better diagnostic accuracy in malrotation not complicated by volvulus, in the neonatal population and enteric fluid administration before US.

Conclusions: Moderate certainty evidence suggests excellent diagnostic accuracy and coupled with the advantages, a strong case exists for the use of abdominal US as the first-line diagnostic test for suspected midgut malrotation with or without volvulus in children and adolescents.
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http://dx.doi.org/10.1136/archdischild-2020-321082DOI Listing
April 2021

The eyes see what the mind knows - a need for midgut volvulus ultrasound education: reply to Strouse.

Pediatr Radiol 2021 Apr 19;51(4):673. Epub 2021 Feb 19.

Singleton Department of Pediatric Radiology, Texas Children's Hospital, 6701 Fannin St., Houston, TX, 77030, USA.

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http://dx.doi.org/10.1007/s00247-021-05001-2DOI Listing
April 2021

Untwisting the complexity of midgut malrotation and volvulus ultrasound.

Pediatr Radiol 2021 Apr 4;51(4):658-668. Epub 2021 Jan 4.

Singleton Department of Radiology, Texas Children's Hospital, 6701 Fannin St., Houston, TX, 77030, USA.

Abdominal US is emerging as an alternative to the upper gastrointestinal (GI) series as the preferred diagnostic imaging test for midgut malrotation complicated by volvulus. Unlike the upper GI series, US is free from ionizing radiation, does not require oral contrast agent, and can be performed portably and at times remotely from the interpreting radiologist, expediting diagnosis. Although some institutions do not have a standardized US protocol for midgut volvulus, many routinely use US in the setting of an infant or child with acute abdominal signs or symptoms to evaluate for common conditions such as hypertrophic pyloric stenosis, intussusception, necrotizing enterocolitis and appendicitis. Because these common conditions can overlap in age and clinical presentation with midgut volvulus, the aim of this pictorial essay is to provide instruction on the technique and diagnostic findings of midgut volvulus on US to ensure confident, accurate interpretation, and prompt treatment.
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http://dx.doi.org/10.1007/s00247-020-04876-xDOI Listing
April 2021

Time-Driven Activity-Based Cost Comparison of Three Imaging Pathways for Suspected Midgut Volvulus in Children.

J Am Coll Radiol 2020 Dec 19;17(12):1563-1570. Epub 2020 Jul 19.

Department of Radiology, Texas Children's Hospital, Houston, Texas; University of Texas, School of Public Health, Houston, Texas. Electronic address:

Objective: To use time-driven activity-based costing to compare the costs of pathways for evaluating suspected pediatric midgut volvulus using either fluoroscopic upper gastrointestinal examination (UGI) or focused abdominal ultrasound (US).

Methods: Process maps were created through patient shadowing, medical record review, and frontline staff interviews. Using time-driven activity-based costing methodology, practical capacity cost rates were calculated for personnel, equipment, and facility costs. Supply costs were included at institutional purchase prices. The cost of each process substep was determined by multiplying step-specific capacity costs by the median time required for each step, and substep costs were summed to generate total pathway cost. Multivariate sensitivity analyses were performed applying minimum and maximum labor costs. Assuming UGI would be used to troubleshoot nondiagnostic US, a break-even analysis was performed to determine the cost impact of varying frequencies of UGI on the total cost of the US-based pathway.

Results: Process maps were created from 105 (48 girls, 57 boys) patient encounters. Base case pathway times were 90 min (UGI) and 55 min (US). Base case cost for UGI was $282.74 (range: $170.86-$800.82) when performed by a radiology practitioner assistant and $545.66 (range: $260.97-$1,974.06) when performed by a radiologist. Base case cost for US was $155.67 (range: $122.94-$432.29) when performed by a sonographer and $242.64 (range: $147.46-$1,330.05) when performed by a radiologist. For a US-based pathway, the total cost break-even pathway mix (percent UGI required for troubleshooting) was 57%.

Conclusion: US can be a faster and less costly alternative to UGI in pediatric patients with suspected midgut volvulus.
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http://dx.doi.org/10.1016/j.jacr.2020.06.023DOI Listing
December 2020

Development of pediatric academic-community radiology services: lessons learned.

Pediatr Radiol 2020 08 2;50(9):1207-1216. Epub 2020 May 2.

Edward B. Singleton Department of Radiology, Texas Children's Hospital and Baylor College of Medicine, 6701 Fannin St., Suite 470, Houston, TX, 77030, USA.

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http://dx.doi.org/10.1007/s00247-020-04662-9DOI Listing
August 2020

Clinico-radiologic features of pleuroparenchymal fibroelastosis in children.

Pediatr Radiol 2019 08 19;49(9):1163-1170. Epub 2019 Apr 19.

Department of Pediatric Radiology, Texas Children's Hospital, 6701 Fannin St., Houston, TX, 77030, USA.

