Benign Hepatic Nodules Mimicking Hepatocellular Carcinoma in the Setting of Fontan-associated Liver Disease: A Case Report.

Fontan procedure, in which systemic circulation is redirected into pulmonary circulation by a baffle, is a palliative surgical strategy for patients born with single ventricle congenital heart disease. Hemodynamic changes secondary to Fontan procedure, also termed as Fontan physiology, result in end-organ damage, especially of the liver. Fontan-associated liver disease (FALD) represents a spectrum of pathologies ranging from mild liver fibrosis to advanced liver cirrhosis and hepatocellular carcinoma (HCC). Hepatic nodules, some of which have been documented as HCC in several case series and reports, are a recognized complicated feature of FALD. Herein, we report a case with benign hepatic nodules mimicking HCC by imaging characteristics, emphasizing the fact that arterially enhancing lesions with delayed washout appearance may reflect benign regenerative or focal nodular hyperplasia-like nodules in patients with Fontan physiology. HOW TO CITE THIS ARTICLE: Çolaklar A, Lehnert SJ, Tirkes T. Benign Hepatic Nodules Mimicking Hepatocellular Carcinoma in the Setting of Fontan-associated Liver Disease: A Case Report. Euroasian J Hepato-Gastroenterol 2020;10(1):42-44.

Retroperitoneal and Pelvic Lymph Nodes in Children: What Is Normal?

OBJECTIVE. The purpose of this study was to evaluate size criteria for retroperitoneal and pelvic lymph nodes in healthy children. MATERIALS AND METHODS. We identified all trauma patients younger than 18 years old without underlying disease and with CT scans without abnormalities in the abdomen and pelvis during 2014-2015. Two pediatric radiologists reviewed the studies independently and recorded the number of retroperitoneal and pelvic lymph nodes with a long diameter 5 mm or greater and the size (two perpendicular diameters) of the largest lymph node in five anatomic locations. Discrepant results were reviewed in consensus. The relationship of short diameter to age and interobserver variability was evaluated. RESULTS. A total of 166 patients (86 boys) with a mean age of 7.2 years old (range, 0.1-18.0 years old) were identified. More than 95% of lymph nodes in the retroperitoneum and pelvis had a short diameter measuring at most 7 and 8 mm, respectively, by consensus. The size of the largest short diameter of lymph nodes did not vary with age. More than four lymph nodes were identified in any anatomic location in only three patients, by only one of the radiologists. Agreement for lymph nodes with largest diameter of 5 mm or greater between radiologists ranged from 70.5% to 97.6% for the five anatomic locations with poor interobserver agreement (κ, 0.2-0.3). CONCLUSION. The size and number of retroperitoneal and pelvic lymph nodes in children are less than in adults. A short diameter threshold of 7 mm (retroperitoneal) and 8 mm (pelvic) and more than four lymph nodes with long diameter of 5 mm or greater in one location may define disease.

A comprehensive severity score for the morbidly adherent placenta: combining ultrasound and magnetic resonance imaging.

BACKGROUND: Ultrasound (US) is the first-line imaging modality to assess the morbidly adherent placenta, but sensitivity and specificity are lacking. OBJECTIVE: This investigation aims to improve diagnostic accuracy with a comprehensive score using clinical history, US, and magnetic resonance imaging (MRI). MATERIALS AND METHODS: We conducted a retrospective cohort study of pregnant women who received both transvaginal US and MRI with suspicion for morbidly adherent placenta between 2009 and 2016. US was scored with the following metrics: (i) previa, (ii) hypervascularity, (iii) loss of retroplacental clear space and (iv) lacunae. MRI was evaluated for (i) intraparenchymal vessels, (ii) abnormally dilated vessels, (iii) fibrin deposition, (iv) placental bulge and (v) bladder dome irregularity. Bayesian analysis was used to estimate the probability of morbidly adherent placenta for a given score. Diagnostic testing parameters were calculated. RESULTS: Among the 41 women with concerning imaging, histologically identified disease was confirmed in 16. The probability of morbidly adherent placenta increased with the score. At the highest US score, the probability of disease was 63.7%. With the highest MRI score, the probability of adherent placentation was 90.5%. Combining the US and MRI findings had a sensitivity of 56% and a specificity of 92%. CONCLUSION: A combined scoring system using MRI and US may accurately identify patients at risk for morbidity associated with morbidly adherent placenta.

Characteristics of testicular tumors in prepubertal children (age 5-12 years).

