Vascular anomalies: nomenclature, classification, and imaging algorithms.

There is a lot of ambiguity in the usage of correct terminology in the description of vascular malformations and tumors. Hemangioma and arteriovenous malformation (AVM) are the most commonly used terms and are the mostly incorrectly used as well! The aim of this review article was to lay out the correct nomenclature and describe the correct usage for the physicians and radiologists involved in diagnosing and managing these lesions. We describe the various classification systems which have been devised to define the multiple entities included under vascular anomalies. The latest classification system that should be adhered to is per the International Society for the Study of Vascular Anomalies, approved at the 20th ISSVA Workshop held in Melbourne in April 2014, last revised in May 2018. The main features of the latest revision have been highlighted. This classification, however, does not list the diagnostic clinico-radiological features for each entity. In addition, guidelines regarding the appropriate use of available imaging modalities are lacking in the literature. We, hereby, aim to address these pertinent issues in this review article.

Vascular anomalies: diagnostic features and step-wise approach.

The first part of this review article emphasized correct nomenclature, classification systems, and imaging algorithm of vascular anomalies. The second part of the review discusses the individual entities, highlighting the characteristic clinico-radiological features of the commonly encountered ones. A step-wise algorithmic approach is also proposed for the evaluation of a suspected case of vascular anomaly.

Chest-Wall Collateral Embolization to Reduce Surgical Blood Loss in Peripheral Aspergillomas.

OBJECTIVES: The objective of this study was to present the procedural details and digital subtraction angiography (DSA) findings of perioperative chest-wall collateral embolization (PCCE) and compare intraoperative blood loss in patients of pulmonary aspergilloma (PA) undergoing lung resection with and without PCCE. MATERIALS AND METHODS: Since November 2017, we have performed PCCE in 17 patients (14 males, three females, age 34.41 ± 12.85 years) before surgery for PA (embolization group). Retrospective evaluation of these patients was done, DSA findings were noted, and perioperative parameters (surgical approach, extent of resection, operative time, blood loss, blood transfusion, morbidity grade, and length of post-operative stay) were compared with a comparative cohort of 24 patients of PA (21 males, three females, mean age 36.13 ± 12.58 years) who underwent thoracic surgery without PCCE (May 2013-November 2017) (control group) using the Mann-Whitney U test. RESULTS: A total of 55 arteries were embolized in 17 patients (mean 3.23 arteries per patient). Technical success could be achieved in all patients without any procedure-related complications. The most common arteries embolized were posterior intercostal arteries (29) followed by costocervical trunk (10), superior thoracic (8), lateral thoracic (5), and internal thoracic arteries (3). The mean blood loss was significantly lower in the embolization group compared with the control group (676.47 vs. 1,264.58 mL, p = 0.015). Within the embolization group, patients who underwent video-assisted thoracoscopic surgery had even lower blood loss compared with open surgery cases (466 vs. 887.50 mL, p-value = 0.046). CONCLUSION: PCCE is a simple and safe procedure, useful in reducing intra-operative blood loss in patients undergoing surgery for peripherally located PA.

Case 283: Breast Filariasis.

History A 44-year-old woman who was a resident of Bihar, which is a state in eastern India, presented to the surgical outpatient department of our hospital with a history of gradually increasing swelling of the right breast associated with redness, pain, and itching over the past month. She reported a general sense of malaise and experienced episodes of chills over the past 6 months; however, she had no documented fever. There was no history of breast trauma. No history suggestive of a possible hypercoagulable state could be elicited (she was a nonsmoker, had undergone uncomplicated normal vaginal delivery 15 years earlier, was not taking oral contraceptives, and had no history to suggest past deep venous thrombosis). General physical examination findings were unremarkable. On local examination, she was found to have diffuse enlargement of the right breast. The skin over the lateral part of the breast was erythematous and showed the presence of prominent superficial veins. On palpation, few ill-defined firm mobile masses were found in the upper outer quadrant with overlying skin induration. No skin ulceration or nipple discharge was present. Few firm and discrete lymph nodes were palpable in the right axilla. Laboratory investigations showed mild anemia (hemoglobin level, 10 g/dL; normal range, 12-15 g/dL), a total leukocyte count of 14 000 cells per microliter (14 cells × 109/L) (normal range, 4500-11 000 cells per microliter [4.5-11 cells × 109/L]), a normal differential leukocyte count (74% neutrophils [normal range, 40%-80%], 24% lymphocytes [normal range, 20%-40%], and 2% eosinophils [normal range, 1%-4%]), and an erythrocyte sedimentation rate of 31 mm per hour (normal range, 0-29 mm per hour). She underwent both mammography and US. Real-time US showed mobile structures on the series of US images obtained seconds apart. On the basis of the imaging findings, US-guided fine-needle aspiration cytology was performed to confirm the diagnosis, and appropriate treatment was instituted.

