Imaging in Erdheim-Chester Disease.

Erdheim-Chester disease (ECD) is a rare, multisystemic, inflammatory, non-Langerhans cell histiocytic neoplasm. The discovery of recurrent and somatic mutations in the mitogen-activated protein kinase signaling pathway, most commonly BRAFV600E, has led to a reclassification of ECD from an inflammatory disorder to a neoplastic process. It is now included in the revised 2016 World Health Organization classification of hematopoietic tumors and in the Langerhans group in the revised 2016 Histiocytosis Classification of the Histiocyte Society. When symptomatic, ECD most commonly manifests with bone pain and fatigue. Also, neurologic manifestations, central diabetes insipidus, exophthalmos, and periorbital xanthelasma-like lesions are frequently encountered. Pathologic findings may vary depending on the site of biopsy and may display a spectrum of features. Thus, due to the diverse clinical presentation and variable histologic findings, imaging can often show the first sign of the disease. Radiologic findings are, however, interpreted in conjunction with clinical and histologic findings to establish the diagnosis of ECD. From providing classic findings that facilitate diagnosis to helping radiologists determine the extent of disease and predicting a prognosis, the role of radiology in ECD has evolved with the understanding of the disease itself. Insights into the molecular pathogenesis and the development of targeted therapeutic agents along with approval of vemurafenib and cobimetinib have necessitated revision of the guidelines for the management of ECD. The authors discuss various radiologic findings of ECD and differential diagnoses by using an organ system-based approach and briefly describe the revised consensus recommendations for evaluation, diagnosis, and treatment based on the International Medical Symposia on ECD from a radiologist's perspective. ©RSNA, 2024 Supplemental material is available for this article. The full digital presentation is available online.

Radiation exposure during sacral neuromodulation lead placement: Multi-institutional descriptive study.

OBJECTIVES: Fluoroscopy has significantly improved lead placement and decreased surgical time for implantable sacral neuromodulation (SNM). There is a paucity of data regarding radiation and safety of fluoroscopy during SNM procedures. Our study aims to characterize fluoroscopy time and dose used during SNM surgery across multiple institutions and assess for predictors of increased fluoroscopy time and radiation dose. METHODS: Electronic medical records were queried for SNM procedures (Stage 1 and full implant) from 2016 to 2021 at four academic institutions. Demographic, clinical, and intraoperative data were collected, including fluoroscopy time and radiation dose in milligray (mGy). The data were entered into a centralized REDCap database. Univariate and multivariate analysis were performed to assess for predictive factors using STATA/BE 17.0. RESULTS: A total of 664 procedures were performed across four institutions. Of these, 363 (54.6%) procedures had complete fluoroscopy details recorded. Mean surgical time was 58.8 min. Of all procedures, 79.6% were performed by Female Pelvic Medicine and Reconstructive Surgery specialists. There was significant variability in fluoroscopy time and dose based on surgical specialty and institution. Most surgeons (76.4%) were considered "low volume" implanters. In a multivariate analysis, bilateral finder needle testing, surgical indication, surgeon volume, and institution significantly predicted increased fluoroscopy time and radiation dose (p < 0.05). CONCLUSIONS: There is significant variability in fluoroscopy time and radiation dose utilized during SNM procedures, with differences across institutions, surgeons, and subspecialties. Increased radiation exposure can have harmful impacts on the surgical team and patient. These findings demonstrate the need for standardized fluoroscopy use during SNM procedures.

Large Solitary Pulmonary Cryptococcoma Mimicking Lung Carcinoma in an Immunocompetent Patient.

Cryptococcosis is a life-threatening mycosis typically seen in immunocompromised patients. Pulmonary cryptococcosis generally presents as multiple or solitary nodular opacities. Cryptococcal infection presenting as a destructing cavernoma (cryptococcoma) without diffuse infiltration of the lung is an extremely rare presentation, even in immunocompromised patients. This report presents a healthy, HIV negative, immunocompetent patient who presented with a large solitary lung mass provisionally diagnosed as a lung malignancy on radiological imaging that proved to be a large cryptococcoma after biopsy. The patient was treated with liposomal Amphotericin B and fluconazole, and the lesion showed regression on serial imaging. This case report thus highlights an unconventional presentation of pulmonary cryptococcosis in an immunocompetent individual.

Skeletal Metastasis From Carcinoma of the Gall Bladder: Need for Bone Scintigraphy Justified?