Background: Pleuroparenchymal fibroelastosis (PPFE) may be underdiagnosed clinically and radiographically in children with a remote history of cancer, leading to a delay in care and unnecessary lung biopsies.

Objective: To describe the characteristic clinical and radiologic findings of PPFE in a cohort of children to facilitate recognition and noninvasive diagnosis.

Materials And Methods: Clinical presentation, history of chemotherapy or radiation therapy, lung or bone marrow transplantation, and lung function testing and outcome were retrospectively extracted from the electronic medical records of eight children treated at our institution's pulmonary medicine clinic with histopathology confirmation of PPFE from 2008 to 2018. Two pediatric radiologists evaluated the chest imaging studies for the presence or absence of published radiologic findings of PPFE in adults, including platythorax, pneumothorax, upper lobe predominant pleural and septal thickening, and bronchiectasis. Platythorax indices were calculated from the normal chest CT exams of eight age- and gender-matched individuals obtained via the radiology search engine.

Results: The mean presentation age was 12.9 years (range: 7-16 years). Seven of the eight had a history of chemotherapy and radiation therapy for cancer. Three of the eight had undergone bone marrow transplantation and none had undergone lung transplantation. The mean time between chemotherapy, radiation therapy, and/or bone marrow transplantation and the presentation of PPFE was 8.4 years (range: 5.6-12.1 years). Most of the patients presented with dyspnea (63%), cough (50%) and/or pneumothorax (38%). The mean percentage of predicted FEV1 (forced expiratory volume in one second) was 14.1 (range: 7.7-27.5). All eight patients demonstrated platythorax, bronchiectasis, pleural and septal thickening (upper lobes in four, upper and lower lobes in four) and six had pneumothorax. Five underwent lung biopsies, four of whom developed pneumothoraces.

Conclusion: Clinical and radiologic findings of pediatric PPFE are similar to those in adults, although a majority of the former have a history of treated cancer. Clinical presentation of restrictive lung disease, dyspnea, cough or spontaneous pneumothorax years after treatment for childhood cancer combined with platythorax, upper lobe pleural and septal thickening and traction bronchiectasis on chest CT establishes a presumptive diagnosis of PPFE.
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http://dx.doi.org/10.1007/s00247-019-04405-5DOI Listing
August 2019

Hormonal, metabolic and skeletal phenotype of Schaaf-Yang syndrome: a comparison to Prader-Willi syndrome.

J Med Genet 2018 05 1;55(5):307-315. Epub 2018 Mar 1.

Jan and Dan Duncan Neurological Research Institute at Texas Children's Hospital, Houston, Texas, USA.

Background: Nonsense and frameshift mutations in the maternally imprinted, paternally expressed gene located in the Prader-Willi critical region 15q11-15q13, have been reported to cause Schaaf-Yang syndrome (SYS), a genetic disorder that manifests as developmental delay/intellectual disability, hypotonia, feeding difficulties and autism spectrum disorder. Prader-Willi syndrome (PWS) is a genetic disorder characterised by severe infantile hypotonia, hypogonadotrophic hypogonadism, early childhood onset obesity/hyperphagia, developmental delay/intellectual disability and short stature. Scoliosis and growth hormone insufficiency are also prevalent in PWS.There is extensive documentation of the endocrine and metabolic phenotypes for PWS, but not for SYS. This study served to investigate the hormonal, metabolic and body composition phenotype of SYS and its potential overlap with PWS.

Methods: In nine individuals with SYS (5 female/4 male; aged 5-17 years), we measured serum ghrelin, glucose, insulin-like growth factor 1 (IGF-1), insulin-like growth factor binding protein 3, follicle-stimulating hormone, luteinising hormone, thyroid-stimulating hormone, free T4, uric acid and testosterone, and performed a comprehensive lipid panel. Patients also underwent X-ray and dual-energy X-ray absorptiometry analyses to assess for scoliosis and bone mineral density.

Results: Low IGF-1 levels despite normal weight/adequate nutrition were observed in six patients, suggesting growth hormone deficiency similar to PWS. Fasting ghrelin levels were elevated, as seen in individuals with PWS. X-rays revealed scoliosis >10° in three patients, and abnormal bone mineral density in six patients, indicated by Z-scores of below -2 SDs.

Conclusion: This is the first analysis of the hormonal, metabolic and body composition phenotype of SYS. Our findings suggest that there is marked, but not complete overlap between PWS and SYS.
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http://dx.doi.org/10.1136/jmedgenet-2017-105024DOI Listing
May 2018

Radiology Resident' Satisfaction With Their Training and Education in the United States: Effect of Program Directors, Teaching Faculty, and Other Factors on Program Success.

AJR Am J Roentgenol 2016 May 9;206(5):907-16. Epub 2016 Mar 9.

2 Department of Diagnostic and Interventional Radiology, University of Texas Health Science Center at Houston, Houston, TX.

