Magnetic resonance imaging patterns and perfusion changes of posterior reversible encephalopathy syndrome in children with clinical outcome correlation.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) in children has a propensity towards atypical features on magnetic resonance (MR) imaging, with limited literature on perfusion changes and clinicoradiological correlation. OBJECTIVE: We aimed to comprehensively study MR imaging patterns of pediatric PRES, including cerebral blood flow variations on arterial spin labeling, and looked for any MR biomarkers of poor clinical outcome. MATERIALS AND METHODS: In this retrospective observational study conducted in a tertiary hospital setting, MR records over a 4-year period (May 2019 to May 2023) were systematically searched along with their clinical details. Patients with an age less than 18 years and a clinicoradiological constellation consistent with PRES were included. MR scans were analyzed by two neuroradiologists with 8 years' and 10 years' experience. Association was sought with poor clinical outcome (defined as modified Rankin Scale score at discharge of > 2). RESULTS: A total of 45 patients (29 boys) were included in the study, with a mean age (± standard deviation) of 11.19 (± 4.53) years. On MR imaging, 95.6% of patients (n = 43) showed atypical features and/or atypical areas of involvement. The superior frontal sulcus (n = 18) was the most predominant MR pattern, and cerebellar involvement was not uncommon (n = 15). Unilateral involvement (n = 3), isolated central pattern (n = 1), and spinal cord involvement (PRES-SCI: n = 1) were also encountered. Brainstem involvement (n = 4) showed a characteristic "V-sign" of anterior medullary hyperintensity. Patchy restricted diffusion (46.6%), punctate hemorrhages (37.7%), and leptomeningeal contrast enhancement (36%) were not uncommon. Arterial spin labeling sequence (available in 24 patients) showed increased cerebral blood flow in the involved areas in 79.2% of patients. Univariate analysis showed a significant association of the presence of hemorrhage (P = 0.003), involvement of brainstem (P = 0.007), deep white matter (P = 0.008), and thalamus (P = 0.026) with poor clinical outcome. Multivariate regression analysis found that hemorrhage on MRI (P = 0.011, odds ratio 8) was an independent factor associated with poor clinical outcome. CONCLUSIONS: The conventionally described atypical features in PRES are common in children and therefore may no longer be considered exceptions. Raised perfusion on arterial spin labeling sequence was seen in the majority of cases. Hemorrhage on MRI was an independent predictor of poor clinical outcome in pediatric PRES.

Dextromethorphan Associated Neurotoxicity with Cerebellar Edema (DANCE) Syndrome in Young Children: Neuroimaging Features.

Dextromethorphan toxicity in young children (especially those with age 4 years or younger) can have an extremely poor prognosis if untreated. However, if timely recognized and optimally managed, it can have a good clinical outcome despite significant initial insult. We present 3 pediatric cases (< 5 years old) with sudden unresponsiveness following ingestion of cough medications containing dextromethorphan. All these children showed cytotoxic edema in cerebellar hemispheres on MR brain, with diffusion restricting foci in supratentorial white matter in 2 patients. These features resemble the recently described acute opioid toxidrome in children, the POUNCE syndrome (Pediatric Opioid Use-associated Neurotoxicity with Cerebellar Edema). Hence, we name this entity "DANCE" (Dextromethorphan Associated Neurotoxicity with Cerebellar Edema) to increase the awareness of dextromethorphan toxicity in young children and the need to promptly recognize it to initiate optimal management.ABBREVIATIONS: POUNCE= Pediatric Opioid Use-associated Neurotoxicity with Cerebellar Edema; DANCE= Dextromethorphan Associated Neurotoxicity with Cerebellar Edema.

Clinico-Epidemiological Analysis of Small Bowel Obstruction in Adults at a Tertiary Care Center in India.

