Portal Steal Syndrome From a Large Linton’s Splenorenal Shunt after Liver Transplantation: Successful Endovascular Management Through Off-Label Application of a 30 mm Amplatzer Cardiac Plug

A 34-year-old patient underwent liver transplantation for progressive hepatic failure in the setting of congenital hepatic fibrosis. In past medical history, the patient had undergone splenectomy with proximal Linton’s splenorenal surgical shunt creation for symptomatic portal hypertension with hypersplenism. The patient developed an early allograft dysfunction, with radiologic evidence of a reduced portal flow associated to portal steal from the patent surgical shunt. The patient was successfully treated through endovascular placement of a 30 mm Amplatzer cardiac plug at the origin of the splenic vein.

Natural History of Untreated Retinoblastoma

Treatment abandonment is a leading cause of death in children with retinoblastoma worldwide. We studied children who abandoned treatment upfront at diagnosis to delineate the natural history of untreated retinoblastoma. Studied were children who received no treatment, diagnosed between 2007 and 2017 at 29 Chinese centers. Data were retrospectively collected from medical chart reviews and interviews with each patient’s family. During the study period, 44 children received no treatment after diagnosis of retinoblastoma. Clinical or radiologic evidence of orbital extension was available for 25 children, and radiologic evidence of systemic metastasis was available for 12 children. Median times from diagnosis of intraocular tumor to orbital disease was 13.7 months, orbital disease to metastasis was 2.6 months, and metastasis to death was 2.0 months. Children with brain metastasis had shorter survival than those with metastasis to other sites (median 1.0 vs. 3.1 months; p = 0.015). Overall, 36% of patients died within 12 months of diagnosis, 77% within 24 months, 95% within 36 months and 100% within 48 months. While multiple factors influence refusal of treatment, insights into the natural history of retinoblastoma derived from real-world evidence can inform clinicians and parents that retinoblastoma is life-threatening and encourage urgent treatment at diagnosis.

Improved detection of air trapping on expiratory computed tomography using deep learning

Background Radiologic evidence of air trapping (AT) on expiratory computed tomography (CT) scans is associated with early pulmonary dysfunction in patients with cystic fibrosis (CF). However, standard techniques for quantitative assessment of AT are highly variable, resulting in limited efficacy for monitoring disease progression. Objective To investigate the effectiveness of a convolutional neural network (CNN) model for quantifying and monitoring AT, and to compare it with other quantitative AT measures obtained from threshold-based techniques. Materials and methods Paired volumetric whole lung inspiratory and expiratory CT scans were obtained at four time points (0, 3, 12 and 24 months) on 36 subjects with mild CF lung disease. A densely connected CNN (DN) was trained using AT segmentation maps generated from a personalized threshold-based method (PTM). Quantitative AT (QAT) values, presented as the relative volume of AT over the lungs, from the DN approach were compared to QAT values from the PTM method. Radiographic assessment, spirometric measures, and clinical scores were correlated to the DN QAT values using a linear mixed effects model. Results QAT values from the DN were found to increase from 8.65% ± 1.38% to 21.38% ± 1.82%, respectively, over a two-year period. Comparison of CNN model results to intensity-based measures demonstrated a systematic drop in the Dice coefficient over time (decreased from 0.86 ± 0.03 to 0.45 ± 0.04). The trends observed in DN QAT values were consistent with clinical scores for AT, bronchiectasis, and mucus plugging. In addition, the DN approach was found to be less susceptible to variations in expiratory deflation levels than the threshold-based approach. Conclusion The CNN model effectively delineated AT on expiratory CT scans, which provides an automated and objective approach for assessing and monitoring AT in CF patients.