Objective: Radiology residency education must evolve to meet the growing demands of radiology training. Resident opinions are a major resource to identify needs. However, few published data are available on a national level investigating the radiology resident perspective on factors that influence the resident experience. Our study investigates factors that affect residents' satisfaction with their residency experience and education.

Materials And Methods: A 67-item survey was sent to all radiology residency program directors and coordinators in the United States to be distributed at their discretion. Questions were multiple choice, free-text answer, or 5-point Likert scale. Statistical significance (p < 0.05) was determined using chi-square test, t test, and logistic regression analysis, respectively.

Results: Two hundred seventeen radiology residents responded to the survey (range, 212-217 responses per question). Overall, 77.8% (168/216) of residents were satisfied with their residency programs. Subcategories that showed a statistically significant correlation with overall satisfaction, in decreasing strength according to the odds ratio (OR), include the program director or administrative office (OR, 72.2; 95% CI, 27.4-221.9), the daily workstation experience (OR, 30.5; 95% CI, 12.8-80.9), the faculty (OR, 19.5; 95% CI, 8.9-45.4), educational conferences (OR, 7.9; 95% CI, 3.9-16.4), work hours (OR, 6.4; 95% CI, 3.2-13.2), teaching opportunities (OR, 6.5; 95% CI, 3.1-13.8), research opportunities (OR, 5.1; 95% CI, 2.6-10.6), personal study (OR, 2.1; 95% CI, 1.1-4.1), and compensation (OR, 1.9; 95% CI, 1.0-3.7).

Conclusion: Our study provides incremental data to the existing literature that offers insight into factors that contribute to a successful radiology residency program.
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http://dx.doi.org/10.2214/AJR.15.15020DOI Listing
May 2016

Primary Epithelioid Angiosarcoma of Bone with Robust Cell Cycle Progression and High Expression of SPARC: A Case Report and Review of the Literature.

Ann Clin Lab Sci 2015 ;45(3):360-5

Department of Pathology and Laboratory Medicine, University of Texas Health Science center at Houston, TX, USA

Epithelioid angiosarcoma of bone is a rare entity. Secreted protein acidic and rich in cysteine (SPARC), or osteonectin, is a secreted glycoprotein that has been implicated in tumorigenesis. We report a case of epithelioid angiosarcoma involving the long bones of the lower extremity showing diffuse and strong expression for SPARC immunohistochemistry in tumor cells. Ki-67 was positive in ~50% of tumor cell nuclei and the accompanying mitotic index was 19 mitotic figures/10 high power fields.Expression of SPARC in tumors has been correlated with sensitivity to nanoparticle albumin-bound paclitaxel (Nab-paclitaxel), particularly in the context of robust cell cycle progression into the mitotic phase. This finding could suggest new therapeutic options for further consideration.
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March 2016

Intussusception revisited: is immediate on-site surgeon availability at the time of reduction necessary?

AJR Am J Roentgenol 2014 Feb;202(2):432-6

1 Department of Diagnostic and Interventional Radiology, University of Texas Health Science Center at Houston, Houston, TX.

Objective: The American College of Radiology recommends that fluoroscopically guided intussusception reduction be performed with a surgeon readily available. At many institutions, this may not be feasible. The purpose of this study was to assess the utilization of immediate surgical services at the time of radiologic intussusception reduction.

Materials And Methods: All radiologic intussusception reductions at a tertiary care children's hospital from November 2007 through August 2012 were reviewed to determine method, complications, clinical status of the child, and time between unsuccessful reduction and operative intervention.

Results: Among 433 patients with intussusception referred for fluoroscopic reduction, 86.1% (n = 373) had successful reductions, and 13.9% (n = 60) had unsuccessful reductions. Five perforations represented 8.3% (5/60) of the unsuccessful and 1.2% (5/433) of the total reduction attempts. Six patients' conditions became hemodynamically unstable during attempted reduction (four perforations, two unsuccessful reductions without perforation), representing 10% (6/60) of unsuccessful and 1.4% (6/433) of total reduction attempts. Percutaneous needle decompression and cardiopulmonary resuscitation restored hemodynamic stability in all cases. The mean time to surgery after perforation was 1.3 hours, after unsuccessful reduction complicated by hemodynamic instability was 2.2 hours, and after unsuccessful radiologic reduction without complication was 4.3 hours.

Conclusion: In this series, complications requiring immediate medical or surgical attention were rare, occurring in 1.6% of cases (five bowel perforations, two cases of hemodynamic instability without perforation). On-site surgeon presence may not be necessary at the time of radiologically guided reduction attempts provided that the attending physician is facile with percutaneous needle decompression and management of hemodynamic instability and that ultimate surgical care can be arranged expeditiously.
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http://dx.doi.org/10.2214/AJR.13.10731DOI Listing
February 2014