BACKGROUND: Acute small bowel obstruction (SBO) is a common surgical emergency. The study aims to provide a comprehensive clinical-epidemiological description of SBO in adults at a tertiary care center in western India. METHODS: This hospital-based cross-sectional study was conducted from July 2020 to June 2022 and enrolled 88 SBO patients requiring surgical intervention. After adequately resuscitating the patients, various surgical procedures were performed based on the intraoperative conditions of the bowel. Patients were assessed postoperatively for the duration of their hospital stay, postoperative complications, and surgical recovery. RESULTS: There was a male preponderance (n=55), with a median age of 50 (18-90) years. Abdominal discomfort was the most frequent symptom, necessitating a hospital visit (97.9%, n= 86), followed by nausea (85.2%, n= 75), constipation (78.1%, n=69), and abdominal distension (51.1%, n=45). Ileal strictures (18.2%, n=16) were the most common etiology, followed by postoperative adhesions (14.8%, n=13) and bands (13.6%, n=12), of which 76.4% (n=9) had past surgical history. Resection and anastomosis were the most frequently performed surgical interventions in this study (36.4%, n=32), followed by stoma creation (27.3%, n=24) and adhesiolysis (17%, n=15). The postoperative 30-day mortality of 11.36% (n=10) was noted, which could be ascribed to the elderly population with comorbidity, postoperative complications, and who required extended stay in the critical care unit. CONCLUSION: Benign ileal stricture was the most common cause of acute SBO in the emergency. Prompt and timely diagnosis combined with a multidisciplinary approach and effective management can improve outcomes and reduce morbidity and mortality in adult patients with SBO.

Aspergillus terreus pulmonary infection in a patient with late-onset combined immunodeficiency: a case report with literature review.

Common variable immunodeficiency (CVID) is the most common humoral immune deficiency in adults, characterized by recurrent sinopulmonary bacterial infections. Invasive fungal infections are rarely associated with CVID. Late-onset combined immunodeficiency (LOCID) is a recently recognized variant of CVID with low CD4 counts and immunoglobulins deficiency. The current study reveals the first documented case of invasive pulmonary aspergillosis (Aspergillus terreus) in a patient with LOCID. A 52-year-old female with a recurrent history of sinopulmonary infections presented with acute onset fever and shortness of breath. Blood culture and bronchoalveolar lavage culture grew A. terreus. Further evaluation revealed low immunoglobulins (IgG, IgM and IgA). Moreover, she also had low CD4 counts (<200 cells/µL). The patient was successfully treated with voriconazole and immunoglobulin therapy. Finally, the study discusses LOCID as a potential risk factor for invasive fungal infections, which can be easily overlooked and cause poor outcomes.

Primary hepatic diffuse large B- cell lymphoma mimicking cholangiocarcinoma.

Primary lymphoma of liver is a rare malignancy with non-specific clinical features and tumor markers. The presentation and imaging features may be indistinguishable from other hepatic malignant lesions. Pathological diagnosis is the gold standard, and early detection is essential to choose the treatment modality. Here, we share an interesting case of Primary Diffuse Large B cell lymphoma of liver and its imaging findings on Computed tomography (CT), Magnetic Resonance Imaging (MRI) and F-18 FDG PET/CT.

Outcomes after Embolization in Pediatric Abdominal Solid Organ Injury: A Trauma Center Experience.

Background Trauma is a significant cause of morbidity and mortality worldwide among children. Nonoperative management is the standard of care in hemodynamically stable children with blunt abdominal solid organ injury. Embolization is a potential pathway, which has shown increasing evidence for benefit in adult trauma patients. However, the data in children is limited. Materials and Methods A retrospective analysis of hospital data of all children (<18 years of age), presenting to a tertiary-care trauma center in India, with history of blunt trauma from January 2021 to June 2023, was performed. Preprocedural imaging, angiographic and embolization details, number of blood transfusions, and length of hospital stay were assessed. Results Two hundred and sixteen children (average age: 11.65 years) presented with a history of abdominal trauma during the study period. Eighty four children were FAST positive, out of whom, 67 patients had abdominal solid organ injury on computed tomography. Liver was the most commonly injured solid organ ( n = 45), followed by the spleen and kidney. Ten children had solid abdominal organ arterial injuries for which eight children underwent embolization. The average length of hospital stay in embolization group ( n = 8) was 4 days, as compared to 11 days in children undergoing operative management ( n = 2). At 6 months follow-up, all children were asymptomatic. Conclusion Superselective embolization is a safe and feasible procedure in appropriately selected children with abdominal injury.

Hepatopulmonary fusion in congenital diaphragmatic hernia: successful management of a lethal variant.