BACKGROUND: Carcinoma of the gall bladder has a guarded prognosis with predominant sites of involvement being liver and regional nodes. Osseous metastasis in carcinoma of the gall bladder is rare and hence bone scintigraphy does not form a part of the routine work-up for such patients. CASE REPORTS: We describe two patients with carcinoma of the gall bladder with osteolytic metastasis (stage 4). Conservative treatment was planned but both of them succumbed to the illness. CONCLUSIONS: We thus highlight the importance of performing a bone scan or PET CT in cases of carcinoma of the gall bladder. Besides, our cases challenge Paget's seed - soil theory for sites of metastasis.

Atraumatic Main-En-Griffe due to Ulnar Nerve Leprosy.

BACKGROUND: Leprosy is the most common form of treatable peripheral neuropathy. However, in spite of effective chemotherapeutic agents, neuropathy and associated deformities are seldom ameliorated to a significant extent. This necessitates early diagnosis and treatment. Clinical examination of peripheral nerves is highly subjective and inaccurate. Electrophysiological studies are painful and expensive. Ultrasonography circumvents these demerits and has emerged as the preferred modality for probing peripheral nerves. CASE REPORT: We describe a 23-year-old male who presented with weakness and clawing of the medial digits of the right hand (main-en-griffe) and a few skin lesions since eighteen months. The right ulnar nerve was thickened and exquisitely tender on palpation. Ultrasonography revealed an extensive enlargement of the nerve with presence of intraneural color Doppler signals suggestive of acute neuritis. Skin biopsy was consistent with borderline tuberculoid leprosy with type 1 lepra reaction. The patient was started on WHO multidrug therapy for paucibacillary leprosy along with antiinflammatory drugs. Persistence of vascular signals at two months' follow-up has led to continuation of the steroid therapy. The patient is compliant with the treatment and is on monthly follow-up. CONCLUSIONS: In this manuscript, we review multitudinous roles of ultrasonography in examination of peripheral nerves in leprosy. Ultrasonography besides diagnosing enlargement of nerves in leprosy and acute neuritis due to lepra reactions, guides the duration of anti-inflammatory therapy in lepra reactions. Further, it is relatively inexpensive, non-invasive and easily available. All these features make ultrasonography a preferred modality for examination of peripheral nerves.

Wolffian Origin of Vagina Unfolds the Embryopathogenesis of OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) Syndrome and Places OHVIRA as a Female Counterpart of Zinner Syndrome in Males.

BACKGROUND: The classical theory of Müllerian origin of upper vagina fails to explain complex urogenital malformations like OHVIRA syndrome; the Acien's hypothesis, however, unravels the hidden embryopathogenesis. As per Acien, Wolffian (mesonephric) ducts instead of Müllerian ducts and sinovaginal bulbs, give rise to the vagina. The new hypothesis, however, retains the concept of origin of the ureters (with ureters inducing renal development) by the former and the uterus by Müllerian ducts. Thus, a failure of development of mesonephros/mesonephric duct gives rise to absent ureters and hence absent homolateral kidney; blind ending (obstructed) ipsilateral hemivagina and cessation of support to paramesonephric ducts which leads to unfused uterus (uterus didelphys). Hence, the new hypothesis explains all components of OHVIRA syndrome. On a parallel track, unilateral anomalous development of the mesonephros in males causes atresia of the homolateral ejaculatory duct that results in obstruction of the proximally placed seminal vesicle. Besides, there is absence of the ipsilateral kidney (Zinner syndrome). CASE REPORT: In this manuscript, we describe four cases of OHVIRA syndrome. Case 1 was a 34-year-old nulligravida, married since fourteen years, who presented with a 5-month history of pelvic inflammatory disease and dyspareunia. Regular menses in the patient and azoospermia in her husband delayed the diagnosis. Case 2 was a 14-year-old girl who presented with dysmenorrhea and lower abdominal pain since a few months. Case 3 was a 27-year-old female who presented with infertility and dysmenorrhea. Case 4 was a 15-year-old female who presented with a one-year history of dysmenorrhea and cyclic pelvic pain. In all cases, one of the uterine horns revealed collection due to a hemivaginal septum and an absent ipsilateral kidney; thus, establishing the diagnosis of OHVIRA syndrome. The case 4 additionally revealed homolateral vaginal agenesis. CONCLUSIONS: On the basis of our 4 cases, we support the Acien's hypothesis of Wolffian origin of vagina which explains the development of OHVIRA syndrome. Besides, we emphasize the need to suspect this syndrome in a female with a pelvic mass and absence of homolateral kidney. Finally, we believe that OHVIRA due to its Wolffian origin is a female equivalent of Zinner syndrome in males. Therefore, we propose OSVIRA (Obstructed Seminal Vesicle and Ipsilateral Renal Agenesis) as an acronym for Zinner syndrome analogous to OHVIRA.