Adult case of giant sacrococcygeal teratoma: case report

Background Sacrococcygeal teratomas are tumors originating from pluripotent embryonic germ cell layers located in the fetal coccyx. These tumors are highly vascular if they undergo malignant transformation. Typically, they are found in infants and children and occasionally can be diagnosed prenatally. Adult cases are very rare, and represent tumors present since birth with delayed detection. Case presentation We describe a case of a giant sacrococcygeal teratoma in a 25 years old female college student presenting with right gluteal swelling of 4 months’ duration. In addition to the huge disfiguring mass on the perineal area, she also had lower abdominal pain, urinary complaints, and difficulty with ambulation. Discussion Pre-operative impression was of a sacrococcygeal mass and histopathology following complete surgical excision revealed a sacrococcygeal teratoma. She recovered well after surgery with no radiologic evidence of recurrence at six months. Conclusion Although rare, sacrococcygeal teratoma should be considered as a differential diagnosis for female adults presenting with perineal and/or pelvic masses. Complete surgical excision remains the mainstay of treatment.

Improved Detection of Air Trapping on Expiratory Computed Tomography Using Deep Learning

ABSTRACTBackgroundRadiologic evidence of air trapping (AT) on expiratory CT scans is associated with early pulmonary dysfunction in patients with cystic fibrosis (CF). However, standard techniques for quantitative assessment of AT are highly variable, resulting in limited efficacy for monitoring disease progression.ObjectiveTo investigate the effectiveness of a convolutional neural network (CNN) model for quantifying and monitoring AT, and to compare it with other quantitative AT measures obtained from threshold-based techniques.Materials and MethodsPaired volumetric whole lung inspiratory and expiratory CT scans were obtained at four time points (0, 3, 12 and 24 months) on 36 subjects with mild CF lung disease. A densely connected CNN (DN) was trained using AT segmentation maps generated from a personalized threshold-based method (PTM). Quantitative AT (QAT) values, presented as the relative volume of AT over the lungs, from the DN approach were compared to QAT values from the PTM method. Radiographic assessment, spirometric measures, and clinical scores were correlated to the DN QAT values using a linear mixed effects model.ResultsQAT values from the DN were found to increase from 8.65% ± 1.38% to 21.38% ± 1.82%, respectively, over a two-year period. Comparison of CNN model results to intensity-based measures demonstrated a systematic drop in the Dice coefficient over time (decreased from 0.86 ± 0.03 to 0.45 ± 0.04). The trends observed in DN QAT values were consistent with clinical scores for AT, bronchiectasis, and mucus plugging. In addition, the DN approach was found to be less susceptible to variations in expiratory deflation levels than the threshold-based approach.ConclusionThe CNN model effectively delineated air trapping on expiratory CT scans, which provides an automated and objective approach for assessing and monitoring air trapping in CF patients.

Features and Outcome of Hepatobiliary Tuberculosis among Filipino Children: Report of a Six-year Experience

Background. The features and outcome of hepatobiliary tuberculosis (HBTB) have not been extensively reportedin children. Objective. To describe the clinical, biochemical, radiologic, microbiologic and histologic features and outcome ofchildren diagnosed with HBTB. Methods. Data of HBTB patients aged 0-18 years were collected by review of medical records and as they wereadmitted. Cases were classified as bacteriologically-confirmed (positive AFB smear, TB culture or PCR of bile/livertissue) or clinically-diagnosed (clinical, histologic and/or radiologic evidence). Results. A total of 36 patients were included (mean age: 13yrs; 64% males): three bacteriologically-confirmed and33 clinically-diagnosed. Most common signs/symptoms were weight loss (69%), fever (67%), hepatomegaly (61%)and jaundice (53%). Of the total, 68% had hypoalbuminemia, 50% increased transaminases and 47% prolongedprothrombin time. Fifteen (42%) patients were AFB positive on various microbiologic specimens. Most commonimaging finding was hepatic calcification (64%). Of 11 patients with liver biopsy, seven (64%) had chronic/granulomatous inflammation. All 36 were managed medically. Eight were lost to follow up, six died, and 22 (61%)are alive, nine with complete resolution of liver disease. Conclusion. Hepatobiliary tuberculosis presents with non-specific clinical and biochemical findings. Severalinvestigations are necessary to confirm the diagnosis. Overall response to anti-TB treatment is satisfactory withpossible resolution of liver disease.