Congenital diaphragmatic hernia (CDH) is a congenital anomaly involving the herniation of intra-abdominal contents into the thoracic cavity. Hepatopulmonary fusion (HPF), an exceedingly rare subtype mainly associated with right-sided CDH, presents unique diagnostic and therapeutic challenges. This case report describes a male infant with right-sided CDH complicated by HPF. The intricate anatomical anomaly involved the fusion of the right lung to the liver, posing challenges during surgical separation. The patient experienced postoperative complications, including prolonged ventilation, tracheostomy and pulmonary issues, which led to a prolonged hospital stay. Intraoperative challenges stem from the absence of demarcation between lung and liver tissues and abnormal vascular structures. In summary, managing HPF in right-sided CDH necessitates a customised, multidisciplinary approach to optimise patient outcomes, highlighting the need for ongoing research to refine understanding and treatment strategies.

Absent or hypoplastic nasal bone: What to tell the prospective parents?

BACKGROUND: Absent or hypoplastic nasal bone (AHNB) on first or second-trimester ultrasonography (USG) is an important soft marker of Down syndrome. However, due to its varied incidence in euploid and aneuploid fetuses, there is always a dilemma of whether to go for invasive fetal testing for isolated AHNB. This study aims to assess outcomes specifically within the context of Indian ethnicity women. MATERIALS AND METHODS: This was a prospective observational study. All patients who reported with AHNB in the first- or second-trimester USG were included. Genetic counseling was done, and noninvasive and invasive testing was offered. Chromosomal anomalies were meticulously recorded, and pregnancy was monitored. RESULTS: The incidence of AHNB in our study was 1.16% (47/4051). Out of 47 women with AHNB, the isolated condition was seen in 32 (0.78%) cases, while AHNB with structural anomalies was seen in nine cases (0.22%). Thirty-nine women opted for invasive testing. Six out of 47 had aneuploidy (12.7%), while two euploid cases (4.25%) developed nonimmune hydrops. The prevalence of Down syndrome in fetuses with AHNB was 8.5% (4/47) and 0.42% (17/4004) in fetuses with nasal bone present. This difference was statistically significant (p = .001). CONCLUSION: The results indicate that isolated AHNB cases should be followed by a comprehensive anomaly scan rather than immediately recommending invasive testing. However, invasive testing is required when AHNB is associated with other soft markers or abnormalities. As chromosomal microarray is more sensitive than standard karyotype in detecting chromosomal aberrations, it should be chosen over karyotype.

Gallbladder perforation: Diagnostic accuracy of new CT difficulty score in predicting complicated laparoscopic cholecystectomy.

PURPOSE: To formulate and evaluate the diagnostic performance and utility of a new CT difficulty score in predicting difficult laparoscopic surgery in cases of gallbladder (GB) perforation. METHODS: This prospective single centre study included a total of 48 diagnosed cases of GB perforation on CT between December 2021 and June 2023, out of which 24 patients were operated. A new 6-point CT difficulty scoring system was devised to predict difficult laparoscopic approach, based on patterns of inflammation around the perforated GB that were found to be surgically relevant. The pre-operative imaging findings on CT were studied in detail and correlation coefficients of various imaging findings were calculated to predict difficult surgery. RESULTS: On CECT, the type of perforation, according to the revised Niemeier's classification could be exactly delineated in all 48 patients. A CT difficulty score of ≥ 3 was found to a good predictor difficult laparoscopic approach, with statistical significance (p = 0.001), sensitivity of 94.44%, specificity of 83.33%, PPV of 94.44% and NPV of 83.33%. Inflammatory changes around duodenum showed maximum correlation coefficient of 0.744 (p = 0.0001), around colon showed a correlation coefficient of 0.657 (p = 0.0005), and in the omentum had a correlation coefficient of 0.5 (p = 0.013)). Inter-observer agreement was also calculated for various findings and it was found to have moderate to strong agreement (κ value 0.5-1.0). CONCLUSION: The CT difficulty scoring system can be an effective tool in predicting difficult laparoscopic surgery in cases of GB perforation in an emergency setting which can help in decision making and improved patient outcome.

The Varieties of Ignorance: Imaging of Congenital Variants of Pancreas and Its Ductal System-A Pictorial Review.

Congenital variants of the pancreas are being increasingly detected with the widespread use of modern imaging techniques. The underlying embryologic aberration predicts the final appearance of pancreatic development. It is essential to recognize these congenital variants, as many of these have been proven to be associated with pancreatic diseases like recurrent pancreatitis and chronic abdominal pain. Cross-sectional techniques like multidetector computed tomography and magnetic resonance cholangiopancreatography are the most used imaging techniques for the pancreas, where a radiologist comes across these variants. This pictorial aims to classify the type of variant anatomy of the pancreas, their imaging appearances, and their clinical significance.

