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.

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.

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.

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.

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.

A Rare Sparkle: A Case of Calcified Kidneys in a Young Infant With Renal Failure.

Primary hyperoxaluria-1 (PH1) is an autosomal recessively inherited rare genetic condition due to the deficiency of the hepatic enzyme alanine:glyoxylate aminotransferase which leads to high systemic levels of oxalate and subsequently, early end-stage renal disease and death. Here, we present a case of a three-month-old male infant who presented with loose stools, reduced oral intake, and decreased activity for 12-13 days along with edema and a peeling rash on cheeks, lips, and genitalia. During the entire duration of the inpatient stay, the child was oligoanuric. Kidney ultrasound (USG) was suggestive of bilateral hyperechoic kidneys with increased cortical echogenicity and a computed tomography scan showed bilateral diffusely calcified renal cortices with well-preserved renal architecture. A diagnosis of "oxalate nephropathy" was made from renal biopsy and genetic testing confirmed it to be "primary hyperoxaluria-1". The child was initially managed conservatively, and then peritoneal dialysis was done, following which the child was shifted to intermittent hemodialysis.

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.

18F-fluoro-2-deoxy-2-d-glucose PET-CT (FDG PET-CT) in staging of high-risk renal and urothelial bladder cancers (COPPER-T) trial protocol.

Background and Study Design: Role of 18F-fluoro-2-deoxy-2-d-glucose positron emission tomography-computed tomography (FDG PET-CT) in evaluation of renal cell cancers (RCC) and urinary bladder cancers is not standardized, and the COPPER-T trial, which is a single centre prospective randomized study, was designed to compare it with conventional imaging for staging of clinically localized high risk RCC and urinary bladder carcinoma (Stage T2 and above). Patients and Methods: There will be two subgroups of patients: RCC and urinary bladder carcinoma. In each of these, the patients will be randomized to either Arm A or Arm B. In each of the arms, each patient will be subjected to diagnostic imaging by FDG PET-CT. The CT scan will be a contrast-enhanced scan like that in conventional staging. A radiologist and nuclear medicine specialist will report the scan independently. The radiologist will not have access to the PET scan sequences and will only review the contrast-enhanced computed tomography (CECT) images. In Arm A, the report of the conventional imaging modality, that is, CECT and bone scan if done, will be reviewed first by the clinician, and based on this report, a management plan will be made. Then, the PET-CT report will be reviewed, and change in the management plan will be noted. New findings or equivocal findings if any in the PET-CT report would be noted. In Arm B, the report of the PET-CT report will be reviewed first by the clinicians, and a management plan will be made. Then, the CECT and/or bone scan reports will be reviewed, and any change in the management plan will be noted. Outcome and Significance: Final analysis of the data after completion of the trial will help in clarifying the role of FDG PET-CT in high risk RCC and transitional cell carcinoma (TCC) of the bladder, its diagnostic accuracy compared with conventional imaging and the impact of using it on patient management.

Thoracoscopy-Assisted Esophagectomy for Esophageal Leiomyomatosis.

INTRODUCTION: Diffuse esophageal leiomyomatosis is a rare esophageal tumor characterized by circumferential thickening of smooth muscle layers of the entire esophagus. CLINICAL CASE: Herein, we describe the case of a 19-year-old girl, who presented with a history of long-standing dysphagia. On evaluation she was found to have diffuse esophageal leiomyomatosis and was managed successfully by thoracoscopy-assisted esophagectomy with intra-nodal indocyanine green injection. DISCUSSION: In this report, we discuss the pre-operative workup and our surgical approach to managing this rare entity. We also discuss the available literature on the subject and the lessons learnt in managing this complex condition.

Nonhypertrophic Pyloric Stenosis in a Neonate: A Rare Presentation.

Gastric outlet obstruction in neonates due to nonhypertrophic pyloric stenosis (NHPS) is a rare cause. We report the case of a 37-day-old baby boy who presented with complaints of vomiting for the last 2 weeks and an inconsolable cry over the last 2 days. He has been vomiting seven to eight times a day, a few hours after breastfeeding. On ultrasonography, the stomach was distended, while the pylorus was not hypertrophied. An upper gastrointestinal (GI) contrast study was done, which was suggestive of gastric volvulus. We performed a laparoscopy for the same. Intraoperatively, the volvulus was already resolved. We performed gastropexy. Postoperatively, he had persistent symptoms, for which an upper GI endoscopy was performed. It demonstrated a narrow pylorus, consistent with the NHPS. We performed a laparoscopic Heineke-Mikulicz pyloroplasty. The patient's symptoms had improved postoperatively. He was discharged after 5 days.

Pediatric Renovascular Hypertension: A Pediatric Interventional Radiologist's Perspective.

Renovascular hypertension (RVH) contributes close to one-fourth of the secondary etiologies of hypertension in children and a delay in diagnosis can result in adverse clinical outcomes. RVH in children is clinically silent with elevations in blood pressure measurements sometimes as its sole manifestation. Only a high index of suspicion by the clinician can prompt its detection. Despite the availability of other imaging modalities like ultrasound, computed tomography, and magnetic resonance imaging, digital subtraction angiography is still considered the gold standard to make a diagnosis of RVH. Angioplasty is considered the treatment of choice in appropriately selected patients. In this article, we shall focus on the various imaging findings, and management of RVH in children, which requires a multidisciplinary approach with a special focus on the role of interventional radiology.

Gangliocytic Paraganglioma of the Duodenum: A Masquerader.

Background: Gangliocytic paraganglioma (GP) is a rare tumor that most commonly arises from the duodenum and is characterized pathologically by 3 cell types: epithelioid, spindle, and ganglion cells. GP is often difficult to differentiate from a neuroendocrine tumor on the basis of preoperative imaging, and the diagnosis is based on final histopathologic and immunohistochemical analysis. Case Report: We report the case of a 28-year-old male who presented with pain in the abdomen, bilious vomiting, and weight loss. Imaging showed a mass involving the first and second part of the duodenum that was likely a neuroendocrine or gastrointestinal stromal tumor. He underwent robotic-assisted pancreatoduodenectomy, and the final pathology report identified GP with lymph node metastasis. The patient was doing well at 1-year follow-up. Conclusion: GP is often a histologic surprise as most cases are diagnosed in postoperative histopathology. While GP has a more benign course than a neuroendocrine tumor, radical surgical resection is warranted in cases of diagnostic dilemma, suspicion of malignancy, or lymph node metastasis. Robotic-assisted pancreatoduodenectomy is a feasible option.