Case 283.

History A 44-year-old woman who was a resident of Bihar, which is a state in eastern India, presented to the surgical outpatient department of our hospital with a history of gradually increasing swelling of the right breast associated with redness, pain, and itching over the past month (Fig 1). She reported a general sense of malaise and experienced episodes of chills over the past 6 months; however, she had no documented fever. There was no history of breast trauma. No history suggestive of a possible hypercoagulable state could be elicited (she was a nonsmoker, had undergone uncomplicated normal vaginal delivery 15 years earlier, was not taking oral contraceptives, and had no history to suggest past deep venous thrombosis). General physical examination findings were unremarkable. On local examination, she was found to have diffuse enlargement of the right breast. The skin over the lateral part of the breast was erythematous and showed the presence of prominent superficial veins. On palpation, few ill-defined firm mobile masses were found in the upper outer quadrant with overlying skin induration. No skin ulceration or nipple discharge was present. Few firm and discrete lymph nodes were palpable in the right axilla. Laboratory investigations showed mild anemia (hemoglobin level, 10 g/dL; normal range, 12-15 g/dL), a total leukocyte count of 14 000 cells per microliter (14 cells × 109/L) (normal range, 4500-11 000 cells per microliter [4.5-11 cells × 109/L]), a normal differential leukocyte count (74% neutrophils [normal range, 40%-80%], 24% lymphocytes [normal range, 20%-40%], and 2% eosinophils [normal range, 1%-4%]), and an erythrocyte sedimentation rate of 31 mm per hour (normal range, 0-29 mm per hour). She underwent both mammography (Fig 2) and US (Fig 3). Real-time US showed mobile structures on the series of US images obtained seconds apart (Fig 4). On the basis of the imaging findings, US-guided fine-needle aspiration cytology was performed to confirm the diagnosis, and appropriate treatment was instituted.

Can Diffusion Weighted Imaging Aid in Differentiating Benign from Malignant Sinonasal Masses?: A Useful Adjunct.

BACKGROUND: To evaluate the role of diffusion weighted imaging (DWI) and apparent diffusion coefficient (ADC) values at 3 Tesla in characterizing sinonasal masses. MATERIAL/METHODS: After ethical clearance, 79 treatment naive patients with head and neck masses underwent magnetic resonance imaging (MRI), including DWI at 3 Tesla using the following b values - 0, 500 and 1000 s/mm2. Thirty-one patients were found to have sinonasal tumours and were subsequently analysed. Image analysis consisted of a morphological evaluation of conventional MR images, qualitative evaluation of DW trace images and quantitative assessment of mean ADC values. Receiver operating characteristic (ROC) curve was drawn to determine a cut-off ADC value for the differentiation between benign and malignant masses. RESULTS: Sinonasal masses showed an overlapping growth pattern on conventional imaging, irrespective of their biological nature. The mean ADC value of benign lesions was 1.948±0.459×10-3 mm2/s, while that of malignant lesions was 1.046±0.711×10-3 mm2/s, and the difference was statistically significant (p=0.004). When a cut-off ADC value of 1.791×10-3 mm2/s was used, sensitivity of 80% and specificity of 83.3% were obtained for characterization of malignant lesions, which was statistically significant. Juvenile nasopharyngeal angiofibroma (JNA) showed distinctly high ADC values, while meningioma was the only benign lesion with restricted diffusion. Atypical entities with unexpected diffusion characteristics included: adenocarcinoma, adenoid cystic carcinoma, meningioma, chondrosarcoma and fibromyxoid sarcoma. CONCLUSIONS: DWI in conjunction with conventional imaging can potentially enhance the diagnostic accuracy in characterizing sinonasal masses as benign or malignant. Some specific entities such as JNA and meningioma showed distinctive diffusion characteristics.

Hypophosphatemic osteomalacia due to cadmium toxicity in silverware industry: A curious case of aches and pains.