Juvenile Granulosa Cell Tumour of the Ovary with Unilocular Pure Cystic Presentation: A Case Report and Review of Literature.

BACKGROUND: Granulosa cell tumours of the ovary are rare, hormonally active, oestrogen-secreting tumours of the ovary existing in two forms: the adult form and the even rarer juvenile form. These tumours present as predominantly solid lesions while the cystic, unilocular presentation is uncommon. CASE REPORT: We present an 18-year-old unmarried girl who presented with complaints of chronic pain, abdominal distension and presence of facial hair. Radiological examination revealed a large, purely cystic, unilocular lesion without any solid components, debris or septations. Histopathological diagnosis was of a juvenile granulosa cell tumour. CONCLUSIONS: Radiological criteria suggestive of malignant ovarian masses include thick, irregular walls and septae; papillary projections and solid, echogenic foci. Nonetheless, we propose that a malignant ovarian lesion should be included in the differential diagnosis of a unilocular, purely cystic ovarian lesion.

Venous complications of pancreatitis: a review.

Pancreatitis is notorious to cause vascular complications. While arterial complications include pseudoaneurysm formation with a propensity to bleed, venous complications can be quite myriad. Venous involvement in pancreatitis often presents with thrombosis. From time to time case reports and series of unusual venous complications associated with pancreatitis have, however, been described. In this article, we review multitudinous venous complications in the setting of pancreatitis and propose a system to classify pancreatitis associated venous complications.

Pancreaticopleural fistula: a review.

Pancreaticopleural fistula is a rare complication of chronic pancreatitis consequent to posterior disruption of the pancreatic duct. The fistulous track ascends into the pleural cavity and gives rise to large volumes of pleural fluid. Pancreaticopleural fistula thus poses a diagnostic problem since the source of pleural fluid is extrathoracic. To further complicate the matter, abdominal pain is seldom the presenting or significant feature. The pleural effusion is typically rapidly accumulating, recurrent and exudative in nature. Pleural fluid amylase in the correct clinical setting virtually clinches the diagnosis. Magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography and computed tomography may delineate the fistula and thus aid in diagnosis. Endoscopic retrograde cholangiopancreatography has emerged both as a diagnostic as well as therapeutic modality in select patients of pancreaticopleural fistula while magnetic resonance cholangiopancreatography is the radiological investigation of choice. Besides delineating the ductal anatomy, magnetic resonance cholangiopancreatography can help stratify patients for appropriate management. A near normal or mildly dilated pancreatic duct responds well to chest drainage with octreotide while endoscopic stent placement benefits patients with duct disruption located in head or body of pancreas. Failure of medical or endoscopic therapy calls in for surgical intervention. Besides, a primary surgical management may be tried in patients with complete ductal obstruction, ductal disruption in tail or ductal obstruction proximal to fistula site.

Wandering spleen with torsion causing pancreatic volvulus and associated intrathoracic gastric volvulus. An unusual triad and cause of acute abdominal pain.

CONTEXT: Wandering spleen is a rare medical entity in which the spleen is orphaned of its usual peritoneal attachments and thus assumes an ever wandering and hypermobile state. This laxity of attachments may even cause torsion of the splenic pedicle. Both gastric volvulus and wandering spleen share a common embryology owing to maldevelopment of the dorsal mesentery. Gastric volvulus complicating a wandering spleen is, however, an extremely unusual association, with a few cases described in literature. CASE REPORT: We present a case of a young female who presented with acute abdominal pain and vomiting. Radiological imaging revealed a intrathoracic gastric volvulus, torsion in an ectopic spleen, and additionally demonstrated a pancreatic volvulus - an unusual triad, reported only once, causing an acute abdomen. CONCLUSION: The patient subsequently underwent an emergency surgical laparotomy with splenopexy and gastropexy.

Spinal Hydatid as a Rare Cause of Posterior Mediastinal Lesion: Understanding Cervicothoracic Sign on Chest Radiography.