INTRODUCTION: Testicular tumors in children have two peaks with different types of tumors; in the first 4 years of life a third to half are benign with increased risk of malignancy during puberty. The pathology of testicular tumors between these peaks, at the age of 5-12 years, is not known. We hypothesized that because of the low level of testosterone at this time, the incidence of malignant tumors is very low. OBJECTIVE: To compare malignancy risk of primary testicular tumors in children in the prepubertal period (5-12 years) compared with younger (0-4 years) and pubertal (13-18 years) children. STUDY DESIGN: We retrospectively (2002-2016) identified patients <18 years with surgery for primary testicular tumor. Patients with testicular tumor risk were excluded. Ultrasound studies were reviewed for contralateral testis volume, tumor morphology, and tumor maximal diameter, for three age groups: 0-4, 5-12, and 13-18 years. The Freeman-Halton extension of the Fisher exact probability test was adopted for categorical outcomes, and one-way ANOVA for continuous outcomes. RESULTS: Fifty-two patients (mean age 11.0 years, range 6 days-18 years) were identified. Malignant tumor prevalence significantly differed (p < 0.01) among age groups (Fig).: 0-4 (72.7%, 8/11), 5-12 (0%, 0/16), and 13-18 years (44.0%, 11/25). The most common tumor types in 5-12 years were epidermoid cyst (31.3%, 5/16) and tumor mimics (37.5%, 6/16). Prevalence of cystic tumors in 5-12 year olds was not significantly different compared with other age groups. Contralateral testicular volume >4 mL (pubertal surge) significantly (p < 0.01) differed among groups: 0-4 years (0/11), 5-12 years (3/16), and 13-18 years (19/20). In children aged 13-18 years the mean tumor maximal diameter (29.8 ± 4.4 mm) was significantly larger (p < 0.01) compared with children 5-12 years (9.3 ± 5.5 mm) and all malignant tumors had contralateral testicular volume >4 mL. DISCUSSION: We found that preadolescent children between the ages of 5 and 12 years have distinctive characteristics compared with the other age groups. Most importantly, no malignant testicular tumors were found in this age group. About a third of the children presented with an incidental testicular mass. The testicular tumors were significantly smaller (9.3 ± 6.7 mm) compared with those in children aged 13-18 years (29.8 ± 4.4 mm). There were limitations because of the retrospective nature of the study. CONCLUSION: We found no malignant testicular tumors in children aged 5-12 years with no risk factors and prior to pubertal surge. Our study suggests use of more conservative treatment in this group of patients.

Effects of distance and transport method on intervention and mortality in aneurysmal subarachnoid hemorrhage.

OBJECTIVE Most patients suffering from aneurysmal subarachnoid hemorrhage (aSAH) initially present to a hospital that lacks a neurosurgical unit. These patients require interhospital transfer (IHT) to tertiary facilities capable of multidisciplinary neurosurgical intervention. Yet, little is known about the effects of IHT on the outcomes of patients suffering from aSAH. In this study, the authors examined the effects of IHT and transport method on the timing of treatment, rebleed rates, and overall outcomes of patients who have experienced aSAH. METHODS A retrospective review of medical records identified all consecutive patients who presented with aSAH at an outside hospital and subsequently underwent IHT to a tertiary aneurysm care center and patients who initially presented directly to a tertiary aneurysm care facility between 2008 and 2015. Demographic, operative, radiological, hospital of initial evaluation, transfer method, and outcome data were retrospectively collected. RESULTS The authors identified 763 consecutive patients who were evaluated for aSAH at a tertiary aneurysm care facility either directly or following IHT. For patients who underwent IHT and after accounting for these patients' clinical variability and dichotomizing the patients into groups transferred less than 20 miles and more than 20 miles, the authors noted a significant increase in mortality rates: 7% (< 20 miles) and 18.8% (> 20 miles) (p = 0.004). The increased mortality rate was partially explained by an increased rate of initial presentation to an accredited stroke center in patients undergoing IHT of less than 20 miles (p = 0.000). The method of transport (ground or air ambulance) was found to have significant effect on the patients' outcomes as measured by the Glasgow Outcome Scale score (p = 0.021); patients who underwent ground transport demonstrated a higher likelihood of discharge to home (p = 0.004). The increased severity of presentation in the patient cohort undergoing IHT by air as defined by the Glasgow Coma Scale score, a need for an external ventricular drain, Hunt and Hess grade, and intubation status at presentation did not result in increased mortality when compared with the ground cohort (p = 0.074). In addition, there was an 8-hour increase in duration of time from admission to treatment for the air cohort as compared with the ground cohort (p = 0.054), indicating a potential for further improvement in the overall outcome of this patient group. CONCLUSIONS Aneurysmal SAH remains a challenging neurosurgical disease process requiring highly coordinated care in tertiary referral centers. In this study, the overall distance traveled and the transport method affected patient outcomes. The time from admission to treatment should continue to improve. Further analysis of IHT with a focus on patient monitoring and treatment during transport is warranted.

Fishtail deformity of the distal humerus: association with osteochondritis dissecans of the capitellum.