Hypophosphatemic osteomalacia in an adult often gives clinical diagnostic challenges. Usually, they are caused by either tumor-induced osteomalacia or due to genetically mediated hypophosphatemia, particularly X-linked hypophosphatemia. However, heavy metal toxicity, leading to global proximal renal tubular dysfunction, is a rare cause, and in particular, cadmium toxicity is rarely encountered in clinical practice. The presence of bony pain and neurological deficit, along with a classical exposure history, provides the diagnostic clue. In this background, here we present a middle-aged man who had severe bony pains all over his body and lower back stiffness for five years. He underwent an initial workup as a suspected spondyloarthropathy but was later on, found to have hypophosphatemic osteomalacia and severe proximal renal tubular dysfunction. Further, the workup revealed elevated FGF-23. His occupational history revealed prolonged exposure to cadmium fumes in the silverware industry. He improved moderately with treatment; however, significant renal damage is still present. This case highlights the importance of considering cadmium toxicity in proper clinical and occupational contexts in the evaluation of hypophosphatemic osteomalacia in an adult.

Biomass Fuel Exposure and Lungs: Unraveling the Imaging Conundrum.

Prolonged exposure to biomass fuel smoke is a proven irritant, known to aggravate chronic lung diseases. Of the myriad spectrum of thoracic manifestations associated with inhalation of biomass fuel smoke, bronchial anthracofibrosis is a recently described entity characterized by bronchial narrowing and visible anthracotic pigmentation on bronchoscopy. Common imaging features include bronchostenosis, peribronchial soft tissue with or without calcification along with peribronchial lymph nodes. Its close similarity to endobronchial tuberculosis and bronchogenic carcinoma in clinical presentation and imaging poses a diagnostic challenge and hence underlines the importance of knowledge about this entity. This review aims to summarize the key imaging features of bronchial anthracofibrosis while also briefly discussing the spectrum of thoracic manifestations including distinct entities associated with biomass fuel smoke exposure.

Clinicoradiologic predictors of sclerotherapy response in low-flow vascular malformations.

OBJECTIVE: To evaluate the clinical effectiveness of sclerotherapy agents in low-flow vascular malformations (LFVM) and identify clinical/imaging features to predict response. METHODS: A retrospective analysis of hospital records of symptomatic LFVM patients who underwent phlebosclerotherapy from January 2015 to April 2018 was done. Patients were subdivided into venous malformations (VM) and lymphatic malformations (LM). Out of 246 cases, 223 patients (132 males, 91 females; age range, 2-52 years) had VM and 23 (13 males, 10 females; age range, 3 months to 45 years) had LM. The clinical response was graded as excellent (>60%), good (30%-60%), and poor (<30%). More than 30% was considered as acceptable response. The χ2 test was performed for correlation between clinical response and clinical, sonographic, magnetic resonance imaging, phlebographic parameters followed by multilinear regression. RESULTS: Cavitary (43%) and spongy (37.7%) were the most common phlebographic patterns seen among VM and a cavitary pattern (87%) was most frequent in LM. Sodium tetradecyl sulphate and bleomycin were most commonly used sclerosants in VM and LM, respectively. The mean number of sessions was 4.35 (range, 1-23) in VM and 2.64 (range, 2-7) in LM. Among VM, 114 patients (51.1%) had excellent response to treatment (>60%) and 75.8% patients had an acceptable response (>30%). All patients with LM had an acceptable response (excellent response in 86.9%). Clinical disfigurement, discoloration, diffuse involvement, dysplastic venous morphology on phlebogram, and late and indirect draining vein correlated with poor response to sclerotherapy in VM (P = .003, P = .036, P = .007, P = .008, P = .003, and P = .035, respectively). Cystic components on ultrasound examination and direct draining vein were seen more often in excellent responders (P = .004 and P = .007) in addition to absence of disfigurement, discoloration, and diffuse involvement (P = .032, P = .003, and P = .002). Mod els comprising clinical disfigurement, dysplastic veins, and late draining vein had the greatest predictive value for poor response (R2 = 0.256). Also, the best model for predicting excellent response comprised presence of direct draining vein and absence of skin discoloration (R2 = 0.109). Eleven instances of minor complications occurred among a total of 1032 sessions, seven with sodium tetradecyl sulphate and four with polidocanol. CONCLUSIONS: Acceptable response to sclerotherapy was achieved in majority of LFVM with extremely low complication rates. Clinicoradiologic features, especially phlebographic findings, correlated with response to sclerotherapy.

A case of an 81-year-old with cough and dyspnea.

It is uncommon to diagnose usual interstitial pneumonitis as a unilateral presentation. We present a case of an 81-year-old current smoker who presented with exertional dyspnea and dry cough. The patient had right sided UIP pattern in the CT chest along with hiatus hernia. The etiology for the unilateral lung involvement was postulated to be due to the hiatus hernia leading to gastro-esophageal reflux disease (GERD) which caused micro aspirations leading to lung injury and fibroblast activation. Whether this can be prevented by anti-reflux medications needs further research. Our patient was managed with pirfenidone, metered dose inhalers containing tiotropium and proton-pump inhibitors Thus, a high index of suspicion for underlying gastro-esophageal reflux must be kept in such patients to arrive at an early diagnosis and start treatment.