Multimodality imaging in cerebral venous thrombosis: a synopsis for emergency radiologist.

Cerebral venous thrombosis (CVT) is an uncommon but potentially fatal condition which presents with a wide range of symptoms. Some of these presenting features are vague thus contributing to the delay in diagnosis. A prompt diagnosis and initiation of appropriate therapy are therefore of paramount importance. In this pictorial, we have tried to illustrate the direct and indirect imaging features of CVT in detail on multiple imaging modalities, along with the potential pitfalls of imaging.

Laparoscopic Management of Auto-Amputated Ovarian Cyst in an Infant.

ABSTRACT: The routine schedule of antenatal ultrasound scans has led to an increased frequency of detection of foetal ovarian cysts. Although most of them regress spontaneously, some may grow into large cysts and undergo torsion followed by auto-amputation. However, pre- and post-natal scans may fail to identify this event. We report a case of a prenatally diagnosed ovarian cyst that failed to resolve conservatively and was increasing in size in post-natal ultrasounds. Pre-operative ultrasound and magnetic resonance imaging failed to detect the auto-amputation. The diagnosis was confirmed on laparoscopy which offers a safe and effective method for the removal of ovarian cysts in neonates and infants.

Large Urethral Diverticular Calculus Clinically Mimicking a Metastatic Deposit in a Case of Ovarian Mass.

Calculus in the urethra of the female is very unusual. The patient remains asymptomatic or uncommonly presents with symptoms of dysuria, post-void urinary dribbling, and dyspareunia. If asymptomatic, it can be diagnosed incidentally on gynecological examination. Being hard in consistency, it may mimic metastatic lesion. We present a case of a female who presented to us for management of ovarian mass. On routine examination there was a hard mass in her vagina which was suspected to be a metastatic lesion. This mass on evaluation came out to be a urethral diverticulum with a large calculus. Very large urethral calculus are a very rare presentation in a female.

Left hepatectomy for hepatic hydatid cyst with intra-biliary rupture: Better to be radical.

Echinococcal liver cysts are predominantly located in the right lobe of the liver and are mostly asymptomatic. A frank intra-biliary rupture (IBR) of hydatid cyst is uncommon, having variable clinical presentation and treatment options. We present a case of a 60-year-old male patient who presented with pain in the upper abdomen associated with vomiting but without jaundice. On investigations, he was diagnosed to have a left lobe hepatic hydatid cyst (HHC) with IBR for which left hepatectomy with bile duct exploration was performed. It highlights the benign nature of the disease for which seldom major hepatectomies have to be performed.

Jejunal mucinous adenocarcinoma: Unusual presentation of a rare pathology.

Mucinous adenocarcinoma of jejunum is a rare tumor of the gastrointestinal tract. Patients usually present after fifth decade of their life with non-specific symptoms. Delayed diagnosis is commonplace and often the reason for advanced disease and poor prognosis. These tumors may masquerade as other common malignancies, with a conclusive diagnosis only after the final histopathological examination. We present a case of jejunal mucinous adenocarcinoma, disguised as cecal malignancy, in an old female patient, managed with radical resection and adjuvant chemotherapy. The report reiterates that the mucinous variant of jejunal adenocarcinoma is a rare pathology with an unusual advanced presentation.

Vascular tumors of the liver: A brief review.

Vascular tumors of the liver are mesenchymal lesions from endothelial cells. They range from common benign lesions such as haemangioma, intermediate tumors like Kaposi sarcoma, and perivascular epithelioid cell tumor to malignant tumors such as hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma in adults. Pediatric vascular tumors of the liver also include benign, locally aggressive, borderline, and malignant masses with haemangiomas being the most common benign tumors and epithelioid hemangioendothelioma being an uncommon pediatric malignancy. The list of these lesions is completed by nodular regenerative hyperplasia, solitary fibrous tumour, and hepatic small vessel neoplasms (HSVN). Some of these tumors are uncommon and rare. This review article aimed to enumerate hepatic vascular tumors along with their imaging, histopathology, molecular findings for accurate diagnosis that can result in better management.