BACKGROUND: Location of an intrathoracic lesion on chest radiograph is facilitated by application of 'silhouette sign'. This helps narrow down the differential diagnoses. The list of probable diagnoses reduces further on determination of the density of the lesion. A spinal hydatid presents as a fluid-density posterior mediastinal lesion on chest radiograph with destruction of the vertebral body and preservation of the disc space. Spinal hydatid is, however, rare. CASE REPORT: We describe a case of a 30-year-old female with gradual-onset paraperesis since six months. Chest radiograph was suggestive of a posterior mediastinal lesion with fluid density and destruction of D4 vertebra. MRI findings were consistent with spinal hydatid. The patient was started on perioperative benzimidazole therapy with resection of the hydatid cyst. The drug therapy was continued for six months post-operatively. CONCLUSIONS: A chest radiograph helps localise the site and possible contents of the lesion. It also guides further investigations. MRI is the imaging modality of choice for spinal pathologies causing cord compression including spinal hydatid. Echinococcal involvement of the spine is a rarity but needs to be considered in the differential diagnoses for spinal causes of gradual-onset paraperesis.

Renal Cysts and Nephrocalcinosis in a Patient Deficient in 11 beta-Hydroxylase Enzyme.

BACKGROUND: Chronic hypokalemia is known to induce renal structural and functional abnormality. The former includes induction of renal cyst formation and interstitial fibrosis while the latter entails urine-concentrating defect. However, these hypokalemia-mediated changes occur in a handful of conditions including primary aldosteronism, distal renal tubular acidosis, Liddle's disease, apparent mineralocorticoid excess syndrome and Bartter's type 3 syndrome. Such a finding has never been described in an 11 beta-hydroxylase deficient individual. CASE REPORT: We describe a case of a 15-year-old male, deficient in 11 beta-hydroxylase enzyme, presenting with hypertensive haemorrhage in basal ganglia and chronic hypokalemia-mediated nephrocalcinosis and renal cysts. To add to the uniqueness, our patient was discovered to harbour bilateral testicular adrenal rests as well. CONCLUSIONS: An early diagnosis could help prevent these sequelae and preserve long-term renal function and safeguard against ill-effects of hypertension. Besides, aetiology of nephrocalcinosis should be sought for and corrected.

Abdominal CT findings characteristic of Castleman disease: multi-centre review of 76 adult cases with abdominopelvic nodal involvement.

OBJECTIVE: Characterize the CT findings of abdominopelvic Castleman disease, including a new observation involving the perinodal fat. METHODS: Multi-centre search at 5 institutions yielded 76 adults (mean age, 42.1 ± 14.3 years; 38 women/38 men) meeting inclusion criteria of histopathologically proven Castleman disease with nodal involvement at abdominopelvic CT. Retrospective review of the dominant nodal mass was assessed for size, attenuation, and presence of calcification, and for prominence and soft-tissue infiltration of the perinodal fat. Hypervascular nodal enhancement was based on both subjective and objective comparison with aortic blood pool attenuation. RESULTS: Abdominal involvement was unicentric in 48.7% (37/76) and multicentric in 51.3% (39/76), including 31 cases with extra-abdominal involvement. Histopathologic subtypes included hyaline vascular variant (HVV), plasma cell variant (PCV), mixed HVV/PCV, and HHV-8 variant in 39, 25, 3 and 9 cases, respectively. The dominant nodal mass measured 4.4 ± 1.9 cm and 3.2 ± 1.7 cm in mean long- and short-axis, respectively, and appeared hypervascular in 58.6% (41/70 with IV contrast). Internal calcification was seen in 22.4% (17/76). Infiltration of the perinodal fat, with or without hypertrophy, was present in 56.6% (43/76), more frequent with hypervascular vs non-hypervascular nodal masses (80.5% vs 20.7%; P < .001). Among HVV cases, 76.9% were unicentric, 71.1% appeared hypervascular, and 69.2% demonstrated perinodal fat infiltration. CONCLUSION: Hypervascular nodal masses demonstrating prominence and infiltration of perinodal fat at CT can suggest the specific diagnosis of Castleman disease, especially the HVV. ADVANCES IN KNOWLEDGE: Abdominopelvic nodal masses that demonstrate hypervascular enhancement and prominent infiltration of the perinodal fat at CT can suggest the diagnosis of Castleman disease, but nonetheless requires tissue sampling.

Comprehensive review of clinical presentation, diagnosis, management, and prognosis of ruptured hepatocellular carcinoma.