A 22-Year-Old Man With Back Pain, Dilated Veins Over Chest, and Mass in Right Atrium.

CASE PRESENTATION: A 22-year-old nonsmoker male, without any previous comorbidity, presented with 4 months' history of right upper back pain. Pain was constant dull aching type, nonpleuritic, aggravated by lying on the right lateral side and partially relieved on taking analgesics. He then developed progressive dyspnea over 2 months and noticed dilated veins over his neck and front of chest. There was associated unintentional weight loss of 6 kg. There was no history of cough, expectoration, wheeze, or hemoptysis, nor any episodes of night sweats or fever.

Imaging protocols for CT chest: A recommendation.

Computed Tomography (CT) is the mainstay of diagnostic imaging evaluation of thoracic disorders. However, there are a number of CT protocols ranging from a simple non-contrast CT at one end of the spectrum, and CT perfusion as a complex protocol available only on high-end scanners. With the growing diversity, there is a pressing need for radiologists, and clinicians to have a basic understanding of the recommended CT examinations for individual indications. This brief review aims to summarise the currently prevalent CT examination protocols, including their recommended indications, as well as technical specifications for performing them.

Orbital Imaging Manifestations of Neurocutaneous Syndromes Revisited.

Neurocutaneous syndromes or phakomatoses represent a heterogeneous group of multisystemic disorders involving structures of ectodermal origin. Characteristic ocular manifestations are described for individual entities that are often the first clues to the underlying diagnosis. However, opaque ocular media or involvement of retrobulbar orbit limits adequate clinical evaluation. This underlines the role of imaging, especially cross-sectional imaging modalities, such as computed tomography and magnetic resonance imaging, which offer a comprehensive evaluation of orbit and its contents. This review aims to summarize the cross-sectional imaging features of orbital manifestations of common neurocutaneous syndromes encountered in clinical practice.

Benign neck masses showing restricted diffusion: Is there a histological basis for discordant behavior?

Diffusion weighted imaging (DWI) evolved as a complementary tool to morphologic imaging by offering additional functional information about lesions. Although the technique utilizes movement of water molecules to characterize biological tissues in terms of their cellularity, there are other factors related to the histological constitution of lesions which can have a significant bearing on DWI. Benign lesions with atypical histology including presence of lymphoid stroma, inherently increased cellularity or abundant extracellular collagen can impede movement of water molecules similar to malignant tissues and thereby, show restricted diffusion. Knowledge of these atypical entities while interpreting DWI in clinical practice can avoid potential misdiagnosis. This review aims to present an imaging spectrum of such benign neck masses which, owing to their distinct histology, can show discordant behavior on DWI.

Diffusion-weighted imaging in extracranial head and neck schwannomas: A distinctive appearance.

PURPOSE: To evaluate the diffusion weighted (DW) magnetic resonance imaging (MRI) features of the extracranial schwannomas of head and neck. MATERIALS AND METHODS: The MRI (including DWI) of 12 patients with pathologically proven head and neck schwannomas (4 men, 8 women, with mean age of 32.6 years; age range 16-50 years) were retrospectively evaluated. Images were analyzed for signal intensity and morphology on conventional sequences followed by the qualitative evaluation of DW images (DWI) and measurement of apparent diffusion coefficient (ADC) values. RESULTS: Majority of the tumors were located in the parapharyngeal space (8/12). All but one showed heterogeneous appearance, with 10 tumors showing scattered areas of hemorrhage. Eight out of 12 tumors showed intensely hyperintense core surrounded by intermediate signal intensity peripheral rim (reverse target sign) on T2-weighted (T2W) images. On DWI, these eight tumors showed a distinctive appearance, resembling target sign on trace DWI and reverse target on ADC map. Out of the remaining four tumors, one showed uniformly restricted diffusion whereas three showed free diffusion. Mean ADC value in the central area of free diffusion was 2.277 × 10(-3) mm(2)/s (range of 1.790 × 10 -(3) to 2.605 × 10(-3) mm(2)/s) whereas in the peripheral area was 1.117 × 10(-3) mm(2)/s (range of 0.656 × 10(-3) to 1.701 × 10(-3) mm(2)/s). Rest of the schwannomas showing free diffusion had a mean ADC value of 1.971 × 10(-3) mm(2)/s. CONCLUSION: Majority of the head and neck schwannomas showed a characteristic appearance of free diffusion in the centre and restricted diffusion in the periphery of the mass.