BACKGROUND: Fishtail deformity is a rare deformity of the humerus exhibiting concavity of the lateral trochlea, resulting in ulnotrochlear joint derangement. We wanted to share our experience that osteochondritis dissecans of the capitellum is a common associated complication. OBJECTIVE: To summarize imaging of fishtail deformity in children centered on complications of the radiocapitellar joint. MATERIALS AND METHODS: From the radiology information system, we identified all patients <18 years with the diagnosis of fishtail deformity. We included only patients with V-shaped deformity of the distal humerus due to concavity at the lateral trochlea (fishtail deformity). Each patient's initial injury, most recent radiograph and available MRI were evaluated for radiocapitellar joint derangement. RESULTS: Seven patients (4 males) with a mean age of 12.9 years (range: 9.7 to 14.4 years) were identified. Radiocapitellar joint abnormalities were identified in six patients including osteoarthritis (n=5), flattened and sclerotic capitellum (n=4), osteochondritis dissecans (2 associated with loose body, n=4) and radial head subluxation (n=2). In 4 patients, MRI detected changes of osteoarthritis (n=4), osteochondritis dissecans (n=2) and loose body (n=1) not identified on radiography. Two patients with osteochondritis dissecans underwent surgery and one patient has planned surgery. CONCLUSION: Radiocapitellar joint abnormalities (particularly, capitellar osteochondritis dissecans) are common in patients with fishtail deformities. MRI should be performed in these patients since some abnormalities, possibly requiring surgery, are not detected on elbow radiographs.

Long-term follow-up analysis of microsurgical clip ligation and endovascular coil embolization for dorsal wall blister aneurysms of the internal carotid artery.

Blister aneurysms at non-branching sites of the dorsal internal carotid artery (dICA) are fragile, rare, and often difficult to treat. The purpose of this study is to address the demographics, treatment modalities, and long-term outcome of patients treated for dICA blister aneurysms. A retrospective review of medical records identified all consecutive patients who presented with a blister aneurysm from 2002 to 2011 at our institution. Eighteen patients (M=7, F=11; mean age: 48.4±15.1years; range: 15-65years) harbored a total of 43 aneurysms, 25 of which were dorsal wall blister aneurysms of the ICA. Eleven (61.1%) patients presented with aneurysmal subarachnoid hemorrhage (aSAH), and 10 (55.6%) patients had multiple aneurysms at admission. Twelve patients had 18 aneurysms that were treated microsurgically. Five (41.7%) of these patients had a single recurrence that was retreated with subsequent repeat clip ligation. Six patients had 7 blister aneurysms that were treated with endovascularly. One (16.7%) of these patients had a single recurrence that was retreated with subsequent coil embolization. Postoperative vasospasm occurred in 8 (44.4%) patients, one of whom suffered from a stroke. This is one of the largest single-institution dICA blister aneurysm studies to date. There was no detected significant difference between microsurgical clip ligation and endovascular coil embolization in terms of surgical outcome. These blister aneurysms demonstrate a propensity to be associated with multiple cerebral aneurysms. Strict clinical and angiographic long-term follow-up may be warranted. STATEMENT OF SIGNIFICANCE: Blister aneurysms are focal wall defects covered by a thin layer of fibrous tissue and adventitia, lacking the usual collagenous layer. Due to their pathologically thin vessel wall, blister aneurysms are prone to rupture. The management of these rare and fragile aneurysms presents a number of challenges. Here, we address the long-term outcome of patients treated for blister aneurysms at non-branching sites of the dICA. The presented data and analysis is imperative to determine the necessary strict long-term clinical and angiographic follow-up.

Reirradiation of Recurrent and Second Primary Head and Neck Cancer With Proton Therapy.

PURPOSE: To report the clinical outcomes of head and neck reirradiation with proton therapy. METHODS AND MATERIALS: From 2004 to 2014, 61 patients received curative-intent proton reirradiation, primarily for disease involving skull base structures, at a median of 23 months from the most recent previous course of radiation. Most had squamous cell (52.5%) or adenoid cystic (16.4%) carcinoma. Salvage surgery before reirradiation was undertaken in 47.5%. Gross residual disease was present in 70.5%. For patients with microscopic residual disease, the median dose of reirradiation was 66 Gy (relative biological effectiveness), and for gross disease was 70.2 Gy (relative biological effectiveness). Concurrent chemotherapy was given in 27.9%. RESULTS: The median follow-up time was 15.2 months and was 28.7 months for patients remaining alive. The 2-year overall survival estimate was 32.7%, and the median overall survival was 16.5 months. The 2-year cumulative incidence of local failure with death as a competing risk was 19.7%; regional nodal failure, 3.3%; and distant metastases, 38.3%. On multivariable analysis, Karnofsky performance status ≤70%, the presence of a gastrostomy tube before reirradiation, and an increasing number of previous courses of radiation therapy were associated with a greater hazard ratio for death. A cutaneous primary tumor, gross residual disease, increasing gross tumor volume, and a lower radiation dose were associated with a greater hazard ratio for local failure. Grade ≥3 toxicities were seen in 14.7% acutely and 24.6% in the late setting, including 3 treatment-related deaths. CONCLUSIONS: Reirradiation with proton therapy, with or without chemotherapy, provided reasonable locoregional disease control, toxicity profiles, and survival outcomes for an advanced-stage and heavily pretreated population. Additional data are needed to identify which patients are most likely to benefit from aggressive efforts to achieve local disease control and to evaluate the potential benefit of proton therapy relative to other modalities of reirradiation.