BACKGROUND: Spontaneous rupture of hepatocellular carcinoma (rHCC) is a life-threatening complication that occurs in 3 % to 15 % of patients with hepatocellular carcinoma (HCC). This review aimed to discuss the most recent updates in the epidemiology, pathophysiology, risk factors, diagnosis as well as presentation, management, and prognostic factors of rHCC. METHODS: A comprehensive systematic review was conducted using Medline/PubMed and Web of Science databases with the end of search date being December 1, 2023 regarding rHCC diagnosis, imaging, and management. RESULTS: Achieving adequate hemostasis and stabilization of the patient remains the primary objective in the management of patients with rHCC. In earlier studies, the mortality rate in the acute phase of rHCC was reported to be 25 % to 75 %. However, more recent studies have demonstrated that transcatheter arterial embolization (TAE)/transcatheter arterial chemoembolization (TACE) followed by elective hepatectomy in select patients may offer improved survival benefits and decrease perioperative complications compared with TAE/TACE alone or emergent/1-stage hepatectomy. CONCLUSION: Although the prognosis for rHCC remains the worst among causes of death related to HCC, more recent studies have demonstrated that improved short- and long-term patient outcomes may be achieved through active surveillance efforts for HCC combined with advanced multimodal diagnostic tools and multidisciplinary management strategies.

Intra-abdominal Venous Thromboses and Their Management.

While a plethora of articles discuss management of deep venous thromboses in extremities, there is a relative scarcity of literature comprehensively describing intra-abdominal venous thromboses, and their management. Intra-abdominal venous thromboses include iliocaval venous obstruction (ICVO), hepatic venous thrombosis (HVT), portal venous thrombosis (PVT), renal vein thrombosis (RVT), splenic vein thrombosis (SVT), and gonadal vein thrombosis (GVT); each of which provides unique microenvironmental challenges to management. Doppler ultrasound is the first line imaging modality for diagnosis, and computed tomography and magnetic resonance imaging can help define the extent of thrombus burden and aid with interventional planning. Systemic anticoagulation remains the common medical treatment for intra-abdominal venous thrombosis, however, catheter directed thrombolysis and thrombectomy show positive outcomes in ICVO, HVT, PVT, and RVT, with transjugular intrahepatic portosystemic shunt (TIPS) creation especially beneficial in HVT and PVT. In this review article, we describe pathophysiology, clinical features, imaging findings, and current management options for intra-abdominal venous thromboses.

Off the wall: incidental paraspinal and pelvic muscle pathology on abdominopelvic imaging.

As the use of cross-sectional abdominal and pelvic imaging has increased exponentially in the past several decades, incidental musculoskeletal findings have become commonplace. These are often unrelated to the indication for the examination and are frequently referred to as the "radiologist's blind spot" on these studies. The differential diagnosis for abnormalities of the paraspinal and pelvic musculature is, in many cases, quite different from the anterior abdominal wall muscles. Furthermore, due to their relatively deep location, pathology involving the former muscle groups is more likely to be clinically occult, often presenting only incidentally when the patient undergoes cross-sectional imaging. Effective treatment of diseases of these muscles is dependent on adherence to a diverse set of diagnostic and treatment algorithms. The purpose of this review article is to familiarize the radiologist with the unique pathology of these often-overlooked muscles of the abdomen and pelvis.

Secondary varicocele caused by pancreatic pseudocyst obstructing testicular venous drainage.

A pseudocyst is a fluid/debris collection that occurs as a complication of pancreatitis. It can be symptomatic and cause compression of the surrounding structures. Our case report highlights a 29-year-old male who presented with secondary varicocele on left side caused by a huge pseudocyst seen to compress the left renal and testicular veins.

Banana in a Condom: An Unusual Cause of Small Bowel Obstruction.

An otherwise healthy, 34-year-old man presented to the emergency department with abdominal pain, nausea, and vomiting, which began a day after he ingested a banana-stuffed condom. A contrast-enhanced computed tomography (CT) abdomen and pelvis revealed a dilated small bowel with a transition point, findings consistent with small bowel obstruction. Abdominopelvic ascites was also noted on imaging, which was concerning for bowel distress. He was taken to the operating room for an exploratory laparotomy, which revealed a high-grade obstruction by a foreign body, a banana stuffed in a condom, in the mid-jejunum. An enterotomy was performed to relieve the obstruction. The postoperative course was uneventful, and he was discharged three days later.

Multiple extrasplanchnic venous thromboses: a rare complication of pancreatitis. A case report.

CONTEXT: Venous thrombosis has been described in patients with acute and chronic pancreatitis. This is especially common in portal vein, splenic vein and superior mesenteric vein. To the best of our knowledge, involvement of superior vena cava and subclavian vessel due to pancreatitis has not been reported. CASE REPORT: We present here a case of an adult male with alcoholic chronic pancreatitis who presented with multiple vessel thromboses involving superior vena cava, inferior vena cava, bilateral subclavian, internal jugular vein, axillary, iliac and renal vein without involvement of portal, splenic and superior mesenteric vein that was effectively treated with i.v. anticoagulation therapy. CONCLUSION: Venous thromboses can occur outside the splanchnic circulation in pancreatitis.